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Master's Programme in Health Informatics
Spring Semester 2015
Degree thesis, 30 Credits
Investigating needs and barriers in developing eHealth
tools supporting self-management of people with Sickle
Cell Disease
Author: David-Zacharie ISSOM
Author: David-Zacharie ISSOM
Main supervisor: Dr, Stefano Bonacina, LIME, Karolinska Institutet
Co supervisor: Prof, Gunnar Hartvigsen, MI&T, University of Tromsø
Examiner: Maria Hägglund, LIME, Karolinska Institutet
1
Master's Programme in Health Informatics
Spring Semester 2015
Degree thesis, 30 Credits
Affirmation
I hereby affirm that this Master thesis was composed by myself, that the work contained
herein is my own except where explicitly stated otherwise in the text. This work has not
been submitted for any other degree or professional qualification except as specified; nor
has it been published.
Tromsø, 15-08-2015
__________________________________________________________
ISSOM David-Zacharie
2
Master's Programme in Health Informatics
Spring Semester 2015
Degree thesis, 30 Credits
Investigating needs and barriers in developing eHealth tools
supporting self-management of people with Sickle Cell Disease
Abstract
Background: Although the specific needs of people with sickle cell disease (SCD) are well
documented, the global burden is increasing and SCD lacks recognition. Despite the use of
eHealth tools for chronic diseases is rising and demonstrates potential positive health
outcomes, only few studies related to SCD have been conducted.
Objective: The presented work focuses on assessing how eHealth tools can support patients’
self-management daily tasks, on determining patients’ unmet needs and on understanding how
they want to use eHealth tools.
Methods: A literature review has been performed to elaborate on areas lacking within the
literature, a web-survey was sent to patients worldwide and focus groups have been conducted
to extract software requirements and to design a prototype of user interface reflecting
patients’ needs.
Results: The literature review highlighted that some of the negative effects of the disease can
be partly reduced and be supported by existing eHealth tools. The survey results confirmed
the sub-optimality of the care received by patients, described their needs in a comprehensive
way and showed their clear interest in using wearable devices and smartwatches to support
their self-care needs. As a product of this research, a prototype of a mobile app has been
designed and combines the use of a smartphone with the convenience and accessibility of a
smartwatch.
Conclusion and future work: The suggested prototype may have the potential to support the
self-management needs of people with SCD and suggests that existing technologies have the
capacity to improve SCD patients’ quality of life. Overcoming the challenges of
interoperability with electronic medical records and wearable devices as well as optimizing
the functionalities for individuals using a comprehensive set of participatory design methods
will be required to begin the implementation. Finally, extensive testing, usability studies and
randomized control trials will help to gain clinical approval and compliance with medical
devices certification requirements.
Keywords: sickle cell disease, self-management, eHealth, wearable devices, wireless
technologies, global health, quality of life
3
Preface
This thesis concludes my two years Master in Health Informatics and has been done in
six months.
The motivation for writing this dissertation comes from many different directions.
First, from my particular interest of individual with sickle cell disease, secondly, from my
interest in health informatics and computer sciences. Those last made me think about the
opportunities of using ICTs for sickle cell disease and how they could help to manage the
daily self-care needs. Thirdly, my desire to help other people with sickle cell disease
having a better life was a permanent motivation.
During the project, I have been hospitalized several times because of the disease. This led
to more stress and forced to delay the project issue. Each hospitalization required
numerous weeks of convalescence and it was then difficult to keep working on the
project.
I was fortunate to get a lot of inspiration and ideas from Prof. Christian Lovis, a great
support and enthusiasm from Prof. Kaveh Samii and encouragements from my family and
my closest friends. Having the opportunity to be in the field for many years and
participate in conferences helped me to get an overview of how health informatics can
empower chronic diseases patients.
I have beneficiated from the invaluable support of my main supervisors Prof. Gunnar
Hartvigsen and Dr. Stefano Bonacina, who directed me, helped me and improve my
competences.
My particular interest has been reinforced by positive and negative emotions, especially
coming up when reading for the first time and analysing patients’ answers from the
questionnaire. Indeed, hearing patients’ voices and distress calls has been extremely
touching and motivating. Spending time and meeting patients’ during the focus groups
also reinforced my will of producing potentially useful work. These emotions also
sometimes slowed down my work. I was often afraid of doing wrong or not enough.
Moreover, I also felt overwhelmed by the quantity of issues and work needed to reduce
the worldwide burden of the disease. Hopefully I felt often reassured when seeing that
other patients’ issues to cope with the disease where in fact very similar to mine and since
I survived, I could keep working.
This thesis offered me the opportunity to take different roles: secretary, principal
investigator, patient, patient-representative, e-Patient, researcher, health informatician,
project leader and moderator. Being the principal investigator, I was responsible of all the
areas. During the data collection processes, I received a vibrant support from Claude Isofa
from the Swiss Sickle Cell Disease Patient Association, from Sandro Speck from the
University Hospital of Zurich but also from Prof. Hartvigsen. Part of the work has also
been published in two conferences.
I hope to be able to continue the work and develop collaborations and partnerships that
could contribute to help sickle cell disease patients and decrease the burden the disease.
4
Acknowledgements
I am extremely grateful to my outstanding supervisor, Professor Gunnar Hartvigsen for
his patience and for having accepted to provide a continuous help, support and an
extremely helpful supervision, I learned a lot.
I thank the team at the Norwegian Centre for Integrated Care and Telemedicine for their
support, especially Eirik, Terje, Timotheus, Meghan, Taridzo, Nils, Line, Alain, Siri,
Ashenafi, Stein Olav, Kasper and Raghnild. They succeeded to keep my motivation up
and support me when needed. I extremely enjoyed my stay in Tromsø.
I acknowledge also my excellent supervisors Dr. Stefano Bonacina, Professor Christian
Lovis, Professor Kaveh Samii, Dr. Christian Kjellander and Professor Sabine Koch for
their precious time, input, ideas and comments.
Special thanks to Prof. Samii from the Geneva University Hospital who enabled the
publication of the survey on the website Haemoglobinopathies.ch.
I thank my co-authors Dr. Frederic Ehrler, Dr. Rolf Wipfli and Alexandra Zosso for their
very important work.
Many thanks to Dr. Dina Vishnyakova, Dr. Emilie Pasche, Dee O’Sullivan, Alex Wyke,
Jacqueline Bowman, Dr. Jacques Durand as well as Colette, Youmna and Churchill for
their vital input and great support. I am grateful also to all the patients’ associations and
patients who gave me their support.
I thank all the people who helped me to create and spread the survey in different
languages, especially Claude Isofa, Dr. Bonacina, Dr. Wipfli, Dr. Kjellander, Lara,
Monika and Edouard. They have been of immense help, especially to improve the quality
of the questions and remove bias.
The research could not have been done without the support and understanding of my
family and from my friends, especially during the struggling days. Thank you Alexandra,
Emmy, Sanni, Anne-Marit, Jeanette, Martin, Muriel, Ermano, Laetitia, Fabrice, Melba,
Millie, Michele, Yvan, Michalis, Ellisiv, Sandra, Xavier, Alexandre, Coline, Nastya,
Anne, Mickaël, Stéphanie, Tatiana, Mariann, Pernille, Sebastien and the SAIH people. I
am extremely grateful and I feel extremely lucky to have them.
Thanks to all people at HIMSS, Health 2.0 and Orphanet for their help and input.
Last but not least, I thank Dr. Lars Daae Horvei, the personal at the University Hospital
of North Norway and also at the St. Olavs Hospital in Trondheim for their good care and
support.
Thank you Diana and Sverker for their energy and support.
This thesis is dedicated to my parents and my sister who have gave me an excellent basis,
supported me and took care of me all the way.
In memory of Ruth and Marion.
5
Table of contents
List of abbreviations ........................................................................................................... 9
List of Figures ................................................................................................................... 10
List of Tables .................................................................................................................... 12
1.
2.
Introduction............................................................................................................... 13
1.1.
History of Sickle Cell Disease .......................................................................... 13
1.2.
Medical geography of Sickle Cell Disease ....................................................... 13
1.3.
Health delivery of Sickle Cell Disease ............................................................. 14
1.4.
Manifestations of the disease ............................................................................ 15
1.5.
Pathophysiology of sickle cell disease .............................................................. 16
1.6.
Symptoms and complications ........................................................................... 20
1.7.
Adult emergency management of vaso occlusive crises ................................... 20
1.8.
Screening and diagnosis.................................................................................... 20
1.9.
Prevention and therapeutic education ............................................................... 21
1.10.
Patients’ associations and support groups..................................................... 21
1.11.
Treatment options ......................................................................................... 22
1.12.
Self-management and self-care ..................................................................... 22
1.13.
Problem definition and project goal .............................................................. 23
1.14.
Research questions ........................................................................................ 24
1.15.
Limitations and risks ..................................................................................... 24
1.16.
Research Goal ............................................................................................... 24
1.17.
Objectives ..................................................................................................... 24
1.18.
Statement of originality................................................................................. 25
Theory and state-of-the-art ....................................................................................... 25
2.1.
Literature review ............................................................................................... 25
2.2.
eHealth .............................................................................................................. 25
2.2.1.
Telemedicine ............................................................................................. 25
2.2.2.
Mobile Health or mHealth ........................................................................ 26
2.2.3.
Barriers of implementing eHealth tools for SCD comprehensive care ..... 26
2.2.5.
Examples of wearable technologies suitable for SCD self-management.. 28
2.2.6.1.
Gamification and games for health ....................................................... 33
2.2.6.2.
mHealth and eHealth systems ............................................................... 33
2.2.7.
Existing eHealth systems for sickle cell disease ....................................... 34
2.2.8.
Publicly available apps ............................................................................. 35
2.2.9.
Systems found in the literature review...................................................... 36
6
2.2.10.
3.
Methods .................................................................................................................... 39
3.1.
Design thinking ................................................................................................. 39
3.2.
Questionnaire .................................................................................................... 39
3.3.
Software engineering ........................................................................................ 40
3.3.1.
3.4.
Evaluation ..................................................................................................... 40
Focus groups ..................................................................................................... 41
3.4.1.
First meeting ............................................................................................. 42
3.4.2.
Second meeting ......................................................................................... 42
3.4.3.
Third meeting ............................................................................................ 43
3.5.
Prototyping........................................................................................................ 43
3.6.
The selection of participants and its limitations................................................ 43
3.7.
Ethical approval ................................................................................................ 45
3.8.
The data collection tools ................................................................................... 46
3.9.
The processing of the collected data ................................................................. 46
3.10.
4.
Limitations, major findings and evaluation .............................................. 36
Summary of the methods used ...................................................................... 47
Results....................................................................................................................... 48
4.1.
General sociodemographic tendencies .............................................................. 48
4.2. General health tendencies ...................................................................................... 48
4.3.
ICT receptiveness ............................................................................................. 55
4.3.2.
Services wanted from a health app ........................................................... 57
4.4.
Self-tracking...................................................................................................... 61
4.5.
Free-text questions ............................................................................................ 62
4.6.
Non-mandatory questions ................................................................................. 65
4.7.
Preliminary mock-ups ....................................................................................... 65
4.7.1.
Whiteboard prototyping ............................................................................ 66
4.7.2.
Non-functional requirements .................................................................... 67
4.7.3.
Preliminary prototype ............................................................................... 67
4.8. Focus groups results analysis ................................................................................. 76
4.9.
5.
Prototype evaluation ......................................................................................... 96
Discussion and future work ...................................................................................... 97
5.1.
Critique of the methods used ............................................................................ 97
5.2.
Major findings................................................................................................... 97
5.3. Achievements and expected health benefits of an implementation of the
prototype ....................................................................................................................... 98
5.3.1.
Ubiquity .................................................................................................... 98
7
6.
5.3.2.
Education .................................................................................................. 98
5.3.3.
Psychological ............................................................................................ 98
5.3.4.
Health promotion (preventive healthcare) ................................................ 98
5.3.5.
Health service delivery: from self-care to healthcare ............................... 99
5.4.
Feedback ......................................................................................................... 100
5.5.
Economy ......................................................................................................... 100
5.6.
Suggestions for future features ....................................................................... 100
5.7.
Financing ........................................................................................................ 101
5.8.
Compliance issues and motivational issues .................................................... 101
5.9.
Regulatory issues and clinical approval .......................................................... 101
5.10.
Extension of the questionnaire .................................................................... 101
5.11.
Essentials for the implementation of eHealth tools for SCD ...................... 102
Conclusion .............................................................................................................. 104
References....................................................................................................................... 106
Appendix 1: Project Plan ................................................................................................ 116
Preliminary ................................................................................................................. 116
Revised ....................................................................................................................... 117
Risks assessment ......................................................................................................... 118
Appendix 2: Survey in English ....................................................................................... 119
Appendix 3: Web-Survey results .................................................................................... 135
Appendix 4: Focus-groups notes and paper prototypes with notes ................................ 147
Appendix 5: Summary of the Focus groups ................................................................... 159
Appendix 6: Emergency summary template ................................................................... 162
8
List of abbreviations
API – Application programming interface
CDC – United States Centres for Disease Control and Prevention
CMM – Capability maturity model
CRP – C-Reactive Protein, protein marking the level of inflammation in the blood
EMR – Electronic medical record
epSOS – European project demonstrating interoperability between EMR across Europe
FDA – American Food and Drug Administration
EMA – European Medicines Agency
IHE – Integrating the Healthcare Enterprise
Hb - Haemoglobin
HIMSS – Healthcare Information and Management Systems Society
HL7 – Health Level 7, set of international standards for clinical data transfer
HON – Health On the Net
ICT (s) – Information and Communication Technologies
IP – Internet Protocol
NGO - Non-Governmental Organization
NHS – British National Health Services
Q - Question
REQ - Requirement
SCD – Sickle Cell Disease
SNOMED – Systematized Nomenclature of Medicine
UN – United Nations
VOC – Vaso occlusive crisis
WHO – World Health Organization
WMA – World Medical Association
9
List of Figures
Figure 1 - Global epidemiology of sickle cell disease in neonates, Piel et al, 2013 ......... 13
Figure 2 - WHO Health System Framework .................................................................... 15
Figure 3 - Sickle cell patient healthcare and social environment ..................................... 15
Figure 4 - Inheritance patter for the sickle cell gene, Humanillnesses, 2015 ................... 16
Figure 5 - normal and sickle red blood cells - Healthwise, 2014...................................... 16
Figure 6 - Illustration of contact lens measuring blood sugar and sending data that are
developing Microsoft and Google .................................................................................... 28
Figure 7 - Wristband Samsung Simband packing six sensors .......................................... 28
Figure 8 - Implantable blood analyser chip, EPFL ........................................................... 29
Figure 9 - Example of NFC implant ................................................................................. 30
Figure 10 - Main steps of the design thinking process ..................................................... 39
Figure 11 - Average results per number of evaluators ...................................................... 41
Figure 12 –Steps for the prototype creation ...................................................................... 41
Figure 13 - Photography taken during the last meeting .................................................... 42
Figure 14 - Geographic profile of the survey respondents................................................ 44
Figure 15 - How would you rate your overall health? ...................................................... 49
Figure 16 - Do you feel you have healthy habits? ............................................................ 49
Figure 17- Do you feel you get the right amount of exercise? ......................................... 50
Figure 18 - Number of crises without hospitalisations during the last 12 months ............ 50
Figure 19 - Number of crises with hospitalisations during the last 12 months ................. 51
Figure 20 - Have you experienced one of these complications?....................................... 52
Figure 21 - What symptoms are the most disturbing for your daily? ............................... 52
Figure 22 - How often do you see your doctor? ............................................................... 53
Figure 23 - Is your healthcare provider (the one you see often) able to see if you are
getting sicker or need a check-up? .................................................................................... 54
Figure 24 - Would you like to share your health information with other people? ............ 54
Figure 25 - Overall, are you satisfied with the way the healthcare system is taking care of
your disease? ..................................................................................................................... 54
Figure 26 - How familiar are you with the use of Smartphones? ..................................... 55
Figure 27 - Do you use on a regular basis any of the following technologies? ................ 56
Figure 28 - What have been the best places to get info about Sickle Cell disease?.......... 56
Figure 29 - Where have you been active on the internet about SCD? .............................. 57
Figure 30 - What kind of technology would you consider the most to use in order to
manage your disease? ....................................................................................................... 58
Figure 34 - What would convince you to use a health app regularly? .............................. 59
Figure 32 - Most important services an app should provide ............................................. 60
Figure 33 - What do patients would like to have automatically collected? ...................... 61
Figure 34 - What is your biggest concern in your daily life? ........................................... 62
Figure 35 - In your own words, what other features or services would you appreciate? .. 63
Figure 36 - What should be improved in the public healthcare system? .......................... 64
Figure 37 - What is particularly good in the healthcare system that takes care of you? ... 64
Figure 38 - Sketches of potential main page ..................................................................... 66
Figure 39 Preliminary mock-ups for discussion with patients (Living well with SCD,
SCD news and, In case of crisis) ...................................................................................... 68
Figure 40 Preliminary mock-ups for discussion with patients (Logbook, Monitor and
Analysis) ........................................................................................................................... 69
10
Figure 41 - Preliminary mock-ups for discussion with patients (Settings, Points and
Schedule) .......................................................................................................................... 70
Figure 42 - Preliminary mock-ups for discussion with patients (Medical record, online
consultation and Sickle Cell Warriors) ............................................................................. 71
Figure 43 - Preliminary mock-ups for discussion with patients (What is SCD and Living
well with SCD) ................................................................................................................. 72
Figure 44 - Preliminary mock-ups for discussion with patients (Usual symptoms, How to
treat SCD) ......................................................................................................................... 73
Figure 45 - Preliminary mock-ups for discussion with patients (live Chat and Support
groups) .............................................................................................................................. 74
Figure 46 - Preliminary mock-ups for discussion with patients (suggested prototype for
informational app)............................................................................................................. 75
Figure 47 - Login, Settings, Home and Side-Menu .......................................................... 87
Figure 48 – Psycho-social features ................................................................................... 88
Figure 49 - Emergency help feature.................................................................................. 89
Figure 50 - Logbook and Monitoring screens................................................................... 90
Figure 51 - Integration with electronic medical record..................................................... 91
Figure 52 - Access to information about living well with SCD........................................ 92
Figure 53 - Access to appointments and remote consultation .......................................... 92
Figure 54 - Challenges screen ........................................................................................... 93
Figure 55 - Smartwatch home scrolling menu .................................................................. 94
Figure 56 - Health features on the smartwatch ................................................................. 94
Figure 57 - Visualisation of the tasks to do on the smartwatch ........................................ 95
Figure 58 - Mood management feature on the Smartwatch .............................................. 95
Figure 59 - Pain management feature on the Smartwatch ................................................ 95
Figure 60 - Social feature on the Smartwatch ................................................................... 96
Figure 61 - Exercise management features on the Smartwatch ........................................ 96
11
List of Tables
Table 1 - Difference of funding between cystic fibrosis and sickle cell disease in America
- Sickle Cell Disease Association of America, 2012 ........................................................ 14
Table 2 - Factors increasing the sickling process ............................................................. 17
Table 3 - Potential eHealth tools for VOC detection ........................................................ 31
Table 4 - Relevant mHealth apps from the publicly available markets and sorted by year
of publication .................................................................................................................... 37
Table 5 - Relevant eHealth tools extracted from the literature and sorted by year of
publication ........................................................................................................................ 38
Table 6 - Socio-Demographic characteristics of respondents ........................................... 43
Table 7 - Opening period of the questionnaire ................................................................. 45
Table 8 - Overview of the methods used .......................................................................... 47
Table 9 - Suggestions for future features ........................................................................ 100
Table 10 - Risks assessment ........................................................................................... 118
Table 11- Sociodemographic details of the study participants ....................................... 135
Table 12 - Answers to health-related questions .............................................................. 136
Table 13 - Receptiveness to eHealth tools and digital literacy ....................................... 140
Table 14 - Extraction of the free-text answers: what is your biggest daily concerns?.... 143
Table 15 - Free-text answers: What other features or services would you appreciate .... 144
Table 16 - Free-text answers extraction: What should be improved in the healthcare
system that takes care of you? ........................................................................................ 145
Table 17 - Free-text answers extraction: What is particularly well in the healthcare system
that takes care of you? .................................................................................................... 146
Table 18 - Free text answers extraction: Critic, comment or suggestions ...................... 146
12
1. Introduction
1.1. History of Sickle Cell Disease
The first case of Sickle Cell Disease (SCD) has been documented in 1910 by Dr. James
B. Herrick. A Jamaican patient has been described with abnormal red blood cells, “large
number of thin, sickle-shaped and crescent-shaped”. Seventeen years later, researchers
found that the disease’s manifestations were linked to blood oxygen levels [1]. In 1910,
the disease was still known for centuries in African tribes and was already named, but due
to the breakout of families and tribes during slavery, the oral traditions have been lost.
Nowadays, poor education and lack of awareness as well as beliefs and organizational
issues are keeping SCD a burden for many patients [2–4].
1.2. Medical geography of Sickle Cell Disease
SCD is the most common blood disorder in the world, but has been for a long time
ignored or misunderstood and still is suffering from a general lack of awareness,
highlighting a situation that has not significantly evolved during the last decades.
Moreover, mainly due to newer interventions and treatment that mostly rich countries can
afford, the life expectancy and quality of life of people with sickle cell disease depends of
where they are born [5]. The morbidity is more significant in low developed countries,
especially in Africa and in South Asia where children have 70% to 90% of chances of
dying before 5 years old [6]. The mean age of death is 45-55 years in Europe, USA and in
the Caribbean, and of 5-11 years in Africa [7].
Originally, the disease was most common in India and equatorial Africa, then in the
southern Mediterranean area, and later, because of slavery transportations, in America
and in the Caribbean. Nowadays, migrations are distributing SCD all over the world,
giving SCD a status of global disease. Around 5% of the world’s population carry the
gene causing sickle cell disease and individuals affected by SCD are about 300 million in
the world and over 300’000 babies are born yearly with SCD [8]. In some regions, in the
tropics and sub-tropics, the prevalence can reach 25% [9,10]. However, SCD is still a rare
disease in the European Union, with a prevalence of 1-5/10’000 cases [11–15]. Figure 2
indicates the regions were SCD is the most prevalent [16].
Figure 1 - Global epidemiology of sickle cell disease in neonates, Piel et al, 2013
13
1.3. Health delivery of Sickle Cell Disease
Research on the disease is scarce and despite SCD has been discovered for more than a
century, the general level of knowledge is poor. The disease’s lack of adequate funding
and sufficient awareness makes patients often stigmatized or misunderstood [17,18]. As
illustrated in Table 1, in the USA, the amount of private funding for the cystic fibrosis, a
disease affecting 6-7 times less patients, was of about 20 times more generous. Moreover,
more than twice more papers were published last 5 years for cystic fibrosis. Studies also
suggest that the reasons of low funding could be due to the lack of public awareness
campaigns, resulting to a lack of educative programmes and healthcare professional
training [5,19,20], but also by the fact that the burden of sickle cell disease charges in
majority poor regions of India and Africa, preventing the disease to get enough funding.
Table 1 - Difference of funding between cystic fibrosis and sickle cell disease in America - Sickle Cell
Disease Association of America, 2012
Condition
Cystic fibrosis
Sickle cell disease
Approx. Number of Total funding
patients affected
in the USA
30’000
$482’530’384
100’000
$76’098’978
Funder per person
affected
$16’084
$761
The day to day situation of people with SCD is in many ways similar for patients in
northern hemisphere and southern hemisphere. In northern hemisphere regions, access to
healthcare is generally good but the lack of awareness prevents patients of receiving
adequate, timely and coordinated treatment. In southern hemisphere countries, the lack of
financial power, beliefs due to a poor knowledge of the disease, lack of information,
research, medical support and medication and the deprived access to healthcare services
add difficulty to patients’ situation [2,21–23]. Beyond these differences, patients suffer
from the same lack of optimal, personalized and quality care. Consequently, the general
health service delivery for sickle cell disease needs to be efficiently improved and SCD
being a chronic disease, the continuity of care is a keystone. In order to make health
inequalities as small as possible, the World Health Organization, in 2006 and 2010,
adopted a resolution putting sickle cell disease in the priority global health issues. They
started to supervise and coordinate initiatives focusing on the prevention and
management needs of SCD around the world. Strategies such as increasing awareness
about the disease, promoting a better access to healthcare and encouraging and supporting
research are ongoing [8]. Only few regions are experiencing a change [10]. The World
Health Organisation (WHO) Health Systems Framework in Figure 1 summarizes and
provides guidance on how a good health system should be organized and what should be
fulfilled to deliver optimal health outcomes and tackle priority health issues like sickle
cell disease. The 2009 report of the WHO resolution for SCD1 describes different lacks
that prevent the access to an optimal healthcare service delivery and defines a strategy for
implementing policies and adopting comprehensive healthcare management programs.
1
http://www.afro.who.int/index.php?option=com_docman&task=doc_download&gid=5436
14
Figure 2 - WHO Health System Framework
1.4. Manifestations of the disease
Sickle cell disease is expressed by the obstruction of small capillaries and then a blockage
of the blood circulation, leading to a failure in oxygen transportation, causing an oxygen
deprivation in tissues (hypoxia). This is called vaso-occlusive episodes (VOC) [24,25].
They lead to severe pains, tissues and organs damages as well as life threatening
complications like acute chest syndrome, multiple organ failures or strokes. The episodes
(or crisis), are managed most of the time at home, but hospitalizations can be necessary
when complications occur or pains become insupportable [26,27].
In their daily life, patients generally have to handle chronic pains or difficult to predict
severe pain episodes, chronic anaemia, fatigue and emotional troubles like fears or
depression [28].
Even though the triggering of the crises is largely unpredictable, experiences reported
from patients and research show that lifestyle actions can reduce the frequency of crises
[13,29–31]. Patients are for instance advised to avoid altitude, variations of temperature,
dehydration, fatigue, infections and should follow a healthy lifestyle and a balanced diet.
In order to avoid complications, they should also seek for prompt care when they feel ill.
While evidence-based guidelines, clinical pathways and recommendations to manage
VOC in hospitals are available and even though patients can be frequent utilizers of the
healthcare system, caregivers don’t own the required knowledge. This leads to patients
receiving sub optimal care [18,32–34].
Guidelines for an optimal and comprehensive longterm care for sickle cell disease shows that it must
be treated like any other chronic disease with
many comorbidities and life-threatening
complications [35]: SCD must be treated
continuously (day-to-day) and urgently in case
Patient
of VOC. The care and follow-up must include
all stakeholders of a healthcare delivery service
Family
and embrace all the actors in the daily life of a
Entourage
patient [36,37]. Caregivers need to receive
Support groups
information about how to handle and advise
patients in a timely way in order to make the healthiest
Healthcare system
decisions.
Figure 3 - Sickle cell patient healthcare and
social environment
15
1.5. Pathophysiology of sickle cell disease
People who inherit only one sickle cell disease gene have
the sickle cell trait whereas people who have two sickle
cell genes have sickle cell disease [38].
The first can pass the trait to their children but
except in extreme form, in rare cases and in
particular environments, sickle cell trait carriers
don’t have complications or symptoms of sickle
cell disease [39].
Figure 4 - Inheritance patter for the sickle cell
gene, Humanillnesses, 2015
SCD is a genetic pathology of haemoglobin (Hb)
[40]. Haemoglobin is the main constituent of the
red blood cell and is used to convey oxygen trough the body in all different organs. There
is many different kinds of Hb in adult caused by genetic mutations. The Hb A, found in
majority among adults, and the Hb F, which is mainly found in foetus. Sickle cell disease
is one of the variants, and the haemoglobin is called Hb S, for Sickle. Hb is formed of
two protein chains, the alpha chain and the beta chain. In Hb S case, beta chains are
abnormal and agglomerates inside the red blood
cells.
Normally, red blood cells have a disc shape but
in haemoglobin S, they tend to take the shape
of a sickle. They also tend to be more rigid and
fragile than normal red blood cells and then are
easily destroyed. This causes a chronic
destruction of the sickle cells (haemolysis) and
patients suffer of chronic anaemia. Sickling
happens in higher quantity and more likely
when the body is exposed to low levels of
oxygen, leading to what is called hypoxemia
and also when the blood is acid or poorly hydrated. People with SCD usually have a
chronic deficiency of saturated oxygen in the blood [41,42]. Table 3 summarizes the
sickling factors.
The severity of sickle cell disease varies from person to person, most people have few
complications and crises per year but some can have frequent and life-threatening
complications, but this can fluctuate from year to year [43]. These differences have been
explained by genetic factors modifying the severity of the symptoms but also by different
lifestyles, by an intake of antibiotic prophylaxis at an early age and by vaccines that
prevent life-threatening infections [44–47].
People with SCD suffer of another blood anomaly called thalassaemia. Thalassaemia is
characterized by a low Hb level in the blood cells and by a low red blood cells number.
Thalassemia can cause an iron overload, which can be fatal. Bone deformities, enlarged
spleen, heart problems, slow growth rate and a higher risk of infections [48,49].
The disease is closely linked to malaria. The genetically mutations of the red blood cells
confer a survival advantage to the carrier when getting malaria [1,10]. But this advantage
is mainly for sickle cell trait carriers or people with thalassaemia only. Malaria remaining
deadly for the carriers of the two genes [48,50].
Figure 5 - normal and sickle red blood cells Healthwise, 2014
16
Table 2 - Factors increasing the sickling process
Factor
Increased Sickling
Decreased
Sickle
Oxygenation
Oxygen (Hypoxemia)
Deoxygenation
Haemoglobin percentage
High Hb S, low Hb A, low Hb F
High Hb F, high
Hb A, low Hb S
Haemoglobin S
High concentration
Low
concentration
Haemolysis
High
Low
Blood circulation
High restriction
Low restriction
Water
Dehydration
Hyponatremia
17
Possible ways
monitoring
Pulse oximeter
of Possible
treatment
Decrease
the
altitude, increase
breathing, oxygen
supplementation,
rest, hyperbaric
chamber [51]
Blood test, anaemia- Hydroxyurea,
related symptoms
blood transfusion,
blood
marrow
transplant, folic
acid
Blood
test, Hydroxyurea,
spectrophotometry,
blood transfusion,
anaemia-related
water intake
symptoms
Blood tests, urine Blood transfusion,
test, eye colour, pulse blood
marrow
rate
regularity, transplant
anaemia-related
symptoms
Body position analysis Body
position
alteration
Perspiration
and Fluid intake
galvanic skin response
Possible
causes
Altitude,
intense
physical
activity [25]
Inherent to
SCD, extreme
temperature
s
Inherent to
SCD, extreme
temperature
s
Inherent to
SCD, extreme
temperature
s
Body
position,
tight clothes
Alcohol,
smoking
monitors
Temperature
Fever
Optimal
Thermometer
pH
Acidosis
Alkalosis
Urine
tests
Nutrition
Deficient diet
Balanced
rich diet
Infections
inflammations
tests,
Fluid
intake,
medication
intake, rest
blood Fluid intake, diet
change, surgery,
medication
and Blood tests, visual Folic
acid,
D
symptoms analysis
vitamin,
zinc,
calcium, iron and
magnesium
supplementation,
balanced diet
and Infectious
diseases,
risky Antibiotics and Tachypnea,
Antibiotics, Fluid
behaviours and bad hygiene
anti-infections
tachycardia,
CRP, intake,
antitreatments
white blood cell, high inflammatory
body
temperature, drugs,
rest,
low blood pressure, vaccines
urine test, blood tests
18
habits,
cortisone ,
diuretics,
intense
physical
activity [25]
Infections,
physical
activity
Renal
problem,
unbalanced
diet,
dehydration
Unbalanced
and
poor
diet, chronic
haemolysis
Viral
or
bacterial
infection,
inherent to
VOC
Physiological stress
High
Low
Heart rate monitor,
electrocardiograms,
galvanic skin response
monitor
and
perspiration monitor
19
Breathing
variations,
emotional
support
Pain,
rest, emotional
factors,
physical
activity,
fatigue
1.6. Symptoms and complications
The disease is generally characterized by several symptoms and complications. Acute and
chronic symptoms are part of the disease. The vaso-occlusive crises occur most of the
time without warning and cause pain anywhere in the body. People with SCD suffer also
of chronic pain, severe anaemia, and the blood vessels clothing can cause several
damages to the spleen, making patients prone to get infections, damages to lungs, causing
acute chest syndrome and pulmonary hypertension, damages to kidneys, heart, brain,
eyes, bones, joints, gallbladder, liver, penis, legs and arms. Pregnancies are also at higher
risk. Women can trigger VOC, experience miscarriages and give birth to underweight or
premature babies. The chronicity of the disease, strokes, organ damages increasing with
age, pain or fatigue may make patients suffer of mental health issues like depression,
social exclusion or cognitive problems [27,35]. A study from Booth et al is very eloquent
at this subject and summarizes the usual complications [52].
1.7. Adult emergency management of vaso occlusive crises
Most of people with sickle cell disease take care of themselves in their daily life but
sometimes require access to acute care and hospital emergency admissions [4]. Patients
are generally admitted in last resort and manage their symptoms at home. Indeed, studies
from Carroll et al claim that SCD patients’ hospitalisations account for only 3.5% of the
crises [43,53].
Emergency Sickle cell patients require a rapid treatment by a well-trained and educated
healthcare professionals able to manage and evaluate the different issues. Currently,
patients often suffer from a wrong triage and have to wait more than the recommended
time for receiving their first doses of analgesic. Furthermore, the doses are often
suboptimal, leading to more patient frustrations and suffering, motivating them to avoid
emergency departments as much as possible [32,34,54–56] [33]. Emergency care can be
summarized in a rapid identification of the risks of complications, strong and aggressive
analgesic administration to relief the pain and treatment limiting tissue damage and
reversing the crisis like oxygen supplementation, adequate hydration and blood
transfusions. Diagnostics exams like chest x-rays, general organs functional test and vital
signs monitoring are also recommended [57] and day hospital and specialized centres
with integrated care lead to less hospitalizations rate and decrease the costs of care
[58,59].
Care processes are complex but several clinical pathways, clinical guidelines,
management strategies or recommendations can be used by clinicians to effectively treat
acute VOC and improve their level of knowledge [34,35,59–61].
1.8. Screening and diagnosis.
Since SCD is a genetic disease, the diagnosis requires screening tests. The screening is
done via blood examination or in case of prenatal screening, by placenta or amniotic fluid
examination. According to the WHO, in most countries, universal new-born SCD
screening is not systematically performed and diagnoses are only done when a serious
complication occur. The disease’s early manifestations are recognizable. They usually
appear after birth as painful swelling of the hands and feet, fatigue, jaundice or pain in the
body [44,62,63]. Subsequently, most people do not know if they are sick or if they are
carrier of the gene that could be passed to their child. As a consequence, patients die
before the age of five, mostly because inappropriate care, infections or complications.
Early management can indeed avoid organ damages that could alter patients ‘quality of
life.
20
Since the burden of the disease is increasing, population needs to be better informed.
Moreover, studies have demonstrated that in many countries, beliefs often prevent parents
to give adequate care to patients [2,3].
The disease cannot be prevented but systematic screening and a good awareness of people
about the disease can help couples make informed decision about pregnancy. For
instance, genetic counselling can help determining if a conjoint is carrier of the sickle cell
trait and in some countries, if the disease is detected during pregnancy, a therapeutic
abortion can be requested [64]. Consequently, support from medical specialists can be
sought in order to find how to take good care of a person with sickle cell disease.
Moreover, genetic counselling and screening should be available as a routine. They can
help to early detect the disease and contribute to provide timely care. If patients have the
required finances and live around of an adequate healthcare delivery service, they can
start early treatment and antibiotic prophylaxis.
1.9. Prevention and therapeutic education
The information needed to manage and treat the disease’s symptoms and complications is
often not known by caregivers exercising where the disease remains rare or were the
population lacks of health education [65]. Studies have shown that following the
recommendations can reduce the burden of the disease and improve patients’ long-term
health outcomes [66]. This can be achieved by installing comprehensive care as soon as
the disease is diagnosed. People with SCD must also learn how to prevent infections.
Children are advised to take penicillin as a prophylaxis and all the patients should receive
vaccines. [67]. Moreover, studies have shown that patients with an appropriate
therapeutic education have less absenteeism and have a lower utilization of the healthcare
system [65,68].
ICTs tools have here a role to play. They can offer the opportunity to link caregivers and
patients by easing the communication. Patients could for instance ask for advices when
travelling, when doing sport or seek advices for their professional careers or scholar
orientations. Additionally, monitoring the factors increasing the sickling risk and
watching the symptoms of infections, for instance via body temperature monitoring,
blood tests or urine tests could help early recognize symptoms of VOC and provide early
warning [50].
1.10. Patients’ associations and support groups
Community-based support groups can provide advices on how to find good care or
support, how to participate to events or camps organized for patients, how to share their
experience with other patients, but also provide adequate therapeutic education [4,30].
Facebook groups and websites like PatientsLikeMe.com, HealthUnlocked.com or
SickleCellSociety.org provide forums where patients can post questions, discuss about
their thoughts or different topics. They can also ask questions, seek for advice or share
information about how they live with sickle cell disease. Some patients’ associations, for
instance in England (Bent centre) provide support groups led by parents of children with
SCD, by psychologists or by groups of patients where patients can meet, learn more about
the disease or get support. Some of those centres provides also day care clinics, where
patients can come when they feel ill and receive prompt care before being discharged on
the same day.
21
1.11. Treatment options
SCD has no real cure yet but it’s possible to reduce the global burden, by preventing
infections, by encouraging patients to adopt behaviours susceptible to reduce the
frequency of crises, by advising them to react promptly to the first manifestations of
complications and by promoting a good health.
The only cure available is hematopoietic stem cell transplant (bone marrow transplant). It
can be done for some children and some adults but requires a heavy treatment. Moreover,
bone marrow transplant is risky, expensive and only accessible to a large minority of
patients [69].
Long term treatment includes recommendations for daily oral prophylactic penicillin up
(children up to 5 years old), transcranial Doppler examinations (2-16 years old children),
red blood cells transfusions when the haemoglobin levels is under 10g/dl, or less
according to patients’ usual basal haemoglobin level [35]. Acute and chronic pain are
treated with strong analgesic such as anti-inflammatory drugs and opiates, oxygen
supplementation, a high fluid intake and rest. When fever occurs, pathogen screening
should be performed. Regular blood transfusions may be recommended in case of
recurrent severe anaemia, frequent crises or stroke.
The only drug approved for treatment is Hydroxycarbamide (Hydroxyurea). This drug is
used for the treatments of some cancers. Hydroxyurea is recommended among SCD
patients with the patients who are experience very frequent crises (more than 3 crises per
year). This treatment has many side effects and must be discussed with the medical
doctors. One side-effect from which SCD can profit is that the drug conducts the bone
marrow to produce more Hb F, compensating the quantity of Hb S and breaks down the
cell that are susceptible to sickle. When the body is responsive, the drug has the potential
to reduce the frequency and intensity of crises [70]. The long-term safety effects are not
well known and many adverse effects, like abnormal sperm parameters or leukaemia have
been often reported [71].
A new drug, Rivipansel is revealed as helping to reduce the dose of opioids when patients
have VOC [72].
Finally, some medicines derived from plants (phytomedicines) have appeared to be
effective in reducing the frequency of VOC and are free from adverse effects. However,
they are not approved yet and still need further clinical trials [73].
1.12. Self-management and self-care
The American Centre for Disease Control (CDC) provides a self-care guide explaining
and summarizing how patients can live well with sickle cell disease [31]. The book
explains what the disease is, how to treat it give tips that can help patients staying as
healthy as possible. The most important advices given by the brochure is that patients
shall find good medical care, get regular check-ups, prevent infections, learn healthy
habits that reduce the frequency of crises, listed as the sickle-cell disease selfmanagement cornerstones that we can find in following list and in the literature:
1. Patients shall eat as healthy as possible and follow a balanced diet
2. Patients shall drink 4 to 6 litres of water per day.
3. Patients shall practice a moderate regular physical activity but exhaustion must be
avoided.
22
4. Patients shall comply to the routine medication (vitamin, folic acid and when
prescribed, hydroxyurea)
5. Patients should get vaccines like influenza and evaluate their health for instance
by controlling the oxygen saturation level that can indicate a decrease blood
oxygen carriage.
6. Patients shall avoid caffeine, alcohol and cigarette consumption must be avoided.
7. Patients shall avoid stressful situations and lack of rest.
8. Patients shall avoid extreme temperatures and temperature variations
Patients shall also, in case of crisis that can be managed at home, patients shall take
analgesics, drink plenty of water, rest and take an oxygen supplementation, go to an
emergency department when pain can’t be treated at home or when the following
symptoms appear: fever, respiratory problems or chest pain, severe headaches, vision
problems or loss of feeling. The guide offers also tips to cope with stress, how to stay
physically active, look for clinical studies and get support from other patients or
community-based organizations.
1.13. Problem definition and project goal
The lack of research, funding and consequently the limited level of knowledge are
currently leading the healthcare system to fail taking care of sickle cell disease in a
comprehensive way. There is no knowledge about what comprehensive care means for
patients nor what they are expecting from the healthcare system. Studies have
demonstrated the dysfunction of the healthcare system building blocks and the low
performance in providing a safe, timely, effective, efficient, and patient-centred care [62].
Information and Communications Technologies (ICTs) can contribute to the spread and
the management of SCD-related health information. ICTs have indeed the potential to
help SCD patients by encouraging the adoption of comprehensive healthcare management
programs and by establishing disease surveillance system. An information system
focusing on sickle cell disease can help producing, analysing, disseminating and using
reliable information to support patients, but also can support the institution of health
policies aiming to decrease the burden of the disease.
Globally, the comprehensive range of services needed by people with sickle cell disease
to implement or follow policies for early screening, health prevention, health promotion
and outbreak reduction or even to find a cure are not available. Furthermore, and even
more now because of the migrations, the delivery of healthcare services needs to be
coordinated and accessible, regardless of language, ethnicity, culture, finances and
geolocation. In high income countries, human resources, materials, equipment, basic
pharmaceuticals, healthcare facilities and the clinical knowledge are available, but their
use and their access for sickle cell disease patients is limited and suboptimal in many
ways. This study will then focus on the WHO recommendation of creating patientcentred comprehensive healthcare management programs and disease surveillance
systems for SCD. One strategy is to understand the day-to-day needs of people with
sickle cell disease in order to develop tailored ICTs based solutions that could help them
maintain their health and wellness over time.
Moreover, the use of mobile applications supporting the comprehensive self-management
of chronic diseases is raising, many inspiring patient-centred and mobile based selfmanagement systems for other chronic diseases like diabetes have been developed but
few are focusing on sickle cell disease.
23
1.14. Research questions
In order to tackle these problems, it will be required to understand the opportunities of
using ICT-based tools (eHealth tools) for people with SCD and to gain knowledge about
which tools are currently used to manage chronic diseases and how they help patients.
Consequently, the research questions are:
Q1: What can eHealth tools offer to help people with Sickle Cell disease in their daily
life?
Q2: What are sickle cell disease patients’ self-care needs in their daily life?
Q3: What do patients want and need from eHealth tools to help them in their self-care?
Q4: What kind of self-operated mobile-based solution could help them in their daily-life?
Answering the first question (Q1) requires to establish how relevant the use of eHealth
tools for the self-management of sickle cell disease is. The research will focus on forming
a collection of the existing tools that are related to SCD and what future possibilities of
application they offer.
The second question (Q2) seeks to establish what patients’ needs in term of day-to-day
self-care are.
The third question (Q3) seeks to identify what SCD patients would want or expect from
the technology in order to support them in their daily life.
The fourth question (Q4) seeks to identify how eHealth tools that back the establishment
of comprehensive healthcare management programs could look like.
1.15. Limitations and risks
The study will be limited by what the current technology can offer. Furthermore, the
timeframe will not permit to develop a working prototype. Also, the scarcity of patients,
especially in Norway, where this study is done will limit the access to a representative
population. It could be difficult to get a significant number of participating patients.
Appendix 1 and Table 12 present the risk assessment and the measures done in order to
reduce the risks.
1.16. Research Goal
The research goal is to give an overview and to conceptualize what a helpful solution for
patients could look like. This will be done by investigating how eHealth tools for selfcare can be useful for sickle-cell disease patients and what the challenges of giving them
self-management ICTs based tools are.
1.17. Objectives
The main objective is to create a prototype (mock-up) of a mobile app that could answer
the requirements of a comprehensive tools to help the self-management of people with
SCD. The second objective is to answer patients’ wishes and needs in term of self-care
and thus find how to support them in the management of the health-related effects of their
disease. The last objective is to get findings that could be reused and be relevant for
future implementation. Such findings could enable and prepare organizational changes in
the healthcare systems in order to deploy patient-centric solutions for sickle cell disease.
Findings could also potentially be informative for patients, giving them an idea of how
technology could help them.
24
1.18. Statement of originality
The first contribution in the literature was a poster for the MEDINFO 2015 conference
and was a review of the existing eHealth tools and systems for sickle cell disease [74].
The second contribution was a conference paper for the Scandinavian Health Informatics
conference (SHI 2015) and focused on the identification of patients’ main needs, wishes
and issues in term of self-care and self-management of their disease [75].
2. Theory and state-of-the-art
2.1. Literature review
This chapter presents a general literature review meant to understand the disease deeply
and to have a broad idea of patients’ needs. It provides a state-of-the-art on the existing
ICTs solutions for chronic diseases in order to understand what eHealth tools can
realistically be used for. The chapter describes the knowledge extraction of the most
relevant tools that could be used to promote and maintain the health of people with SCD.
To do so, the research was focused on understanding how eHealth tools could support the
disease’s self-management cornerstones and on comparing the existing solutions for other
diseases like COPD or diabetes. Relevant papers about existing solutions for sickle cell
disease have been extracted to leverage the understanding of what is existing for other
diseases and what is done for SCD. It was a very important step to gain an idea of the
potential applications for SCD.
The search strategy for the Background and the general literature review was to use
Google Scholar, PubMed, Cochrane and Science Direct with the following criteria:
“sickle cell”, “sickle cell management”, “sickle cell living”, “sickle cell quality of life”,
“sickle cell mobile”, “sickle cell software”, “sickle cell self-management”, “sickle cell
self-care”, “sickle cell problems”, “sickle cell literacy” and “sickle cell health literacy”.
The search yielded more than 20’000 articles. The papers before 1990 were exclude and
the one I reviewed were mainly papers about the quality of life of patients and papers
explaining what sickle cell disease is.
The background and the literature review about SCD have finally been used to generate
accurate questions for the survey once enough knowledge acquired.
2.2.
eHealth
According to the European Commission, electronic processes in health (eHealth) refer to
the use of information and communication technologies for health. In the context of
sickle cell disease, ICTs can be applied for instance to educate patients or health
workforces, to improve the access to care and the quality of care, the prevention, the
diagnosis and treatment of the disease but also the self-care. Such tools can be used also
for the monitoring and the surveillance of SCD related symptoms, helping the general
management of SCD and improving communication and information flow between
healthcare providers and patients [76].
2.2.1. Telemedicine
Telemedicine is the remote communication between patients and health professionals.
Applications can enable distant consultations, using voice, text, data, images or videos
and have the potential to improve the communication between sickle cell patients and
health services. Some studies have demonstrated great opportunities in using such
25
technologies to facilitate the consultations by providing free telephone services or video
calling, easing the contact with caregivers and reducing the burden of sickle cell disease
in remote areas [77,78].
2.2.2.
Mobile Health or mHealth
Some studies for other chronic diseases have demonstrated encouraging results and longterm possibilities of use of mobile health tools. For instance, Årsand et al have developed
a patient-operated mobile application for smartphones, where people with diabetes can
self-manage the most important aspects of their disease [79,80]. Some others mobile selfmanagement tools like WellDoc [81] have shown a positive impact on the management
of diabetes.
By facilitating communications between patients and healthcare providers and in offering
patients’ tools that for instance give them feedback about their health, cell phones and
smartphones have the potential to empower patients in the management of their health.
Mobile systems can also help patients to comply with their treatments, remind them for
check-ups appointments, ease the information sharing about their health and ease the
contact of emergency services, transferring their important health information to health
services. Such systems, by giving patients access to their own data can also support the
decision making processes and enhance patient’s understanding of their disease.
2.2.3.
Barriers of implementing eHealth tools for SCD comprehensive care
To enable patients to self-manage sickle cell disease in an optimal way requires to access
information from several disciplines. An optimal healthcare system should be able to
provide patients the tools and the information needed for their self-care and should
provide an optimal access to health services. The material and the content exist, but the
bidirectional transfer of knowledge from caregivers to patients is deprived [82]. The use
of eHealth tools can offer the opportunity to support the information flow between the
different stakeholders and have the potential to empower patients and help structuring and
organizing the care around patients.
2.2.3.1.
Health and digital literacy, quality and security of online health
information
People are seeking more and more health information online [83]. The quality of
information can vary dramatically between different sources of information like forums,
website or online groups and since people use the information they find to make healthrelated decisions, this can have bad consequences. People don’t always have the skills
and knowledge to evaluate the quality of information they find on Internet and can lack of
digital literacy to use such eHealth tools. Indeed, studies suggest that low health literacy
and digital literacy level play a role in people’s ability to evaluate online health
information quality [84]. Moreover, many people, have never used internet or are digital
illiterate. In order to provide patients digital content, they can use and information they
can trust and understand, a prior assessment of SCD patient’s digital literacy and health
literacy level would be of interest. The material given to patients should take into account
their level of digital and health literacy in order to provide good quality, accurate, reliable
and understandable information. Some organizations like the foundation Health on the
Net (HON) are taking care of assessing the quality of health information on Internet,
26
evaluating websites that provide health information and giving them a seal of quality2.
Other measures like education programmes about how to interpret information on the
Internet can also be put in place.
Beside patients' digital literacy and health literacy, but also healthcare professionals
training and education, another important challenge is to integrate such tools in the
healthcare system.
2.2.4. Medical devices, standards, certifications, approval and compliance
The following list summarizes some of the eHealth tools and medical devices that could
be used by sickle cell disease patients and would require certifications and regulation by
the FDA [85]34.
- Contact lenses (lenses tracking the tears chemistry and monitoring the blood
pressure)
- Active implantable medical devices (chips that can analyse and monitor an
individual’s blood chemistry or chips that can be used to store and transfer data)
- Devices used to inject liquid or gas medication into the body
- Mobile apps that can present a risk to patients if the app does not work as
intended:
o Mobile apps used as an accessory to an already FDA regulated medical
device like an app that reads data from a pulse oximeter and converts
them.
o Mobile app that transforms a mobile platform into a regulated medical
device like an app intended to be used for diagnosis, cure, treatment,
mitigation or prevention of disease
Some electronic medical records provide an interface (API) for external software that
want to access healthcare information. These APIs can be integrated in mobile apps and
can enable the retrieval of chosen information. Other standards for data and health
documents interoperability like HL7 (health information data exchange standard), EPSOS
(European project demonstrating interoperability between EMR across Europe), IHE
(initiative to improve health information data interoperability), SNOMED (nomenclature
for meaningful exchange of health information) or the EMA Structure (standard for
pharmaceutical information mapping) shall be integrated in any system that want to
enable the import and export of healthcare information. Finally, standards from the
Continua Healthcare Alliance (CHA), a company that provides interoperability standards
for medical devices should be taken into account.
A rise of solutions brought by the Quantified-Self (QS) movement opened the door to the
use of ICTs as a resource for patients to cope and track their chronic condition. QS
illustrates the opportunities offered by ICTs in enabling individuals self-tracking their
daily life parameters. Usually people track their physical activity, their health status, their
fatigue, their diet, their psychological changes or their mood. Furthermore, existing
studies have demonstrated that the use of techniques for patients’ self-reporting of pain
symptoms, moods or fatigue have good impact on their health [86–88].
2
http://www.hon.ch/
http://www.regulations.gov/#!docketDetail;dct=PS;rpp=10;po=0;D=FDA-2011-D-0530
4
http://www.d4.org.uk/research/regulation-of-health-apps-a-practical-guide-January-2012.pdf
3
27
Such digitized information can be seen from a mobile phone or for more convenience
from a smartwatch. They help the self-tracker to be more self-aware and to understand
himself better in any day-to-day situation.
When health-related information are transferred to physicians, they can enable new
possibilities of early detecting problems or finding new evidences or hidden insights [89–
91]. We can see potential applications for sickle cell disease, where patients can use
standard medical devices (CHA) to track disease-related parameters like physical activity,
vital signs, blood parameters, mental and social variables like mood or stress,
environmental values like weather or air quality and then receive feedback on their
health, their actions or their behaviours.
2.2.5. Examples of wearable technologies suitable for SCD self-management
Devices that can be worn permanently by patients in a non-disruptive way are interesting.
They can assist their self-care and self-management needs in their everyday life. The
following devices can be used to monitor patients’ vital signs, symptoms, health status,
wellness or physiological parameters but also be used for automatic injection of drugs
[92,93]. Such devices can even take several form like contact lenses (Figure 7), clothes,
watches (Figure 8), bands, jewellery or earbuds.
Figure 6 - Illustration of contact lens measuring blood sugar and sending data that are developing Microsoft
and Google
Figure 7 - Wristband Samsung Simband packing six sensors
28
Such devices can be wearable, semi-invasive or invasive, for instance contact lenses or
implantable micro-chips and be more or less smart. They can for instance be able to
monitor an individual’s environment, control it and learn from it. They can also be
unreactive to the environment and be used for data exchange or storage.
In the case of a patient with SCD, carrying the health information important in case of
emergency can accelerate the care processes, providing caregivers important information
in a timely way. Implantable chips that can store information and transfer it when put in
contact with a smartphone (NFC chips) have the potential of being helpful in emergency
situations. They can embed for instance information about allergies or a summary of the
medical history, usual treatment, healthcare issues and complications. A study from
Neumayr et al [94] about the use of an electronic card worn by patients that enables
healthcare professionals to retrieve medical information could be adapted to such
implantable smart devices. A healthcare provider could be able to read the chip that
contains the patient’s life-saving information with his mobile phone and get instructions
on how to take care of the person.
We can easily see other applications for the treatment and self-management of sickle cell.
Medical implants (Figure 9 and Figure 10) or wearable devices meant for the continuous
monitoring some blood chemicals or physiological variables like hydration level, pulse
oximetry, blood pressure, respiration rate, temperature or heart rate (Figure 8), but also
devices that deliver drugs or transfer data could be used for the early detection of VOC
[92,95,96]. Table 4 summarizes the type of devices that could be used to track SCDrelated physiological parameters.
Figure 8 - Implantable blood analyser chip, EPFL
29
Figure 9 - Example of NFC implant
30
Table 3 - Potential eHealth tools for VOC detection
Factor
Oxygen (Hypoxemia)
Type
Wearable pulse oximeter
Haemoglobin percentage
Haemoglobin S
Haemolysis
Blood circulation
Water
Temperature
pH
Nutrition
Infections
inflammations
Publicly available tools
Nonin
Onyx
9650,
Contec
CMS50FW, Withings Pulse 02,
iHealth Pulse 02, Samsung SimBand,
Amiigo,
Hexoskin,
Omsignal,
Zensorium Tinké, Viatom CheckMe
Portable haemoglobin analyser (blood count),
Hemocue 201+, Stat-Site M
Portable
haemoglobin
analyser, Hemocue 201+, SmartScope
microscope(blood smear)
Portable haemoglobin analyser, urine analyser, Hemocue 201+, BioHarness
pulse rate meter
Posture analyser, smart clothes
Kinect, Microsoft Band, Zephyr
BioHarness 3
Smartband, smart patches
Kenzen Echo H2
Wristband with thermometer
Amiigo, Viatom CheckMe
Prototype-based tools
Scanadu Scout, Bragi
The Dash, [97,98]
[99]
[100–104]
Scanadu
Urine,
Samsung SimBand,
QuickPosture
Samsung SimBand
Bragi
The
Dash,
Samsung SimBand
Urine or blood pH Meter
Sensorex pH Meter
Scanadu Urine
Diet journaling, nutrition monitor system
MyPlate
Livestrong,
Calorific, [105]
MyFitnessPal, Fitocracy, Fooducate
and Blood pressure monitor, heart rate monitor, H2Care, Hexoskin, Omsignal, Zephyr Samsung
SimBand,
heart rate variability monitor, thermometer, BioHarness,
Zensorium
Tinké, Bragi
The
Dash,
implantable blood analyser, respiration rate Viatom CheckMe
Scanadu Scout
monitor
31
Physiological stress
Blood pressure monitor, heart rate monitor, Zensorium Tinké, BioHarness, Spire, Samsung Simband
heart rate variability monitor, thermometer, Quardiocore, Empatica
respiration rate monitor, perspiration and
galvanic skin response
32
2.2.6.
Existing eHealth systems used for chronic diseases self-management
The number of mobile health apps to manage chronic diseases is escalating. We can find
for instance some good quality apps for diabetes self-management. Some health apps can
be used with smartwatches or wearable devices, like NST Diabetes Diary or mySugr.
Studies have also shown an improvement in patient quality of life, helping patients track
their condition simply and efficiently [106,107].
2.2.6.1.
Gamification and games for health
Maintaining patient engagement and motivation for using an app is often an issue,
including items from games are sometimes used as a strategy but patients usually feel
mixed about that [108]. The implementation of such techniques should be discussed with
patients. Some apps like mySugr, MangoHealth or Fitocracy implement gamification to
try making apps that can create long lasting healthy habits [109].
2.2.6.2.
mHealth and eHealth systems
Solutions for the management of chronic diseases like diabetes are rising and their
usefulness has been proved [79]. A quick look on the websites StartupHealth,
iMedicalApps.com, MobiHealthNews and MyHealthApps already revealed interesting
systems and apps. There is a potential of doing similar solutions for SCD.
Some of the main existing commercial systems and mobile health apps that can be used
for chronic diseases self-management or health promotion are listed below and could
serve as inspiration, for instance the Figure 12 illustrates a system that estimates an
individual’s risk of cardiovascular disease, systems that estimate risk of triggering a VOC
can be similarly done for SCD.
- Open Health Network: generic mobile health app platform enabling to create
mHealth apps using a set of existing modules
- Cellnovo: mobile diabetes self-management system
- MediSafe: medication reminder
- Emotiv Insight: Mood tracking and mood altering app
- Thync: mood altering devices
- eVisit: mobile telemedicine system
- CheckedUp: educational app for pre and post-operative care
- FitBit: Fitness app to track activity, diet, sleep and weight
- Glooko: mobile diabetes self-management system
- ASCVD Risk Estimator: app to estimate the risk of cardiovascular disease
- MyChart: app to access data from Epic electronic medical record
- MangoHealth: medication reminder
- Welldoc BlueStar: mobile diabetes self-management system
- Dexcom G4: mobile diabetes self-management system (Figure 15)
- Little Byte’s blood sugar tracker : diabetes self-management app (Figure 13)
- HelloHeart: blood pressure monitoring app (Figure 11)
- MySugr Diabetes Logbook: mobile diabetes self-management system with
gamifications items
- HealthTap: patient-provider communication app
- Oxitone Medical: blood oxygen monitoring platform
- Diabetes Diary (NST): mobile diabetes self-management system (Figure 14)
- Firechat: communication app that can be used to create chatrooms for patients
- Fitocracy: fitness app with gamification items to enhance users’ motivation
33
2.2.7.
Existing eHealth systems for sickle cell disease
Few papers are available on what patients expect on this subject, but the actual studies
show a great interest from patients [110].
A literature review has been performed on the regular data sources such as PubMed,
IEEE Xplore, Cochrane, Web of Science and PLOS ONE. The total of unique papers is
24. They have been found using the terms “sickle cell disease” in combination with the
keywords “mobile”, “electronic”, “self-management”, “system”, “mHealth and “cellular”.
The review highlighted a project about a smartphone-based microscope that can be used
for sickle cell disease screening and the imagery processes that can be applied in order to
detect sickle cells [111,112]. As suggested by Breslauer et al and Youngchan et al, this
could be used in addition with imaging techniques that potentially simplify the screening
process. Applications in low income countries could have a great impact on health
outcomes [111,113].
Additionally, some studies from Woods et al about telemedicine systems for SCD have
been done in the early 2000s. They have demonstrated a greater clinical productivity as
well as a good patient satisfaction. Isolated patients in rural and remote areas were able to
receive care [114–116].
Furthermore, the search highlighted a system monitoring the school attendance and the
daily activities of teenagers with sickle cell disease in order to understand the
repercussions of the disease on their quality of life. Indeed, SCD can make patients miss
many days of school which can have consequences on their level of education [117,118].
The team of Jacob et al has developed a system using text messages to help piloting
psychotherapy interventions and to monitoring pain symptoms remotely [93,119,120].
The same team also developed a web-based diary for youth with SCD with a main focus
on enhancing the pain management of patients by giving them feedback and advices
[121,122]. As well, a project aims to enhance the communication between SCD patients
and healthcare provider in paediatric environments [123].
Other papers about the use of wearable sensor and embedded systems for sickle cell
disease illustrated the potential uses of data collecting systems [124,125].
Two recent papers have shown the challenges and opportunities of using physiological
data coming from physiological sensors [90,126].
The last paper found is describing a system, called Chart Card intended to provide
patients a plastic card including their medical information and instructions for healthcare
provides on how to take care of them in case of emergency. This paper is a feasibility
study and demonstrated the advantages of providing emergency information and care
protocols to healthcare professionals that lack of training and education about SCD [94].
The chapter below summarizes the literature review findings on eHealth tools intended to
be patient-operated and to manage the self-management issues of people with SCD.
2.2.7.1. Research criteria
In order to find existing systems meant to help patients to self-care, the terms used for
research were less broad that for the precedent research.
34
The research has been performed first on the publicly available app stores and then on the
most common literature databases such as Cochrane, PubMed, IEEE Xplore and Web of
Science.
The inclusion criteria for a system are as follows:
- Patient-operated
- Focused only on sickle cell disease
- Offering at least one of the following:
o Help the patient to better understand the disease (educational content)
o Manage at least one of the SCD self-management cornerstones (diet,
hydration, exercise, routine medication, advices on what should be
avoided)
o Manage one of the symptoms like pain or fatigue
o Help for a psychological or social issue of SCD patients
o Provide an assistance in case of VOC or emergency
2.2.8.
Publicly available apps
The research has been done from the Norwegians app stores of Google (Android),
Microsoft, BlackBerry and Apple. The impossibility to circumvent the geolocation
limitations for all the stores could prevent some apps to appear in the results. For that
reason an additional research has been done with the term “sickle cell app” on the Google
search engine, the website “MyHealthApps.com”, “iPhoneAppStorm.com” and
“AppAnnie.com”.
2.2.8.1. Google Play Store:
The first research with “sickle cell” returned 64 results. 60 of the app returned were not
focused on sickle cell disease only.
2.2.8.2. Microsoft Windows Phone store:
A research with “sickle cell” returned two results. Both of them are excluded.
2.2.8.3. Microsoft Windows Store
The Windows App store returned 0 result for “sickle cell”.
2.2.8.4. BlackBerry World
BlackBerry World returned 1 result. It’s an app providing tutorials and introduction to
blood pathologies. It has been excluded.
2.2.8.5. Apple iTunes Store:
The research on the iTunes store returned 9 results with the term “sickle cell” and 5 with
the terms “sickle cell disease” and ”sickle cell anaemia”.
2.2.8.6. Google Search Engine:
The search with the keywords “sickle cell app” returned 3 new results. See Figure 16 and
Figure 18.
2.2.8.7. Other websites:
A research on AppAnnie and iPhoneAppStorm with “sickle cell” returned 4 apps.
Among the 4 apps, 2 have been removed of the market, and 2 are still available.
35
2.2.9.
Systems found in the literature review
To perform the literature review, the terms used were “sickle cell system”, “sickle cell
app” and “sickle cell telemedicine”. Researching with the same terms than on the public
platforms would retrieve too many results.
2.2.9.1. PubMed
1486 results have been retrieved and no paper passed the inclusion criteria.
2.2.9.2. IEEE Xplore
The first term returned 13 results. Six relevant apps have been extracted.
The second research returned a unique result that did not pass the inclusion criteria.
The last research returned 3 results that are already described above.
2.2.9.3. Web of Science
With the first term, 1477 results have been retrieved and no paper passed the inclusion
criteria. The second term research retrieved 1 paper. The paper, written by Leonard et al
passed the inclusion criteria [127]. The last term research did not retrieve any result.
2.2.9.4. Cochrane:
The first research retrieved 5 results and none was relevant.
The second research retrieved no results.
The last research retrieved 3 results, 2 of them were the same papers.
2.2.10. Limitations, major findings and evaluation
As explained before, some systems have been missed because of the geographic
limitations of the different public app markets. This pointed out that nearly no apps were
available worldwide. “SiKL” could only be used in the UK and “VOICE” only in the
USA. The only system available worldwide was the game “Zoo Crush”.
The research performed on Google and on the other websites, as wells as the attempts to
circumvent the geographical limitations, decreased the risk of missing an app.
Furthermore, the term “sickle cell system” used on the common literature databases was
too broad to retrieve any successful results and should not be reused for future research.
The literature review of eHealth systems for SCD demonstrated that mobile technologies,
such as smart clothes, wearable devices, web apps and smartphone apps are already used
to tackle some of the main issues faced by patients.
The literature review of mHealth systems for SCD showed a total of 13 relevant existing
systems. 6 were publicly available and 7 were research reports.
In total, 4 were games and all of them provided a basic educative content about SCD.
One of the app, “VOICE”, helped the patient track pain symptoms and give the
possibility to alert someone in case of emergency.
The last app, SiKL, is a summary of the patient medical record and patients can set
reminders for medication. Except this app, all publicly available apps and games have
been designed to be used exclusively by children.
In addition, 5/7 of the literature extracted systems were focused on youth or children with
sickle cell disease. Three of the systems found in the literature are focused on the pain
36
management, one on medication management and the other focused on other issues faced
by patients.
Among all the systems found, none offered a comprehensive support for SCD selfmanagement needs. The one that offers the most functionalities is the study from Jacob et
al. Further investigations have shown that nothing has been updated since 2012. In
addition, the system is not publicly available.
Table 5 and Table 6 summarize the findings and include the name of the apps, the type of
application, the platform of availability, the last year of update, the ranking given by users
and the type of issue managed. Figure 17 and Figure 18 illustrate the three self-operated
apps that have been retrieved from the literature and from the public markets.
Sickle Cell
Invaders
SiKL
BloodFeud
Iron Game
App
Game
iPhone
iPhone
2013
2012
2014
2014
2013
37
Rank
n/a
4.4
5, 3.8
5, 4.4
4.5
n/a
5
Medication
App
Year
2014
2014
Pain management
Voice Crisis Alert
Platform
iPhone
iPad
iPhone,
Android
iPhone,
Android
iPhone
Hydration
Type
App
Game
Game
Emergency
Title
Beyond Sickle Cell
BloodBot
Zoo Rush
Educative content
Table 4 - Relevant mHealth apps from the publicly available markets and sorted by year of publication
X
X
-
-
-
-
X
-
-
-
-
-
X
-
X
-
X
-
-
-
-
X
X
-
-
-
X
-
Leonard et al [127]
Brown C et al [120]
Venugopalan et al [117]
Jacob et al [93,121–123]
Woods et al [114–116]
38
-
X
Other issues or symptoms
-
Nb of users
Panneerselvam et al [125]
Year
2014
Medication
Cheng et al [119]
Title
Usability and Feasibility of a mHealth Intervention for
Monitoring and Managing Pain Symptoms in Sickle Cell
Disease: The Sickle Cell Disease Mobile Application to Record
Symptoms via Technology (SMART)
iACT--an interactive mHealth monitoring system to enhance
psychotherapy for adolescents with sickle cell disease
Application of embedded system for a genetic disease, sickle
cell anaemia
The Use of Mobile Technology for Intensive Training in
Medication Management in the Paediatric Population
SickleREMOTE: A two-way text messaging system for
paediatric sickle cell disease patients
Activity and school attendance monitoring system for
adolescents with sickle cell disease (SickleSAM)
Web-based mobile e-Diary for youth with Sickle Cell Disease
Sickle cell disease telemedicine network for rural outreach
Pain
management
Author / Reference
Jonassaint et al [128,129]
Hydration
Table 5 - Relevant eHealth tools extracted from the literature and sorted by year of publication
-
15
2014
-
-
Psychological (psychotherapy)
-
60
2014
-
-
Blood chemicals and vital signs
-
0
2014
-
-
-
X
8
2012
-
X
-
-
0
2012
-
-
Social (school attendance)
-
10
2012
2000
X
-
X
X
Mood, Sleep
Remote patient consultation
-
30
1000
3. Methods
In the interest of creating a solution that can be innovative, the first technique used to organize the
ideas and the work was the method of design thinking.
3.1. Design thinking
Design thinking was first used in architecture [130] but has been adapted to software development.
It’s a method of solution-based thinking that emphasizes the creativity by reflecting about how to
conceive global solutions for specific problems. The first step consists in identifying the problem and
in understanding its environment. The second step focuses on finding a concept that can solve the
issue. The third step emphases the design of a conceptual prototype and the final step is the testing
with the final users. The timeframe of the study was too compact to perform the last steps of
implementing, developing and testing with the patients. Figure 19 illustrates the design thinking
process.
Figure 10 - Main steps of the design thinking process
In order to compensate the compact timeframe, the choice has been made to involve patients as much
as possible. Especially by performing a participatory design study where patients can share their
needs, opinions and suggestions but also where they can give feedback.
The strategy used was then a triangulation of methods. The first phase was to complete a literature
review and then to perform an exploratory research with a questionnaire. The survey questions are
available in Appendix 2. The second step was to conduct focus groups. Focus groups are interviews
with a small group of patients where ideas and work can be produced during meeting and discussions.
The next phase was the software engineering phase, were requirements have been extracted from the
discussions with patients and from the survey results. Finally, a conceptual prototype has been
designed.
3.2. Questionnaire
The questionnaire was intended to get a comprehensive idea of patient needs and wishes in term of
eHealth tools to support them.
First by writing a legal consent for patients and then writing demographics questions, questions about
their health issues, their quality of life, their digital literacy, their habits, and their needs in term of
self-management. Additional questions about the features they want from eHealth tools and the
devices they may agree to use have been asked. Finally they had the opportunity if they wished to
write how they would imagine a perfect healthcare system and in what manners they are struggling or
disappointed by the healthcare system taking care of them.
39
To create the online survey and especially for the questions about the features they might want,
inspiration has been taken from the survey available on “MyHealthApps.net” and on
“PatientView.org” titled “What do patients and carers want from health apps”. The website
“SurveyMonkey.com” and especially the surveys about quality of life and health questions have also
been observed.
A clinical expertise was assured and advices, remarks and important state of the art information about
the ongoing research on the disease provided by sickle cell disease specialists. Clinical experts
reviewed, corrected and approved the survey in order to remove errors and ensure accuracy.
3.3. Software engineering
The last step was the software engineering part. After analysis of the preliminary results, a proposal of
user interface mock-up covering the usual self-management needs of SCD patients has been designed
and shown to patients during the focus group meetings. When the final survey results were available,
the requirements were extracted from the results analysis and a final proposition of prototype has been
designed, including the results from the surveys, discussions from the focus groups and feedback from
patients.
3.3.1. Evaluation
The prototype has been inspected using a limited heuristic evaluation method. The heuristic
evaluation is the iterative and systematic inspection of a user interface, in order to find usability
problems and designs that do not comply to the usability principles (heuristics) [131]. To comply with
the heuristics, an interface should:
- “be simple and the information appear in a natural and logical order”
- “speak the users’ language”
- “minimize the user’s memory load”
- “be consistent” (a user should not wonder if different word, situations or actions mean the
same thing)
- “give appropriate feedback” (a user should now what is going on)
- “provide clearly marked exits”
- “provide shortcuts”
- “show good error messages”
- “prevent errors”
- “provide help and documentation”
In order to use the evaluation method at its full potential (Figure 20), it is advised to have around
12 people evaluating the user interface. Single evaluators can find around 35% of the issues. The
heuristic evaluation has been done on each screen several times (max. 7 times) to minimize the
limitation of being a single evaluator.
40
Figure 11 - Average results per number of evaluators
The prototype design has been done in 4 steps, as shown in Figure 21. First the screen was created to
meet the specific requirement, the screen was then evaluated in order to find usability problems like
navigation, content, coherence or a design problem and in case of issue, a solution has been identified.
Subsequently, the solution was implemented and the prototype modified. Finally, a last reading of the
patient requirement was done in order to validate if the screen meets the requirement.
Feature
creation
Problem
finding
Solution
identification
Feature
modification
Validation
Figure 12 –Steps for the prototype creation
3.4. Focus groups
The focus groups were intended to discuss the questionnaire results and specify the requirements and
the prototype design. Three focus groups have been planned to explore what were the most important
features that the system should provide, get more insights on the survey questions and clarify the
needs.
Prior to the focus group, an informal interview has been conducted with the president of the
Norwegian patient association. The goal of the preliminary interview was to present the project and
hear the first impressions and spontaneous feedback. The meeting taught about the situation of people
with SCD in Norway, about the approximate number of patients and about the activities done by the
41
association. Finally, discussions were conducted about the president’s general medical history and the
challenges of leading a sickle cell disease patient association. Four patients were enrolled in the focus
groups. During the focus groups, a paper prototyping technique with thinking aloud techniques have
been used. Throughout this meetings, patients could tell loudly what they were thinking while writing
or drawing their ideas. This technique permitted to get instant feedback from them, to understand their
ideas and priority, to reflect about possible concepts of user interface and to get specific requirements
and detailed features.
At the beginning of the first meeting, patients were explained the project’s goal and focus. A
PowerPoint Presentation has been used to support the explanations.
Three patients (two female and one male) who have been recruited by the president and the principal
investigator attended the meetings, bringing the number of patients at four. No one withdrew.
A bias is induced by the fact that the author took several roles; patient, moderator, lead designer and
interviewer. In order to release the workload, the meetings were separated in 3 separated discussions
of about 1 hour and a half with a 15 minutes break in the middle.
For additional information, a summary of the meetings and notes can be found in Appendix 4 and
Appendix 5.
Figure 13 - Photography taken during the last meeting
3.4.1. First meeting
The first half of the first meeting was composed of interviews focused on questions about patients’
history, quality of life and health related issues, as well as their interest in using eHealth tools for their
self-care. We also discussed about how broad were their digital and health literacy. Finally, we talked
about the opportunities eHealth tools for SCD self-management could bring and we discussed about
their main concerns and needs, following the survey questions.
The second half of the meeting was a discussion about their routine health habits, a discussion of their
problem with the healthcare system and the acquisition of the main requirements and suggestions of
features. We frequently compared their opinions with the survey preliminary results.
3.4.2. Second meeting
During the second meeting, patients have been given the task to rank each prototype screen by order
of priority. They had to choose and prioritize functionalities according to their preferences, by giving
a score from 1 to 6 and explaining why. Patients were provided papers and could sketch how they
would imagine the system and add their ideas or suggestions. Then, a discussion about their findings
has been planned for the next meeting and discussions about the preliminary results of the
questionnaire continued. Finally, they provided feedback and ideas on the mock-ups and highlighted
some important issues or requirements that should be taken into account in the revised prototype. At
the end, a new meeting has been planned.
42
3.4.3. Third meeting
The last meeting was focused on the discussion about the modifications to bring into the revised
prototype. We also discussed about the rating they gave on the prototypes in order to clarify the
specifications. The paper prototypes they had done were discussed and a summary on how the
prototype could be improved written. Figure 56 is a photography that has been taken during this
meeting.
3.5. Prototyping
To illustrate the results, the mock-up software “Balsamiq Mockups” has been used to create the User
Interface prototype.
The mock-ups have been designed in reference to the user requirements, taking a user-centred
approach and using the technique of rapid prototyping [132,133], a method allowing to design initial
non-functional prototypes quickly in order to allow the final users to see how the system could look
like and subsequently give feedback. Some requirements have not been designed because they had a
too high or too low level of granularity. Therefore, only the most demonstrative screens have been
designed.
The heuristic requirement of having an easy to use and simple app has been taken into account.
Mainly by dividing the app in various screens with fewer functionalities while keeping a coherent and
intuitive navigation scheme.
3.6. The selection of participants and its limitations
The group of patients who are more susceptible to own mobile devices or being digitally literate are
youth patients and adult patients. The choice has been made to focus only on adult patients (over 18
years old), rather than children. This choice has been made for several reasons:
1. Most of the current research and the existing tools are done for children.
2. The lifespan of patients is increasing, making adult patients more and more numerous
3. Nearly no studies in eHealth are done for adults with SCD
Regarding the sample description, most of the respondents were young adult female and most of the
patients have a high education. Table 6 illustrates the socio-demographic characteristics of
participants.
Respondents are coming from 11 different countries and the majority of the respondents is from
France.
54.5% of respondents have a child living on the same roof but it is unclear if the child is their own or
a sibling. The geographic repartition is illustrated by Figure 23.
Table 6 - Socio-Demographic characteristics of respondents
Distribution
% (N=33)
Respondents
Male
Female
Age
Average
Median
Marital status
Single
Married
Widowed
Separated
Education
Bachelor
24.2
75.8
31.9
31
42.4
45.5
3
9.1
24.2
43
Master
Superior professional degree
High school graduate
Employment status
Student
Employee
Unable to work
21.2
15.2
12.1
30.3
26.4
12.1
Geographic profile of the respondents
33,33%
12,12%
9,09% 9,09%
6,06%
3,03% 3,03% 3,03%
3,03%
3,03% 3,03%
Figure 14 - Geographic profile of the survey respondents
Family and entourage are important in the life of a person with SCD, especially for youth. But since
the study will focus on the creation of a patient-operated system, they will not be allowed to answer
the survey nor taken into account in this study, except for patients who are not able to give their
consent themselves.
No explanation of medical terms, for instance for patients with a low level of health literacy has been
done. It was assumed that the target patients had enough knowledge. It was probably a mistake since
one patient commented at the end of the survey by saying that he had to search for the definition of
some medical terms.
Concerning accessibility issues, people who were not able to answer the questionnaire were given the
option to let a third person answering the survey for themselves and paper copies were available.
The survey was first supposed to be conducted in Sweden, Switzerland and Norway but in order to get
more answers, it has finally been sent globally to patients through patients’ associations globally.
More patients’ associations have been contacted and a researched on Google performed with the
terms “sickle cell anaemia” OR “sickle cell disease” OR “drepanocytosis + “association” in English,
German, Italian and French. About 40 sickle cell disease patients’ associations have been found.
The patients’ associations that are only taking care of children have been excluded, a research on
Facebook retrieved 16 patients’ groups and survey have also been sent to them.
The person of contact on the Facebook group was the group administrator. And the person of contacts
for the patients’ associations were the secretary or the presidents. This was the main way of patient
recruitment (focus groups). To summarize, the questionnaires have been sent to the Facebook groups
and by mail directly to the patients’ associations found on Google and on the website “Orpha.net”.
Surveys have been conducted in France, UK, Canada, USA, Germany, Greece, Switzerland, Canada,
44
Italy, Benin, Belgium, Mali, Niger, Senegal, Mauritania, Madagascar, Martinique, Guadeloupe, Haiti,
Guyana, Cameroon and Burkina Faso. A paper version has been sent to the Swiss patient association
in order to allow patients to fill the questionnaire when they come to hospital for a visit but finally all
patients used the web-survey.
The first deadline for the data collection has first been fixed two months after the beginning but in
order to reach more patients, has been extended to five months later due to the initial low rate of
answers. The opening period are summarized in Table 7 below.
Table 7 - Opening period of the questionnaire
Language
French
English
German
Italian
Activation date
24.02.2015
24.02.2015
08.03.2015
24.06.2015
Deactivation date
24.07.2015
24.07.2015
24.07.2015
24.07.2015
An interview with the president of the now inactive Norwegian patient association, formerly named
the Norwegian Sickle Cell Association has been conducted in order to organize focus groups with
Norwegian patients, this due to the geographical proximity of the study setting. The study has also
limitations due to the low number of participants available. Since in Norway people with SCD are
scarce, dispersed and difficult to find. It decreased the opportunities of patient involvement and only
four focus groups participants have been enrolled. When joining the study (focus groups and survey),
participants had the option to join for further participations on the user interface design, but no
patients made use of this option.
According to reports from the Swiss Patient Association, the reason why some people would not
accept the legal consent was because of the focus on adults. Respondents were not aware of that
before reading the terms and conditions and then dropped.
Nonetheless, the objective being to create a general idea of how a comprehensive self-management
system for SCD patients could look like and not to develop a working system, this method was
sufficient for the scope of the study. However, if the time would allow it and thanks to the SIMED
who provided the material needed, a functional prototype could have been created. The material
available for an implementation of the conceptual prototype was the software development tool Visual
Studio 2015, a smartphone HTC One M7 and an eHealth devices platform Libelium eHealth
Biometric Sensor Platform for Arduino.
3.7. Ethical approval
The study involves patients for the completion of the questionnaire and eventually for the evaluation
of mock-ups of User Interface. There is no cohort done. There is an announcement for patients and a
legal consent page in the beginning of the questionnaire informing patients that they have the ability
to withdraw at any time and in this case, data would be deleted. However, there is a limitation. Since
data are anonymous, the only way to find the patient’s data after they validated their answers is to get
knowledge about when (date and hour) the completion has been done. The questionnaire approval
page has been done following the rules that would apply to a survey requiring an approval from
ethical committee and according to the Revision 2 (October 2013) of the WMA Declaration of
Helsinki5.
The Patient is informed that all data are collected anonymously and the patient has to give an
electronic consent before starting the survey. According to the Swiss Federal Act on Research
involving Human Beings, the Act “does not apply to research which involves anonymously collected
or anonymized health-related data”. Therefore, no seeking of Ethical Committee is needed in
Switzerland. The same rule applies in Sweden, according to the Section 3 of the Personal Data Act
5
http://www.wma.net/en/30publications/10policies/b3/
45
(1998:204) and the ethical Review act as well as in the Norwegian Act relating to medical and health
research, chapter 1 art.206. However, an examination of the International Ethical Guidelines for
Biomedical Research Involving Human Subjects showed that any research using data from human
subjects must seek for Ethical review and the Swedish legislation says that “Ethical approval is
needed if sensitive personal data is handled or intervention affecting a person, who is participating in
the research”. The European directive 2001/20/CE on the best practices in conducting clinical trials
and drugs adds the obligation to give to people who are not able to give their consent, the ability to be
represented legally or to give an oral consent, if a witness is present. In conclusion and considering
that the study will not handle sensitive information, no ethical approval has been sought.
3.8. The data collection tools
Most of the data came from notes taken during the focus groups, from the questionnaires results, the
interviews and from the literature review.
The questionnaire has been created in four languages. All the translations have been made available
on the website “Haemoglobinopathies.ch”.
The questionnaire has been created first using the website Google Forms, then the website Typeform
and finally they have been written in Microsoft Word. The questionnaires have been accessible only
via the website Haemoglobinopathies.ch and linked to the online survey hosted by Typeform.com
Once the questionnaire partly filled by patients, giving enough data to perform a preliminary analysis,
the interview and the focus groups have been conducted in order to assess the relevance of the
questions, to refine them for a future questionnaire and to compare the results of the focus groups with
the results of the questionnaire. See Appendix 4.
3.9.
The processing of the collected data
The analysis of the collected data could give insights about the main patients’ needs, lifestyle,
behaviours, health status, problems and issues faced, digital literacy and also inform about their
interest on eHealth tools to manage their disease. The results of the survey have been analysed by
taking into account the literature review, comparing patients’ needs stated in the literature with
patients’ survey answers and focus groups interviews.
The tool “TypeForm” provided a set of charts that could be used for easier data interpretation and a
Microsoft Excel file is also produced. Basic statistics operation (mean, median, max and min) have
then been performed in order to interpret the results.
6
http://lovdata.no/lov/2008-06-20-44/§20
46
3.10. Summary of the methods used
Table 8 presents an overview of the design and research methods used and their purpose.
Table 8 - Overview of the methods used
Research
question
Q1, Q2
Q1, Q4
Q2
Q2
Q2
Q2, Q3
Q2, Q3
Q2, Q3
Q2, Q3
Q3
Q3
Q3
Issue
Method
Patients’ demographics
Opportunities and relevance
General knowledge about SCD
Patients QOL
Patients self-care
Patients’ health related issues
Patients’ healthcare experience
Patients’ lifestyle
Patients’ motivation and interests
Feedback from patients
Patients’ digital and health literacy
Patients’ needs assessment
Q3
Q4
Patients’ suggestions collection
Prototyping
Q4
Q4
Requirements analysis
Requirements engineering
Questionnaire
Questionnaire, Literature Review
Literature review, Focus groups, physicians
Questionnaire, Focus group, Literature review
Questionnaire, Literature review
Questionnaire, Focus groups
Questionnaire, Interview, Focus groups
Questionnaire
Questionnaire, Focus groups
Questionnaire, Focus groups
Questionnaire
Questionnaire, Focus groups, Interview, Paper
prototyping
Questionnaire, Focus groups
Whiteboard, mock-ups, heuristic design, Focus
groups
Questionnaire, Focus groups
Questionnaire, Focus groups
47
4. Results
4.1. General sociodemographic tendencies
Forty-two people participated to the survey. The questions and the results are available in Appendix 2
and Appendix 3. On the 42 people who participated, 33 people agreed to the conditions and responded
to the mandatory questions. They were then included in the study. Some questions, non-mandatory
have been left unanswered by some patients, but they don’t conflict with the analysis.
Further investigations would be required to find whether if women are affected more regularly by the
disease. Indeed, the results showed that only one man was suffering of the symptoms monthly.
Women must be taken into account for instance by adding information about pregnancy and maternal
health related to SCD. Also, the average age suggests that adult patients might be able to pay for
eHealth tools such as wearable devices. In addition, most of them declared being willing to pay for
such services.
We can assume that the patients who are married would receive assistance from their conjoint in case
of VOC and then would appreciate to let their significant other use the system. Since patients come
from all over the world, the system must be available in their own language. A strategy is to make the
system available in the European languages, but in priority in English. The low number of
respondents from Africa could be explained by the lack of Internet access but more investigations are
needed. The fact that most respondents are titular of a high education diploma shows that most of
them are probably educated enough to understand health information and their disease. Furthermore,
only one person complained about the lack of medical terms explanations. Part of respondents also
have a job, despite studies suggest that sickle cell patients could struggle to get a normal job [23,134].
Some studies show that higher numbers of unemployment are correlated with high number of
hospitalisations due to sickle cell pain crises [134]. The relatively low number of crisis that are facing
the respondents should be taken into account in this reasoning.
4.2. General health tendencies
This first group of question was intended to understand how patients live with sickle cell disease and
what the impact of the disease on their everyday life is. Results show that patients tend to have an
average health status. They are not following so much healthy habits and nearly none of them is
practising physical activity. As some studies suggest, this is probably due to the difficulty for SCD
patients to practice sports or physical activity without the fear of being exhausted and risking to
trigger a crisis, but also probably due to the cardiomegaly [25]. Some studies have shown that being
unable to practice sport affects the moral of patients, making them feel abnormal and isolating them
from social activities. More education, coaching and psychosocial support can make them more
secure and help them gradually practice a physical activity [135].
Respondents think that their following of healthy habits is good enough but not excellent. It would
then probably be useful for patients to receive some information and help about how to live healthy.
4.2.1. Healthy habits and lifestyle
As illustrated in Figure 18, patients feel having a good overall health, the mean score is 6.1/12. 24.2%
of the respondents feel having a very good overall health, as well 21.2% perceive having a good
overall health and also 21.2% feel having an average overall health. The focus groups confirmed this
result. Patients during the meetings reported feeling in good health.
48
How would you rate your overall health?
11 (EXCELLENT)
3,03%
10
3,03%
9
24,24%
8
9,09%
7
21,21%
6 (GOOD)
6,06%
5
21,21%
4
6,06%
3
3,03%
2
0,00%
1 (POOR)
0,00%
Figure 15 - How would you rate your overall health?
The results have shown that most of patients are judging themselves as practicing good healthy habits.
Patients explained during the focus groups that despite temptations from time to time, they were
trying to respect a healthy lifestyle.
Do you feel you have healthy habits?
11 (EXCELLENT)
0,00%
10
0,00%
9
12,12%
8
9,09%
7
30,30%
6 (GOOD)
21,21%
5
9,09%
4
9,09%
3
2
1 (POOR)
6,06%
3,03%
0,00%
Figure 16 - Do you feel you have healthy habits?
In contrast, people perceived practicing an insufficient amount of exercise, the average score is of
2.78/12. The average score is of 5.25/11 with 30.3% of the respondents scoring 7/11. Results are
illustrated by Figure 24, Figure 25 and Figure 26.
49
Do you feel you get the right amount of
exercise?
11 (EXCELLENT)
0,00%
10
0,00%
9
0,00%
8
3,03%
7
6,06%
6 (GOOD)
9,09%
5
12,12%
4
21,21%
3
21,21%
2
1 (POOR)
27,27%
0,00%
Figure 17- Do you feel you get the right amount of exercise?
4.2.2. Impact of SCD on respondents’ daily life
During the last year, 48.5% of the respondents had less than 4 manageable at home VOC. 24.2 % had
between 4 and 7 crises that did not require a hospital admission and the same number had between 8
and 10 manageable at home crises. The maximum was 30 and the minimum 0. The median is 4 and
the mean 5. Results are illustrated by Figure 27 and Figure 28.
Approximately 80% have been hospitalized less than 4 times, which shows that most crises are
managed at home.
57.6% have missed less than 4 days of work or school during the last year. The mean number of day
of work or school missed is of 6.25 days and the maximum is 41 days.
Crises without hospitalisations (last 12 months)
3,03%
24,24%
48,48%
24,24%
0-3
4-7
8-10
Above 10
Figure 18 - Number of crises without hospitalisations during the last 12 months
50
Crises with hospitalisations (last 12 months)
21,21%
78,79%
0-3
4-7
Figure 19 - Number of crises with hospitalisations during the last 12 months
The results point out the difficulty for patients to follow normal day-to-day activities. This requires
further investigations to understand which specific activities make patients struggle the most.
Respondents also reported having difficulty to comply with their medication prescriptions, to sleep, to
avoid infections, pain symptoms or simply would like to feel free of experiencing psycho-social issues
due to the disease. Indeed, as studies suggest [23] and the survey results confirmed, patients often fear
experiencing VOC. Here a mobile health app could have an impact.
Results also confirmed that patients don’t have many crisis requiring hospitalisations and most of the
crises are managed at home. This endorses the relevance of a self-care system that fit in patient’s daily
life. Such a system should then be able to ease the access to healthcare services in case of VOC, since
crises can have devastating consequences.
4.2.3. Symptoms and issues
Most patients are regularly disturbed by pain episodes (81.8%), fatigue (66.7%), headaches (27.3%),
and coldness of the hands (24.2%).
Most of the time, symptoms interfere with patients daily (27.3%), every 2-3 months (24.2%) or
monthly (12.1%).
Patients have experienced plenty of complications related to SCD. Infections have touched 75.9% of
respondents, chronic pains account for 72.7%, acute chest syndrome for 60.6% and gallstones for
39.4%. The majority of patients (54.55%) also expressed difficulty having a normal physical activity
and 36.4% had troubles practicing self-care actions like taking their routine medication regularly.
Moreover, 33.3% have difficulty completing normal day-to-day activities. Many patients (30.3%),
experience psycho-social issues like having a low level of self-confidence, missing school or work on
a regular basis (27.3%), being treated differently by others (27.3%) or having difficulty to sleep
(24.2%). Finally, 94% of the respondents know at least one other person with SCD.
For these questions, respondents were asked to provide several answers. Figure 29 and Figure 30
shows the complete results.
51
Have you experienced one of these
complications?
INFECTIONS / FEVER
CHRONIC PAIN
ACUTE CHEST SYNDROME
GALLSTONES
SPLENIC CRISIS
EYES PROBLEMS
PULMONARY EMBOLISM
PULMONARY INFARCTION
HAND-FOOT SYNDROME
KIDNEY STONES
PULMONARY EDEMA
NONE
MYOCARDIAL INFARCTION
PULMONARY HYPERTENSION
STROKE
MULTIPLE ORGAN FAILURES
75,76%
72,73%
60,61%
39,39%
24,24%
21,21%
21,21%
12,12%
12,12%
9,09%
9,09%
6,06%
6,06%
6,06%
3,03%
3,03%
Figure 20 - Have you experienced one of these complications?
Results also show that patients have experienced many life-threatening complications. This
accentuates their fragility, especially in case of frequent rehospitalisation. For these reasons, the
system should help patients to avoid crises.
The low number of hospitalisations reported by patients can explain why most patients are able to get
a normal job and follow a normal education. For patients with high rates of hospitalisations as well as
for patients who are relatively healthy, messages sent to the employer, awareness campaigns and
schedule arrangements may help employers to deal with the sickest patients and thus decrease the
discrimination.
What symptoms are the most disturbing
for you daily?
PAIN EPISODES
81,82%
FATIQUE
66,67%
HEADACHES / DIZINESS
27,27%
FASCICULATIONS
24,24%
COLDNESS OF THE HANDS OR FEET
24,24%
INFECTIONS / FEVER
15,15%
RAPID OR IRREGULAR PULSE
12,12%
THE DISEASE DOES NOT AFFECTS ME
9,09%
PALENESS
9,09%
OTHER: AMNESIC EPISODE
3,03%
Figure 21 - What symptoms are the most disturbing for your daily?
The results generally relate to what studies suggest, most patients are suffering from anaemia related
symptoms and from recurrent pain symptoms during their daily life. This put forward the need to
52
regularly monitor the symptoms. There is no need to monitor the symptoms continuously since few
patients are reporting suffering all the time, but daily or several times per month should be sufficient.
Collecting and analysis such self-tracked information could help managing the most frequent
symptoms and propose feedbacks or advices for their pain management or sleep management. The
advices can be based on the documents about self-care management and self-care guides [136] in
combination with information and health data collected from patients’ associations. Such information
could be included in the system as well as reminders for instance to keep patients hydrated enough or
being assisted when following healthy habits. Most patients were feeling interested about learning
more about their disease and about patient communities able to support them. This kind of
information should then be included too. In addition, some patients need to travel a long distance in
order to contact a healthcare provider or to meet another patient. As shown in the literature, remote
communications tools can have an impact.
4.2.4. Relationship with the healthcare system and health literacy
Patients are interested in learning more about the disease, especially about corresponding support
groups and communities (60.6%), the cause of triggering of VOC (48.5%), the drugs and their sideeffects (48.5%) and other particularities of the disease like research and news (42.4%). Websites like
the British National Health Services (NHS) specialized care centre can serve as inspiration (See
Background and theory).
Most patients see their doctors about 3-4 times per year (39.4%), 5-7 times per year (18.2%) or
monthly (27.3%). Results are illustrated by Figure 31. In order to see their healthcare provider, patient
have to travel in average 28km. The median distance is 9.5km and the maximum is 296km.
Patients feel that the healthcare provider they see the more often is never (27.3%) or rarely (33.3%)
able to see when they are getting sicker or would need a check-up, while 27.3% feel their healthcare
providers is sometimes able to see it. See Figure 32.
The results show that 27.3% are extremely likely willing to share their health information, 24.2% very
likely to share it 21.2% are not at all willing to share it. See Figure 33.
Overall, 27.3% of patients are not at all satisfied with the way the healthcare system is taking care of
their disease, 30.3% are poorly satisfied and 33.3% have a normal satisfaction level. See Figure 34.
In addition, 42.4% declared having a moderate trust in their healthcare providers about making
decisions that are in their best interest.
How often do you see your doctor?
2-4 TIMES PER YEAR
39,39%
MONTHLY
27,27%
5-7 TIMES PER YEAR
ALMOST NEVER
18,18%
9,09%
8-10 TIMES PER YEAR
3,03%
DAILY
3,03%
Figure 22 - How often do you see your doctor?
53
Is your healthcare provider able to see if
you are getting sicker or need a check-up?
4 (ALWAYS)
12,12%
3
27,27%
2
33,33%
0 (NEVER)
27,27%
Figure 23 - Is your healthcare provider (the one you see often) able to see if you are getting sicker or need a check-up?
Would you like to share your health
information with other people?
5 (EXTREMELY LIKELY)
27,27%
4
24,24%
3
18,18%
2
9,09%
0 (NOT AT ALL LIKELY)
21,21%
Figure 24 - Would you like to share your health information with other people?
Overall, are you satisfied with the
way the healthcare system is taking
care of your disease?
5 (EXTREMELY)
4
0,00%
9,09%
3
2
0 (NOT AT ALL)
33,33%
30,30%
27,27%
Figure 25 - Overall, are you satisfied with the way the healthcare system is taking care of your disease?
54
4.3. ICT receptiveness
The following answers relate to respondents’ digital literacy and to their interest in using eHealth
tools to manage their disease.
4.3.1. Digital literacy
Patients identified themselves as extremely well familiar (57.6%) with the use of Internet, very well
familiar (27.3%) and moderately (15.1%) well familiar. See Figure 35.
They identified themselves as extremely well familiar (72.7%) with the use of Smartphones, very well
familiar (18.2%), moderately (15.1%) well familiar (6%) and poorly familiar (3%). See Figure 36.
Most of patients were receptive about the use of eHealth tools for their health, 60.1% answered very
positively, 21.2% positively and 18.2% were neutral.
When asking about what technology they use on a regular basis, 100% declared they used
smartphone, 94% using a Computer and 64% are using a Tablet. 85% use a messaging software like
WhatsApp or SMS and 67% use a communication software like Skype.
Respondents declared using those technologies for seeking health information everyday (18.2%), 3-6
times per week (12.1%), 1-2 times per year (15.2%) or every 3-4 months (12.1%).
To get information about SCD, 88% declared having preferred contacting patients group that meet in
person, 72.7% liked using websites or search engines to retrieve health information and 55% preferred
Facebook groups. See Figure 37.
Respondents are active on the Internet to talk about SCD. They mostly use online support groups
(81.8%), Facebook (63.6%), online forums or chats (12.1%). 30.3% of respondents are not active on
the Internet but are interested to become (30.3%). See Figure 38.
Regarding the use of ICTs by their entourage to search for health information, 81.2% of the
respondents declared using Tablets, Computers (54.55%) or Smartphones (24.2%). Respondents were
asked to provide several answers.
How familiar are you with the use of
Smartphones?
4 (EXTREMELY WELL)
72,73%
3
18,18%
2 (MODERATELY WELL)
6,06%
1
0 (NOT AT ALL WELL)
3,03%
0,00%
0,00% 10,00% 20,00% 30,00% 40,00% 50,00% 60,00% 70,00% 80,00%
Figure 26 - How familiar are you with the use of Smartphones?
55
Do you use on a regular basis any of
the following technologies?
NONE OF THE ABOVE
0,00%
MESSAGING SOFTWARE
84,85%
COMMUNICATION SOFTWARE
FITNESS TRACKER
SMARTWATCH
66,67%
6,06%
0,00%
TABLET
63,64%
SMARTPHONE
100,00%
COMPUTER
93,94%
Figure 27 - Do you use on a regular basis any of the following technologies?
Most respondents are used to access digital health information through smartphones and computers.
They also responded being regular users of the most common messaging and communication
software. This suggests the learning curve they would have to escalate in order to use a mobile app
designed for them would be short.
What have been the best places to get info
about Sickle Cell disease?
FAMILY / FRIENDS WHO HAVE SEARCHED FOR ME
24,24%
PATIENTS GROUPS THAT MEET IN PERSON
87,88%
FACEBOOK GROUPS
54,55%
ONLINE CHAT ROOMS / FORUMS
6,06%
ONLINE PATIENT COMMUNITIES
18,18%
ONLINE SUPPORT GROUPS
18,18%
WEBSITES / SEARCH ENGINES
72,73%
DOCTOR'S APPOINTMENTS
33,33%
Figure 28 - What have been the best places to get info about Sickle Cell disease?
Since many patients preferred using Facebook groups and meeting patients in person to retrieve health
information about SCD, integrating Facebook groups and patients’ associations’ content, as well as
implementing a social network engine should be one of the main requirements of the system.
56
Few patients were using wearable devices and none of them declared using a smartwatch. Despite
that, the majority said they would prefer to use their smartphone but also showed a very pronounced
interest in using wearable devices and smartwatches to track and manage their health. Also, nearly no
patients declared using an app for managing their health, which can be related to the absence of sickle
cell focused apps on the worldwide market.
Where have you been active on the
internet about SCD
OTHER
0,00%
6,06%
NONE OF THE ABOVE AND I'M NOT INTERESTED
NONE OF THE ABOVE BUT I'M INTERESTED
30,30%
0,00%
TWITTER
ONLINE SUPPORT GROUPS / PATIENTS
COMMUNITY
81,82%
BLOG / FORUMS / CHATS
12,12%
FACEBOOK OR OTHER SOCIAL NETWORK
63,64%
Figure 29 - Where have you been active on the internet about SCD?
4.3.2. Services wanted from a health app
About the kind of technologies respondents would consider the most to use to manage their disease,
82.8% declared preferring using a Smartphone app, 45% would prefer to use a website, 34% would
use a remote monitoring or telemedicine device and 27.3% would use a smartwatch. In order to be
convinced to use an app regularly, 72.7% prioritized a system that provide accurate and trustworthy
information, 48.5% would like to have a system that is simple to use and well designed, 45.4% would
like the system to raise awareness about the disease and educate people and patients, 36.3% want a
future proof system that work over time and 33.3% wish a system that allows them to provide support
and help to other patients. Concerning the costs of such a system, 30% would like a free of charge
system. The rest of patients did not prioritize this.
Among all respondents, only 9% are currently using an app to manage their disease.
As Figure 39 illustrates, 36.3% of them would be extremely interested in owning a wearable device
that can track their health status, 27.3% would be very interested and 24.2% moderately interested.
Only 12% are currently wearing a smartwatch or a fitness tracker but 54.6% have declared being
interested in. See Figure 40.
The most important functionalities patients would like to have from a health apps are to receive help
to early detect the signs of VOC (75.8%), receive general information about the disease (75.8%),
receive advices and feedback about how to self-care in their daily life (60.6%), receive help to prevent
the triggering of VOC (57.6%), receive information and feedback about their current health status
(51.5%), ease the access to care in case of emergency (42.42%) and receive assistance to conduct a
healthy lifestyle (42.4%). See Figure 41.
Most of the respondents find particularly important to carry their health information on themselves.
54.5% found it extremely important, 24.24% very important and 18.2% important. Respondents were
asked to provide several answers.
57
What kind of technology would you consider the
most to use in order to manage your disease?
81,82%
45,45%
39,39%
27,27%
24,24%
24,24%
21,21%
15,15%
9,09%
3,03%
Figure 30 - What kind of technology would you consider the most to use in order to manage your disease?
Their biggest motivation and concern about using an app to manage their health was to be sure to get
accurate and trustworthy information. Indeed, providing accurate advices that are not harmful for
them is a priority requirement, but also in order to comply with regulatory authorities such as FDA. In
addition, patients would like to use a future-proof, well-designed and easy to use system that can help
them raise awareness about the disease and educate other people. Patients have selected and
prioritized specific functionalities, the most important being to receive assistance and help to learn
how to detect early signs of VOC. They wished in order to succeed, to get live information about their
health status, general information about the disease and feedback on their lifestyle as well as on the
way they self-care. This confirms again that the main concern of patients is to avoid triggering a crisis
as much as possible. Here, ICTs tools and data mining techniques can be used to analyse patients’
health information and give them feedback.
58
What would convince you to use a health app
regularly?
PROVIDE ACCURATE AND TRUSTWORTHY INFORMATION
72,73%
BE EASY TO USE AND SIMPLE / WELL DESIGNED
48,48%
RAISE AWARENESS ABOUT THE DISEASE AND EDUCATE PEOPLE
45,45%
WORK EFFECTIVELY AND CONSISTENTLY OVER TIME
36,36%
ALLOW YOU TO PROVIDE SUPPORT AND HELP TO OTHER
PATIENTS
33,33%
BE FREE
30,30%
PROVIDE GUARANTEES OF DATA SECURITY AND INTEGRITY
27,27%
HAVE NO ADVERTISEMENTS
ALLOW YOU TO RATE OR COMMENT ON A HEALTHCARE
SERVICE YOU RECEIVED
BE LESS EXPENSIVE THAN ANY OTHER FORM OF HEALTHCARE
SUPPORT
OTHER: I DON'T KNOW
24,24%
18,18%
15,15%
3,03%
Figure 31 - What would convince you to use a health app regularly?
Another concern is to receive help, when they can’t avoid a crisis and are in pain, especially when
requiring access to emergency healthcare. They think it’s extremely important to be able to facilitate
the care process, concretely by carrying their health information all the time. This feature would
require the use of devices such as stationary, portable, worn or implanted medical device, but could
also be digitized information on their smartphone.
Respondents declared not journaling their symptoms or hospital admissions. We can suspect it’s
because it’s not a convenient process and because no tools are specifically adapted for this purpose.
Providing patients, a tool that could help them automatically and simply journal their health status,
symptoms, vital signs or others factors during their daily- activity could have a positive impact on
their motivation, especially if, as they asked, alerts and reminders (not disruptive) could be sent to
them. Moreover, and since they showed a good interest in using self-tracking devices, this should be
easily accepted.
59
What are the most important services a health
app should offer you?
HELP TO DETECT THE EARLY SIGNS OF CRISIS
75,76%
OFFER GENERAL INFORMATION ABOUT YOUR DISEASE
75,76%
ADVICES AND FEEDBACK ABOUT HOW TO SELF-CARE IN
YOUR DAILY LIFE
60,61%
HELP TO AVOID THE TRIGGERING OF CRISIS
57,58%
INFORMATION ABOUT YOUR CURRENT HEALTH STATUS
51,52%
EASE THE ACCESS TO MEDICAL ASSISTANCE IN CASE OF
EMERGENCY
42,42%
HELP TO HAVE A HEALTHY LIFESTYLE
42,42%
OFFER A PERMANENT CONTACT WITH HEALTHCARE
PROVIDERS
33,33%
PROVIDE INFORMATION ABOUT YOUR DISEASE IN CASE OF
EMERGENCY
30,30%
ASSIST WHEN PERFORMING PHYSICAL ACTIVITIES
30,30%
ACCESS YOUR MEDICAL RECORD AND ALLOW TO KEEP IT
UP TO DATE
27,27%
ARRANGE AND MANAGE YOUR MEDICAL APPOINTMENTS /
CHECK-UPS / FOLLOW-UP
24,24%
HELP TO CONTACT SUPPORT GROUPS IN CASE OF CRISIS
18,18%
PROVIDE INFORMATION THAT EDUCATES YOUR FRIENDS /
FAMILY / PEOPLE YOU MEET
15,15%
OFFER AN EMOTIONAL / PSYCHOLOGICAL SUPPORT
OUTSIDE CRISIS
12,12%
HELP TO CONTACT SUPPORT GROUPS OUTSIDE CRISIS
9,09%
OFFER AN EMOTIONAL / PSYCHOLOGICAL SUPPORT IN
CASE OF CRISIS
9,09%
EASE THE ACCESS TO MEDICAL ASSISTANCE IN EVERYDAY
LIFE
9,09%
Figure 32 - Most important services an app should provide
60
4.4. Self-tracking
The following answers help to understand to which extent patients are tracking their health status and
to identify what patients would wish to track. Respondents were asked to provide several answers.
4.4.1. Logging habits
Most of respondents never (66.7%) or nearly never (12.1%) log their pain symptoms. A similar result
was reported regarding their hospital admissions, 54.5% declared never reporting them and 12.1%
nearly never did it. 51.2% declared tracking none of their health or wellness information when 24.2%
are tracking their health status, 21.2% their pain symptoms and 18% their vital signs or physiological
data.
4.4.2. Self-tracking wishes
When asking patients what data they would like to automatically collect, 57.6% responded they want
to track their health status, 57.6% would like to have their pain and clinical symptoms collected,
57.6% are interested in tracking other factors, like environmental factors that can affect their health,
51.5% are willing to collect their vital signs and physiological data, 48.5% want to have their fatigue
and tiredness level track, 39.4% their medicine intake, 33.3% their diet, 33.3% their mood and anxiety
level, 24.2% their sleep and 21.2% wish to track their physical activity. See Figure 42.
42.4% want in high priority to receive alerts when their health status is worsening and 36.4% really
want it but prioritize it less.
Regarding the reception of alerts or reminders when their medicine should be taken, 45.4% want it in
high priority.
Which of the following would you like the
most to be automatically collected?
HEALTH STATUS
57,58%
PAIN AND CLINICAL SIGNS OR SYMPTOMS
57,58%
FACTORS THAT CAN AFFECT YOUR HEALTH STATUS
57,58%
VITAL SIGNS AND BIO-DATA
51,52%
TIREDNESS
48,48%
MEDECINE INTAKE
39,39%
DIET
33,33%
MOOD / ANXIETY LEVEL
33,33%
SLEEP
24,24%
PHYSICAL ACTIVITY
21,21%
NON-SICKLE CELL RELATED PAINS
21,21%
DAILY ROUTINES
18,18%
MOTIVATION
12,12%
FEELINGS AND EMOTIONS
15,15%
SOCIALIZING LEVEL
9,09%
NONE
6,06%
Figure 33 - What do patients would like to have automatically collected?
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4.5. Free-text questions
The next answers are non-mandatory free-text answers given by patients at the end of the survey.
They have been categorized by similarity.
Firstly, respondents reported being mainly worried about avoiding crises and secondly they were
concerned about following a healthy lifestyle that could help them avoid the crises. Those two
concerns are partly linked. They demonstrate that eHealth tools supporting their concerns about
following a healthy lifestyle in order to avoid VOC as much as possible and solutions supporting the
management of the cornerstones of sickle cell disease self-care should be prioritized.
Examples of solutions for others chronic diseases have shown that existing technologies are able to
solve those issues, providing information to patients when needed.
4.5.1. In everyday life, what is your biggest concern (about the disease)?
On a total of 29 respondents, around 41% fear to trigger a vaso-occlusive crisis that would lead to
complications like stroke or death. They are scared not to be able to detect them, not be able to
manage the crises at home and being forced to be admitted to hospital. About 21% of the respondents
recognized also the difficulty to manage their chronic pain and to take their medication. See Figure
43.
Patients' biggest concerns
AVOID VOC AND THE POTENTIAL HOSPITALIZATIONS
AND LIFE-THREATENING COMPLICATIONS
41,38%
TREATMENT, MEDICATION AND HEALTHY
BEHAVIORS
20,69%
DISTANCE FROM FAMILY AND ENTOURAGE IN CASE
OF VOC
10,34%
PAIN MANAGEMENT
10,34%
PREGNANCY
10,34%
HEALTH STATUS MAINTENANCE
6,90%
Figure 34 - What is your biggest concern in your daily life?
4.5.2. In your own words, what other features or services would you appreciate?
Respondent’s answers have been divided in 6 categories. Patients would like to have a selfmanagement mobile app that takes into account the cornerstone of sickle cell disease selfmanagement (31.4%), a system that would help them to get a timely and effective help in case of
VOC (22.9%) and a system that monitor the potential triggers of VOC and alert them in case of
worsening (17.1%). See Figure 44.
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In your own words, what other features
or services would you appreciate?
SELF-MANAGEMENT APP MANAGING THE
CORNERSTONES
31,43%
ASSISTANCE TO GET FAST AND EFFECTIVE
EMERGENCY CARE
22,86%
MONITORING OF POTENTIAL TRIGGERS WITH
ALERT (HEALTH STATUS, VITALS SIGNS,
ENVIRONMENT, ETC.)
17,14%
FACILITATE COMMUNICATION WITH OTHERS
11,43%
COACHING FOR SPORT, HEALTHY BEHAVIOR
8,57%
PSYCHO-SOCIAL SUPPORT
8,57%
Figure 35 - In your own words, what other features or services would you appreciate?
Additionally, since patients felt isolated and reported receiving sub-optimal and slow care, as well as
complained about the lack of awareness and education of healthcare professionals, solutions
answering those issues could be developed. Solutions such as eHealth tools enabling them to get a
better access to healthcare and to avoid as much as possible using the healthcare system by educating
them and providing timely and useful information to healthcare providers. Finally, patients reported
liking the quality of care when they receive them, the main difficulty being to get access to healthcare.
4.5.3. What could or should be improved in the healthcare system that takes care of you?
After sorting the free-text answers in 3 categories, a general trend is marked. Most patients (73%)
think that the healthcare system need to be improved. Many people complained about the general lack
of education and training of caregivers, the slowness and bad quality of emergency care as well as the
often wrong triage and the long waiting times. Also, respondents felt often that caregivers would not
listen to them and that the number of specialized and trained nurses or doctors was too low.
16% of respondents are wishing to have improved follow-ups, getting an easier and better organized
access to ambulatory exams and an optimized care by day hospitals in order to allow hospitalisations.
Finally, 12% wished that the public health authorities would raise the awareness of the disease, by
creating for instance TV advertisements or awareness campaigns in schools or hospitals. Some
patients also reported wishing to receive a financial help for their daily medication. See Figure 45.
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In your own words, what could or should be
improved in the public healthcare system?
15%
Awareness of population
12%
Healthcare professionals
education/awareness and hospital
organization
Support for the follow-up
73%
Figure 36 - What should be improved in the public healthcare system?
4.5.4. In your own words, what is particularly well in the healthcare system that takes care of
you?
23 patients answered this question. 39.13% of the respondents appreciate the availability of
caregivers, treatments and the general quality of care. 26% also liked their follow-up. See Figure 46.
What is particularly good in the
healthcare system?
AVAILABILITY AND QUALITY OF CARE
39,13%
FOLLOW-UP
26,09%
HEALTHCARE PROFESSIONALS EDUCATION
COST
17,39%
8,70%
DIAGNOSIS
4,35%
PAIN MANAGEMENT
4,35%
Figure 37 - What is particularly good in the healthcare system that takes care of you?
Moreover, patients reported a general dissatisfaction with the healthcare system. They wished to be
able to trust their healthcare provider more and they emphasized that healthcare professionals and
even their entourage were rarely able to anticipate when they would become sick. Consequently, they
would appreciate the possibility to share their health information with other people.
This emphasizes the need of a regular remote monitoring system that can transfer automatically data
to healthcare provider or other recipients. A solution integrating and analysing data from wearable
64
devices, allowing patients to perform remote consultations or discuss issues through online chats and
forums, can be embedded on a mobile app to help patients wherever they are and then have the
potential to answer their issues and show that sickle cell disease patients can actually feel abandoned
and sub-optimally receiving care.
4.6. Non-mandatory questions
These are the last questions, they were non-mandatory in order to decrease the survey length and
because not entirely related to patients’ self-care needs. However, they can possibly give more insight
about what patients would wish.
4.6.1. If a service answering your needs, wish or suggestions was available today, would you use
it?
Around 35% would use it with an extreme enthusiasm, 41% would use it with a very pronounced
enthusiasm and approximately 19% would use it with a neutral enthusiasm. Nobody did express a
negative opinion.
4.6.2. Would you pay for it?
34.4% would pay for it without special enthusiasm and are rather neutral. 18.8% would very likely
pay for it, 12.5% would pay, while 25% would rather not pay for it, and 9.4% would certainly not pay
for it.
4.6.3. Would you recommend it to a patient community or to your doctor?
53.1% would recommend it with enthusiasm, 28% would recommend it and approximately 19% are
rather neutral. Nobody did express a negative opinion.
4.6.4. When you think about this project, is it rather something patients need or want?
Approximately 72% of the respondents think this would be something needed while about 28% would
think it’s something rather wanted. Some free-comments from patients said it would rather be both;
needed and wanted.
4.6.5. What has been your first reaction when you heard about this project?
About 50 % of the respondents had a very positive reaction, 18.8% were neutral, 15.6% had an
extremely positive reaction and 15.6% had a positive reaction. Nobody did express a negative
opinion.
4.6.6. What is your opinion about this survey?
16.7% of respondents are extremely positive, 20% are very positive, 36.7% are positive and 26.7%
are neutral. Nobody did express a negative opinion.
4.6.7. Feel free to write any critic, comment or suggestion
Among the free critics and suggestions, most were encouragements and congratulations. Some people
suggested to improve the questionnaire, by making it shorter, adding more symptoms like yellow
eyes, give a small explanation of the symptoms and provide assistance for illiterate people. People
would wish to receive more emotional support and psycho-social support. Some also suggested to
modify the question 4.7.4 about the need of eHealth tools for SCD self-management by allowing too
choose both answers.
4.7. Preliminary mock-ups
These are the prototypes made in order to illustrate the preliminary results and the SCD selfmanagement best practices. They served as thinking base and feedback platform during the focus
groups.
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4.7.1. Whiteboard prototyping
After having read in the literature the biggest concerns of patients, extracted the cornerstones of sickle
cell disease self-management and reflected about how an app could look like, a preliminary
whiteboard mock-up has been sketched. The sketch takes the form of a simple journal page that
summarizes the self-tracked information from a medical device. One can see the level of oxygen, the
pulse rate, the temperature, the last quantity of food ingested, the quantity of water intake, the
medication taken, the altitude, the quantity of sleep and level of fatigue, the quantity of physical
activity performed, and the quantity of pain symptoms that occurred.
The idea is to present in one page some of the most important elements of data that should be taken
into account to avoid triggering a VOC. The middle panel shows the current probability of triggering
a crisis, in order to give instant feedback to the patient. Figure 47 contains the draft of a chart showing
the sleep patterns, the quantity of rest, the quantity of physical activity performed and the fluctuations
of oxygen level. The thinking was to give the possibility to show custom information, blood related
level, stress level or pH level could be added. A tap on any element would open detailed information
and a user could enter data.
After having received the first questionnaire results and in order to prepare the focus-groups meeting,
the design reflection has been pushed forward. Consequently, rapid prototypes that could be more
representative of a potential solution for patients have been created.
Figure 38 - Sketches of potential main page
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4.7.2. Non-functional requirements
This chapter describes the non-functional requirements extracted from the literature review and the
questionnaire results.
- Affordability
o The system shall not be expensive for patients (out of pocket), this in order to be
accepted by people who were not willing to pay or for people with limited financial
resources. Although, payers like welfare programmes or insurances could subsidize
the fees.
- Compliance, Privacy, Security, Certification
o According to the survey results, few patients are concerned about the security and
privacy of their health information, but since they would like to have health
information sent to caregivers and to receive their medical record information, a
compliance to countries specific norms would be required.
o The system shall be for instance ready to be compliant with data privacy regulations.
Measures of data protection that ensure the integrity, confidentiality and security of
health information would also have to be put in place. Implementing them could be
facilitated by existing commercial solutions for data encryption, access and access
logging.
o The system, since features such as giving feedback on patients’ health status could
have a direct impact on patients’ health, shall be certified as a medical device.
- Effectiveness and quality
As requested, the system shall provide accurate, trustworthy and non-harmful
information
4.7.3. Preliminary prototype
This section shows the mock-ups that have been created after the preliminary analysis of the survey
results and based on the whiteboard prototype.
4.7.3.1. Menu
The concept of this navigation menu is to be a side-menu that would appear on demand and being
hidden most of the time. This is thought like that in order to maximize the room on the screen. The
menu incorporates a panic button that would send the user to the screen “In Case of Crisis”.
The elements “SCD News”, “Living well”, “Logbook”, “Points”, “Monitor”, “Analysis”, “Medical
record”, “Schedule”, “Ask a doctor”, “Warriors”, “Settings” and “Panic” are meant to be links to the
respective screens.
The element “About SCD” would link to general information about sickle cell disease in order to
inform patients and other interested people about the disease. The section “What is SCD” illustrates
what kind of information could be integrated.
The pages “Profile”, “Manual”, “Help”, “Legal” have not been created because their granularity level
is too small. Additionally, they can be interpreted as standard features and then patients could imagine
how they could look like relatively easily.
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4.7.3.2. Living well with Sickle-Cell Disease
This function, illustrated in Figure 48, demonstrates the need of getting trustworthy information and
advices about how to live well with the disease. The navigation on this screen would be as follows;
the main page would offer quick information about the cornerstones of SCD self-management and
selecting a section would send to a more detailed page where the patient could also share the
information with chosen people. Scrolling down the main page would show more sections.
The sections “Living well with SCD”, “Usual Symptoms”, and “What is SCD” show in more details
what could be the information displayed when a user would select an item. In order to fill the different
screens with content, information found on the American Centre for Disease Control (CDC) self-care
toolkit, on the websites of the Sickle Cell Disease American Association, the American Mayo Clinic
hospital, the NHS, the WHO, MedLinePlus and WebMD have been summarized and synthetized.
4.7.3.3. SCD News
This page illustrates how patients could receive or share location-based news about SCD, information
about patients associations, hospitals, pharmacies or specialized care centers. The user could choose
from which sources he desires to retrieve information. The mock-up shows information from Google
News. See Figure 48.
4.7.3.4. In case of crisis
This page would assit patients when they need to get healthcare assistance. They would be able to
locate and describe their pain, tell what medication they took, send their vital sign that would have
been taken by wearable sensors, say if they experienced complications symptoms and alert the people
they want or ask for an ambulance. See Figure 48.
Figure 39 Preliminary mock-ups for discussion with patients (Living well with SCD, SCD news and, In case of crisis)
4.7.3.5. Logbook
This page would illustrate the dashboard where patients could get information about their instant
health metrics, their health status or feedback about their lifestyle. They could also see any kind of
collected information from sensors, from third party systems or from their environment. For instance,
they could see their physiological data and the data coming from electronic health record but also
check their live overall health or the risks of triggering a crisis relatively to their behaviour. This page
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could also give them feedback on their health and advices on how they should behave to improve it.
Encouragements could also be given if patients are doing well and advices to keep them feeling good.
See Figure 49.
4.7.3.6. Monitor
This page would give users more detailed information on data they tracked or on data coming from
their medical record. The page could also show them alerts when they take too much medicine or a
reminder for their medication intake. The page would also give them additional information like tips,
feedback or information about drugs-drugs interactions. They could also order a blood analysis or a
blood transfusion if they blood parameters require it. Additionally, they could refill their
prescriptions, order exams, or simply track their mood, their diet, their physical activity or other
parameters. See Figure 49.
4.7.3.7. Analysis
This page would give insights on the data collected like statistics on their past or trends based on the
past information and the most recent data. Users would have the possibility to send these to their
caregivers or other users. See Figure 49.
Figure 40 Preliminary mock-ups for discussion with patients (Logbook, Monitor and Analysis)
4.7.3.8. Schedule
This page is intended to help patients manage their medical appointments. They could book
appointments, search for appointments and their directions, retrieve more information about a
scheduled exam, modify it or cancel it. See Figure 50.
4.7.3.9. Points
This page is intended to illustrate the possibility of implementing gamification items such as points to
motivate people using the app. The information displayed on this page would be related to patient’s
health habits, by giving them incentives, feedback and statistics on the way they behaved. The idea
was to allow patients to receive recompenses if they would behave in a healthy way. Rewards could
be materialized as vouchers to buy items in a pharmacy or suggest them to make a donation to a
foundation or Non-Governmental Organization (NGO). See Figure 50.
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4.7.3.10. Settings
This page would concentrate all the parameters that users could set. The most important are the
possibility to set reminders, write medical information and emergency information in order to
accelerate the care process in case of emergency. See Figure 50.
Figure 41 - Preliminary mock-ups for discussion with patients (Settings, Points and Schedule)
4.7.3.11. Online consultation
This page shows how patients could communicate remotely with a care provider. The screen offers
the possibility to get a conversation with a doctor, via a live chat or with a video call. A patient could
choose his interlocutor from a list of people available. See Figure 51.
4.7.3.12. Sickle Cell Warriors
This page is designed to help users getting support or providing support to others. This page could
answer some of the psychosocial issues faced by patients. The users have the possibility to create and
participate in group discussions, social activities, chat rooms with other patients or could wrote
private messages. They could also access a list of the existing patient communities in their area or in
any place they would wish to go. See Figure 51.
4.7.3.13. Medical record
This page would summarize the data that patients synchronize with their electronic medical record.
Patients could also write or modify documents, like their emergency summary and their medical
notes. They could print them, send them to their caregiver or share them data with a third person.
They would also have the option to export their data for their own use. The mock-up illustrates the
keystones of a medical record; the emergency card, the medical summary and history, the diagnoses,
the interventions done, the laboratory results, the prescriptions, the allergies, the vaccines, the people
who access the medical record or take care of the individual but also other documents like discharge
letters or radiographies. The laboratory results section shows the most important SCD related
parameters that can be extracted from a blood analysis. See Figure 51.
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Figure 42 - Preliminary mock-ups for discussion with patients (Medical record, online consultation and Sickle Cell
Warriors)
71
4.7.3.14. What is SCD
This page gives a more detailed information about SCD like the risk factors, the key facts and how to
monitor a SCD patients. See Figure 52.
4.7.3.15. Living well with SCD
This page gives information about what kind of healthy behaviour could prevent or diminishes the
triggering of VOC. See Figure 52.
Figure 43 - Preliminary mock-ups for discussion with patients (What is SCD and Living well with SCD)
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4.7.3.16. Usual symptoms
This page would provide general information about the symptoms that face SCD patients. See Figure
53.
4.7.3.17. How to treat SCD
This page would provide general information about how SCD patients are and how they can be
treated. See Figure 53.
Figure 44 - Preliminary mock-ups for discussion with patients (Usual symptoms, How to treat SCD)
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4.7.3.18. Live chat
This page was a simplified prototype of what a live-chat could look like in order to communicate with
a care provider for getting simple advices. See Figure 54.
4.7.3.19. Support Groups
This page is an early prototype of an information page about the display of available support groups
or communities. See Figure 54.
Figure 45 - Preliminary mock-ups for discussion with patients (live Chat and Support groups)
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4.7.3.20. Nearby
This page would give location-based information about medical specialists, pharmacies or support
groups that could be helpful for SCD patients. See Figure 55.
4.7.3.21. Sickle Cell News
This page would give information about the ongoing research or provide updates concerning sickle
cell disease like new advancements or events. See Figure 55.
Figure 46 - Preliminary mock-ups for discussion with patients (suggested prototype for informational app)
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4.8. Focus groups results analysis
Patients reported feeling alone and not receiving care or help in an optimal way, especially regarding
pain management. They reported that this made their mood fluctuating constantly. Studies confirm
these findings, SCD patients often reporting mood and psychological issues [137].
Participants liked the idea of a mobile health app and, similarly to the survey respondents, stressed
needing and wanting solutions to support their self-care. Participants wanted mainly to track their
SCD related symptoms, the factors that could affect their health and wished to receive psychological
support from professionals and social support from other patients. Additionally, they hoped that
technologies could help them getting information on their current health status in order to make better
informed decisions.
Patients felt under-informed about the long-term consequences of the disease and about the adverse
effects of the medication they had to take (hydroxyurea). Indeed, the research on the field is lacking
but the access to information that is already known could be facilitated by a mHealth solution. Indeed,
participants said they would like to have the right information, at the right time and if possible in their
pocket.
Participants altogether showed a surprising interest in wearing a smartwatch giving them prompt
information about their health status and the majority felt motivated to have an implantable device
that could collect their health data.
They also said wanting to have a system with particularly comprehensive features, but stated they
would have preferred to have a highly versatile and customizable interface where they could be able
to pin or arrange features in a different layout and order. They justified this requirement after
discussing how every aspect of the disease is important, but could vary from patients to patients and
from day-to-day. For instance, some patients said they would prefer to regularly get information about
their medication while others would prefer to stay in contact with other patients.
They felt that not having access to their electronic health records was limiting their self-care tasks and
then they alleged wishing to get permanent access to their own medical data as soon as possible.
They discussed also the importance of getting inspired by others existing self-management app for
different chronic disease like diabetes.
They found important to have a simple to use and professionally designed system that would give
them access to good quality and trustworthy information. Prior to be accessible, the content should
then be extracted from evidence-based sources, verified by organizations like HON or validated by
healthcare professionals.
We also discussed about the financial affordability of such system and even though they reported
being willing to pay, they suggested the creation of collaborations with other organizations or
companies that could help developing the app and maybe subvention it. They have suggested also to
collaborate with pharmaceutical companies producing drugs for SCD in order to get funding or to find
a business model allowing to contribute to the funding of patient organizations in low income
countries. We discussed also about research institutes and different European projects like
RenewingHealth7, Project Precious8 and Shifo9 that are doing similar eHealth tools for other health
issues.
They also discussed the importance of creating a strong local patient association that is linked with
European patients’ associations and hospitals. We discussed about the importance of creating
specialized sickle cell centres and references centres. We agreed that patients’ associations can have
here a strong role of advocacy to play.
They also suggested to seek help from the WHO and UNICEF to get support for the creation of
solutions in low-income settings, and we discussed about the fact that SCD being a priority global
health issue, this could be relevant. We discussed that this could be especially pertinent for eHealth
7
http://www.renewinghealth.eu/
http://www.thepreciousproject.eu/?page_id=27
9
https://shifo.org/
8
76
solutions improving the screening processes and we discussed about the models of mobile clinics that
could bring care, and information to remote patients.
They also suggested to try partnerships with NGOs for the creation of a specific self-management
solution intended to be used by children or by people with low literacy.
In conclusion, all their comments and ideas showed a great interest and emphasized the need and the
potential of creating solutions for SCD patients.
4.8.1. Features of the apps by order of importance.
The list below describes the discussions about what features would be appreciated and prioritized.
In order to help them organize their preference order, the specific questions asked were:
- What would you think are the most important features that should be created in a first place?
- What are the most important features for your daily self-care?
- Why have you decided it like that?
1. A patient-connecting set of functionalities:
This functionality, named by patients “Sickle Cell Vikings” or “Sickle Vikings”, is the
most wanted.
Patient said they felt lonely and found difficult to get help and support from doctors
and in general, from the society. They felt painful that even though the community of
patient is numerous, people are dispersed, disconnected and scattered. This is
especially true in countries where the prevalence of the disease is low like in Norway.
They felt problematic to get access to well-trained and specialized healthcare
professionals. Some reported having to wait for years before getting a regular followup.
They reported the same problems when they travelled and lived in Africa or in
Northern America. This can be related to the fact that the survey results generally show
a clear tendency for most questions. Patients are then most probably facing the same
issues all over the world. For these reasons, they would like to get connected with other
patients in order to grow a self-supportive community that could make them feel better
and help them increase the awareness about the disease. They felt the need to advocate
it and to improve the therapeutic education of all patients. They recommended that this
should be done in collaboration with patients’ associations. The absence or low activity
of patients’ associations in the countries where they lived or the regions they visited
emphasized the determination of having physical or virtual contact with other patients.
A rapid observation of patients’ associations’ websites and Facebook groups illustrated
that few were existing in the world. Which is a paradoxical, considering the number of
patients worldwide and especially in the countries were the prevalence of the disease is
the highest.
Patients emphasized solutions like discussions groups with members of patients’
associations, general discussions, chats or forums with other patients with topics they
could control freely.
Patients also said they felt healthier and more normal when meeting other patients. For
those reasons, they wished to create and participate to events or meetings.
Here also eHealth tools have the potential to facilitate this. Participants reported feeling
guilty and sometimes ashamed, sad or excluded. They exemplified it by the fact that
when their friends or sporty people would ask them to participate to physical activities,
they would not understand why they can’t perform as well as the others. They found an
interesting opportunity in the use of mHealth tools to get the possibility to do this kind
of activities with other patients or people who would more probably understand them.
In addition, they reported a lack of educated moderators and health literate patients in
the existent online forums. They felt that the quality of the conversations is often low
and believed that many patients were not health literate enough to understand deeply
the disease. This emphasized the importance of getting trustable and understandable
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information from clear sources that could comfort or reassure them. They said that
meeting or discussing in online groups or discussions other patients would be an
empowering and motivating tool and that it could give a good environment to raise the
awareness about the disease and increase their health literacy level. They also stressed
the importance of having the possibility to get a moderation or advices. Indeed, they
would like that someone of trust, such as a very literate patient, a caregiver or a sickle
cell disease specialist moderate the online discussions.
They also suggested that default topics of discussion can be added and serve as an
educative platform, giving patients more information and updated information about
their disease.
The following list shows the specific features wanted. The list is ordered by importance
according to patients:
o
o
o
o
o
o
o
o
A patient community where can be seen information that patients made public,
(health information, contact information, profile, topics of discussions, etc.), the list
of all patients, currently available patients and the discussion they (patient operating
the app) are in.
Public discussions with other patients but also open for patients’ associations (like
forums that could be moderated by a person of trust).
Private and semi-private discussions where people can create private forums where
people could only be invited or only join when the discussion is set as publicly
accessible.
The possibility to organize events or activities with patients.
A list of local and global patients’ associations where they could get information
about meetings, contact them or being contacted.
A connection with the tools they use the most such as Facebook, Twitter or
WhatsApp. Ultimately, they specified preferring to have all the discussions integrate
in the same app and not on Facebook or another social network. They justified it by
wishing to keep their health-related issues private with other patients and health
professionals.
A young adults and youth section that would be created for people under 18-year-old
has been asked. Patients could not decide if it should be only accessible for children
or if any user could access it. In order to be more flexible, the decision should be
given to the user. For instance, by default, someone over 18 could access the public
information available for people under 18. If a user under 18 creates a discussion, he
should have the possibility to make it invisible for people over 18.
Add the following sections in the general discussions: Prevention, Quality of Life,
Pregnancy, Youth with SCD and Patients’ Recommendations
2. A Logbook to monitor their health data:
This set of functionality has been ranked second by patients. They justified it because
they felt more important to be able to rely on a strong community in order to receive
effective support when needed. They could learn healthy behaviour and support each
other and then think it would minimize the need to have a tool that support their selfcare. Patients still thought it was very important but felt that it would come in second
order. They wished to have an easy to use tools to manage and understand better their
disease by having more information about their current health status. Participants said:
“Try to get inspiration from the best health apps available and design something
simple!”.
They wished also to track their symptoms and their life in order to learn the triggers of
crises and would find helpful to have the ability of linking and comparing their own
data with data from other patients. Even though they feel having already a good self78
awareness level about their general health status, they wanted to improve it. They are
interested in tracking their health data and would appreciate to show it to their doctor,
compare it with other patients and have an idea on how their health is going over time.
They felt important to have this kind of information available during their everyday
activities and would feel informative to have a look in the present, past and the future.
For instance, by having an organized view of the different factors and parameters that
have affected, that affects or that could affect their life.
They prioritized some aspects like the creation of an activity plan with challenges that
motivate them to conduct a healthy lifestyle. This should be linked to their current
health status and would be more useful if it indicates for instance, the quantity of water
intake, the quantity and quality of exercising they practice or the medication they take.
Participants reported that having the possibility to have access to data from their own
health record, especially the blood chemistry results and specifically the basal
haemoglobin level would be interesting for them. Other parameters coming from their
routine exams such as lung function (spirometry and oxygen saturation level in activity
and when resting), cardiac function or radiology images could also be integrated.
They also thought that logging their pain symptoms would be important, especially if
linked to information about their mood. Indeed, they explained the influence of pain on
their mood and on their social behaviours and interactions.
They reported also the influence of the quality of sleep and fatigue level on their health
and the impact of other factors like their stress level, the external temperature or the
current altitude. They said that some of these factors have often contributed in the
triggering of crises and they said they could feel sometimes feel a health worsening
coming.
They believed that a comprehensive set of information could help them to know
themselves better and to adapt their daily lifestyle, such as a different diet or the
practice of physical exercise. The following list describes what this feature should
include:
o
o
o
10
11
An activity plan
 Give advices relatively to a combination of data coming from medical record,
medical devices and manually inputted data could help providing
personalized information. For instance, by giving evidence-based
recommendations and tips about what one should eat according to a previous
meal or lab results or what exercises they should and could perform
according to their lung capacity (related to the level of lung damages and
physical training) or oxygen saturation level taken when performing physical
activity (i.e. 6 minutes’ walk test). Some European projects are doing similar
things for other chronic diseases like Diabetes or COPD1011.
Give the ability to see the most important information at a glance. Patients saw an
interest in the use of a smartwatch for more convenience.
Some of the data that should be monitored, sorted by interest (patients):
 Hydration (quantity of drink and time of intake)
 Amount of sport or physical activity performed
 Information about the medicine intake
 Quantity of sleep
 Level of fatigue
 Lab results:
 Blood-related data
http://www.renewinghealth.eu/services
http://www.is-active.eu/
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 Lung-related data
 Pain symptoms (location and level
 Other symptoms (dryness, cold, etc.)
 Mood
 Diet and nutrition
 Body temperature
 Immunizations and vaccinations taken
 External weather and altitude
 Consumption of alcohol
 Consumption of cigarettes
Some data like the altitude are not prioritized by patients but can have an impact on
their health. These data could then be used for a default layout of information on the
screen, but for monitoring purposes, the entirety of data should be taken into account
and their weight and importance should be evaluated by sickle cell disease specialists.
o Overall, patients found important to have an understanding of their current health
status, that could be calculated with the self-collected data and help them to
understand better the triggers of their crises.
They detailed this feature by expressing the need of learning when their health is
fluctuating (better or worse, good, bad, ok, improving). They liked the idea of a
smiley, graphs, horizontally scrolling text or semaphores.
o Receive general insight, feedback, alerts and warnings based on the self-tracked data
 Warnings when too much or too little medication is taken
 Consequences of medication intake (alerts if too much or not enough)
 Drugs interactions
 Explanations on blood chemistry level such as ferritin or haemoglobin level
 Warning when one should drink more
 Recommendations like the medicine or actions that should be taken when in
pain
o Receive challenges for altering a behaviour (for instance a 10 days’ challenge to
encourage them to eat 5 fruits and vegetables a day, do a 45mn walk every day, etc.)
o See the history and trends of the last daily, weekly, monthly, yearly logbook data.
o Have the ability to order medical exams (blood test, transfusions, medication, etc.)
and the option to integrate the results in the system.
o Have the ability to input information about drugs, advices or behaviours that did work
or did not work well
o Receive alerts and reminders if they reported drinking alcohol or smoking
3. Online consultation:
Focus groups participants want to have an always available English speaking
interlocutor and when not immediately available, they want to see the waiting time.
They also want in a second time, to have the ability to speak in their native language.
We discussed and suggested that it could be done by matching the default interlocutor
to the phone language. They proposed to enrol specialists, medical students or other
trustworthy professionals of trust. In order to recruit them, they recommended to
conducts surveys in order to find people interested in being involved in such
programme. Patients reported they would agree to pay a monthly subscription in order
to be able to get an answer as soon as possible from a healthcare professional. They
would like to rate the people who answer them in order to help other patients’ choices.
The following list describes the features prioritized by patients
o
Get the contact information from the nearest specialized doctors (Must be accessible
without Internet connectivity).
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o
o
o
o
o
o
o
o
o
o
o
o
o
Get the contact information from the nearest specialized healthcare facility. (Must be
accessible without Internet connectivity).
Have the ability to call the medical specialist who follow regularly the patient.
Have the ability to call the nearest available medical specialists.
Have the ability to call another available medical specialist.
Have the ability to chat with the medical specialist who follows the patient.
Have the ability to chat with the nearest available medical specialists.
Have the ability to chat with another medical specialist.
Have the ability to send a picture or sound record to the medical specialist who
follows the patient.
Have the ability to send a picture or sound record to the nearest available medical
specialists.
Have the ability to send a picture or sound record to another available medical
specialist.
Have the ability to video call the medical specialist that follows the patient.
Have the ability to video call the nearest available medical specialists.
Have the ability to video call another available medical specialist.
4. Access to the Electronic Health Record
Participants would like to see pertinent information that can be used when or before
visiting a new doctor or the regular healthcare provider. The information they would
like to have is the blood type, the basal haemoglobin level, the type of sickle cell
disease, the prescribed medicine and a summary of their medical history. They were
also interested in seeing information about their diagnoses, health problems and
conditions, their family related problems, their procedures and interventions, the last
transfusions with the last results, the last crises with the discharge summary, the
complications they faced, the co-morbidities they experienced, the vaccines and
immunizations they have taken, their psychological antecedents, information about the
different stakeholders that took care of them, an access to their radiology images and
their emergency information. They want this section to be linked with the Logbook
feature. It should then be implemented in parallel to the second requirement.
5. Get news about sickle cell disease
Even though patients are already used to get information from medical books, on the
Internet or when visiting their doctors, they expressed the importance of getting the
latest news about the disease. They would like a unified access to the most recent news
about SCD disease. This can include, among others, papers published in the literature,
press releases, news and articles, information about clinical trials or general
information about healthcare services delivery and should include:
o
o
o
o
o
o
o
o
Automatically updated information.
Location-based or global information.
News related for instance to the recent possibilities of treatments like transplants.
News about the advances in research, most recent age of survival, screening
procedures, recommendations for instance for diet or sport.
Information about disease prevention.
Information about the Quality of Life of people with SCD.
Information about different healthcare systems and how they treat SCD.
The possibility to change the sources of information.
6. Travelling with Sickle Cell Disease
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This has been judged very important by patients travelling a lot and patients wished to
have included location-based information:
o
o
o
o
o
o
o
o
o
Information about the medical specialists in the area of travel.
Patients associations and support groups.
Hospitals and healthcare facilities.
Pharmacies.
Names of the drugs and substances of the prescribed medicine in a target language.
Translation of own important health information in a target language.
 Translation of what is SCD, the usual symptoms, how to treat it.
Information about vaccines that should be taken.
Information about epidemiological issues.
Information about factors like altitude or weather that could be important.
7. In case of crisis (Panic button)
This could allow patients to get help when they are having a crisis. It has not been
prioritized by patients because they can generally use their phone to alert people or
they can be transported to an emergency room. All participants reported to avoid
passing by an emergency admission and have the habit to go directly in the specialized
ward. Moreover, patients felt more challenging to implement this kind of features
because it’s closely dependent on the healthcare delivery services. Although, they
thought it could help to raise awareness about the disease and motivate health
authorities to make patients’ data available for themselves.
o
o
o
o
o
o
o
o
o
o
o
o
o
o
o
o
Alerts should be done via text messaging, email or phone call.
Alerts and emergency help should be performed with as few manipulations as
possible (i.e. one button when acute emergency and more manipulations when less
acute emergency).
Have the possibility to alert the “in case of emergency” default contact people
(family, next of kin, doctor who follow the patient, hospital ward).
Have the possibility to alert another patient from the SCD community.
Have the ability to contact other people of choice (office, school, etc.).
Have the ability to contact a transport service (ambulance, taxi, etc.).
Have the ability to inform about the pain level and the location.
Have the ability to inform the receivers about other symptoms.
Have an emergency card with the most useful information in case of crisis.
Have the ability to send information about the prescribed medication.
Have the ability to send information about the medication taken.
Have the ability to send information about the vital signs.
Have the ability to send information about previous complications.
Have the ability to send information about other information that could be of
importance for caregivers (allergies, drug interactions, comorbidities, etc.).
Be forced to fill the personal emergency information during the app first
configuration.
Have the ability to send information about the current location.
8. Information about scheduled appointments
Patients reported sometimes forgetting about appointments and would like for instance
to receive alerts. They would like also to organize annual check-ups where all the
exams are organized the same day. This functionality has been judged less central and
more difficult to implement with an automatic data retrieval because also very
dependent to tight links with electronic medical records but should include:
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o
o
o
o
The ability to see the past and future appointments
The ability to order appointments and exams
The possibility to receive alerts on when to ask for annual/bi-annual/trimestral checkups
The possibility to receive reminders of appointments
9. Get information about how to live well with the disease.
This part is wanted as highly customizable (patients want to organize the order of items
and the layout) and must be grounded on best-practices, guidelines and
recommendations from evidence-based data sources, by doctors or by other trustworthy
patients. Participants suggested the creation of a quiz or another kind of game that can
help them improve their knowledge. They suggested it could be shown when installing
the app and proposed from time to time.
The most requested wish is to have information about how to avoid bad health, for
instance information about the consequences of swimming into cold water, smoking or
drinking alcohol. They wish to have the required knowledge to prevent behaviours
susceptible to trigger VOC. Participants felt health literate enough but said that
sometimes the social pressure made them choose to have bad or not recommended
behaviours. Subsequently, they didn’t wish to be reminded of the consequences of such
bad behaviours all the time but think it is important to receive reminders from time to
time or to have access to information when they wish to. They feel that getting input
from the patient community can be of high value. For instance, by giving the
possibility to patients of discussing, commenting or ranking a recommendation or a
lifestyle habit.
The following items are as well listed by priority given by patients.
o
o
o
o
o
o
o
o
o
o
o
o
o
o
o
Information about emotional and psycho-social support.
Information about the medicine that should be taken.
Information about the common types of medicine that can be taken.
Information about the less common types of medicine that can be taken.
Information about where any medicine is available.
Information about the medicine (since when it has been prescribed, if it has been
found after research focused on SCD or related disease and why they are commonly
used).
Information about the importance of finding good medical care.
Information about how to avoid crises and triggers including information about the
most susceptible triggers (behavioural, environmental, infectious, etc.).
A section about the importance of immunization and vaccination.
A section with important advices when travelling.
Tips given by patient communities.
Information about the importance of getting regular check-ups.
Information about staying updated about the disease.
General information and specific information for males and females.
Information about recommended types of diet (should be linked to personal
information as much as possible).
10. The integration of gamification items, rewards and incentives
Patients didn’t wish to put this feature as a central functionality. They felt that receiving
rewards or gain points when they use the apps may help them to keep using it in a long term
but they argue that because they feel willing and needing to have such a system, they would
not need more incentives. Indeed, they stated that they would be anyway motivated but would
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appreciate to receive, for instance gift cards or coupons. They liked the idea of helping to
decrease the global burden by donating medicine to patients who do not have access to
medical care. They also suggested to contribute to the funding of camps where children with
sickle cell disease could participate. They thought this could help to improve the quality of
life of patients, help their psycho-social issues and raise awareness about the disease.
11. A help and FAQ section
12. Lock Screen feature
They suggest to add this functionality. They would like an app that provide a simple and rapid
access to their emergency information without unlocking the phone:
o Patients’ emergency information
o Sickle cell disease information
o In case of emergency contact information
o Interoperable with invasive or non-invasive device
13. A section where users can give feedback about the system
14. Settings
This section will have to be improved before implementation of each functionality and could
be modified in function of the features implemented.
o Emergency Information (settings for panic button)
o Possibility to add normal values and set threshold (alerts for instance when too low
physical activity, sleep, haemoglobin, etc.)
o Have the ability to set the role and age of the user and if parent, patient, etc.
o Reminders
o Notifications
o Location tracking
o Personal information
o Medical contacts
o Other personal contacts
o Storage management
o Data synchronization
o User interface and layout
o Units and format
o Have the possibility to use and create different profiles of users
4.8.2. Suggestion for other apps
The discussions led to think that a second separated app was needed. They emphasized the utility of
another system, specifically able to provide information about the disease and intended to be used by
parents (parental guide), friends, children, care providers who do not know about the disease. They
said that such a system should be available for free. This system could be integrated in the same
comprehensive system, but should simply prevent access to the whole features to users who are not
logged in. When a user logs in, he would have access to the complete set of features, and if the user
does not log in, he could access to the basic features. It could also be approached like a freemium
system, allowing an access to more features if an individual pays for.
Patients decided that except the Live Chat feature and the nearby information feature, the entire of the
suggested interface could be important. Information could be displayed in a user-friendly and very
simplified way.
They also suggested to develop an interface for low income countries, optimized for people with lowliteracy that could be used, with less functionalities, on a GSM phone or low-end smartphone. They
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suggested to incorporate infographics, games or video and they stated that the system should only be
used by people who are interested by the disease or by people who had the disease. This reason made
them emphasize even more the need to have accurate and trustworthy information.
The following lists shows the suggested features with patients’ comments:








What is SCD
o They found this screen relevant but thought it should be made more attractive and heavily
simplified, for instance with infographics or small video and animations.
Living well with SCD
o This part should include tips and information to promote a healthy lifestyle and inform
people how to minimize the triggering of vaso-occlusive crises and how to handle them
when they happen. They suggested that the information on this page could be given also
as a leaflet to patients, relatives or next of kin during hospital visits. They suggested that
the information could optionally be completed by the patient community but should
mainly come from evidence-based data sources.
 Information about what factors are susceptible to trigger crises (can be completed
by the patient community, for instance with a link to a page “what does patients
say”)
 Information about how to avoid triggers and what are the triggers (could be
commented by patients)
Usual symptoms
o This information could also be completed by patients but should mainly be grounded by
evidence-based information.
How to treat SCD
o Information about the common type of medicine and the alternative medicines should be
available.
o Information about the medical specialists.
Nearby
o This part should show the specialized centres, the healthcare specialists, the pharmacies,
the patients associations and the hospitals in a chosen area.
Live chat
o This part has been judged as potentially very useful, especially if people could receive a
fast answer. The limitations are that it would require an access to Internet and available
medical specialists. They said this part should be prioritized but stay optional (or paid).
This screen should permanently provide a contact page showing the phone numbers and
address of the nearest competent centres or healthcare specialists. See Nearby prototype.
Support groups
o This part should mainly display information about the available patients’ associations and
support groups in a selected area, but also about the main online forums or blogs.
Sickle Cell News
o This has been judged as the lowest priority feature and could show the latest news coming
from a simple google search or chosen sources of information. Prototype design
The figures and the list below summarize and describe the improved screens that have been created.
Login
This page allows the user to connect either with created credentials or with a Facebook account. Many
patients have an account and are active on Facebook groups and then connecting with such account
would add convenience.
The screen offers also the possibility to retrieve a forgotten password or to create a new account. A
profile for the doctors who would like to communicate with patients should be created too.
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Settings
Compared to the previous prototype and with the goal of increasing simplicity of use, items have been
removed and grouped under different labels. During the first configuration of the application, a set of
screen shall allow the user to parameter the basic behaviour of the application, like the notifications,
location tracking, wearable device coupling, the connection with the chosen electronic medical record
and the filling of the emergency information.
Device management
Parametrization of the wearable devices and Bluetooth connection
Emergency settings
Setting of the emergency summary information and information that would appear on the lock screen.
Import/Export
Parametrization and management of the importation of data from third apps like electronic medical
records and for the exportation to plain text files or spreadsheets
Notification management
This allows to manage how the reminders and notifications should be handled
Profile management
Place to add, edit or delete an account
Parametrization of the Alerts thresholds
This feature is especially important when there is no data retrieval from any electronic medical record.
Data could be for instance the normal values for oxygen saturation, used in order to know when the
system should send an Alert.
Share my location
This setting would allow or not the system to share the user’s location with others. This automatically
would activate in case of emergency.
The following refined prototype mostly shows an extended version of the first mock-up and adaptions
that were requested by patients during the focus-groups.
4.8.3. Homepage
This is the homepage of the application. By default, the layout would be as on Figure 57. This mockup was inexistent in the previous prototype and has been added to show a more direct access to the
main functions, in complement to the side-menu and the bottom-menu. Users would have the
possibility to organize the order by pressing the reordering button in the top-right corner or by longselecting an icon.
4.8.4. Bottom-Menu
The menu groups the favourite quick access functionalities. Users would have the possibility to
rearrange them by a long-selection or by taping the top-right corner button.
4.8.5. Side-Menu
This navigation menu has been completed by more items and the SOS button has been enlarged. The
menu would appear anytime the user performs a swipe on the screen from the left-edge to the right.
This would reveal the entirety of the additional features; the challenges (points earned, health
challenges and games), the frequently asked questions (FAQ), a user manual, the legal disclaimer, a
button to allow users to rate and give feedback and a section about us.
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Figure 47 - Login, Settings, Home and Side-Menu
4.8.6. Sickle Vikings Community
Sickle Vikings is the name given to the social feature of the system. It is divided in 4 sections, the
homepage, the discussions page, the community page and the events page. A sharing button allows
user to recommend a discussion topic on a social network for instance by quoting a sentence and
sharing it.
4.8.7. Sickle Vikings homepage
- New members
o This screen shows the newest members of the sickle community.
- Recent activity
o This would allow people to see the most recent activity on the social network, filtered
by most popular or by most recent posts.
4.8.8. Discussions
- Topics
o This screen would show the different topics of discussion created by users.
- Popular
o This screen shows the most popular discussions. They are sorted by number of
replies.
- Newest
o This shows the most recent discussions, ordered by order of creation.
- Chat
o The screen illustrates a common chat room where all users can express themselves in
a chat room.
- Messages
o This would show the last private messages or messages from private groups.
- All
o This would show all the discussions.
4.8.9. Community and Events
This part, as illustrated by Figure 58, differs substantially of the previous prototype. Details have been
added to illustrate better focus groups suggestions and ideas. It is intended to group to manage the link
with the different users and the patients’ communities or support groups. Where people could contact
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patients’ associations worldwide, or find them in any country. People could also share the contact
information of the associations on a social network. The events screen allows users to join, create or
share user-created events. The page shows the past events, all the events ordered by date or the most
imminent events.
Figure 48 – Psycho-social features
4.8.10. Emergency (SOS Button and Lock Screen)
The two emergency features designed here and extracted from the requirements were the need to
always have on themselves their important medical information and the need to get help in case of
insupportable crisis.
4.8.10.1. In Case Of Emergency
This part was inexistent and has been added after discussion and suggestions of patients during focusgroups. In order to access this functionality. Users would have to configure their emergency
information in the settings during the first start of the application. The screen allows the user to
change the language of the page. In order to be understandable by a person who does not understand
the phone language. The screen shows the personal Information, a summary of what has the patient,
the blood type, and the persons of contact. In addition, there is a link to the emergency summary
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report extracted from the medical record, see Figure 59 and Appendix 7 for an example of how could
look an emergency summary.
4.8.10.2. Panic
This screen has been developed and detailed. It now illustrates the entire process needed to receive
help in case of emergency. The page is accessed from the side-menu by clicking on the red button
SOS. The first page asks the users where he has pain and how intense is the pain. The second screen
allows the user to check the symptoms he is experiencing and who he wants to alert. The last page
shows the user the possibility to confirm his last self-tracked health data and the medication he has
taken Data would optimally be collected from a wearable device like a smartwatch or with the help of
notifications regularly asking the patient how he feels and allowing him to enter his pain level as well
as the actions he has taken in order to relieve it. It could also be added in another screen asking what
actions has the user taken in order to relief the pain. Finally, in order to be more usable, these two
pages should be designed differently, with less items and be perhaps separated in two pages.
Figure 49 - Emergency help feature
4.8.11. Logbook
This screen is a refined version of the first prototype. It includes and shows a more configurable
screen and allow users to define how the page should look like.
- Dashboard (See Figure 60)
o This screen allows the user to create his own dashboard with the collected health
information he would judge the most useful. Data could be the oxygen level, the level
of sleep, the physical activity, etc. One click on the graph would send to a screen
showing more details about the variable.
o The screens show on the top, an illustration of the current health status of the user.
Since the factors and the parameters triggering a VOC are well known, the
calculation could be done by comparing the normal values from the aggregated data
coming from wearable devices and electronic medical record. Furthermore, by
calculating a probability of triggering a crisis. The values that could be taken into
account are before a crisis, in the beginning of a crisis, in the middle of a crisis, at the
end of the crisis, and after the crisis. The estimation should be readjusted and adapted
day-to-day.
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o
-
The top-button would allow to access additional parameters to monitor. But a
probably more user-friendly way would be to have a more visible button on the
screen
Monitor (See Figure 60)
o This part would show the entirety of the variables that could be tracked, computed
and analysed over time in order to improve the current health status estimation
feature. The user would have the possibility to rearrange the order and to put the one
he wants on the dashboard.
Figure 50 - Logbook and Monitoring screens
4.8.12. Medical Record
Now the page is decoupled from the Logbook and is an entire subsystem. It reflects the wish of
patients to get access to their medical record and allows the idea to use a third-party system or retrieve
data from existing EHRs.
- Homepage (See Figure 61)
o This lists, not exhaustive, would display the important information that could be
retrieved from the medical record such as the lab results, the prescriptions, the
vaccines or the radios. It would but also show document like a summary of previous
episodes, the anamnesis or an emergency summary.
- Medications (See Figure 61)
o The user could select the button prescriptions in the lists and see a summary of his
intake, the pills he has to ingest and a history of the doses taken. It could also show
additional information like the interactions with other drugs and alerts if a person
takes too much drugs.
- Lab results (See Figure 61)
o The user could also choose to see the laboratory results that are important for sickle
cell disease. This page shows the information relative to kidney issues, the common
vitamin deficiencies, the infections and the data relevant to detect the triggering of a
crisis, as well as the general shape of the patient. The page shows the possibility to
export the results and to see trends in a timeline, as illustrated in the 4th screen.
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Figure 51 - Integration with electronic medical record
4.8.13. Travelling with SCD
This page illustrates the requirement asked during a focus group of getting useful information when
travelling. The information could be about the specialized health centres, the vaccines needed, the
existing patient community, the local pharmacies that can get the needed medication and a translation
of the prescriptions in the target language. See Figure 62.
4.8.14. SCD News
This part is an extended view of the previous prototype. It would show information about the disease,
general information (what is SCD, what are the risk factors, the key facts, the exams and tests, the
treatments, the usual symptoms and how to react to them, patients’ needs to improve their quality of
life and stay healthy, etc.), news, research advancement and how to have a good quality of life. This
part could be made in another separated app but could also be integrated. See Figure 62 and the
section focus groups analysis.
4.8.15. Living well with SCD
This part is showing patients a list (that can be reorganized) of the important actions to take in order to
stay as healthy as possible. Discussions during the focus-groups have shown the importance of a
simple to use app without information overload. Here patients could start challenges related to the
different actions, for instance a 5 days’ challenge where the user should avoid stress or sleep enough.
Data could be tracked using wearable devices measuring stress level or the quantity of sleep. See
Figure 62.
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Figure 52 - Access to information about living well with SCD
4.8.16. Ask a doctor
This illustrates the online consultation functionality. Patients could start a conversation with a doctor
and ask questions via voice, video, or text. They would also have the possibility to send an attachment
like a sound or a picture. The waiting time to get an answer should be displayed somewhere on the
screen when a patient desire to contact a doctor. See Figure 63.
4.8.17. Schedule
This screen allows the user to see the scheduled appointments with caregivers, like exams or checkups. The user could request a new appointment or cancel it. The screen could also show the directions
to the appointments and give more information about the purpose and the process. See Figure 63.
Figure 53 - Access to appointments and remote consultation
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4.8.18. Challenges
- Challenges
o This would list the available incentives set help patients following a healthy lifestyle
and would show the scores and performances. See Figure 64.
- Games
o This would link to the existent games findable on the Internet or in various app stores.
- Points
o This would summarize the number of points earned by the patient when following a
healthy lifestyle and could for instance give more point when the user takes his
medication on time. Reward patients with gift cards or opportunities of donation for
charity causes could be interesting to help other sickle cell patients around the world.
Figure 54 - Challenges screen
4.8.19. Smartwatch features
Since patients were responsive to the idea and interested in using wearable devices. Here are some
examples of how it could be adapted for more convenience, to a use with a smartwatch combined with
a wearable device for health-parameters self-tracking. We could imagine a system that gives patients
an accurate and continuous insight on their health by analysing their blood results at their wrist.
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Figure 55 - Smartwatch home scrolling menu
4.8.20. Home menu
This would be a scrolling menu where people could navigate easily and reach the information they
want quickly. It has been divided as presented in Figure 65.
4.8.21. Health
This part has been imagined to enable patients to see in a glance what their relevant instant health
metrics are. They could also receive motivating sentences to encourage their self-care and give them
an overview of their current health-status as well as information on how well they are behaving. See
Figure 66.
Figure 56 - Health features on the smartwatch
4.8.22. To-Do
This menu would group the daily tasks a user needs to perform. It could be a challenge, a medication
to take, a water intake or answering a note or a message from a doctor. It could also be for instance a
second display where the user would see the scheduled appointments and receive reminders and
notifications on this daily to-do list. Reminders could tell a person when to drink and how much to
drink, as exemplified in Figure 67.
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Figure 57 - Visualisation of the tasks to do on the smartwatch
4.8.23. Mood and pain
The mood and pain menu shows an example of how a user could enter his mood. If the answer is
indicating a bad mood, the system could then ask them if he has pain and recommend him to take a
certain quantity of medicine. If the pain level is too high, the system could propose the user to get help
from a healthcare professional or support from another patient around. See Figure 68, Figure 69 and
Figure 70.
Figure 58 - Mood management feature on the Smartwatch
Figure 59 - Pain management feature on the Smartwatch
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4.8.24. Social
This part relates to the Sickle Vikings part of the app. It could, as illustrated, serve as alert system for
other users when some patient would need help, or simply to receive notifications on the recent social
activity, private messages or ongoing events. See Figure 70.
Figure 60 - Social feature on the Smartwatch
4.8.25. Exercise
This feature is intended to help patients during their practice of physical activity. It would give them
tips to preserve their health and assist them in order to avoid exhaustion. It could give them warnings
when they are at risk of tiredness. For instance, when an activity is too exhausting, their level of
oxygen would decrease, and they would sweat, existing wearable devices are able to detect that. A
notification could warn them and advise them to reduce the effort and hydrate. See Figure 71.
Figure 61 - Exercise management features on the Smartwatch
4.9.
Prototype evaluation
The evaluation of the prototype has not been done by patients. Patients from the focus-group just had
a quick look on the mock-ups and did not find anything to improve. They also stated it was accurately
reflecting the discussions and their thoughts. They reported wishing to use similar system if they had
the possibility.
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5. Discussion and future work
The goal of this thesis was to gather the required amount of knowledge and the needed information
that could enable the creation of a mobile app designed to answer sickle cell disease patient’s selfmanagement needs. To achieve that, have been done a literature review on the issues caused by the
disease, an investigation on what are the health informatics and the medical related pre-requisite as
well. In addition, to be able to propose patients a prototype potentially helping them support their
disease in a comprehensive way, an acquisition of patients’ main requirements and problems faced
has been performed. The multi-disciplinary knowledge required to achieve this tool, the design
process in collaboration with patients as well as the collection of their multiple opinions and the
gathering of the requirements are discussed in this chapter.
5.1. Critique of the methods used
Bias: The lack of other users to provide feedback and input on the design has probably limited the
quality of the mock-ups. Receiving more input and opinions from patients would allow to refine it.
Not all screens have been designed, but the most relevant have been considered in order to illustrate
how the system could work.
An additional bias can be found in the questionnaire, since most of the respondents were female,
further studies need to explore if women and men prefer a significantly different kind of interface or
features. The analysis performed on the survey answers did not reveal anything substantial.
Alternative methods: Focus groups have been privileged over interviews because they allow a
certain freedom in assessing patients’ issues. It allows to understand what people feel about their
disease and how they think of a proposed solution. It also allows to take patients’ needs in a creative
process. Users can exchange ideas, discuss experiences and feelings and can comment on each other’s
while proposing creative solutions. One issue, compared to interviews is that it forces to meet patients
frequently in person and it can be exhausting for patients to attend many meetings. Questionnaire
have been used to identify general trends that could be then specified during focus groups. The use of
questionnaires can be debatable. More respondents could have given a better insight but it was
difficult to get patients. Despite that, the results allowed to identify sufficiently clear trends.
Usability assessment and testing: The prototype proposes a comprehensive set of features. Keep this
complexity usable in a simple way is not trivial. Therefore, a cohort of patients should receive the
system and test it. Subsequently, a standardized questionnaire (system usability scaled) for usability
testing, feedback questionnaires, techniques like eye-tracking or software allowing to monitor users’
behaviours would be needed to evaluate the usability.
Heuristic design: In this study, the main limitation of the heuristic evaluation process is that it
requires a recommended number of five to twelve reviewers to evaluate the prototype and to identify
most usability issues. Only one person who was the designer, evaluated it, thus limiting the scope and
the precision of the analysis. Further studies will be required to give more credibility to the
evaluation.
Paper prototyping: Some paper prototypes have been created during the focus-groups and can be
seen in Appendix 4. They helped to put ideas in place and to illustrate the thinking. However, they
were not advanced enough to create an entire screen, but were sufficient to illustrate what information
patient would like to visualize and what new features they would like to have.
5.2. Major findings
The survey results and the focus groups confirmed what the literature review suggested about
patients’ needs and issues. In addition, the results are adding more knowledge to the field of mHealth
for SCD as well as relevant and thorough details about patients’ specific needs and concerns. The
focus groups allowed to define very precise requirements. Even though the number of respondents in
this study is limited, the results show clear tendencies. The results analysis gives also a precise insight
on what features should offer an app for people with sickle cell disease and the prototype illustrate
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how patients would appreciate it. Although, respondents are coming from different horizons, they are
still expressing the same needs and issues. Further research and more work will be required to
implement the features with the close participation of patients and also to put in place a solid health
information network focused on the self-management of sickle cell disease patients.
5.3. Achievements and expected health benefits of an implementation of the
prototype
The system has the potential to have good health outcomes on the disease, helping patients to learn
more about themselves. Propositions of solutions tackling the principal complications faced by SCD
patients such as organs damages and pain symptoms prevention (education interventions, health status
monitoring, VOC avoidance and rapid detection, timely alert in case of emergency), as well as the
psychological and the social issues that patients suffer from are stated in the requirements and
illustrated by the mock-ups. However, the work focuses on patients’ education and daily life
assistance. If entirely and carefully taken into account and integrated when developing the system, the
requirements and suggestions of implementation could have positive health outcomes.
5.3.1. Ubiquity
By creating a system that could fit different platforms such as computers, smartwatches, mobile phone
and smartphones, the system has the ability to be continuously and everywhere available for patients.
Mobile phone and smartwatches being constantly worn by people, would allow them to support their
self-management in every activity of their daily life.
5.3.2. Education
The system has the potential to educate patients about their health, especially when combined with a
wearable medical device, by giving them live insights on their disease. The SCD News and living
well with SCD features can educate patients, their entourage, caregivers and the general population.
5.3.3. Psychological
The social features and the online consultation features of the system could contribute to decrease the
psychological burden of patients, helping them to communicate their moral issues with other patients
and offering the opportunity to get support when needed.
5.3.4. Health promotion (preventive healthcare)
Disease prevention 12 are the actions that can be taken in order to avoid poor health and death. The
implementation of the prototype may have the potential to impact the different levels of preventive
healthcare. Especially by advising patients to follow a healthy lifestyle and by having healthy
behaviours. Additionally, by helping them to anticipate a worsening of their health status, monitoring
their health variables regularly.
Primary prevention (health promotion): The app has the potential to avoid the occurrence of the
disease. It would improve partly the primary prevention processes by increasing the general public
knowledge, helping improving the quality of life of patients and promoting immunizations, physical
exercise, healthy diet, and discouraging of following unhealthy behaviours.
The system does not focus on eliminating the disease (curing, screening and genetic counselling) but
this could be a future work.
Secondary prevention (disease prompt treatment): The system focuses mainly on supporting people in
their daily life. This kind of prevention, by monitoring (and potentially early detecting) the risk factors
12
https://iom.nationalacademies.org/~/media/Files/Activity%20Files/Quality/IntegrativeMed/Preventive%20Medi
cine%20Integrative%20Medicine%20and%20the%20Health%20of%20the%20Public.pdf
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of health status worsening and by advising for check-ups or treatment when needed (psychological
status and vaso-occlusive crisis), can potentially prevent complications or disabilities due to organ
damages.
Tertiary prevention (damages reducing): The monitoring features and the social features of the
system can reduce the negative impact of the disease (psychological, social and health-related issues)
by giving patients’ the information they could need to take actions improving their health or can
transfer the information healthcare providers able to help them or provide treatment or care.
5.3.5. Health service delivery: from self-care to healthcare
-
-
-
-
-
-
-
Surveillance and research
o The system, by collecting several kind of self-generated data (mental health,
physiological data, etc.), has the potential to enhance the reutilization of patients’
self-generated data. Data could be useful for research purposes and may contribute to
the early detection of outbreaks.
Since most SCD patients have a low resistance to infections, massive data mining and
analysis of database incorporating the day to day effects of SCD as well as the
clinical symptoms can help to create insights. This could also simplify the conduct of
epidemiology studies focusing on discovering new triggers of vaso-occlusive crisis.
Data could help to evaluate the healthcare utilization and enable healthcare providers
to act in among in order to reduce the suffering caused by pain episodes (anticipating
care delivery). Such data could also enable governance to make decisions and
leverage health policies to better scale screening and genetic counselling
programmes, advocacy, awareness and prevention campaigns.
Partnerships
o In order to strengthen the opportunities and build long-term possibilities, developing
the system would require strong partnerships with patients, healthcare professionals,
support groups and governments.
Healthcare organisation
o By integrating in one place, the different stakeholders who are linked to patients’
care, the system has the ability to give patients and their care-providers, the
information they need to install efficient care processes and workflows.
For instance, by organizing check-ups when needed, routine exams regularly or
providing resources only when required.
Follow-up (consultations)
o The potential of improving patient-provider communication through an increased
patients’ participation in their own care could enable doctors to make better informed
decisions for their patients.
Pharmaceuticals
o Monitoring patients’ medication intake and the effectiveness of medical drugs can
help to scale doctors’ prescriptions according to the real usage and real needs of
patients and evaluate their effectiveness.
Materials and equipment
o The system could benefit patients, by helping healthcare professionals to understand
the day-to-day patients’ self-care needs. For instance, by knowing when a patients
could need oxygen supplementation or a blood transfusion. To enable this, the home
usage of advanced medical devices like blood tests devices and the remote
transmission of data can strengthen the efficiency of healthcare usage, decreasing
patients’ need to reach healthcare facilities and giving information to caregivers.
Resources (training and investment)
o Gaining knowledge about patients’ health issues and the importance of the disease
can help scaling the investment required to improve healthcare professionals training
and education but can also aid to understand when and where the workforce is
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-
-
required and should be mobilized. It could help to highlight suboptimal resources
utilization (i.e. by providing comprehensive statistics about patients’ daily life-issues
or acute care needs or placing resources where needed the most).
Health financing
o The system could emphasize the use of affordable ICTs tools to monitor the
healthcare utilization of patients’ and enable them to monitor the costs and direct
financial consequences of their disease in their daily life. More precise data can help
to understand better the burden of the disease and then give evidence for policy
makers and health economists.
Healthier behaviours
o Healthy behaviours can be increased if care providers and patients are more educated
to follow standards and best practices of care, potentially decreasing the morbidity
and the mortality.
5.4. Feedback
More feedback is needed for the usability acceptance tests. Clinical trials with more and different
patients would be required and could give different opinions and suggestions. Also, a precise
feasibility study of each requirement will have to be performed in order to plan the development and
find adequate technologies.
5.5. Economy
A financial evaluation of the costs of developing such a system would be required to estimate a
budget. Additionally, the costs of the solution should be as low as possible in order to be affordable
by regions with low-financial resources.
5.6. Suggestions for future features
The questionnaire, the focus-groups and the requirements analysis helped to come up with
suggestions of future features. As presented in Table 9, suggestions should be then evaluated by a
group of patients in order to find out their relevance and their interest.
Table 9 - Suggestions for future features
Suggestion
App to teach and train the healthcare professionals
Web platform with statistics, trends and insights on the selfgenerated patients data
Disease surveillance platform accessible with a website providing
statistics and trends as well as an open API providing anonymised
data linked with geographical data and time-based information.
Target group
Caregivers
Caregivers
Caregivers,
public
health
authorities,
disease
surveillance
centres,
governments and politicians
Informative app for citizens
General population
Website to manage the disease with more statistics and trends
Patients
Medical terms explanation
Patients
Medical record translation (Emergency summary and Patients
prescriptions)
Automatic posts and sharing information on social networks
Patients
Platform to provide people keen to enrol randomized control Patients
trials
Integration of existing social networks like Facebook or patients Patients
associations forums
Simplified design
Patients
Ensure a psychological and moral support
Patients
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Simplified content for illiterate (use of voice recognition)
Simplified content for low-income settings
Mobile screening system
Gamified and simplified content for children
Patients
Patients
Patients, Caregivers
Youth patients
Features like video consultation, medical advices or social networks can be seen as a bigger picture,
where existing solutions could already be used. In order to avoid waste of resources when developing
the system, search for the existing apps, API and templates that could answer some of the
requirements is recommended. Then, only the non-existing functionalities would be developed.
5.7. Financing
Financially, the development of the demonstrated system is challenging. Although sickle cell disease
is part of the top 4 priorities of the WHO and UN health-focused Millennium Development Goals
initiative, the lack of public interest and public funding is observable.
The fact that globalisation is increasing the spreading of the gene responsible of sickle cell disease
around the world could facilitate fund-raising and leverage the global political willing, the research
funding and the organisation of public-private partnerships.
5.8. Compliance issues and motivational issues
The challenges of creating an app that can benefit all the patients and engage them in a long term are
numerous. Issues like health literacy, digital literacy, motivation and financial power have to be taken
into account. One possible strategy, is to create a system that can be prescribed my medical doctors,
proving patients the proof of the health benefits for them. Other strategies like the inclusion of
gamification items have proven to be effective in keeping patients motivated in the long run. The
website of the patient advocacy company PatientView presents some advices for application
developers that could help them build systems addressing patients’ needs13. Some of the
recommendations are to involve patients in the development process, to identify their needs, to bring
the solution to a healthcare provider level, to make them sustainable and adaptable and to get
regulatory and clinical approval. Also, the integration of behaviour modifying items like those used in
games for health should be evaluated. Even though patients judged that unnecessary, creating a
system that can be used in a long-term perspective is challenging. Especially if the system is effective,
patients would feel less the need to utilize it, and then health outcomes could be deteriorated.
5.9. Regulatory issues and clinical approval
In order to help the system to get prescribed, supported and approved by healthcare professionals’
approval, the system would require to be tested in clinical trials, patient’s acceptability studies would
need to be performed, usability studies would be required and studies on the costs and the potential
health outcomes established. A paper from Boulos et al summarize the needs and issues that must be
tackled and the processes required to develop a medical app that can comply to the regulatory
authorities in the USA and in EU [138].
5.10. Extension of the questionnaire
In the future, it will be needed in order to refine the needs, to extend the questionnaire to more
patients all over the word. Facebook group’s representatives, patients’ associations and hospitals can
help getting updated answers and a more representative insight. The scarcity of patients will make it
challenging. Extending and adapting the questionnaire for children and illiterate people should be
done as well.
13
https://alexwyke.wordpress.com/2015/06/24/press-release-what-do-patients-and-carers-need-in-health-appsbut-are-not-getting
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5.11. Essentials for the implementation of eHealth tools for SCD
The list below exposes the prerequisite for the implementation of such tools. They can be applied
specifically for SCD, but also to other kind of health apps:
Needs:
- Managed and accessible information complying to the standards and API:
o Digital literacy
 In order to access and make use of digital information, patients and health
professionals need to have enough digital literacy.
 Health information can then be digitized and used for informing, teaching or
training health professionals and patients.
o Health literacy
 Patient’s health literacy level need to be adequately evaluated in order to
understand which kind of health information they can understand. Measures
can be taken to improve patients’ health literacy
o Quality of health information
 The health information provided must be accurate, trustworthy and accurate
in order to not harm patients and ease the access to quality content.
o Security of information
 Patients’ information need to stay safe. The healthcare environment need to
provide the framework that keeps patients’ information confidentiality,
integrity and availability protected.
- Medical Ancillaries (SCD specific treatment support)
The multidisciplinary nature of sickle cell disease requires a system to manage and provide
access to different types of health information. Such data can generally be retrieved from the
electronic medical records containing patients’ information.
o Laboratory
 Information from laboratory tests help to identify the acuteness of haemolysis
and dehydration, detecting infection, inflammations and oxygenation level.
 Vital signs monitoring such as temperature (fever), or oxygen saturation
(hypoxemia) are good indicator of a VOC, as well as pulse rate (tachycardia),
respiration rate (tachypnea) blood pressure (hypotension) and therefore
should be manageable and accessible.
o Radiology and imagery
 Radiology data that allow the surveillance of the health of the eyes, heart,
bones, lungs, spleen and liver should be integrated
o Pain and disease management
 The integration of pharmaceutical information, helping patient gain access to
antibiotics, analgesics, or treatment like hydroxyurea or folic acid is
important.
 Information on other approaches that can help patient manage their disease
and their symptoms like heat packs, hypnosis, psychosocial support,
cognitive behavioural therapy, guided imagery should be provided.
 Information facilitating red blood cell transfusions like blood availability for
sickle cell patients and campaign promoting blood donation should be taken
into account.
 Information about health promotion and prevention should be available for
patients, his entourage, and the caregivers. Information could be about the
preventive measures like high altitudes avoidance, temperature variation and
extremes temperature avoidance, good practice like hydration, physiologic
and emotional stress avoidance, promotion of resting and relaxing, promotion
of moderate physical activity, prevention of infection and information about
when to seek for medical care can promote health.
- Digital health environment
o General infrastructure
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
Electricity, a secured and timely access to Internet, computers and mobile
devices are required to access health information online.
o Interoperability
 Measures to allow a system to communicate with the systems providing the
healthcare information and other useful information related to patients’
environment such as schools, work, health insurance should be put in place.
o Maturity
 A mature system is required to have access to health information coming
from an EMR.
- Workforce
o Health
 Information regarding the available and trained healthcare professionals,
information regarding the logistic, for instance for medicine access and
information about the places and facilities that can treat patients are needed.
o ICT
 Digitally literate people with enough skills are needed to manage the systems
and services but also the infrastructure needed to manage the connectivity
and electricity demand.
o Legal and ethical considerations
 A legal framework is necessary in order to allow communication with
healthcare providers like online consultations and to prevent or penalize
fraudulent access to patients’ medical information.
- Governance framework
o Governing bodies have to put in place the public health strategies, the legal
framework and the prioritization policies that can help developing sickle cell day
hospital and care centres and the eHealth services needed for that. Policies must be
focused on public health strategies. For instance to promote systems and studies that
allow to generate more data about prevalence, mortality, loss of productivity and
absenteeism, psychosocial issues, healthcare utilization, risk factors of VOC,
complications, health outcomes of intervention like hydroxyurea, the level of
awareness and education of patients, community, healthcare providers and entourage,
indications about the wellness and quality of life of patients, the use of clinical
practice guidelines as well as the development of new therapies and investments in
research and development.
- Financing
o High costs
 In 2009 in the USA, the total cost of SCD was of about 2 billion USD for
approximatively 100’000 patients. One quarter of the cost is in
hospitalizations and rehospitalisation [139]. Costs will have to be taken into
account in order to implement the system.
o Funding
Worldwide, the financing of sickle cell disease needs is public, private, donor based,
public-private partnerships based or patients based and no study are available about
the total costs. There is an interest in defining the total average costs. This could
facilitate the payment of healthcare services and reduce the economic burden. The
disease being chronic, the needs and services can be rationalized and centralized.
Barriers:
Sickle cell disease patients need access to healthcare and healthcare information but the system that
take care of them may face several barriers including:
- Poor infrastructure and technical expertise
- Lack of trained workforce
- Lack of funding
- Absence of health facilities and material
- Poor political willing and low awareness
- Lack of governance policies
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-
6.
Unavailability of data needed to develop and implement strategies
Absence of strategies that prioritize SCD as a public health issue
Lack of collaborations and coordination between the different care providers
Lack of trained healthcare professionals
Lack of educated family and entourage
Low patient health literacy
Low digital literacy
No inclusion of public and private health information
No interoperability and standardization of health information
Conclusion
The state of the art research and the results have shown what eHealth tools could offer to people with
Sickle Cell Disease, namely, the ability to better self-manage their symptoms with a mobile
application tailored to meet their needs.
This thesis gives a very thorough description of what kind of support sickle cell patients want and
need from mHealth tools. To my knowledge, there is no comprehensive and detailed collection of
such information.
The worldwide scope and the expanded type of questions asked to patients during the survey and the
focus groups have given the opportunity to recognize voices that have not been given the opportunity
to be heard before. Declarations of patients have been collected through long and thorough
discussions of several topics.
A large amount of information has been gathered about their digital literacy, their health literacy, their
health situation, their concerns, their unmet needs and the issues they face in their daily life. Together,
this knowledge brings a novel understanding to healthcare professionals, researchers, IT specialists,
policy makers and patients’ entourage regarding the daily issues, concerns, wishes and expectations of
people with SCD.
Patients reported receiving sub-optimal and often delayed care, especially in terms of painmanagement. This resulted in an obvious lack of trust in the healthcare delivery services, and eventual
avoidance of any utilization of health care services. Even though day care hospitals in SCD exist and
demonstrate their usefulness to patients when needed, they are not common and then rarely accessible
to patients.
Most participants had maintained a relatively healthy status but were worried about worsening
symptoms when performing day-to-day activities.
They reported wanting continuous access to their medical data to better understand their disease and
allow them to make consistently healthy decisions. They also stressed the importance of establishing a
strong patient community through the use of mHealth tools.
Given that, today, wearable and implantable devices are capable of gathering health parameters, they
have demonstrated their potential of helping people to receive accurate information about their health
status. Therefore, it is certain that most of the requirements can be met by using existing technologies
in an appropriate way. Data mining techniques such as machine learning algorithms can be used to
give patients accurate feedback on the gathered data. Subsequently, smartphone apps can use these
techniques to direct patients to change their behaviour, thus having a direct impact on their health and
SCD symptoms.
The data generated by such self-tracking devices can also be used to gain deeper knowledge of sickle
cell patients by health care providers and researchers.
The data collected can be important for disease surveillance programmes and contribute to a better
understanding of the mechanisms of vaso-occlusive crisis.
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The prototype that resulted from this project succeeded in staying true to patients’ original
requirements, exemplifying how eHealth tools could assist them in a comprehensive way.
The solution has shown the feasibility of developing tools likely helpful in educating patients to take
actions that can reduce the frequency of crises or to identify the triggers of VOC. The proposed
solution could also assist them in their daily life and facilitate the access to emergency care when
required.
Although more research is needed, first impressions given by the patients on this prototype were
positive and prefigure a good acceptance level.
In the future and in parallel with the development of the prototype, extensive tests and clinical trials
would be required. In order to gain clinical approval and achieve compliance with regulating
authorities, such as the FDA. In doing so, these measures will offer the system the opportunity to be
prescribed by clinicians and help a greater number of people with SCD.
The potential of successfully developing this project is high, especially in high income countries
where the technology is accessible. However, as the survey results have illustrated, patients with SCD
are facing similar issues and needs with their self-management all over the world. Especially in areas
where healthcare resources, even sub-optimal options, are not accessible. This study demonstrates
how patients can take a greater role in their self-management to supplement a lack of limited health
care resources, thus bringing new opportunities for regions where the access to healthcare is
problematic.
Nevertheless, challenges like the lack of systematic screening, poor awareness and health literacy, a
reduced Internet connectivity, political instabilities and a low access to water and electricity will have
to be overcome in the poorest regions. Furthermore, monetary issues prevent the poorest to access
necessary health care. However, because this may call for prices to drop or the opportunity for other
solutions to be offered as alternatives, such limiting situations create an even greater role and
opportunity for positive impact for such eHealth tools as the one proposed by this project. While
creating a system that is capable of meeting every person’s needs, is adaptable to specific socioeconomic and cultural settings, and that can be integrated into the various health systems is
challenging, it is our hope that, with few adaptations, such solution could be accessible and used
worldwide.
In conclusion, the world is seeing a growth in the mHealth market, indicating good future
opportunities. In parallel, the extremely positive feedback given by patients at the end of the survey
and during focus groups gives an endless motivation to pursue the work.
I am confident in the future influence of eHealth tools to enable individuals with SCD to achieve
positive outcomes. I believe that the capabilities of such tools can facilitate patients to make more
informed daily decisions, improve their social needs and offer them a better quality of life in the long
run. On the other hand, I think the biggest challenge while creating such a system will be to collect
data enabling the establishment of meaningful associations between what patients feel and how they
feel.
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References
[1]
Savitt TL. The invisible malady: sickle cell anemia in America, 1910-1970. J Natl Med Assoc
1981;73:739–46.
[2]
Okwi AL, Byarugaba W, Ndugwa CM, Parkes A, Ocaido M, Tumwine JK. Knowledge gaps,
attitude and beliefs of the communities about sickle cell disease in Eastern and Western
Uganda. East Afr Med J 2009;86:442–9.
[3]
Gustafson SL. Knowledge and Health Beliefs of Sickle Cell Disease and Sickle Cell Trait: The
Influence on Acceptance of Genetic Screening for Sickle Cell Trait 2006.
[4]
Nimgaonkar V, Krishnamurti L, Prabhakar H, Menon N. Comprehensive integrated care for
patients with sickle cell disease in a remote aboriginal tribal population in southern India.
Pediatr Blood Cancer 2014;61:702–5. doi:10.1002/pbc.24723.
[5]
Odame I. Perspective: we need a global solution. Nature 2014;515:S10. doi:10.1038/515S10a.
[6]
Piel FB, Hay SI, Gupta S, Weatherall DJ, Williams TN. Global burden of sickle cell anaemia
in children under five, 2010-2050: modelling based on demographics, excess mortality, and
interventions. PLoS Med 2013;10:e1001484. doi:10.1371/journal.pmed.1001484.
[7]
Prasad R, Hasan S, Castro O, Perlin E, Kim K. Long-term outcomes in patients with sickle cell
disease and frequent vaso-occlusive crises. Am J Med Sci 2003;325:107–9.
[8]
WHO | Sickle-cell disease and other haemoglobin disorders n.d.
[9]
Network GSCD. Global SCD Distribution n.d.
[10]
Chakravorty S, Williams TN. Sickle cell disease: a neglected chronic disease of increasing
global health importance. Arch Dis Child 2014. doi:10.1136/archdischild-2013-303773.
[11]
Orphanet:
Sickle
cell
anemia
n.d.
http://www.orpha.net/consor/cgibin/OC_Exp.php?Lng=EN&Expert=232 (accessed December 22, 2014).
[12]
Rares M. Les Cahiers d ’ Orphanet Prévalence des maladies rares : Données bibliographiques
Classement par prévalence décroissante ou n.d.
[13]
Constantinou C, Payne N, Inusa B. Assessing the quality of life of children with sickle cell
anaemia using self-, parent-proxy, and health care professional-proxy reports. Br J Health
Psychol 2014. doi:10.1111/bjhp.12099.
[14]
Aygun B, Odame I. A global perspective on sickle cell disease. Pediatr Blood Cancer
2012;59:386–90. doi:10.1002/pbc.24175.
[15]
Oniyangi O, Cohall DH. Phytomedicines (medicines derived from plants) for sickle cell
disease.
Cochrane
Database
Syst
Rev
2015;4:CD004448.
doi:10.1002/14651858.CD004448.pub5.
[16]
Piel FB, Patil AP, Howes RE, Nyangiri OA, Gething PW, Dewi M, et al. Global epidemiology
of sickle haemoglobin in neonates: a contemporary geostatistical model-based map and
106
population estimates. Lancet (London, England) 2013;381:142–51. doi:10.1016/S01406736(12)61229-X.
[17]
Adeyemo TA, Ojewunmi OO, Diaku-Akinwumi IN, Ayinde OC, Akanmu AS. Health related
quality of life and perception of stigmatisation in adolescents living with sickle cell disease in
Nigeria: A cross sectional study. Pediatr Blood Cancer 2015;62:1245–51.
doi:10.1002/pbc.25503.
[18]
Haywood C, Diener-west M, Strouse J, Carroll CP, Bediako S, Lanzkron S, et al. Perceived
Discrimination in Health Care is Associated With a Greater Burden of Pain in Sickle Cell
Disease. J Pain Symptom Manage 2014. doi:10.1016/j.jpainsymman.2014.02.002.
[19]
Aygun B, Odame I, Asia S. REVIEW A Global Perspective on Sickle Cell Disease 2012:386–
90. doi:10.1002/pbc.
[20]
Ollila E. Global health priorities - priorities of the wealthy? Global Health 2005;1:6.
doi:10.1186/1744-8603-1-6.
[21]
Grosse SD, Odame I, Atrash HK, Amendah DD, Piel FB, Williams TN. Sickle cell disease in
Africa: A neglected cause of early childhood mortality. Am J Prev Med 2011;41:S398–405.
doi:10.1016/j.amepre.2011.09.013.
[22]
Serjeant GR. Mortality from sickle cell disease in Africa. BMJ 2005;330:432–3.
doi:10.1136/bmj.330.7489.432.
[23]
Barbarin OA, Christian M. The Social and Cultural Context of Coping with Sickle Cell
Disease: I. A Review of Biomedical and Psychosocial Issues. J Black Psychol 1999;25:277–
93. doi:10.1177/0095798499025003002.
[24]
Bookchin RM, Balazs T, Landau LC. Determinants of red cell sickling. Effects of varying pH
and of increasing intracellular hemoglobin concentration by osmotic shrinkage. J Lab Clin
Med 1976;87:597–616.
[25]
Diaw M, samb A, Diop S, Sall ND, Ba A, Cissé F, et al. Effects of hydration and water
deprivation on blood viscosity during a soccer game in sickle cell trait carriers. Br J Sports
Med 2014;48:326–31. doi:10.1136/bjsports-2012-091038.
[26]
Brousseau DC, Owens PL, Mosso AL, Panepinto JA, Steiner CA. Acute care utilization and
rehospitalizations
for
sickle
cell
disease.
JAMA
2010;303:1288–94.
doi:10.1001/jama.2010.378.
[27]
Silva IV, Reis AF, Palaré MJ, Ferrão A, Rodrigues T, Morais A. Sickle cell disease in
children: chronic complications and search of predictive factors for adverse outcomes. Eur J
Haematol 2014. doi:10.1111/ejh.12411.
[28]
Wallen GR, Minniti CP, Krumlauf M, Eckes E, Allen D, Oguhebe A, et al. Sleep disturbance,
depression and pain in adults with sickle cell disease. BMC Psychiatry 2014;14:207.
doi:10.1186/1471-244X-14-207.
[29]
Molter BL, Abrahamson K. Self-Efficacy, Transition, and Patient Outcomes in the Sickle Cell
Disease Population. Pain Manag Nurs 2014. doi:10.1016/j.pmn.2014.06.001.
107
[30]
Telfer PT. Management of sickle cell disease: out-patient and community aspects. Paediatr
Child Health (Oxford) 2011;21:357–62. doi:10.1016/j.paed.2011.03.005.
[31]
Toolkit S. Living Well With Sickle Cell Disease: Self-Care Toolkit n.d.
http://www.cdc.gov/ncbddd/sicklecell/documents/livingwell-with-sickle-cell-disease_selfcaretoolkit.pdf (accessed December 22, 2014).
[32]
Jenerette CM, Brewer CA, Ataga KI. Care seeking for pain in young adults with sickle cell
disease. Pain Manag Nurs 2014;15:324–30. doi:10.1016/j.pmn.2012.10.007.
[33]
Smith WR, Bovbjerg VE, Penberthy LT, McClish DK, Levenson JL, Roberts JD, et al.
Understanding pain and improving management of sickle cell disease: the PiSCES study. J
Natl Med Assoc 2005;97:183–93.
[34]
Adewoyin AS. Management of sickle cell disease: a review for physician education in Nigeria
(sub-saharan Africa). Anemia 2015;2015:791498. doi:10.1155/2015/791498.
[35]
Yawn BP, Buchanan GR, Afenyi-Annan AN, Ballas SK, Hassell KL, James AH, et al.
Management of sickle cell disease: summary of the 2014 evidence-based report by expert
panel members. JAMA 2014;312:1033–48. doi:10.1001/jama.2014.10517.
[36]
Yawn BP, Buchanan GR, Afenyi-Annan AN, Ballas SK, Hassell KL, James AH, et al.
Management of Sickle Cell Disease. JAMA 2014;312:1033. doi:10.1001/jama.2014.10517.
[37]
Krishnamurti L, Smith-Packard B, Gupta A, Campbell M, Gunawardena S, Saladino R. Impact
of individualized pain plan on the emergency management of children with sickle cell disease.
Pediatr Blood Cancer 2014;61:1747–53. doi:10.1002/pbc.25024.
[38]
Bloom M. Understanding Sickle Cell Disease. Univ. Press of Mississippi; 2009.
[39]
Lervolino LG, Baldin PEA, Picado SM, Calil KB, Viel AA, Campos LAF. Prevalence of
sickle cell disease and sickle cell trait in national neonatal screening studies. Rev Bras
Hematol Hemoter 2011;33:49–54. doi:10.5581/1516-8484.20110015.
[40]
Manwani D, Frenette PS. Vaso-occlusion in sickle cell disease: pathophysiology and novel
targeted therapies. Blood 2013;122:3892–8. doi:10.1182/blood-2013-05-498311.
[41]
Pathophysiology and Principles of Treatment - Sickle Cell Information Center n.d.
https://scinfo.org/additional-online-books-and-articles/pathophysiology-and-principles-oftreatment (accessed July 24, 2015).
[42]
France E, Noire EA. La drépanocytose 2011.
[43]
Carroll CP, Haywood C, Fagan P, Lanzkron S. The course and correlates of high hospital
utilization in sickle cell disease: Evidence from a large, urban Medicaid managed care
organization. Am J Hematol 2009;84:666–70. doi:10.1002/ajh.21515.
[44]
De Montalembert M. [Management of sickle cell disease]. Rev Prat 2004;54:1557–64.
[45]
Gregory TB. Chronic pain perspectives: Sickle cell disease: Gaining control over the pain. J
Fam Pract 2012;61:S5–8.
108
[46]
Amid A, Odame I. Improving outcomes in children with sickle cell disease: treatment
considerations and strategies. Paediatr Drugs 2014;16:255–66. doi:10.1007/s40272-014-00744.
[47]
Haywood C, Beach MC, Lanzkron S, Strouse JJ, Wilson R, Park H, et al. A systematic review
of barriers and interventions to improve appropriate use of therapies for sickle cell disease. J
Natl Med Assoc 2009;101:1022–33.
[48]
Serjeant GR. The natural history of sickle cell disease. Cold Spring Harb Perspect Med
2013;3:a011783. doi:10.1101/cshperspect.a011783.
[49]
Wajcman H, Moradkhani K. Abnormal haemoglobins: detection & characterization. Indian J
Med Res 2011;134:538–46.
[50]
Meade MS, Emch M. Medical Geography. Guilford Press; 2010.
[51]
Stirnemann J, Letellier E, Aras N, Borne M, Brinquin L, Fain O. Hyperbaric oxygen therapy
for vaso-occlusive crises in nine patients with sickle-cell disease. Diving Hyperb Med
2012;42:82–4.
[52]
Booth C, Inusa B, Obaro SK. Infection in sickle cell disease: a review. Int J Infect Dis
2010;14:e2–12. doi:10.1016/j.ijid.2009.03.010.
[53]
Lanzkron S, Carroll CP, Haywood C. The burden of emergency department use for sickle-cell
disease: an analysis of the national emergency department sample database. Am J Hematol
2010;85:797–9. doi:10.1002/ajh.21807.
[54]
Price JH, Khubchandani J, McKinney M, Braun R. Racial/ethnic disparities in chronic diseases
of youths and access to health care in the United States. Biomed Res Int 2013;2013.
[55]
Allen Liles E, Kirsch J, Gilchrist M, Adem M. Hospitalist management of vaso-occlusive pain
crisis in patients with sickle cell disease using a pathway of care. Hosp Pract (1995)
2014;42:70–6. doi:10.3810/hp.2014.04.1105.
[56]
Yale SH, Nagib N, Guthrie T. Approach to the vaso-occlusive crisis in adults with sickle cell
disease. Am Fam Physician 2000;61:1349–56, 1363–4.
[57]
Reddin CDRC, Cerrentano E, Tanabe P. Sickle cell disease management in the emergency
department: what every emergency nurse should know. J Emerg Nurs 2011;37:341–5; quiz
426. doi:10.1016/j.jen.2010.04.014.
[58]
Benjamin LJ, Swinson GI, Nagel RL. Sickle cell anemia day hospital: an approach for the
management of uncomplicated painful crises. Blood 2000;95:1130–6.
[59]
Evidence-Based Management of Sickle Cell Disease: Expert Panel Report, 2014 - NHLBI,
NIH
n.d.
http://www.nhlbi.nih.gov/health-pro/guidelines/sickle-cell-disease-guidelines
(accessed May 6, 2015).
[60]
Clinical T, Guideline P, Cpg T, Children D. CLINICAL PRACTICE GUIDELINES Sickle
Cell Pain Clinical Practice Guideline 1815.
109
[61]
Ender KL, Krajewski JA, Babineau J, Tresgallo M, Schechter W, Saroyan JM, et al. Use of a
clinical pathway to improve the acute management of vaso-occlusive crisis pain in pediatric
sickle cell disease. Pediatr Blood Cancer 2014;61:693–6. doi:10.1002/pbc.24864.
[62]
Carey PJ. Addressing the global health burden of sickle cell disease. Int Health 2014.
doi:10.1093/inthealth/ihu045.
[63]
What Is Sickle Cell Disease? - NHLBI, NIH n.d. http://www.nhlbi.nih.gov/health/healthtopics/topics/sca (accessed July 22, 2015).
[64]
Lebensburger JD, Grosse SD, Altice JL, Thierry JM, Ivankova N V. Understanding and
Improving Health Education Among First-time Parents of Infants With Sickle Cell Anemia in
Alabama : A Mixed Methods Approach 2014;00:1–8.
[65]
Shahine R, Badr LK, Karam D, Abboud M. Educational Intervention to Improve the Health
Outcomes of Children With Sickle Cell Disease. J Pediatr Health Care 2014.
doi:10.1016/j.pedhc.2014.06.007.
[66]
Galeotti C, Courtois E, Carbajal R. How French paediatric emergency departments manage
painful vaso-occlusive episodes in sickle cell disease patients. Acta Paediatr 2014.
doi:10.1111/apa.12773.
[67]
McGann PT, Nero AC, Ware RE. Current management of sickle cell anemia. Cold Spring
Harb Perspect Med 2013;3. doi:10.1101/cshperspect.a011817.
[68]
Shahine R, Badr LK, Karam D, Abboud M. Educational intervention to improve the health
outcomes of children with sickle cell disease. J Pediatr Health Care 2015;29:54–60.
doi:10.1016/j.pedhc.2014.06.007.
[69]
DeBaun MR. The challenge of creating an evidence-based guideline for sickle cell disease.
JAMA 2014;312:1004–5. doi:10.1001/jama.2014.11103.
[70]
Sickle Cell Disease : Is Hydroxyurea the Final Answer ? 2014:2014.
[71]
Berthaut I, Guignedoux G, Kirsch-noir F, Larouziere V De, Ravel C, Bachir D, et al. Influence
of sickle cell disease and treatment with hydroxyurea on sperm parameters and fertility of
human males 2008;93:988–93. doi:10.3324/haematol.11515.
[72]
Telen MJ, Wun T, McCavit TL, De Castro LM, Krishnamurti L, Lanzkron S, et al.
Randomized phase 2 study of GMI-1070 in SCD: reduction in time to resolution of vasoocclusive events and decreased opioid use. Blood 2015:blood – 2014–06 – 583351.
doi:10.1182/blood-2014-06-583351.
[73]
Oniyangi O, Cohall DH. Phytomedicines (medicines derived from plants) for sickle cell
disease.
Cochrane
Database
Syst
Rev
2010:CD004448.
doi:10.1002/14651858.CD004448.pub4.
[74]
Issom D-Z, Zosso A, Ehrler F, Wipfli R, Lovis C, Koch S. Exploring the Challenges and
Opportunities of eHealth Tools for Patients with Sickle Cell Disease. Stud Health Technol
Inform 2015;216:898.
110
[75]
Issom D-Z, Zosso A, Wipfli R, Ehrler F, Lovis C. Meeting Sickle Cell patients ’ unmet needs
with eHealth tools : a preliminary study. Proc from 13th Scand Conf Heal Informatics (SHI
2015), June 15–17, 2015, Tromsø, Norw 2015:12.
[76]
Kelley H, Chiasson M, Downey A, Pacaud D. The Clinical Impact of eHealth on the SelfManagement of Diabetes: A Double Adoption Perspective. J Assoc Inf Syst 2011;12.
[77]
Bediang G, Perrin C, Ruiz de Castañeda R, Kamga Y, Sawadogo A, Bagayoko CO, et al. The
RAFT Telemedicine Network: Lessons Learnt and Perspectives from a Decade of Educational
and Clinical Services in Low- and Middle-Incomes Countries. Front Public Heal 2014;2:180.
doi:10.3389/fpubh.2014.00180.
[78]
Årsand E, Frøisland DH, Skrøvseth SO, Chomutare T, Tatara N, Hartvigsen G, et al. Mobile
health applications to assist patients with diabetes: lessons learned and design implications. J
Diabetes Sci Technol 2012;6:1197–206. doi:10.1177/193229681200600525.
[79]
Arsand E, Frøisland DH, Skrøvseth SO, Chomutare T, Tatara N, Hartvigsen G, et al. Mobile
Health Applications to Assist Patients with Diabetes: Lessons Learned and Design
Implications. J Diabetes Sci Technol 2012;6:1197–206.
[80]
Tatara N, Arsand E, Skrøvseth SO, Hartvigsen G. Long-term engagement with a mobile selfmanagement system for people with type 2 diabetes. JMIR mHealth uHealth 2013;1:e1.
doi:10.2196/mhealth.2432.
[81]
Quinn CC, Clough SS, Minor JM, Lender D, Okafor MC, Gruber-Baldini A. WellDoc mobile
diabetes management randomized controlled trial: change in clinical and behavioral outcomes
and patient and physician satisfaction. Diabetes Technol Ther 2008;10:160–8.
doi:10.1089/dia.2008.0283.
[82]
Haywood C, Bediako S, Lanzkron S, Diener-West M, Strouse J, Haythornthwaite J, et al. An
unequal burden: poor patient-provider communication and sickle cell disease. Patient Educ
Couns 2014;96:159–64. doi:10.1016/j.pec.2014.05.013.
[83]
Ramanathan N, Swendeman D, Comulada WS, Estrin D, Rotheram-Borus MJ. Identifying
preferences for mobile health applications for self-monitoring and self-management: focus
group findings from HIV-positive persons and young mothers. Int J Med Inform 2013;82:e38–
46. doi:10.1016/j.ijmedinf.2012.05.009.
[84]
Diviani N, van den Putte B, Giani S, van Weert JC. Low Health Literacy and Evaluation of
Online Health Information: A Systematic Review of the Literature. J Med Internet Res
2015;17:e112. doi:10.2196/jmir.4018.
[85]
Regulations.gov
Notice
Document
n.d.
http://www.regulations.gov/#!documentDetail;D=FDA-2011-D-0530-0001 (accessed July 20,
2015).
[86]
Panepinto JA, Torres S, Bendo CB, McCavit TL, Dinu B, Sherman-Bien S, et al. PedsQLTM
Multidimensional Fatigue Scale in sickle cell disease: feasibility, reliability, and validity.
Pediatr Blood Cancer 2014;61:171–7. doi:10.1002/pbc.24776.
[87]
Adegbola MA, Barnes DM, Opollo JG, Herr K, Gray J, McCarthy AM. Voices of Adults
Living with Sickle Cell Disease Pain. J Natl Black Nurses Assoc 2012;23:16–23.
111
[88]
Smith WR, Penberthy LT, Bovbjerg VE, McClish DK, Roberts JD, Dahman B, et al. Daily
assessment of pain in adults with sickle cell disease. Ann Intern Med 2008;148:94–101.
[89]
Swan M. The Quantified Self: Fundamental Disruption in Big Data Science and Biological
Discovery. Big Data 2013;1:85–99. doi:10.1089/big.2012.0002.
[90]
Banaee H, Ahmed MU, Loutfi A. Data mining for wearable sensors in health monitoring
systems: a review of recent trends and challenges. Sensors (Basel) 2013;13:17472–500.
doi:10.3390/s131217472.
[91]
Shaywitz D. Do Data From Wearables Belong In The Medical Record? n.d.
http://www.forbes.com/sites/davidshaywitz/2014/09/07/do-data-from-wearables-belong-inthe-medical-record/ (accessed December 22, 2014).
[92]
Farra R, Sheppard NF, McCabe L, Neer RM, Anderson JM, Santini JT, et al. First-in-human
testing of a wirelessly controlled drug delivery microchip. Sci Transl Med 2012;4:122ra21.
doi:10.1126/scitranslmed.3003276.
[93]
Jacob E, Duran J, Stinson J, Lewis MA, Zeltzer L. Remote monitoring of pain and symptoms
using wireless technology in children and adolescents with sickle cell disease. J Am Assoc
Nurse Pract 2013;25:42–54. doi:10.1111/j.1745-7599.2012.00754.x.
[94]
Neumayr L, Pringle S, Giles S, Quirolo KC, Paulukonis S, Vichinsky EP, et al. Chart Card:
feasibility of a tool for improving emergency department care in sickle cell disease. J Natl Med
Assoc 2010;102:1017–23.
[95]
Andreu Perez J, Leff D, Ip H, Yang G-Z. From Wearable Sensors to Smart Implants - Towards
Pervasive and Personalised Healthcare. IEEE Trans Biomed Eng 2015.
doi:10.1109/TBME.2015.2422751.
[96]
Olivo J, Carrara S, De Micheli G. IronIC patch: A wearable device for the remote powering
and connectivity of implantable systems. 2012 IEEE Int. Instrum. Meas. Technol. Conf. Proc.,
IEEE; 2012, p. 286–9. doi:10.1109/I2MTC.2012.6229168.
[97]
Altini M, Penders JF, Amft O. Estimating Oxygen Uptake during Non-Steady-State Activities
and Transitions Using Wearable Sensors. IEEE J Biomed Heal Informatics 2015;PP:1.
doi:10.1109/JBHI.2015.2390493.
[98]
Jin W, Wu L, Song Y, Jiang J, Zhu X, Yang D, et al. Continuous intra-arterial blood pH
monitoring by a fiber-optic fluorosensor. IEEE Trans Biomed Eng 2011;58:1232–8.
[99]
Ding H, Lu Q, Gao H, Peng Z. Non-invasive prediction of hemoglobin levels by principal
component and back propagation artificial neural network. Biomed Opt Express 2014;5:1145–
52. doi:10.1364/BOE.5.001145.
[100] Switz NA, D’Ambrosio M V, Fletcher DA. Low-cost mobile phone microscopy with a
reversed
mobile
phone
camera
lens.
PLoS
One
2014;9:e95330.
doi:10.1371/journal.pone.0095330.
[101] Breslauer DN, Maamari RN, Switz NA, Lam WA, Fletcher DA. Mobile phone based clinical
microscopy
for
global
health
applications.
PLoS
One
2009;4:e6320.
doi:10.1371/journal.pone.0006320.
112
[102] Skandarajah A, Reber CD, Switz NA, Fletcher DA. Quantitative imaging with a mobile phone
microscope. PLoS One 2014;9:e96906. doi:10.1371/journal.pone.0096906.
[103] Smart phone blood test device wins international award | Scope Blog n.d.
http://scopeblog.stanford.edu/2014/11/12/stanford-developed-smart-phone-blood-testingdevice-wins-international-award/ (accessed July 24, 2015).
[104] SmartScope:
Using
Mobile
Microscopy
to
Characterize
Anemia
http://sh.rice.edu/elec419reports/SmartScope Final Report.pdf (accessed July 24, 2015).
n.d.
[105] Kalantarian H, Alshurafa N, Sarrafzadeh M. A Wearable Nutrition Monitoring System. 2014
11th Int. Conf. Wearable Implant. Body Sens. Networks, IEEE; 2014, p. 75–80.
doi:10.1109/BSN.2014.26.
[106] Tatara N, Årsand E, Skrovseth SO, Hartvigsen G. Long-term engagement with a mobile selfmanagement system for people with type 2 diabetes. J Med Internet Res 2013;15.
doi:10.2196/mhealth.2432.
[107] Årsand E, Muzny M, Bradway M, Muzik J, Hartvigsen G. Performance of the First Combined
Smartwatch and Smartphone Diabetes Diary Application Study. J Diabetes Sci Technol 2015.
doi:10.1177/1932296814567708.
[108] Hilliard ME, Hahn A, Ridge AK, Eakin MN, Riekert KA. User Preferences and Design
Recommendations for an mHealth App to Promote Cystic Fibrosis Self-Management. JMIR
mHealth uHealth 2014;2:e44. doi:10.2196/mhealth.3599.
[109] Miller AS, Cafazzo JA, Seto E. A game plan: Gamification design principles in mHealth
applications for chronic disease management. Health Informatics J 2014.
doi:10.1177/1460458214537511.
[110] Shah N, Jonassaint J, Castro L De. Patients Welcome the Sickle Cell Disease Mobile
Application to Record Symptoms via Technology (SMART) 2014.
[111] Breslauer DN, Maamari RN, Switz NA, Lam WA, Fletcher DA. Mobile phone based clinical
microscopy
for
global
health
applications.
PLoS
One
2009;4:e6320.
doi:10.1371/journal.pone.0006320.
[112] Shah S, Dhameliya V, Roy AK. ImPatho - an image processing based pathological decision
support system for disease identification and a novel tool for overall health governance. 2014
IEEE Reg. 10 Humanit. Technol. Conf. (R10 HTC), IEEE; 2014, p. 64–9. doi:10.1109/R10HTC.2014.7026311.
[113] Youngchan Kim, YongKeun Park. Quantitative phase imaging and spectroscopy techniques
for the study of sickle cell diseases 2012:1–2.
[114] Woods K, Kutlar A, Grigsby RK, Adams L, Stachura ME. Primary-care delivery for sickle cell
patients in rural Georgia using telemedicine. Telemed J 1998;4:353–61.
[115] Woods KF, Kutlar A, Johnson JA, Waller JL, Grigsby RK, Stachura ME, et al. Sickle cell
telemedicine and standard clinical encounters: a comparison of patient satisfaction. Telemed J
1999;5:349–56. doi:10.1089/107830299311916.
113
[116] Woods KF, Johnson JA, Kutlar A, Daitch L, Stachura ME. Sickle cell disease telemedicine
network for rural outreach. J Telemed Telecare 2000;6:285–90.
[117] Venugopalan J, Brown C, Cheng C, Stokes TH, Wang MD. Activity and school attendance
monitoring system for adolescents with sickle cell disease. Conf Proc . Annu Int Conf IEEE
Eng Med Biol Soc IEEE Eng Med Biol Soc Annu Conf 2012;2012:2456–9.
doi:10.1109/EMBC.2012.6346461.
[118] Schwartz LA, Radcliffe J, Barakat LP. Associates of school absenteeism in adolescents with
sickle cell disease. Pediatr Blood Cancer 2009;52:92–6. doi:10.1002/pbc.21819.
[119] Cheng C, Brown RC, Cohen LL, Venugopalan J, Stokes TH, Wang MD. iACT--an interactive
mHealth monitoring system to enhance psychotherapy for adolescents with sickle cell disease.
Conf Proc . Annu Int Conf IEEE Eng Med Biol Soc IEEE Eng Med Biol Soc Annu Conf
2013;2013:2279–82. doi:10.1109/EMBC.2013.6609992.
[120] Brown C, New T, Stokes TH, Dampier C, Wang MD. SickleREMOTE: A two-way text
messaging system for pediatric sickle cell disease patients. Proc. 2012 IEEE-EMBS Int. Conf.
Biomed. Heal. Informatics, IEEE; 2012, p. 408–11. doi:10.1109/BHI.2012.6211602.
[121] Jacob E, Gerla M. Web-based mobile e-Diary for youth with Sickle Cell Disease. 2012 IEEE
Consum. Commun. Netw. Conf., IEEE; 2012, p. 385–9. doi:10.1109/CCNC.2012.6181012.
[122] Jacob E, Stinson J, Duran J, Gupta A, Gerla M, Ann Lewis M, et al. Usability testing of a
Smartphone for accessing a web-based e-diary for self-monitoring of pain and symptoms in
sickle
cell
disease.
J
Pediatr
Hematol
Oncol
2012;34:326–35.
doi:10.1097/MPH.0b013e318257a13c.
[123] Jacob E, Pavlish C, Duran J, Stinson J, Lewis MA, Zeltzer L. Facilitating Pediatric PatientProvider Communications Using Wireless Technology in Children and Adolescents With
Sickle Cell Disease. J Pediatr Heal Care 2013;27:284–92. doi:10.1016/j.pedhc.2012.02.004.
[124] Durfee RA, Venugopalan J, Ren J, Wang MD. Med-vest: A wearable sensory platform. 2014
IEEE Healthc. Innov. Conf., IEEE; 2014, p. 199–202. doi:10.1109/HIC.2014.7038909.
[125] Panneerselvam P. Application of embedded system for a genetic disease, sickle cell anemia.
2014 Int. Conf. Adv. Electr. Eng., IEEE; 2014, p. 1–4. doi:10.1109/ICAEE.2014.6838446.
[126] Ehrler F. Agrégation de données d’un réseau de capteurs physiologiques. Swiss Med
Informatics 2014;30. doi:10.4414/smi.30.313.
[127] Leonard S, Jonassaint J, Anderson L, Shah N. The Use of Mobile Technology for Intensive
Training in Medication Management in the Pediatric Population. Blood 2014;124:4842.
[128] Jonassaint CR, Shah N, Jonassaint J, De Castro L. Usability and Feasibility of an mHealth
Intervention for Monitoring and Managing Pain Symptoms in Sickle Cell Disease: The Sickle
Cell Disease Mobile Application to Record Symptoms via Technology (SMART).
Hemoglobin 2015;39:162–8. doi:10.3109/03630269.2015.1025141.
[129] Shah N, Jonassaint J, De Castro L. Patients welcome the Sickle Cell Disease Mobile
Application to Record Symptoms via Technology (SMART). Hemoglobin 2014;38:99–103.
doi:10.3109/03630269.2014.880716.
114
[130] Rowe PG. Design Thinking. MIT Press; 1991.
[131] Mobile
Usability
|
Book
by
Nielsen
Norman
http://www.nngroup.com/books/mobile-usability/ (accessed April 21, 2015).
Group
n.d.
[132] Holzinger A. Rapid prototyping for a virtual medical campus interface. IEEE Softw
2004;21:92–9. doi:10.1109/MS.2004.1259241.
[133] Johnson CM, Johnson TR, Zhang J. A user-centered framework for redesigning health care
interfaces. J Biomed Inform 2005;38:75–87. doi:10.1016/j.jbi.2004.11.005.
[134] Pereira SADS, Brener S, Cardoso CS, Proietti ABDFC. Sickle Cell Disease: quality of life in
patients with hemoglobin SS and SC disorders. Rev Bras Hematol Hemoter 2013;35:325–31.
doi:10.5581/1516-8484.20130110.
[135] Al-Rimawi H, Jallad S. Sport participation in adolescents with sickle cell disease. Pediatr
Endocrinol Rev 2008;6 Suppl 1:214–6.
[136] Jenerette CM, Murdaugh C. Testing the theory of self-care management for sickle cell disease.
Res Nurs Heal 2008;31:355–69. doi:10.1002/nur.20261.
[137] Ballas SK. Current issues in sickle cell pain and its management. Hematology Am Soc
Hematol Educ Program 2007:97–105. doi:10.1182/asheducation-2007.1.97.
[138] Boulos MNK, Brewer AC, Karimkhani C, Buller DB, Dellavalle RP. Mobile medical and
health apps: state of the art, concerns, regulatory control and certification. Online J Public
Health Inform 2014;5:229. doi:10.5210/ojphi.v5i3.4814.
[139] Kauf TL, Coates TD, Huazhi L, Mody-Patel N, Hartzema AG. The cost of health care for
children and adults with sickle cell disease. Am J Hematol 2009;84:323–7.
doi:10.1002/ajh.21408.
115
Appendix 1: Project Plan
Preliminary
116
Revised
117
Risks assessment
Table 10 - Risks assessment
Risk
Aspects
Measures
Time management
Getting answer from different stakeholders
(patient communities, patients, caregivers,
etc.) can then be time consuming
Contact stakeholders and send the survey
to patients as early as possible and extend
the deadline in order to be able to get
enough results and analyse them
Travelling from Northern Norway to meet
people in different areas can delay the
project
Schedule
meeting
Getting the answers from the patients can
take time and be difficult
Send notifications and reminders to
participants
Sickness (VOC) is a non-negligible factor
Try to stay as healthy as possible and if
not possible, delay the project
Ethical approvals would take time to be
received and can be refused
Try to avoid mandatory ethical approval
by reviewing law and asking help to
people with experience
Questionnaire analysis is time consuming
Prepare potential scenarios analysis about
potential tendencies
Market the project differently and use all
possible
medias,
letters,
e-mail,
telephone, visits in hospital, in patients’
associations, etc.
Availability
Participants and stakeholders can be too
busy to answer e-mails, meet or respond to
the survey
Patients are rare and hidden. They might be
difficult to reach, but also to motivate
appointments
when
and
skype
possible
Try to find them with all possible medias.
Facebook,
patients’
associations,
hospitals.
Patients can lack of interest
Methods
Results
Exploratory research via web survey can be
tough because data collection can take time
and it’s difficult to force patients to answer.
Results will be analysed using an
appropriate software
Results and answers can be late
Try to motivate and understand them and
talk to them without being forceful
Try alternatives and ask for feedback
from supervisors
Analyse the results as soon as answers
arrive
Results can be messy
Organize the results and use software for
data organization
Results can be insufficient or insignificant
Ask to more patients and report the
limitations in the analysis
Results can be contradictory or irrelevant
Ask patients
explanations
Results can be overwhelming
Limit the scope of analysis and find
hypothesis.
Delegate the work to potential co-authors
or refocus on the most important and
significant results
Results can be sensible or raise feelings
Avoid judgement and publish raw results
118
for
feedback
and
Appendix 2: Survey in English
Meeting patients with Sickle Cell disease unmet needs
The study targets adults who have Sickle Cell disease and aims to help them self-manage their
disease.
Sickle Cell disease is a complex disease. The pain episodes (Sickle Cell crisis) it causes are hardly
predictable. Even though some patients have shown the ability to early feel a worsening of their
general
condition.
Project goal is to find solutions using eHealth technologies (smartphone, wearable devices, selfmonitoring devices, etc.) in order to give patients the opportunity to obtain in their daily life, the
ability to increase the knowledge of themselves, help them to cope with the symptoms and to better
manage
their
disease.
One of the biggest challenge that Information and communication technologies could overcome is
helping
patients
to
early
detect
and
prevent
the
triggering
of
crisis.
This could be done for instance by providing advice for good self-care, information about their
current health status or an assistance when they perform physical activity.
The following survey contains 47 questions. The completion will require approximately 30 minutes of
your time.
I am interested!
119

Survey
n°
290115
Investigating the needs and barriers in using eHealth tools to support the self-management of
Sickle cell disease patients*
I confirm that I have been informed that this study involves me in a research that is conducted and
organized by David-Zacharie ISSOM (principal investigator), a Master student in Health Informatics
at Karolinska Institutet (Stockholm, Sweden) in order to obtain his degree early august 2015.
The study is done in collaboration with the Karolinska University Hospital, the Norwegian Centre for
Integrated Care and Telemedicine, the Geneva University Hospitals, the Swiss Association of Sickle
Cell Disease and the Swiss Haemoglobinopathy Society. The study takes place in the three aforesaid
countries.
I understand that this project is designed to investigate the needs and barriers in developing eHealth
tools
supporting
the
self-management
of
patients
with
Sickle
Cell
disease.
The study includes all the interested patients above 18 years old and all the published results and
collected
data
will
be
anonymous.
I understand that my participation in this study will involve the voluntary completion of a
questionnaire
that
will
take
approximately
30
minutes
of
my
time.
I understand that I have the possibility to give my contact information if I wish to be contacted.
I understand that my identity as a research participant in this study will be kept in strict confidence
and that no information that identifies me will be released without my separate written approval.
I understand that I may refuse to participate or withdraw from this study before submission of the
final set of responses without any consequences. My responses would be immediately deleted.
As a research participant, I authorize the principal investigator to use and disclose my information
such
as
demographic
information
or
health
information.
I
understand
that
I
have
the
right
to
oppose
it.
I understand that I may contact David-Zacharie Issom ([email protected]) or his
supervisor Dr. Stefano Bonacina ([email protected]) if I have any question about this project or
my
participation
in
this
study.
I understand that I can receive if I wish to, a summary of the results, propositions for further
participation in the prototyping process (testing and evaluation) or additional information about the
study
from
David-Zacharie
Issom.
I consent to participate to this study by clicking on "I consent" or pressing the "Y" touch.
(Thanks to Alexandra Wyke and Dee O'Sullivan from myhealthapps.net and Patient View who
authorized the use of some of their surveys as inspiration)
120
We thank you for your collaboration and wish to assure you that we strive to provide Sickle Cell
patients innovative self-help solutions.
1. What is your gender?
a. Male
b. Female
c. Other: _____________
2. What is your age?
a. ____________
3.
What is your marital status
a. Single
b. Married or domestic partnership
c. Widowed
d. Divorced or separated
e. Prefer not to answer
4.
What is the highest degree or level of school you have completed?
a. No schooling completed
b. Some high school, no diploma
c. High school graduate, diploma or the equivalent
d. Trade / Technical / Vocational training / Professional degree
e. High / superior professional degree diploma
f.
Some university credit, no degree
g. Bachelor's degree
h. Master's degree
i.
Doctorate degree
j.
Prefer not to answer
121
5.
What is your employment status?
a. Employed
b. Self-employed
c. Out of work and looking for work
d. Out of work but not currently looking for work
e. Homemaker
f.
Student
g. Retired
h. Unable to work
6.
Are there any children under the age of 18 currently living in your household?
a. Yes
b. No
7.
Where
do
Ex: Geneva, Switzerland
you
reside
actually?
a. ___________________
The 3 following questions are about your lifestyle as well as your general health status.
8. How would you rate your overall health?
1 (Poor)
2 3 4 5 6 (Good)
7 8 9 10
11 (Excellent)
9. Do you feel you get the right amount of exercise?
1 (Much too little)
2 3 4 5 6 (About the right amount)
7 8 9 10
11 (Much too much)
10. Do
you
feel
you
have
healthy
habits?
Ex: healthy diet, drinking a lot of water, non-smoking, sleeping enough, staying physically active,
get flu shots, etc.
0 (Not at all)
1 2 3 4 5 (Moderately)
122
6 7 8 9
10 (Extremely)
The 15 next questions relate to your experience with Sickle Cell disease
11. In the last 12 months, how many crisis that did NOT require hospital or emergency room
admission
have
you
had?
You can give an estimation.
a. ___________
12. In the last 12 months, how many crisis that DID require hospital or emergency room admission
have
you
had?
You can give an estimation.
a. ___________
13. In the last 12 months, how many days crisis made you miss work or school?
You can give an estimation.
a. ____________
14. What symptoms are
Multiple choice question
the
most
disturbing
for
you
a. Pain episodes (chest or unspecified pains)
b. Rapid or irregular pulse
c. Fatigue
d. Infections / Fever
e. Headaches / Dizziness
f.
Coldness of the hands or feet
g. Paleness
h. Fasciculation (Muscle twitching)
i.
The disease does not affect me in my daily life
j.
Other: ______________________
15. In the last 12 months, how often symptoms interfered with your daily life?
a. Daily
b. Weekly
c. Monthly
d. Every 2-3 months
123
in
your
daily
life
e. About 3-4 times a year
f.
Almost never
g. Never
16. Overall,
have
you
Multiple choice question
experienced
one
of
these
complications?
a. Hand-Foot syndrome
b. Splenic crisis
c. Infections / Fever
d. Chronic pain
e. Acute chest syndrome
f.
Pulmonary infarction
g. Pulmonary embolism
h. Pulmonary oedema
i.
Pulmonary hypertension
j.
Multiple organ failures
k. Myocardial Infarction (Heart attack)
l.
Gallstones
m. Kidney stones
n. Eyes problems
o. Stroke
p. None
q. Prefer not to answer
r.
I don't know
s. Other: _____________________
17. Which would best describe
Try to choose maximum 3 answers
how
you
feel
living
with
a. I have difficulty completing normal day to day activities
b. I have difficulty having a normal physical activity
c. I have difficulty practicing self-care (medication, hydration, etc.)
d. I can't sleep at night
124
Sickle
cell
disease?
e. I am treated differently by others
f.
I feel like a burden to others
g. I have a low level of self-confidence
h. I feel alone
i.
I can't go to work / school on a regular basis
j.
Social life and relationships with friends are restricted
k. Family and close-friends don't understand my suffering
l.
I feel stronger than other people
m. I feel somewhat good
n. I feel very good
o. Sickle Cell disease has not affected my life or does not anymore
p. I prefer not to answer
18. Do you know other people with Sickle Cell disease?
a. Yes
b. No
19. Are you specially interested in learning more about:?
a. Causes of triggering of Sickle Cell crisis
b. Symptoms of the disease
c. Other particularities of the disease
d. Drugs and side-effects
e. Blood transfusions
f.
Support groups and communities
g. None of the above
h. Other
20. How often do you see your doctor?
a. Daily
b. Weekly
c. Monthly
d. 8-10 times per year
125
e. 5-7 times per year
f.
2-4 times per year
g. Almost never
h. Never
21. How
far
are
you
Give an estimation in kilometres
travelling
to
visit
your
healthcare
provider?
a. ______ km
22. How much do you trust your healthcare providers to make decisions that are in your best interests?
1 (I distrust them greatly)
2 3 4 5 (I trust them greatly)
23. Is your healthcare provider (the one you see often) able to see if you are getting sicker or need a
check-up?
0 (Never)
1 2 (Sometimes)
3 4 (Always)
24. Are your family / carers / significant others / close-friends able to see if you are getting sicker or need
a check-up?
0 (Never)
25. Would
you
Assuming you
1 2 (Sometimes)
3 4 (Always)
like
to
share
your
health
information
choose what kind of information and with
with
whom
other
people?
you share it.
(For instance your patient record or wellness status with family members, friends, healthcare
providers, other patients, researchers, health organizations and authorities, personal trainers, etc.)
0 (Not
likely)
at
all 1 2 3 4 5
(Extremely
likely)
26. Overall, are you satisfied with the way the healthcare system is taking care of your disease?
Ex: In your daily life, in case of emergency, global care, psychological and social support, financially,
insurances system, etc.
0 (Not
likely)
at
all 1 2 3 4 5
(Extremely
likely)
126
The 21 following questions are meant to assess your receptiveness and literacy to Information and
Communication Technologies (ICTs), their integration in healthcare (for instance assistive technologies)
and how a health app would suit you the best.
27. How familiar are you with the use of Internet?
0 (Not at all well)
1 2 (Moderately well)
3 4 (Extremely well)
28. How familiar are you with the use of Smartphones?
0 (Not at all well)
1 2 (Moderately well)
3 4 (Extremely well)
29. How receptive are you about the use of ICTs for healthcare purposes?
0
(Very 1 2 (Neutral)
negatively)
30. Do
you
use
on
Multiple choice question
a
regular
3 4
(Very
positively)
basis
any
of
the
following
technologies?
a. Computer
b. Smartphone
c. Tablet
d. Smartwatch
e. Fitness tracker (Smart band or other wearable device)
f.
Communication software (Skype, FaceTime or other)
g. Messaging software (SMS, WhatsApp or other)
h. None of the above
31. How
frequently
are
you
using
Information about Sickle Cell anaemia
them
a. Everyday
b. 3-6 times per week
c. 1-2 times per week
d. Once a month
e. Twice a month
f.
Every 2-3 months
127
to
seek
for
health
information?
g. Every 3-4 months
h. 1-2 times per year
i.
Only during my doctor's appointment
j.
Never
32. What have been the
Multiple choice question
best
places
to
get
info
about
Sickle
Cell
disease?
about
Sickle
Cell
disease?
a. Doctor's appointments
b. Websites / Search engines
c. Online support groups
d. Online patient communities
e. Online chat rooms / Forums
f.
Facebook groups
g. Patients groups that meet in person
h. Family / friends who have searched for me
i.
Other: _______________
33. On what places are
Multiple choice question
you
active
on
the
Internet
a. Blogs / Forums / Chats
b. Twitter
c. Facebook or other social media
d. Online support group / patient community
e. None of the above but I'm interested in health campaigning
f.
None of the above and I'm not interested
g. Other: _______________
34. Does one of your relative or carers use some of these technologies to search for health information
about
Sickle
Cell
disease?
Multiple choice question
a. Computer
b. Smartphone
c. Tablet
128
d. Communication software (Skype, Face Time, etc.)
e. Messaging software (SMS, WhatsApp, etc.)
f.
I don't know
g. None of the above
h. Other: ___________
35. What kind of technology would you consider the most to use in order to manage your disease?
Try to choose maximum 4 answers
a. Internet (Website)
b. Smartphone application
c.
Tablet application
d.
Telemedicine / Remote monitoring device
e.
Smartwatch
f.
Smart band (wristband with sensors or other wearable technologies)
g.
Communication software (Skype, Face Time, etc.)
h.
Messaging software (SMS, WhatsApp, etc.)
i.
Smart TV
j.
None of the above
36. What
would
convince
Try to choose maximum 2 answers
you
to
use
a
health
a. Provide accurate and trustworthy information
b. Be easy to use and simple / well designed
c. Work effectively and consistently over time
d. Allow you to provide support and help to other patients
e. Raise awareness about the disease and educate people
f.
Allow you to rate or comment on a healthcare service you received
g. Provide guarantees of data security and integrity
h. Be free
i.
Be less expensive than any other form of healthcare support
j.
Have no advertisements
k. Other: __________
129
app
regularly?
37. Do you use smartphone / tablet apps to manage your disease?
a. Yes
b. No
38. Which?
If you don’t remember the name, you can describe it
a. ___________________
39. Would you be interested in having a wearable device that tracks your health status?
For ex. a smartwatch, wristband or other wearable device that tracks your physical condition, activity,
sleep, symptoms, biological data, vital signs, etc.
0 (Not at all)
1 2 (Moderately)
3 4
(Extremely)
40. Do you wear a Smartwatch or Fitness tracker?
a.
Yes
b. No but I'm interested
c. No and I'm not interested
41. What are the most important services a health app (or website) should offer you?
Try to choose maximum 6 answers
a. Offer general information about your disease (best-practices, new drugs, advances in
research, choices of treatment, etc.)
b. Advices and feedback about how to self-care in your daily life (pain management, drugs,
best behaviours, etc.)
c. Information about your current health status
d. Help to detect the early signs of crisis
e. Help to avoid the triggering of crisis
f.
Help to have a healthy lifestyle
g. Assist when performing physical activities
h. Offer a permanent contact with healthcare providers
i.
Access your medical record and allow to keep it up to date
j.
Arrange and manage your medical appointments / check-ups / follow-up
k. Ease the access to medical assistance in case of emergency
130
l.
Ease the access to medical assistance in everyday life
m. Offer an emotional / psychological support in case of crisis
n. Offer an emotional / psychological support outside crisis
o. Help to contact support groups in case of crisis (patient community, family, carers, etc.)
p. Help to contact support groups outside crisis (patient community, family, carers, etc.)
q. Provide information about your disease in case of emergency (quick overview of my
medical records and history)
r.
Provide information that educates your friends / family / people you meet
42. What do you think of always carrying your important health information on you?
For instance a card in your wallet or a device carrying your important medical information
0 (Not at all important)
1 2 3 4 (Extremely important)
Thank you for time, you are close to the end!
Following questions are generally about the self-tracking of your health condition
43. Do you report or log your pain symptoms?
0 (Never)
1 2 (Sometimes)
44. Do
you
report
Included your emergency visits
0 (Never)
or
1 2 (Sometimes)
45. Are you tracking some
Try
to
Multiple choice question
3 4 (Always)
of
log
your
hospital
admissions?
3 4 (Always)
the
following
health
choose maximum
/
wellness
information?
5 answers
a. Pain and clinical signs or symptoms
b. Non-Sickle Cell related pains
c. Vital signs and bio-data (pulse, oxygen saturation, temperature, etc.)
d. Factors that can affect your health status (altitude, hydration, pollution, weather, alcohol
consumption, etc.)
131
e. Health status (sickness, infections, etc.)
f.
Daily routines
g. Physical activity
h. Sleep
i.
Diet
j.
Feelings and emotions
k. Tiredness
l.
Motivation
m. Mood / anxiety level
n. Socializing level
o. Medicine intake
p. None
q. Other: ____________
46. Which of the following would
Try to choose maximum 5 answers
you
like
the
most
to
be automatically collected?
a. Pain and clinical signs or symptoms
b. Non-Sickle Cell related pains
c. Vital signs and bio-data (pulse, oxygen saturation, temperature, etc.)
d. Factors that can affect your health status (altitude, hydration, pollution, weather, alcohol
consumption, etc.)
e. Health status (sickness, infections, etc.)
f.
Daily routines
g. Physical activity
h. Sleep
i.
Diet
j.
Feelings and emotions
k. Tiredness
l.
Motivation
m. Mood / anxiety level
n. Socializing level
o. Medicine intake
132
p. None
q. Other: _______________
47. Would you like to receive alerts about when your health status is getting lower
For instance when you should drink, rest, take drugs, get oxygen, etc.
0 (Not at all)
1 2 (Neutral)
3 4 (Yes definitely)
48. Would you like to receive alerts when your medicine should be taken?
0 (Not at all)
Thank
1 2 (Neutral)
3 4 (Yes definitely)
you
again!
The next and last 10 questions are not mandatory but can help us to understand and meet your needs and
wishes.
Feel free to share your thoughts, suggestions or ideas!
49. In everyday life, what is your biggest concern (about the disease)?
a. _____________________________________
50. In your own words, what other features
For instance a Smartphone App, a website or other
or
services
would
you
appreciate?
a. _______________________________________
51. In your opinion, what should or could be improved in the healthcare system that take cares of you?
a. __________________________________________________
52. In your opinion, what is particularly good in the healthcare system that take care of you?
a. __________________________________________________
53. If an electronic service meeting your needs, wishes or suggestions was available today, would you use
it?
0 (Not likely at 1 2 3 4 5
(Extremely
all)
likely)
133
54. Would
you
pay
For instance a subscription or a permanent acquisition
for
it?
0 (Certainly 1 2 3 4 (Of course!)
not)
55. How likely would you recommend it to your healthcare provider or a Sickle Cell disease patient
community?
0 (Not likely at 1 2 3 4 5
(Extremely
all)
likely)
56. When you think about this project, do you think of it as something people might need or might want?
a. Need
b. Want
c. Other: __________________________
57. What was your first reaction when you heard about this research project?
1
(Very 2 3 4 5
(Very
negative)
positive)
58. Feel free to write any other comment or suggestion
a. ____________________________________________
59. How would you rate this survey?
1 (Extremely bad)
2 3 4 5 6 (Excellent)
Thank you for your participation as well as for the time and energy you spent!
You are encouraged to visit the website Haemoglobinopathie.ch where some results will be published.
For further participations to the study or for more information, please contact David-Zacharie ISSOM:
E-mail: [email protected]
Phone 1: +41 (0) 76 306 45 35
Phone 2: +46 (0) 72 037 13 56
Visit the website – www.haemoglobinopathie.ch
134
Appendix 3: Web-Survey results
Table 11- Sociodemographic details of the study participants
Variables
Gender
Male
Female
Age
Median age
Mean age
Mean age female
Mean age male
Median age female
Median age male
18-25
26-35
36 and above
Marital situation
Married
Separated
Single
Widowed
Highest education completed
No diploma
High school graduate
Professional degree
Superior professional degree
Bachelor
Master
Doctorate
Refused to answer
Employment status
Student
Unemployed but in search
Employed
Housewife
Unable to work
Self-employed
Children living in household
Country
Belgium
Cameroon
Canada
France
#
%
8
25
24,24 %
75,76 %
31
32,9
32,2
36,1
31
33
8
18
7
135
15
3
14
1
24,24 %
54,55 %
21,21 %
0,00 %
45,45 %
9,09 %
42,42 %
3,03 %
1
4
1
5
8
7
3
4
3,03 %
12,12 %
3,03 %
15,15 %
24,24 %
21,21 %
9,09 %
12,12 %
10
3
12
3
4
1
18
30,30 %
9,09 %
36,36 %
9,09 %
12,12 %
3,03 %
54,55 %
1
1
1
11
3,03 %
3,03 %
3,03 %
33,33 %
Germany
Guadeloupe
Norway
Rdc
Senegal
Switzerland
Usa
1
2
4
1
1
3
3
3,03 %
6,06 %
12,12 %
3,03 %
3,03 %
9,09 %
9,09 %
Table 12 - Answers to health-related questions
Variables
How would you rate your overall health?
1 (poor)
2
3
4
5
6 (good)
7
8
9
10
11 (excellent)
Mean
B
1 (poor)
2
3
4
5
6 (good)
7
8
9
10
11 (excellent)
Mean
Do you feel you have healthy habits?
1 (poor)
2
3
4
5
6 (good)
7
136
#
%
0
0
1
2
7
2
7
3
8
1
1
6,1
0,00 %
0,00 %
3,03 %
6,06 %
21,21 %
6,06 %
21,21 %
9,09 %
24,24 %
3,03 %
3,03 %
18,48 %
0
9
7
7
4
3
2
1
0
0
0
2,78
0,00 %
27,27 %
21,21 %
21,21 %
12,12 %
9,09 %
6,06 %
3,03 %
0,00 %
0,00 %
0,00 %
8,42 %
0
1
2
3
3
7
10
0,00 %
3,03 %
6,06 %
9,09 %
9,09 %
21,21 %
30,30 %
8
9
10
11 (excellent)
Median
Mean
Minimum
Maximum
Crises without hospitalisations last 12 months
Median nb of crises last 12 months
Mean nb of crises last 12 months
Minimum nb of crises last 12 months
Maximum nb of crises last 12 months
0-3
4-7
8-10
Above 10
Crises with hospitalisations last 12 months
Median
Mean
Minimum
Maximum
0-3
4-7
8-10
Above 10
Day of school / work missed last 12 months
Median
Mean
Minimum
Maximum
0-3
4-7
8-15
16-25
Above 25
What symptoms are the most disturbing for you daily?
Pain episodes
Rapid or irregular pulse
Fatigue
Infections / fever
Headaches / dizziness
Coldness of the hands or feet
Paleness
137
3
4
0
0
5,5
5,25
2
8
9,09 %
12,12 %
0,00 %
0,00 %
4
5
0
30
16
8
8
1
48,48 %
24,24 %
24,24 %
3,03 %
2
2,18
0
7
26
7
0
0
78,79 %
21,21 %
0,00 %
0,00 %
3
6,25
0
41
19
8
2
2
2
57,58 %
24,24 %
6,06 %
6,06 %
6,06 %
27
4
22
5
9
8
3
81,82 %
12,12 %
66,67 %
15,15 %
27,27 %
24,24 %
9,09 %
Fasciculation
The disease does not affects me
Other: amnesic episode
Yellow eyes?
How often did symptoms interfered with your daily life?
Daily
Weekly
Monthly
Every 2-3 months
About 3-4 times a year
Almost never
Never
Overall, have you experienced one of these complications?
Hand-foot syndrome
Splenic crisis
Infections / fever
Chronic pain
Acute chest syndrome
Pulmonary infarction
Pulmonary embolism
Pulmonary oedema
Pulmonary hypertension
Multiple organ failures
Myocardial infarction
Gallstones
Kidney stones
Eyes problems
Stroke
None
Prefer not to answer
I don't know
Other: bone infarctions (1), ostheomy (1), blood clots on arm and thigh
(1), phlebitis (1), hip replacement (1), hematite c (1), hemochromatosis
(1)
Which would best describe how you feel living with sickle cell disease?
I have difficulty completing normal day to day activities
I have difficulty having a normal physical activity
I have difficulty practicing self-care (medication, hydration, etc.)
I can't sleep at night
I am treated differently by others
I feel like a burden to others
I have a low level of self-confidence
I feel alone
I can't go to work / school on a regular basis
Social life and relationships with friends are restricted
138
8
3
1
24,24 %
9,09 %
3,03 %
0,00 %
9
3
4
8
3
3
3
27,27 %
9,09 %
12,12 %
24,24 %
9,09 %
9,09 %
9,09 %
4
8
25
24
20
4
7
3
2
1
2
13
3
7
1
2
0
0
12,12 %
24,24 %
75,76 %
72,73 %
60,61 %
12,12 %
21,21 %
9,09 %
6,06 %
3,03 %
6,06 %
39,39 %
9,09 %
21,21 %
3,03 %
6,06 %
0,00 %
0,00 %
0,00 %
11
18
12
8
9
11
10
6
9
5
33,33 %
54,55 %
36,36 %
24,24 %
27,27 %
33,33 %
30,30 %
18,18 %
27,27 %
15,15 %
Family and close-friends don't understand my suffering
3
9,09 %
I feel stronger than other people
3
9,09 %
I feel somewhat good
2
6,06 %
I feel very good
5
15,15 %
Sickle cell disease has not affected my life or does not anymore
4
12,12 %
I prefer not to answer
0
0,00 %
Do you know people with sickle cell disease?
Yes
30
90,91 %
No
2
6,06 %
Are you specially interested in learning more about:
Causes of triggering of sickle cell crisis
16
48,48 %
Symptoms of the disease
7
21,21 %
Other particularities of the disease
14
42,42 %
Drugs and side-effects
16
48,48 %
Blood transfusions
9
27,27 %
Support groups and communities
20
60,61 %
None of the above
3
9,09 %
Other: pregnancy
1
3,03 %
How often do you see your doctor?
Daily
1
3,03 %
Weekly
0
0,00 %
Monthly
9
27,27 %
8-10 times per year
1
3,03 %
5-7 times per year
6
18,18 %
2-4 times per year
13
39,39 %
Almost never
3
9,09 %
Never
0
0,00 %
How far are you travelling to visit your healthcare provider?
Mean distance
28,125
Median distance
9,5
Minimal distance
1
Maximum distance
296
How much do you trust your healthcare providers to make decisions that are in your best
interests?
1 (i distrust them greatly)
1
3,03 %
2
4
12,12 %
3
14
42,42 %
4
5
15,15 %
5 (i trust them greatly)
5
15,15 %
Is your healthcare provider (the one you see often) able to see if you are getting sicker or need a
check-up?
1 (never)
9
27,27 %
2
11
33,33 %
3
9
27,27 %
4 (always)
4
12,12 %
139
Are your family / carers / significant others / close-friends able to see if you are getting sicker or
need a check-up?
1 (never)
5
15,15 %
2
7
21,21 %
3
11
33,33 %
4 (always)
10
30,30 %
Would you like to share your health information with other people?
1 (not at all likely)
7
21,21 %
2
3
9,09 %
3
6
18,18 %
4
8
24,24 %
5 (extremely likely)
9
27,27 %
Overall, are you satisfied with the way the healthcare system is taking care of your disease?
1 (not at all)
9
27,27 %
2
10
30,30 %
3
11
33,33 %
4
3
9,09 %
5 (extremely)
0
0,00 %
Table 13 - Receptiveness to eHealth tools and digital literacy
Questions
How familiar are you with the use of internet?
0 (not at all well)
1
2 (moderately well)
3
4 (extremely well)
How familiar are you with the use of smartphones?
0 (not at all well)
1
2 (moderately well)
3
4 (extremely well)
How receptive are you about the use of icts for healthcare purposes?
0 very negatively)
1
2 (neutral)
3
4 (very positively)
Do you use on a regular basis any of the following technologies?
Computer
Smartphone
Tablet
Smartwatch
Fitness tracker
140
#
%
0
0
5
9
19
0,00 %
0,00 %
15,15 %
27,27 %
57,58 %
1
2
6
24
0,00 %
3,03 %
6,06 %
18,18 %
72,73 %
0
0
6
7
20
0,00 %
0,00 %
18,18 %
21,21 %
60,61 %
31
33
21
0
2
93,94 %
100,00 %
63,64 %
0,00 %
6,06 %
Communication software
Messaging software
None of the above
How frequently are you using them to seek for health information?
Everyday
3-6 times per week
1-2 times per week
Once a month
Twice a month
Every 2-3 months
Every 3-4 months
1-2 times per year
Only during my doctor's appointment
Never
What have been the best places to get info about sickle cell disease?
Doctor's appointments
Websites / search engines
Online support groups
Online patient communities
Online chat rooms / forums
Facebook groups
Patients groups that meet in person
Family / friends who have searched for me
Where have you been active on the internet about scd
Facebook or other social network
Blog / forums / chats
Online support groups / patients community
Twitter
None of the above but i'm interested
None of the above and i'm not interested
Other
Does one of your relative or carers use some of these technologies to search
for health information about sickle cell disease?
22
28
0
66,67 %
84,85 %
0,00 %
6
4
3
2
2
3
4
5
2
2
18,18 %
12,12 %
9,09 %
6,06 %
6,06 %
9,09 %
12,12 %
15,15 %
6,06 %
6,06 %
11
24
6
6
2
18
29
8
21
4
27
0
10
2
0
33,33 %
72,73 %
18,18 %
18,18 %
6,06 %
54,55 %
87,88 %
24,24 %
0,00 %
63,64 %
12,12 %
81,82 %
0,00 %
30,30 %
6,06 %
0,00 %
Computer
Smartphone
Tablet
Communication software
Messaging software
I don't know
None of the above
What kind of technology would you consider the most to use in order to
manage your disease?
18
8
27
4
8
16
0
54,55 %
24,24 %
81,82 %
12,12 %
24,24 %
48,48 %
0,00 %
Internet (websites)
Smartphone application
Tablet application
15
27
8
45,45 %
81,82 %
24,24 %
141
Telemedicine / remote monitoring device
Smartwatch
Smart band
Communication software
Messaging software
Smart tv
None of the above
What would convince you to use a health app regularly?
Provide accurate and trustworthy information
Be easy to use and simple / well designed
Work effectively and consistently over time
Allow you to provide support and help to other patients
Raise awareness about the disease and educate people
Allow you to rate or comment on a healthcare service you received
Provide guarantees of data security and integrity
Be free
Be less expensive than any other form of healthcare support
Have no advertisements
Other: i don't know
Do you use smartphone / tablet apps to manage your disease?
Yes
No
Which?
Phone, internet, tablet, don't know
Would you be interested in having a wearable device that tracks your
health status?
13
9
7
5
8
1
3
39,39 %
27,27 %
21,21 %
15,15 %
24,24 %
3,03 %
9,09 %
24
16
12
11
15
6
9
10
5
8
1
72,73 %
48,48 %
36,36 %
33,33 %
45,45 %
18,18 %
27,27 %
30,30 %
15,15 %
24,24 %
3,03 %
3
30
9,09 %
90,91 %
0,00 %
0,00 %
0 (not at all)
4
1
0
2 (moderately)
8
3
9
4 (extremely)
12
Do you wear a smartwatch or fitness tracker?
Yes
4
No but i'm interested
18
No and i'm not interested
10
What are the most important services a health app (or website) should offer you?
Offer general information about your disease
25
Advices and feedback about how to self-care in your daily life
20
Information about your current health status
17
Help to detect the early signs of crisis
25
Help to avoid the triggering of crisis
19
Help to have a healthy lifestyle
14
Assist when performing physical activities
10
Offer a permanent contact with healthcare providers
11
Access your medical record and allow to keep it up to date
9
12,12 %
0,00 %
24,24 %
27,27 %
36,36 %
142
12,12 %
54,55 %
30,30 %
75,76 %
60,61 %
51,52 %
75,76 %
57,58 %
42,42 %
30,30 %
33,33 %
27,27 %
Arrange and manage your medical appointments / check-ups / follow-up
8
Ease the access to medical assistance in case of emergency
14
Ease the access to medical assistance in everyday life
3
Offer an emotional / psychological support in case of crisis
3
Offer an emotional / psychological support outside crisis
4
Help to contact support groups in case of crisis
6
Help to contact support groups outside crisis
3
Provide information about your disease in case of emergency
10
Provide information that educates your friends / family / people you meet
5
What do you think of always carrying your important health information on you?
0 (not at all important)
0
1
0
2
1
3
6
4
8
5 (extremely important)
18
24,24 %
42,42 %
9,09 %
9,09 %
12,12 %
18,18 %
9,09 %
30,30 %
15,15 %
0,00 %
0,00 %
3,03 %
18,18 %
24,24 %
54,55 %
Table 14 - Extraction of the free-text answers: what is your biggest daily concerns?
Raw answers (french, english, german)
Grossesse
Ne jamais savoir qd une crise se déclenchera
Faire attention a tout
Mes douleurs de hanche
Mon traitement hydrea et mes douleurs chroniques
état de mon corps
La peur du lendemain ne pas savoir si je vais faire une crise ou pas
Prendre mes médicaments
Infection
Je vis dans un autre pays pour mes études et avoir une crise sans mes parents à mes côté
Boire
Reconnaître lorsque j'atteint mes limites et que je dois me reposer pour ne pas déclencher de crise
Savoir si j'aurai mal et si je vais arriver à gérer cette douleur
D'etre seule en cas de crise c pas evident
...rester en vie le lendemain matin, d'où la peur de dormir
Les crises, les hospitalisations
Douleur
Ne pas pouvoir m'occuper de mes enfants
Eviter la fatigue
Avoir une crise
When i'm i going to get a stroke or a bloodclot in the head
How can i avoid a crisis to trigger, know when could a crisis happen
That people don't receive the healthcare information they need for prevention of health issues.
I have come to terms with my scd.
My biggest concern is having a crisis and how to stop one before it gets to bad where i have to
hospitalized.
143
Severe pain and its management
Stroke, pregnancy and child birth
Getting sick
Tod
Aujourd'hui , ma plus grande préoccupation est l'évolution de mon foie et de mes poumons.
Table 15 - Free-text answers: What other features or services would you appreciate
Raw answers (french, english, german)
Application
Assistance morale, important
Application
Une application sur smartphone
Une application smartphone aidant à gérer la maladie au quotidien
Appli
Une application qui m'aide à gérer ma maladie
Un suivi de mon état de santé et de mes constantes (températures, oxygène, etc...)
Une
mise
à jour
en
fonction
de
mon
environnement
(altitude,
etc...)
Un résumé de mes antécédents médicaux en cas de crise en milieu inconnu pour une prise en
charge
rapide
et
efficace.
Un
contact
avec
un
professionnel
en
cas
d'urgence
un système d'alerte en cas de grosse crise qui entraîne une incapacité de se mouvoir.
Un système de prise en charge si, dans le cas précédent je ne peux pas ouvrir la porte, savoir que
tel
organisme
a
un
double
de
mes
clés,
par
exemple...
Un "coach" pour mettre en place des habitudes de vie saine (introduire le sport, car je n'en ai
jamais
fait
;
coaching
alimentaire,
etc...)
Une alerte au cas où je ne fait plus attention et que ma santé se déteriore (qui pourrait être en 3
temps : le 1er ça m'alerte moi, le 2ème je ne sais pas trop et le 3ème ça alerte ma mère! (ou un
proche))
Une application capable de me prévenir si une crise peut-etre anticipée et combien de temps elle
peut durer
Que la douleur soit prise en charge plus vite
Aucune idée maintenant
Application comme un carnet de santé a noter les crises les medicaments a prendre ou prises les
rendez vous les hospitalisations et en cas de crise en etant seule simplifier les prise en charge
sans perte de temps!
Applications pour smartphones et tablettes
Alerter le service des urgences de mon arrivée
Être suivi médicalement et psychologiquement en dehors des crises. Être alerté chaque jour de ma
santé.
Chatapp where you can comunicate with other sicklers about your worries and exchange
information
Social support. For instance from the closest person around in case of crisis, in case of need of
drugs, etc. A mapp allowing me to live as normally as possible.
A moble app
I would like to see a app where i can communicate with the doctor.
Not sure
Tips on increasing ones lifespan
144
Dont k
Wie ich selbständig werden könnte
D'une application ou d'un bracelet qui alerte l'évolution de l'état de ma santé
Table 16 - Free-text answers extraction: What should be improved in the healthcare system that takes care of you?
Raw answers (french, english, german)
La prévention en écoles lycées et s la tv spot tv ect
Prise en charge
Pas.d'idée
Mon opération de hanche
Un savoir plus approfondi de la maladie
Connaissance maladie / transmission des antécédents
Faire plus confiance aux patients qui eux connaisse leur maladie
Information
Plus réactif et plus informé sur la drepanocytose
La
prise
en
charge
:
être ballotée de service en service est très désagréable car on peut se retrouver n'importe où...
Hospitalisée en gastrologie ou en rhumatologie et être traitée par un docteur ou un (ou plusieurs)
interne(s) qui estime que "ce n'est rien de grave" et qu'il peut très bien "gérer ça"...
On a jamais vu un patient atteint du cancer se retrouver en gastrologie ou en médecine générale,
je
crois,
non
?
Et le fait de ne pas être en contact avec son médecin spécialiste durant l'hospitalisation
parcequ'on est dans un autre service... Ca aussi c'est dur. On se sent abandonné et seul.
Prévoir des aides financières pour aider à l'achat de médicaments, accessoires médicaux ou non
améliorant notre qualité de vie
Cela depend des guarde a l'hopital, il y en a ils en ont rien a foutre
Réduire la superpuissance des soignants qui savent et décident de tout sans la moindre
considération pour les patients et leurs proches. En sus, améliorer la communication entre
professionnels de santé dans la gestion de patients..
La prise en charge a l hopitale pour soulager la douleur eviter lattente beaucoup trop long avoir la
priorité
Le parcours des examens hors du chu ou du centre spécialisé
Prise en charge plus rapide aux urgences
Acces plus facile au medecin specialiste
Le suivi de la drepanocytose pour les adultes. Passés 25 ans si on est mal on a du mal à convaincre
les pompiers ou autres personnes ne connaissant pas la maladie. Il ya beaucoup de
drepanocytaires déprimés et tout le monde s'en fout. Sans ma femme je serais déjà plus de ce
monde. J'ai 37 ans déjà 2 embolies pulmonaires et des douleurs chroniques chaque jours
La prise en charge
The psychological aspect. We do have a lot on our mind but no help to receive!!!
More research and more education to caregivers (and patients). More resources and a better
recognition of the disease.
Healthcare provider education
I think everything run smoothly with the system.
More knowledgeable medical personnel
145
The variety on annual checkup tests completed and check up process that allows the doctor to
detect any issues with the patient can be improved.
Information about sickle c
Informationen
Cela dépend du système dans lequel vous êtes pris en charge. Etre suivi dans un système ou
hôpital universitaire vous soumet au suivi des différents médecins. Il s'avère que le suivi par un
médecin qui vous est connu, renforce la confiance et libère l'expression, facilite la discussion
Table 17 - Free-text answers extraction: What is particularly well in the healthcare system that takes care of you?
Raw answers (french, english, german)
Ald
La connaissance des médecins
Échanges sanguins
Bon suivi dans l'ensemble des crises ont pu être éviter!
Je sais pas
La
prise
en
charge
de
la
douleur.
Ca, ça a bien été intégré.
La pose du diagnostic et le suivi
Une bonne prise en charge mais pas a chaque fois
Le système de soins et la disponibilité des médecins spécialiste dans la prise en charge de la
drépanocytose.
Les medecins qui sont compétents ainsi que les infermiers
Prise en charge à 100% pour les médicaments
Bon suivi
Le suivi et le cout
Le suivi pour les enfants. Quand je l'étais j'avais beaucoup de crises et le personnel médical voulait
m'aider et était réactif. Étant adultes je dois lutter pour que mes maux soient reconnus et pris en
charges
La greffe de la moelle osseuse pour les patients drepanocytaires ayant un donneur compatible.
I really don't know. They do try though i guess
The effectiveness of treatment (relatively to the resources) and the willingness to listen to the
patient
My nurses
The time the nurse and doctor spend with the patient.
Fast response
Access to medication and blood transfusions when required.
Check
Nichts
Avoir des médecins de référence aujourd'hui est quelque chose de bien.
Table 18 - Free text answers extraction: Critic, comment or suggestions
Raw answers (french, english, german)
Courage
Oui
Questionnaire trop long
Très bonne idée hate d'essayer cette application
146
Bon
courage
pour
la
suite
et
j'espère
que
votre
projet
aboutira
s'il est gratuit je serais la première à l'utiliser rires
Parmi les questions sur les symptômes, certains symptômes m'étaient encore inconnus... Peut être
faudrait-il mettre en parenthèse une explications accessible aux personnes qui n'ont pas le
vocabulaire...
Concernant les diplômes, j'ai le baccalauréat, mais je ne connaît pas l'équivalence du diplôme pour
la suisse, j'ai donc choisi "maturité"...
1. Qui va remplir pour un illettré, un enfant ou une personne avec capacité limitée?
2. La question 56 devrait comporter le choix pour les deux car c'est hyper important à mon avis. Il
s'agit à la fois d'une volonté et d'un énorme besoin.
Je n'est pas de commentaire à ajouter
Oui
Bravo
Je l'ai déjà dit être adulte drepanocytaire est cause de dépressions dautomedications fortes et de
désengagement des personnel de santé in/hors crises.
I've been living in norway for 20 years and today for the first time i was able to hope again
because of a person i met and this project. If there is so much information out there about for
example diabetes, why not for sickle cell disease?
I am a public health doctoral student. I do not have sickle cell disease or trait but am planning to
conduct my research on sickle cell. Thank you for this research. I look forward to your
developments. All the best to you!
No other comments
Positiv überrascht
La connaissance de son organisme est un des éléments qui améliore la prise en charge aussi. Un
outil qui contribue à cette pratique est une avancé surtout pour les enfants qui n'ont pas encore
toute la conscience dur la maladie et ses aléas.
Appendix 4: Focus-groups notes and paper prototypes with notes
147
148
149
150
151
152
153
154
155
156
157
158
Appendix 5: Summary of the Focus groups
Focus group with patients: N°1
Date: 30.04.2015
Begin: 15h00
End: 20:45 (3 discussions of 1h30 has been planned with 15mn break)
Secretary / Moderator: Same person, DZ Issom
Patients: 4 patients
Location: Coffeehouse in town
Participants’ demographics: F=3; M=2; Age: 14 (left at 16h and only observed the discussions), 33,
53 (left at 17h), 23 and 27
Programme:
1) Project presentation with a PowerPoint
a. Can be downloaded from the following link: http://1drv.ms/1DhHgmr
2) Presentation of the attendees
a. In total 4 patients attended the meeting. 3 patients have been recruited from the
database of the Norwegian Sickle Cell Disease Association. Additionally, a member
of the family, the daughter of one patient, joined the group as observer but did not
participate actively. The fourth patient is also the researcher, study conductor,
interviewer and meeting leader.
3) Discussion of patients’ history: a preliminary discussion with patients in order to understand
them better, know their needs and problems, connect with them and better provide solutions
for what they are looking for
4) Discussion of patient’s main concerns and problems
5) Presentation of the objectives of the study
6) Presentation of the opportunities that could bring technologies
7) Discussion about the context of use of solutions wanted by patients
a. All the time
8) Discussion of what is necessary for the long-term and short term routine use of a self-help
tool
a. More education about the disease, tips
9) Presentation of the draft of the paper with the preliminary results
a. Patients Need and want some ICT solution to help them
b. Questionnaire:
10) Discussion of the preliminary results
a. Give advertisement to doctors
11) Discussion of patients main concerns
a. No specialists available and huge lacks of knowledgeable people
b. No people in general aware and educated about the disease
c. No specialists motivated to make efforts, learn and often not listening to patients
d. Doctors not taking patients seriously
e. No support groups - meeting – discussions from/with patients that can understand
f. Willing to get experience from other patients
g. Not everyone gets information nor feedback on the long-term and short term
consequences of the crises
h. Patients are tired to educate caregivers at every meeting, crisis, hospital visits
i. Lack of information about the consequences of taking the only evidence-based
medicine, hydroxycarbamide.
12) Discussion of patients’ main requirements
159
a. Patients want a system that provides accurate, evidence-based and trustworthy
information
b. Education for themselves and for caregivers
c. Discussions with educated patients groups / forums
d. Discussions with informed specialists
e. Discussions with advisers / educated nurses / psychology specialists
f. Setting videoconferences with remote specialists
g. Patients willing to pay a subscription for that
h. In ER settings
i. Raise awareness and education of doctor and nurses
ii. Inform about complications, history, medication taken and needed
iii. Have a health card like the French card
iv. Educate parents and paediatricians
i. Reach other patients
13) Discussion of half of the suggested prototypes (see Figure 2 and Figure 3) and questions
about what patients want specifically
a. Access to medical record
b. Information to help follow-up
c. Information about side-effects of normal treatments
d. Info about alternative medicine
e. Info about studies they could participate in
f. Get information about best practices and guidelines
g. Communicate among others patients
14) Homework for patients
a. Rank the prototypes they didn’t
Focus group with patients: N°2
Date: 05.05.2015
Begin: 15h30
End: 20:00 (3 discussions of 1h30 have been planned)
Secretary / Moderator: Same person, DZ Issom
Patients: 4 patients
Location: Older patients’ apartment
Participants’ demographics: F=2; M=2; Age: 33, 53, 23, 27
Programme:
1) Summarizing previous week meeting
2) Discussion of the SHI paper with a patient that left at 17h on the previous meeting
a. Confirming the findings and specifying concerns
3) Discussion of the second half of the prototypes
a. Discussion of the previous week findings
i. Rating and comments
b. Discussion of the second half
i. Rating, comments, suggestions
ii. Design of new functionalities
c. Discussion of the prototypes for the information app
d. Discussion of the ratings
4) Roundtable to generate new questions
5) Planning a new meeting for the day after
6) Homework for Moderator
a. Combine results and create / modify prototype
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Focus group with patients: N°3
Date: 06.05.2015
Begin: 17h00
End: 19:00 (1 discussions of around 1h30 has been planned)
Secretary / Moderator: Same person, DZ Issom
Patients: 3 patients
Location: Older patients’ apartment
Participants’ demographics: F=1; M=2; Age: 53, 23, 27
Programme:
1) Discussion of the modified prototypes and newly created prototype
2) Discussion of the information app for parents/friends
3) Patients want a specific solution to increase doctor awareness
4) Patients want a specific app for kids, parents and family
5) General discussion and greetings
6) Discussion about a video for MEDINFO poster presentation
7) Discussion about a version for low-income countries that is more accessible and simpler
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Appendix 6: Emergency summary template
EMERGENCY SUMMARY
SICKLE-CELL DISEASE
VASO-OCCLUSIVE CRISIS
The chronic patient is prone to have acute and life threatening complications.
A quick and priority admission in emergency followed by a pain assessment and
fast transfer in acute care unit is required!
Name: Write name here
Birthdate: Write birthdate here (n years old)
Nationality: Write nationality here
Languages spoken: Write the language you are able to communicate with
Condition: Sickle Cell Disease S/S
Blood Type: A+
Allergies: None
Usual Symptoms: Extreme, unbearable, acute and debilitating pains throughout the body, especially
in bones (chest, legs, arms but often everywhere), shortness of breath (suspicion of acute chest
syndrome)
Warning: the patient has difficult to access veins, use preferably the side of the arms.
Usual treatment:
-
Intravenous hydration (4 litres the 2 first hours, then 1 litre per hour)
-
Oxygen supplementation (10 litres)
-
Strong and aggressive analgesia (extreme pain, opiate tolerant patient (no addiction))
o
Fentanyl injections (50 ml) or / Hydromorphone (4-8mg IV).
o
If none available, start with very aggressive morphine IV doses (30-80mg).
o
Install a PCA pump (80 mg/h for morphine) or a sub cutaneous pump.
o
Give anti-inflammatory (first ibuprofen 1 g then diclofenac 75 mg)
o
Give paracetamol per IV (Perfalgan 1 g).
o
Assess the pain regularly and adjust the doses
o
Discharge with Palladon (1-4 mg), Paracetamol (1g), Diclofenac 75, Aspirin cardio
100
Put in place a respiration rate surveillance. The sedation being high, the patient usually stops
breathing and thus requires to be kept awaken by a nurse. The best practice is a quick transfer to
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intermediate intensive cares unit and transfer in normal units when the pain is manageable with lower
doses of analgesia.
-
Do blood tests for Hb level, RBC count, CRP, pH, arterial blood 02, C02 and creatinine.
-
Give a red blood cell transfusion as soon as possible (2 bags).
-
The patient needs to rest.
-
Fever might occur and the CRP usually increase until 200 mg/l (due to the pathophysiology of
the disease and rarely due to an infection). But always useful to screen for bacterial or viral
infection.
-
The CRP will decrease by itself after few days.
-
Plan another blood transfusion 1-2 days later. And one month later
Basal levels:
-
Hb (g/dl) is 9.5
-
MCV (u3): 80 fL
-
RBC count (pg): 30
-
B-Leucocytes (per mm3): 9.1
-
Bilirubin: (uMol/l): 44
-
Folic acid: chronic deficit
-
Magnesium: chronic deficit
-
D Vitamin: chronic deficit
Contraindications and recommendations:
-
DO NOT hyper hydrate
-
Oral or parenteral corticosteroids should be administered with particular care
-
Avoid use of vasoconstrictive medication
-
In case of general anaesthesia, ensure continuous intravenous hydration and plan for a
preoperative transfusion
Usual complications:
If morphine intake, strong hitching in the back, give treatment for that. History of acute chest
syndromes and frequent lungs infiltration. Perform a scanner and a chest radio. If infiltrated, give
diuretics and C-PAP. Watch for pulmonary and heart infarction. Treat the usual opioids related side
effects like constipation.
Who to contact
Next of Kin: +1 555 555
Significant other: + 1 777 777
Following doctor: Prof. Jack Dawson, +46 888 88 88 88 (Karolinska Solna, Stockholm)
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