Survey
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
* Your assessment is very important for improving the workof artificial intelligence, which forms the content of this project
Special needs dentistry wikipedia , lookup
Infection control wikipedia , lookup
Patient safety wikipedia , lookup
Race and health wikipedia , lookup
Health equity wikipedia , lookup
Public health genomics wikipedia , lookup
Rhetoric of health and medicine wikipedia , lookup
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? 61 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. 62 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. 63 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. 65 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 66 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. 67 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 68 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. 69 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. 70 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) 72 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) 73 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) 74 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) 75 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 77 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/ 79 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). 80 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 81 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: 82 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 83 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 84 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. 85 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. 86 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 87 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 88 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. 89 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. 90 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. 91 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 92 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. 93 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. 94 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 95 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. 96 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 97 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 98 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 99 - - 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 100 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 101 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 102 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 103 - 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. 104 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. 105 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 160 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 161 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 162 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) 163