Download inherited bleeing disorders

Community Oral Health Project
Exploring the Oral Health of
People with Haemophilia and
Investigation into the role of the
Dental Hygienist.
Diploma in Dental Hygiene
School of Dental Science
Faculty of Health Sciences
Trinity College, Dublin
Class of 2013
Diploma in Dental Hygiene
Trinity College Dublin
May 2013
i
Acknowledgements
Firstly, and most importantly, I would like to thank my supervisor Dr. Alison
Dougall, for taking the time out of her busy schedule to meet with me on several
occasions to discuss my project and to help steer me in the right direction. Thank
you Dr. Dougall for your patience and support throughout this project, it was
greatly appreciated.
Finally, I would like to thank my family and friends who have taken an interest in
this topic while listening to me discuss it over the past several weeks. Thank you
for your encouragement and support.
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Abstract
Individuals living with haemophilia can experience the same oral diseases as
those without. Dental treatment can be more complicated due to potential for
prolonged dental bleeding and also the reluctance of general dentists willing to
treat this group of patients. With such barriers in place people with haemophilia
are at increased risk of having worse oral health than the general population.
There is very little research on the oral health of individuals with haemophilia.
There are limited conclusions that can be made about the oral health of this group
of patients as the evidence that is available is of poor quality. In order to improve
the oral health of people with haemophilia a ‘shared-care approach’ must be
implemented. This will provide the necessary specialist care where required but
will also decrease unnecessary travel times to Haemophilia Centres, decrease
waiting lists and as a result will help improve the oral health of these individuals.
Education of both students and qualified health care workers is imperative in order
to reduce the feeling of fear when treating this group of patients. Caries and
periodontal disease are preventable oral diseases; therefore the patient must be
educated on the best preventable measures at home as well as preventable
treatment that can be given by the dentist and the dental hygienist, such
treatments will reduce the likelihood of invasive treatment and the requirement of
factor replacement therapy which also comes with its risks.
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Table of Contents
Declaration of Academic Integrity
ii
Acknowledgements
iii
Abstract
iv
Introduction
1
General characteristics of people with haemophilia
2
Oral health of people with haemophilia
3
Barriers to primary dental care for people with haemophilia
5
Solutions to Barriers
6
Model of Dental Service – Shared Care
7
Special High Risk Group
8
Dental Treatment Considerations for the Dental Hygienist
8
Local measures for the Dental Hygienist to control bleeding
11
Discussion and Conclusions
12
References
13
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Introduction
The types of inherited bleeding disorders that are going to be discussed
throughout this project include Haemophilia A, Haemophilia B and Von Wilebrand
disease, all of which come under the umbrella of Haemophilia. Haemophilia is an
X-linked disorder in which a clotting factor is greatly reduced or even absent
(Freedman et al., 2009). This condition is usually hereditary; however thirty
percent of cases are caused by spontaneous mutations with no family history
(Arruda and High, 2008).
Haemophilia A is a deficiency of factor VIII, which affects ninety percent of suffers.
Haemophilia B is a deficiency in factor IX. Both A and B will result in long-term
bleeding for the patient. The most common type of bleeding disorder is von
Willebrand disease, affecting up to one percent of the world’s population (Lillicrap
& James 2009). Von Willebrand disease is characterised by mucosal bleeding, this
disease affects both males and females equally. Individuals experience reduced
amounts of von Willebrand factor, or abnormal forms in circulation (Lillicrap &
James 2009).
People with haemophilia (PWH) will experience the same oral
diseases as those without. However, dental treatment for PWH may be more
complicated due to the risk of prolonged bleeding due to an inherent platelet
dysfunction. Throughout this project I will include a literature review on the general
characteristics of PWH, oral health of these individuals and the hygienist’s role in
treating these patients. I will also discuss barriers to primary dental care for people
with haemophilia (PWH) and possible solutions to these barriers.
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Literature Review
General Characteristics of People with Haemophilia:
The most severe form of haemophilia affects males only, the majority of the time.
While it is extremely rare, females can be seriously affected but only if their father
suffers from Haemophilia and their mother is a carrier. Women who are carriers
may suffer from symptoms of mild haemophilia (National Haemophilia Council).
“Haemophilia describes a group of inherited blood disorders in which there is a life
long defect in the clotting mechanism of the blood. PWH are at a risk of
haemorrhaging for longer periods of time, compared to individuals whose blood
factor levels are normal” (Irish Haemophilia Society).
Haemophilia can be classified into three different categories such as severe,
moderate and mild. Severe is when an individual has one percent or less factor,
moderate, between two and five percent factor, and mild between six and forty
percent factor.
Living with Haemophilia:
Haemophilia can be characterised by prolonged bleeding and at times,
spontaneous bleeding (especially into the joints in severe cases). Bleeding into the
joints and muscles is the most common problem for those individuals living with
haemophilia which, in time may lead to joint damage and loss of mobility if
bleeding occurs in the same joint repeatedly (Arruda and High 2008). Individuals
with mild haemophilia may experience spontaneous bleeding and the disorder
may not be diagnosed unless they suffer major trauma or till a time where they
require surgical intervention (Roberts et al., 2010).
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Oral Health of People with Haemophilia:
Several studies have shown oral health and oral health related quality of life is
poor in this group of patients when compared to the general population.
Dental Caries:
In 1982, the World Dental Federation and the World Health organisation jointly
advocated that certain goals in relation to dental health be achieved by the year
2000 (Carcao et al., 2010). These goals stated “that at least fifty percent of
children aged five to six years should be free of dental caries and on average,
children aged twelve years should have a dmft/DMFT (decayed, missing and filled
teeth because of caries, DMFT representing adult teeth) scores no more than
three. As well as these goals it was also stated that eighty five percent of the
population should still have all their teeth at the age of eighteen. In most high
income countries, these goals are being achieved in healthy children (Carcao et
al., 2010).
Petersten et al. (2005) noted “in Demark there was sixty to seventy percent of five
to twelve year olds free of dental caries”. It has also been shown that these targets
are also being achieved by healthy children and also boys with haemophilia in
other high income countries. Unfortunately, patients with von Willebrand disease
(VWD) are not meeting these targets as only thirty eight percent of the patients
were caries free and had a mean dmft score of 6.8 and DMFT score of 4.0. Sonbol
et al (2001) demonstrated, “that boys with haemophilia have better dental health
than normal healthy children”. It was also concluded that this was a result of
regular
dental
check-ups,
including
comprehensive,
preventative
dental
programmes given to children and parents with haemophilia from their
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haemophilia centres. Individuals with VWD have experienced dental disease
significantly higher than what is seen in patients with haemophilia or in the normal
paediatric population. According to Carcao et al (2010) it appears that patients
with type three VWD are at a greater predisposition to the development of
gingivitis and dental caries, leading to the requirement of dental extractions,
restorations and other procedures that places the patient at increased risk of
bleeding.
Periodontal Disease:
Patients with VWD have experienced significant mucosal bleeding in comparison
to PWH. Consequently when they brush their teeth they are at increased risk of
gingival bleeding which results in negative reinforcement brushing behaviour. This
leads to a vicious circle of persistently poor oral hygiene, and consequently
gingivitis and also risk of further gingival bleeding (Carcao et al., 2010). Ziebolz et
al (2011) carried out a case control study to investigate the effect of congenital
coagulation disorders (CCD) on oral health and periodontal bone loss on
individuals with CCD when compared to healthy individuals. It was concluded that
there was “no clinical relevant difference of oral health between patients with CCD
and controls despite better oral hygiene of patients with CCD”. It was also
observed that there was a significant difference in periodontal bone loss; however,
the observed difference was not clinically meaningful. There was no evidence that
oral health or the periodontal status in patients with CCD is worse when compared
to healthy subjects. However, Ziebolz et al (2011) suggested that they needed
larger studies and longitudinal studies to confirm their results.
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Barriers to primary dental care for people with haemophilia:
According to Kalsi et al. (2012) “PWH may face difficulty in accessing primary
dental care either due to disease specific or patient related barriers”. Access to
dental care for this group of patients may not only be about location or distance
away from the dental surgery. Kalsi et al. (2011) showed that twenty percent of
individuals with bleeding disorders in the UK have experienced being refused
treatment by a dentist. A study carried out between December 2010 and July 2011
included 105 anonymous patient surveys and 50 GDP surveys. The patient survey
demonstrated more patients were affected by patient-related barriers, than
disease specific barriers to access dental care. The GDP survey showed that just
under half of GDP’s were not confident in the dental management of PWH (Kalsi
et al., 2012).
Fear from both the patient and the dentist also contributes to the barriers for PWH
in accessing primary dental care. Avoidance of dental care from this group of
patients is common as well as neglect of oral care. Early intervention and ongoing
prevention is therefore imperative in order to avoid high risk and invasive
procedures (ID Hewson et al., 2011). A study carried by Hitchings (2012)
assessed whether PWH felt they attended a dental professional often enough and
if not why. The study consisted of 12 adults who had not done so in the previous
year and reasons were given throughout the study as to why they had not
attended. Twenty percent of the patients felt that a dentist would not treat them in
case they bled. Other reasons included extrinsic factors such as time and cost,
intrinsic values such fear, additional medical interventions, patients did not feel the
need, and risk of joint bleeds and finally unwilling dentist (Hitchings 2012).
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Solutions to Barriers:
Many of the barriers to care can be removed by facilitating care in general practice
settings for those patients with a mild haemophilia (Freedman et al., 2009).
“The cost of providing factor replacement therapy (FRP) is substantial. By
reducing the number of patients requiring FRP, more time and resources can be
allocated to patients with severe bleeding disorders while access to care for all is
improved” (Freedman et a.,l 2009). A protocol was designed at the National
Centre for Hereditary Coagulation Disorders, St. James’ Hospital Dublin, to reduce
the number of patients requiring factor replacement therapy. Since the introduction
of the protocol, the waiting time for dental treatment has been reduced
dramatically (Freedman et a.,l 2009).
Education of dental students regarding treatment of patients with special
healthcare needs (SHCN) is a subject of great importance, due impact on future
interactions they will have with these patients (Pani et al., 2012). A study was
carried out on Saudi Arabian dental students to assess their perception on the
adequacy of their education in special care dentistry. It also assessed the
willingness and confidence to carry out effective dental care to patients that
require SHCN. The survey demonstrated that most students were satisfied with
the theoretical training they received; however, the vast majority of the students
felt they lacked clinical experience to treat a patient with SHCN confidently.
Overall, when the willingness of the student was compared to their confidence, the
group of students showed to be more willing to treat individuals with SHCN than
they were confident of their ability to do so (Pani et al., 2012). This study suggests
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that the more exposure and education students have when training, the more
confident and less fear the students will feel in the future when treating patients
with SHCN. This in turn, helps to reduce barriers for PWH to attend primary dental
care.
Model of Dental Service – Shared Care
‘Shared care’ is essential in order to treat PWH. Brewer et al (2003) states “there
is a need to simplify the process and identify what can be safely carried out on a
shared care basis in General Dental Practice or Community Dental Service”. A
document formulated by Brewer et al (2003) proposes guidance for both doctors
and dentists to help establish what patients require comprehensive dental
treatment in a hospital and those patients that can have some of their treatment
carried out safely in a primary care setting by a general dental practitioner. It has
been suggested that people with severe haemophilia have all of their dental
treatment except for prosthetics such as dentures, carried out in a hospital
environment or in a General Dental Practice or Community Dental Service, but
only if special arrangements had been made with the Haemophilia Centre.
Moderate suffers of haemophilia should be treated in the same manner as severe
patients. Individuals with mild haemophilia may have their dental treatment carried
out in a General Dental Practice or Community Dental Service. However, Brewer
et al (2003) suggests that it is very important that the patient with mild haemophilia
is seen at two year intervals by a specialist team of the Haemophilia Centre to
ensure that they are maintaining their dental health.
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Special High Risk Group:
Patients with severe haemophilia and von Willebrands Disease (VWD) – type
three, are considered to be at increased risk and also increased exposure to
systemic treatment. Therefore these patients are considered to be high priority
patients (Hewson et al., 2011).
One major issue is the development of inhibitors. Both, patients with severe
haemophilia and type three VWD who require multiple transfusions are at risk of
developing inhibitory antibodies to factor replacement therapy (Carcao et al.,
2010). It is important that prevention is optimised, dental treatment is minimised
and management regimes are in place for all patients with haemophilia in order to
reduce the impact of their oral health on their medical condition, but even more so
in patients with inhibitors to clotting factors.
Dental Treatment Considerations for the Dental Hygienist:
It is the responsibility of the Dental Hygienist as well as other members of the
dental team to develop an understanding of the nature of the condition, how
severe the condition is and also the patient’s previous response to treatment in
order to carry out an effective and safe treatment plan for the patient (Hewson et
al., 2011).
Prevention:
Both dental caries and periodontal disease are two oral diseases in which the
Dental Hygienist can play a significant role in the prevention and in turn prevent
the need of a clotting factor (Brewer et al., 2003). Oral health promotion is the
responsibility of the dental hygienist; this helps to educate PWH the best oral care
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to prevent the occurrence of oral diseases. Instruction such as brushing the teeth
and supporting tissues twice daily using a fluoride tooth paste, dietary advice
including limiting sugar intake to meal times should be given to this entire group of
patients (Brewer et al., 2003). Appropriate dental care is important for all types of
patients; however, dental care is of significant importance for those individuals
living with haemophilia as it affects the general health of these patients
(Mohammad et al., 2009).
Periodontal Treatment:
The dental hygienist also plays a significant role in the maintenance of periodontal
health, it is imperative that adequate home care is instructed to the patient as well
as regular prophylaxis visits of up to six months in order to prevent bleeding and
tooth loss (Brewer et al., 2003). Periodontal treatment may need to be carried out
over a number of visits in order to prevent excessive bleeding. Chlorhexidine
gluconate mouthrinse may also be used as an adjunct therapy to help with initial
inflammation and is required antibiotics may also be prescribed by the dentist
(Brewer et a.,l 2003). The protocol designed for the management of patients with
hereditary coagulation disorders recommended that an antimicrobial mouthwash
to be used for all treatments to reduced the likelihood of post operative infection
(Freedman et al. 2009). “Post procedure dental pain can normally be controlled
with a minor analgesic such paracetamol, possibly in conjunction with codine”
(Hewson et al., 2011).
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Treatment of the elderly patient:
In the past, the average life expectancy for PWH was forty years of age (Jones
2000). The life expectancy for PWH is much greater now; this is the first
generation of individuals to live into old age. For this reason the dental hygienist
will also be involved in the management of treating oral conditions associated with
the elderly such as Xerostomia, angular chelitis, denture stomatitis and root caries.
These oral conditions may also lead to inadequate vitamin intake, which in turn
may cause atrophy of the oral mucosa and other problems throughout the body.
Medical issues associated with elderly such as cardiovascular conditions, diabetes
mellitus, arthritis, cancer, osteoporosis, dementia and depression may also have
further implications on the treatment a dental hygienist will carryout for this group
of patients. The dental hygienist is a useful and valuable member of the health
promotion team. Educating these patients on the common oral risks linked to
smoking, cardiovascular conditions and diabetes mellitus is imperative
(Holm-Pedersen and Loe, 1996).
Local anaesthesia and pain control:
Hewson et al., (2011) advises that the use of local anaesthetic for this group of
patients must always be discussed with the Haemophilia Centre. Oral injections
with local anaesthetic show varying degrees of risks for PWH. Typically,
infiltrations can be given without systemic haemostatic cover. A local anaesthesia
that contains a vasoconstrictor is advised as it will provide additional local
haemostasis. In individuals with severe haemophilia, the use of regional nerve
blocks, lingual infiltrations and floor of the mouth injections should be discussed
with the haematologist. “In the past, bleeding following deep nerve injections has
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been implicated in airway obstruction due to haematoma formation in the
retromolar or pterygoid space; therefore inferior alveolar nerve blocks have been
associated with a greater risk”. However, due to modern fine-gauge single use
needles the risk of a haematoma is now considered to be lower (Hewson et a.,l
2011). Where possible, a long-acting local anaesthesia such as bupivacaine
hydrochloride should be used as long lasting local anaesthetics can provide the
patient with up to eight to ten hours of effective pain relief. Patients must be
warned against lip, cheek or tongue biting while numb to reduce the risk of selftrauma (Hewson et al., 2011).
Local measures for the Dental Hygienist to control bleeding:
Routine dental procedures such as scaling may often cause soft tissue trauma,
resulting in localised haemorrhage (Franchini et al., 2005). “In PWH this may result
in uncontrollable bleeding and therefore may require prophylactic measures preoperatively, special caution peri-operatively and special management postoperatively (Brewer et al., 2006). Local pressure to the area of interest with gauze
may help to control bleeding; if this method is not successful, the use of
tranexamic acid may be introduced. Tranexaminc acid (TA) which is a synthetic
amino acid used in a mouthwash formation has also been used to control oral
bleeding (Lee et al., 2005). Lee et al (2005) carried out a study including thirteen
patients undergoing gingival scaling to assess the effectiveness of tranexamic acid
against factor replacement therapy. The study concluded that “the use of
tranexamic acid after dental scaling is well tolerated by patients and is as effective
as using factor replacement therapy before dental scaling in controlling gingival
bleeding”. By taking this approach, it will also avoid the need for factor
replacement therapy.
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Discussion and Conclusions
It is evident that most routine dental procedures for this special needs group can
be carried out, and safely managed in a general dental setting following
consultation with the patient’s haematologist and adherence to simple protocols.
‘Shared care’ is a concept that when used, will help in the management of the oral
health of this group of patients. Recognising misconceptions and barriers in
accessing primary dental care will enable further development of the shared-care
approach between general dental services, hospital or community dental services
and the haemophilia centre, optimising regular preventative advice and follow ups
to prevent dental disease and invasive dental treatment requiring haemostatic
treatment.
It is evident that education of the healthcare care workers as well as the patient is
highly important, including early intervention and prevention to make the most
positive changes in the oral health of a PWH. With a team approach amongst
health care workers, the patient and where children are involved, parents and
guardians can help in improving oral health of those PWH. Pani et al (2012) shows
that the more exposure students get in treating patients with special healthcare
needs the more comfortable these students become in treating these patients, and
this is important as these students are the future of dentistry and the more
confident these students become, results in less barriers for patients that require
special healthcare needs to access dental care. There is little research on the oral
health of individuals with haemophilia. There are limited conclusions that can be
made about the oral health of this group of patients as the evidence that is
available is of poor quality, which suggests there is plenty of scope for further
research.
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