MEDITERRANEAN JOURNAL OF HEMATOLOGY AND INFECTIOUS DISEASES www.mjhid.org ISSN 2035-3006 Review Articles Prophylaxis of Malaria The Center for Geographic Medicine and Tropical Diseases, The Chaim Sheba Medical Center, Tel Hashomer 52621, Israel Correspondence to: Prof. Eli Schwartz MD, DTMH. The Center for Geographic Medicine and Tropical Diseases, The Chaim Sheba Medical Center, Tel Hashomer 52621, Israel. Tel: 972-3-5308456; Fax: 972-3-5308456. E-mail: email@example.com
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Guideline for oral healthcare of adults with Huntington's disease Graham Manley1, Helen Lane1, Annette Carlsson2, Bitte Ahlborg2, Åsa Mårtensson2, Monica B Nilsson2, Sheila A Simpson3,4 & Daniela Rae*3,4; On behalf of the contributing members of the European Huntington's Disease Networks Standards of Care Dental Care Group A preventive dentistry regime should be implemented at the earliest possible opportunity and maintained throughout development of the condition. The use of high fluoride toothpaste is essential.
Discuss with dieticians their recommendations for use of highly cariogenic foods. Work closely with all professional and nonprofessional groups concerned with care of the person with Huntington's disease.
In the early stage of onset of the condition, consider carefully before providing advanced conservative treatment (i.e., crowns, bridges, implants). In the later stages, maintenance of complex restorative dentistry may present real problems for dentists and caregivers. This may be a difficult issue for patients and/or caregivers to accept will depend upon each individual clinical situation.
The use of intravenous conscious sedation is an extremely valuable tool for dentists when treating people in the mid and late stages of the condition. It may avoid complicated general anesthetic hospital admissions. In the case of a recurrent lip trauma from choreic mouth movements discuss with caregivers frequency and severity of event and options for prevention, using a mouth guard (if appropriate and safe) or extractions if necessary.
As a dentist with experience of treating people with Huntington's disease you must consider it an essential responsibility to become involved in training other colleagues.
Always assume (unless told or from experience) that the patient with Huntington's disease understands and can communicate. Respect his/her wishes even if they do not comply with what you as a dentist considers to be in the interest of good oral health. Summary These guidelines present an overall strategy for oral healthcare based on
the principles of achieving a disease-free, pain-free and safe mouth. The standards of care
referred to in the document seek to provide guidelines for the care and treatment that is no
less a standard provided for an individual that does not have this condition. Such care and
treatment will take into account the health and safety of each individual within the context
of their condition. Particular emphasis is placed on dental professionals working within a
multidisciplinary team, focusing on prevention of oral disease and providing treatment
appropriate to the various stages of the progression of this condition. It is intended that by
providing and subsequently promoting these guidelines, it will focus the dental professions
work on this condition and the implementation of good care for people with Huntington's
1The Royal Hospital for Neuro-disability, London, UK 2 Mun-H-Center, Goteborg, Sweden 3 Department of Clinical Genetics, NHS Grampian, Aberdeen, Scotland, UK 4University of Aberdeen, Aberdeen, AB25 2ZA Scotland, UK *Author for correspondence: Tel.: +44 1224 552 120; firstname.lastname@example.org 10.2217/NMT.11.68 2012 Future Medicine Ltd Neurodegen. Dis. Manage. (2012) 2(1), 1–xxx
SPecial rePort Manley, Lane, Carlsson et al.
Huntington's disease (HD) is an adult-onset, disease. It is clear that much work must be
familial disorder that can affect both men undertaken in order to clarify some of the
and women . Symptom onset usually occurs aspects of dental management.
by the age of 40 years, but this can be very
variable. Age of onset is related to the size of Methods
the mutation, which is an unstable expansion A systematic literature search was performed
of a CAG sequence in the gene . The classical electronically using Embase, Ovid MEDLINE
triad of clinical features includes a movement and Ovid MEDLINE® In-Process & Other
disorder, cognitive impairment, personality Non-Indexed Citations.
and psychiatric disorder. These features
There is very limited literature about the cause complex management problems, which oral health of people with HD. The majority of adversely affects oral health. For example, the literature is confined to individual patient case provision of mouth care and dental care may reports and their management strategies. Few of be physically limited by the movement disorder the papers identified proved relevant and none and furthermore oral hygiene may be neglected were above evidence grade IV (expert opinion). or declined. Described by George Huntington in Interestingly, a review of the literature 1873, this disease continues to devastate families focusing on multidisciplinary working in HD and perplex the clinicians who care for them.
found only one paper that included dentistry in When working with people with HD and the multidisciplinary team . A further paper their families it is important to provide a states that ‘‘ideally a dentist should be part of cohesive and multidisciplinary service. The the multidisciplinary care team with progressive disease affects many different aspects of the neurogenic disorder from diagnosis, so that oral individual's abilities and they require assessment care can be planned throughout the disease process by appropriately trained healthcare professionals. and does not become crisis management in the No one professional will have all the skills final phase of the condition'' . A chapter within a needed to help any one individual. It is therefore multidisciplinary publication considers oral health of importance that the service providers take a care . This presents a literature review rather multidisciplinary approach to HD in order to than discussing the issues involved in including identify the best way to assist individual patients this aspect of care within multidisciplinary work. by taking into account their differing needs.
The provision of oral healthcare for people Huntington's disease & oral health
with HD is dependent on the dental team There are no innate dental features of HD that working together with various agencies that make an individual more susceptible to dental provide care . Interdisciplinary working caries or periodontal disease . However, a study is essential to the effective management of of the oral health of a group of individuals with people with HD. Team members will include mid-late stage HD in a specialized residential care general medical practitioners, neurologists, facility showed that this group of individuals had psychiatrists, neuropsychologists, specialist significantly more missing and decayed teeth, nurses, genetic counselors, social workers, care fewer sound and restored teeth and fewer pairs of managers, palliative care consultants, dieticians, contacting posterior teeth when compared with physiotherapists, occupational therapists and the general population .
speech and language therapists.
There are several contributory factors that The European Huntington's Disease Network may detrimentally affect the oral health of (EHDN) was formed in 2003, to provide a people with HD. These are:platform for clinicians and families to work Nutritional factors together to find a cure for Huntington's disease. In addition, working groups were created to Percutaneous endoscopic gastrostomy (PEG) address various themes; one of which is the Standards of Care (SoC). Within the Standards of Care Group, as part of the EHDN  the dental section has proposed these guidelines Access to and provision of dental care based on the currently available evidence as well as expert consensus on the provision of oral Additional dental considerations health care for individuals with Huntington's Neurodegen. Dis. Manage. (2012) 2(1)
future science group Guideline for oral healthcare of adults with Huntington's disease SPecial rePort
The 1945–1953 Vipeholm study is one of In the later stages of HD, a decision may be the largest and most influential single studies made to feed via PEG. The decision to insert a investigating the association between sugar PEG may be dependent on a number of factors consumption and dental caries . It is notable including:that such a study which concluded results that An advance directive regarding PEG insertion have proved essential to the prevention of dental caries involved manipulation and ‘use' of adults Dysphagia, which poses a significant risk of with learning disability in a residential institute. aspiration pneumonia It concluded that consumption of sugary food Dysphagia, which significantly limits the and drinks both between meals and at meals is associated with a large caries increment. For ethical reasons, this study has never been It should additionally be recognized that a repeated but the conclusions have been ratified person with a PEG may also consume some oral by more recent national reports [10–12].
foodstuffs providing it is not an aspiration risk.
Several dietary factors are associated with There is very little literature about the caries incidence: oral health of people fed via PEG. Historical Amount of fermentable carbohydrate experimental research on animals helped to demonstrated that ‘tube-fed' animals experienced less dental caries . This view has Sugar concentration of food been upheld by recent research that has shown Physical form of carbohydrate that ‘tube feeding had a remarkably lower odds ratio for new dental caries relative to regular Oral retentiveness (length of time teeth are meals' .
exposed to decreased plaque pH) Several papers have reported a higher rate Frequency of eating meals and snacks of plaque and calculus accumulation in people fed via PEG , but there have been no papers Length of interval between eating published that have specifically investigated Sequence of food consumption periodontal disease in this group. However, it is widely known that gingivitis is a reversible People with HD are also known to retain inflammatory reaction of the gingiva to plaque food within the mouth after swallowing [13,14], accumulation, and that gingivitis precedes which will also increase the likelihood of dental periodontitis. Not all gingivitis cases will caries. It is thought that the bolus retention is develop into periodontitis- approximately 10% predominately due to dysphagia, but may also of individuals are highly susceptible and 10% are be due to deficient purposeful lingual searching highly resistant to periodontitis. The difference activity . Thickened fluids can also be difficult in susceptibility is for the most part attributed to clear from the mouth  which increases the to genetic factors . caries risk.
The presence of dental plaque is the principle In order to maintain and/or increase the causative factor in the etiology of periodontal calorific intake of a person with HD, many disease. It could therefore be suggested that
dietitians recommend eating frequent snacks this group of individuals who have shown a
high in calories and to consume lots of sugar or higher reported rate of plaque and calculus
sugary substances, such as honey, jam, syrup accumulation will show a higher prevalence
and treacle [102,103]. It is widely accepted that of periodontal disease. It is clear that this is an
a high frequency of consumption of sugary area that deserves more research, particularly
foods and drinks increases the likelihood to clarify the exact prevalence of periodontal
of dental caries. However, it is of concern – disease in those people who are fed via PEG.
for the consequences of this strategy on oral
health – that people with HD are encouraged Medication
to consume a diet high in sugar. Foods that are An assessment by the EHDN found that
high in sugar often contain ‘empty calories', and approximately 84% of HD registered patients
there is an association in people with HD with receive symptomatic treatment . The
nutritional deficiency .
indications for prescribed medications were: future science group SPecial rePort Manley, Lane, Carlsson et al.
Depression, 50% in a specialized residential care facility found that these individuals had more plaque than the age-matched general population . All of these Irritability and aggression, 13% individuals had gingival inflammation. Some of Sleep disturbance, 9% these individuals provided their own mouth care, and some required assistance with mouth care.
Rigidity, apathy, mania, obsessive–compulsive Self-provided oral hygiene may be poor in this disorders, less than 2% The drugs prescribed varied depending Apathy and poor self care  – toothbrushing upon the country; in the UK, citalopram, may not be a priority and their interest in hygiene tetrabenazine, olanzapine and clonazepam diminishes as the disease progresses [7,21,27]; are frequently prescribed. Citalopram is the single most prescribed antidepressant (41%), Impairment of voluntary motor function  and olanzapine was the neuroleptic chosen in and impaired manual dexterity . 55% of cases in the UK. A common side-effect Toothbrushing may be difficult and of these two medications is xerostomia [22,23]. inadequately performed due to movements and Xerostomia has also been a reported as a side- effect of tetrabenazine . Xerostomia presents As the disease progresses people with HD a significant problem in maintaining good oral become fully dependant on carers for activities health. Its presence is associated with a high of daily living, which includes oral care. Several prevalence and incidence of caries in several papers have elucidated barriers to the provision of papers, and has additionally been associated with mouth care provided by a third party including:tooth loss and periodontitis .
Lack of training [28,29] A study of the oral health of a group of individuals with mid-late stage HD in a Time constraints [28,30,31] specialized residential care facility found that the Lack of knowledge about oral care/disease majority (33/35) of individuals were prescribed one or more medications with the potential to cause xerostomia as an undesirable side effect Lack of oral care supplies  The average number of medications prescribed Uncooperative residents/patients [30,31] which can cause xerostomia as a side effect was Lack of staff  3.89, ranging from none to seven per patient. This study also noted further medications Need for/lack of an oral assessment tool [29,32] prescribed to these individuals with the potential Low priority duty [29,31] to cause undesirable oral side effects. Gingival enlargement is a known side effect of sodium Unrewarding/unpleasant task [29,31] valproate and sertraline. Glossitis is an oral Fear of causing pain  side effect of olanzipine, diazepam, sertraline Use of ineffective mouth care equipment, for venlafaxine and temazepam.
example, foam swabs instead of toothbrush While it is appreciated that such medications may be important in the management of HD These barriers may lead to the inadequate their implications for oral health present yet provision of mouth care and subsequently poor another challenge for preventing dental disease oral health. Although registered nurses (RN) and maintaining good oral healthcare. It is a often receive some mouth care training, RNs continuing concern to the dental profession were unlikely to provide mouth care. Mouth care that liquid medicines may contain cariogenic is often delegated to healthcare assistants or their sweeteners. For some people with HD the oral equivalent . It is therefore possible that mouth intake of a variety of liquid medications on a care is performed by providers with receive no frequent daily basis, if sugar containing, is another formal training in this field. detrimental factor in preserving the dentition.
access to & provision of dental care
There are many well recognized barriers of A study of the oral health of a group of access to dental care for people with disabilities, individuals with mid-late stage HD with teeth such as: Neurodegen. Dis. Manage. (2012) 2(1)
future science group Guideline for oral healthcare of adults with Huntington's disease SPecial rePort
Access to the dental surgery – such as the the successful use of general anesthesia and availability of transport to and from the conscious sedation to provide dental treatment building , wheelchair access  for patients with HD who are unable to accept Financial constraints treatment under local anesthesia. Several anesthetic techniques have been described in Dentist's lack of experience treating people the literature including the uneventful use of with disability  isoflurane , succinylcholine , mivacurium Caregivers' priorities for care  [37,38], forane , nitrous oxide , sevoflurane [38,39] and propofol [39,40]. General anesthesia People with HD may experience additional has been used for a range of dental treatment: barriers to the receipt of dental care: full mouth extractions, restorations, implant Communication difficulties  insertion and endodontics [7,27,37,39,40]. In one case, dental treatment was completed over five Lack of dental professional's knowledge about continuous hours of anesthesia .
HD and consequential reluctance to provide Substantial consideration must be taken before general anesthesia is contemplated for HD Difficulties providing treatment to patients affected patients. It is more likely that treatment with overt choreic movements [4,27,36] under general anesthesia is necessary in the later stages of the disease when there is a higher risk Apathy and resentment in the patient and/or of complication. Dysphagia increases the risk of family . Following diagnosis, people with aspiration in the immediate postoperative period HD may lose self-interest and may choose not  and these patients are often malnourished [27,41] to attend dental appointments or look after and dehydrated . Certain anesthetic agents their oral health. An individual with HD may are avoided, anticholinergic drugs may increase resent family members who may or may not choreic movements , and sodium thiopental have HD and complex psychological and has been reported to cause prolonged apnea. behavioral issues can lead to the subsequent Dental treatment under conscious sedation breakdown of family units. Conversely, family with intravenous midazolam has been recently members may be the prime carers, but may used as an alternative to general anesthesia. It also become apathetic towards or resent the can be used effectively often in small, titrated individual with HD. The family carer may not doses, to control movements and allow effective want to or be able to provide the mouth care dentistry [4,7,42,43]. It has been suggested required for that individual, or assist them to that conscious sedation can help to manage their dental appointments as would be swallowing problems and reduce the risk of necessary to help maintain good oral health. aspiration . Cannulation may be difficult An additional reason for family resentment in and may preclude a patient from receiving relation to dental care may be because of the conscious sedation if movements are so great inability of the dental treatment proposed to that intravenous access cannot be secured . meet the expectations of the patient and/or In such cases the use of oral or transmucosal caregivers. For example, difficulty or (particularly intranasal) sedation may facilitate impossibility in providing advanced restorative cannulation [42,43]. However, if sedation is not care (crowns, bridges, implants).
appropriate effective general anesthesia with a Ability to tolerate dental treatment due to gaseous induction may be the only treatment involuntary movements [4,36].
option. One paper has described the use of diazepam administered through a patient's Regardless of these barriers, it is entirely gastrostomy tube to facilitate dentistry .
feasible to provide a wide range of dental care to
people with HD. Bradford et al.  performed additional dental considerations
complex endodontics under local anesthetic Trauma
alone: it was noted that the choreic movements People with HD often display a disturbance in
prevented the full use of the dental operating gait, can lose balance and are more susceptible
microscope and the length of appointments were to falls . There has been one reported case of
limited, however treatment was successful. There a fractured mandibular condyle following a fall
have been several papers that have discussed where initial impact was the chin . It should
future science group SPecial rePort Manley, Lane, Carlsson et al.
be noted that this group are prone to trips and patient. A more pragmatic approach to treatment falls, which can result in facial injuries.
may well be in the interests of the patient albeit not necessarily meeting patient and or relatives' Dentures become increasingly difficult to retain as the disease progresses due to xerostomia and the stages of HD
reduced muscular control [7,27,46]. It is therefore There have been several attempts to define important to retain functional teeth for as long the stages of progression of HD . Current as possible. Should a denture be considered a research may provide further options :careful risk benefit assessment must be made Early-stage disease cannot be defined as the taking into account the possible instability and time of diagnosis since diagnosis can be made likelihood of airway obstruction. at variable times. Most individuals present with early neurological and psychiatric features, which may have started to cause One paper reported three cases of severe bruxism difficulties at work and home. which increased in severity alongside the progression of their HD. The bruxism resulted Mid-stage disease would be seen to have been in severe tooth wear and jaw pain . Others reached when the affected person has to cease have also reported on its occurrence .
work, or at least change employment to a post that is less challenging. Executive function a guideline for care
decline and some cognitive deficit would be The overall strategy for oral healthcare is based demonstrated. Involuntary movements would on the principle of achieving a disease-free, pain- be obvious but it is most likely that individuals free and safe mouth. We seek to provide care would still be able to feed themselves.
and treatment that is no less a standard than Late-stage disease occurs when employment that provided to an individual that does not have becomes impossible, the individual is no HD. Such care and treatment should take into longer able to live independently, self care account the health and safety of each individual ceases and cognitive decline is obvious. Some within the context of their status.
patients maintain the ability to feed themselves Huntington's disease provides particular to a late stage, but swallowing difficulties and challenges for oral healthcare. As with all involuntary movements create increasing aspects of healthcare, prevention should be introduced before the onset of the condition where possible. Some adults with the condition It is recognized that HD is a continuum of choose not to be formally diagnosed, and the varying clinical features. To interpret into lack of early identification of affected individuals practical care and relate to the needs of the may not allow for the instigation of early oral individual it is suggested that for the purpose health prevention. In addition, the provision of of these guidelines the condition of HD is dental treatment may be difficult because of the described by stages. Different authorities divide clinical features of HD – particularly the choreic HD by three or five stages.
movements. Such treatment difficulties make the need for prevention even more significant. relation to oral healthcare
For these reasons the importance of assessment It is important to work with the family and carers and practical treatment planning cannot be as well as the affected individual and to provide overemphasized. as much choice as possible for care to each When planning and carrying out restorative individual. Much of the care suggested will relate care for people in the early stages careful to the dependence on others for support to the attention however should be given to the fact person with HD. For this reason the guidelines that the effective provision of oral hygiene and will relate to the degree of dependence on care therefore maintenance of advanced restorations, and services of that individual throughout the that is, crowns and bridges, may not be possible progression of their condition. Thus at the for those who will move into the advanced stages early stage the individual will be minimally of the condition. Such issues may provide more dependent on others for support, he/she will be difficulties for the dental practitioner and for the able to carry out good and effective oral hygiene Neurodegen. Dis. Manage. (2012) 2(1)
future science group Guideline for oral healthcare of adults with Huntington's disease SPecial rePort
and will accept all dental procedures with local assessment
anesthetic within primary care. At the middle It is important that essential information about
stage, dependency will increase. The individual oral healthcare is available as soon as possible
may still be able to carry out oral hygiene but from non-dental agencies (medical, nursing,
will need occasional support (occasional physical social care staff). An oral health assessment
help) for this process. Oral examination and must also be carried out by a dental practitioner
treatment will be accepted using local anesthesia specializing in the care of adults with disability,
in the conventional manner; however, some more preferably experienced in the dental care and
complex procedures may need to be carried out management of people with HD. In the early
using sedation or general anesthesia. At the third stages of this condition patient involvement
stage, dependence will be at the highest level. in treatment planning and long term care is
Effective oral hygiene cannot be maintained essential. This should include a discussion about
by the individual alone who must have regular the patient's expectations for their oral health
and effective daily physical support with this and treatment. Individuals with HD may be
task. Oral examination is difficult and only the encouraged to make advance decisions as is
simplest treatment can be carried out with local the case with other areas of their life . This
anesthetic. The use of conscious sedation or would then facilitate long-term planning of oral
general anesthesia would be the most appropriate care between the individual and dental surgeon.
option for treatment if suitable.
oral care regime
Provision of treatment should be appropriate preventive care
to each individual case. This may include Much of the literature about HD and oral restorative care with local anesthetic, conscious health discusses the importance of prevention. sedation and/or general anesthetic. Careful Boyle et al. make the point that at-risk families consideration must be given before providing should be educated about the implications for advanced restorative care as this may well be oral health and encouraged to seek out regular detrimental to the long-term patient care. care . At-risk families and in particular newly Case studies form the bulk of literature diagnosed individuals should be referred at an written about HD and oral health. Although early stage to a dental provider experienced complex dentistry, including endodontics and in the condition. A thorough, preventative implantology, has been described, long-term regime can then be implemented to reduce follow-up of these complex treatments have the risk of oral diseases and their sequelae as not been assessed nor how the oral health of the disease progresses. Good oral health has these individuals is maintained as the disease positive benefits for health, dignity and self- progresses. In view of the fact that implants are esteem, social integration and general nutrition becoming more a part of the provision of dental . While people with HD are generally care, careful consideration should be given to encouraged to eat a highly cariogenic diet and their advantages and disadvantages. Ideally take medications which cause xerostomia to assessment should be provided by a specialist control or reduce their symptoms, the oral implantologist. A more pragmatic approach is health of people with HD is at risk. Without to provide a more easily maintained dentition multidisciplinary and interdisciplinary team with good sound restorations following the working that includes a dental input, this onset of advanced HD. The provision of risk is unlikely to change. The need for the removable prostheses may be appropriate implementation of an oral health program as in the early stages, however if provided it is early as possible cannot be over-emphasized. essential that regular assessment be made of This should include appropriate preventive the potential risk that a denture may provide measures with close support from a hygienist. to airway obstruction. As such, all removal Sound guidance on good oral care is appliances should be clearly marked with additionally available from the HD Association radiopaque inserts.
. To further emphasize its importance a The maintenance of good oral healthcare is practical useful document is available online very important for such patients and therefore (www.futuremedicine.com/doi/suppl/10.2217/ regular and frequent oral assessments by an experienced practitioner are recommended.
future science group SPecial rePort Manley, Lane, Carlsson et al.
Introduction of oral hygiene aids, for example, early stage/phase 0–2
Collis-curve toothbrush, Dr Barman's Superbrush, enlarged and/or weighted Initial thorough examination (including handles/grips, wrist weight cuffs, mouth angle radiographs and photography as appropriate) to obtain a baseline for future planning and Oral hygiene instruction for carers using to help with patient motivation. Review on a written instructions and/or pictures and other regular 6-monthly basis or as required aids (e.g., DVDs) Oral hygiene instruction with the use of Consider introduction of a saliva substitute adjunctive oral hygiene aids where appropriate Individual assessment for the use of the most Restorative treatment appropriate form of fluoride, for example, high Delivery of treatment becomes more f luoride toothpaste, chlorhexadine and fluoride toothpaste , fluoride mouthwash If caries is active consider a shortened dental Preventative advice to improve understanding arch to provide a more easily maintained, of the significance of maintaining good oral functional dentition health and the importance of smoking cessation Treatment in a semi-reclined position with the Dietary advice to limit exposure to sugary use of support cushions help to reduce foods and snacks. This should involve liaison involuntary movements and relax body with dieticians (see Dietician's Guidelines) Discussion of long-term care needs/treatment Treatment becomes more likely to require the needs (future wishes) use of conscious sedation techniques including oral/transmucosal sedation, intravenous Restorative treatment sedation, or general anesthesia for delivery of care Restorative treatment should be high quality and low maintenance Frequent re-assessment of oral care plan, as per Maintenance of existing dentition by sound restorations, endodontics and prosthodontics Consider patients capacity to consent to Identify key teeth (e.g., canines, molars and treatment and the need for consultation occluding pairs) and restore to function regarding all aspects of treatment Advanced restorative treatment planned so Provision of simple and easily maintained that, when the individual can no longer provide oral self-care, a care-giver is prepared and able to take on this role late stage/phase 4–5
Use of local anesthetic with sedation as appropriate. Sedation may be administered Daily oral care will be carried out by carers orally/transmucosally, (intranasal/buccal), supported by regular input from dental intravenously or inhalation sedation using Written instructions and use of pictures as an Use of radio-opaque identification on acrylic aid to oral hygiene and to demonstrate preventive measures Extraction of any unrestorable teeth Regular swabbing of mouth with water or saliva substitutes (cooking oils may help) to Middle stage/phase 2–4
reduce effects of xerostomia. This is especially required before meals and at night time As above with more frequent (e.g., 3–4 Avoid mouthwash which may pose an Use of fluoride varnish Neurodegen. Dis. Manage. (2012) 2(1)
future science group Guideline for oral healthcare of adults with Huntington's disease SPecial rePort
Restorative treatment to be exposed to and involved in this group in a Any interventive treatment is likely to require mentored and structured learning process. This conscious sedation or general anesthesia. The can take place through post graduate courses patient's medical condition will be and be part of undergraduate curriculum. The compromised so liaison with physicians and courses should include other health professionals anesthetists will be required to help assess risk and caregivers to demonstrate the importance of and palliative management multidisciplinary work. The specialty of special care dentistry is becoming more recognized If edentulous or significant tooth loss, do not for its valuable contribution within the Dental consider the provision of removable prostheses Profession through international (IADH) and which may pose an aspiration risk national (e.g., BSDH) bodies. More dentists The patient may be fed via PEG so emphasis working within this specialty will have should be placed on maintenance of a healthy experience in the care and treatment of people and comfortable mouth There is an urgent need for practically When dental treatment using conscious based research which focuses on the needs of sedation and general anesthesia is no longer the individual, their family and caregivers. In appropriate, dental interventions should be as addition research should be concerned with non-invasive as possible, for example,. using the effectiveness of preventive and treatment Carisolve for caries removal, atraumatic strategies that are tailored to the developing restorative techniques, such as glass ionomer stages of the condition.
cement restorations These guidelines present both a focus of this Ensure a safe oral environment. For example, condition to the attention of the dental profession extract mobile teeth to prevent the risk of and a source of support for implementing good inadvertent inhalation oral healthcare. The implementation of these guidelines should be an important task for Otherwise sound and periodontaly secure dentists working in the area of disability and teeth may require extraction to prevent lip, oral health.
tongue or cheek soft-tissue trauma resulting The brochure/information leaflet is included from uncontrolled movement or biting as it serves to emphasize the importance and Balance the benefits of providing treatment value of other health professionals and caregivers against the difficulties in terms of co-operation, in being involved in oral healthcare. It also consent, and restraint. No treatment may be provides useful information for those individuals the appropriate option in palliative care.
An example of an information leaflet for acknowledgements
carers is provided in the Supplementary brochure.
The authors would like to acknowledge the contributing members of the European Huntington's Disease Network (EHDN) Standards of Care Dental Care Group to the The oral healthcare of a person with HD should writing of the Guideline document.The EHDN had no
be based on effective, appropriate and continuing role in study design, data collection and analysis, decision
preventive care. Careful planning and provision to publish, or preparation of the manuscript.
of treatment needs to be implemented by
clinicians experienced in the care of this patient Financial & competing interests disclosure
group. Dental treatment must be appropriate The authors have no relevant affiliations or financial
to the stage of the condition. It should also be involvement with any organization or entity with a finan-
mindful of factors that relate to the progression cial interest in or financial conflict with the subject matter
of this condition that have an oral implication or materials discussed in the manuscript. This includes
(e.g., risk of aspiration).
employment, consultancies, honoraria, stock ownership or Central to every aspect of effective oral options, expert t estimony, grants or patents received or healthcare is the need to work as part of an pending, or royalties.
interdisciplinary team. These core principles can No writing assistance was utilized in the production of only be developed through education, training this manuscript. and research. More dental care professionals need future science group SPecial rePort Manley, Lane, Carlsson et al.
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32 Adams R. Qualified nurses lack adequate dental care for people with disability.
knowledge related to oral health, resulting in 18 Idaira Y, Nomura Y, Tamaki Y et al. Factors inadequate oral care of patients on medical Fiske J, Frenkel H, Griffiths J, Jones V. British affecting the oral condition of patients with wards. J. Adv. Nurs. 24, 552–560 (1996).
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33 Berry AM, Davidson PM. Beyond comfort: the development of local standards of oral oral hygiene as a critical nursing activity in 19 Jawadi AH, Casamassimo PS, Griffen A, health care for people with dementia. the intensive care unit. Intensive Crit. Care Enrile B, Marcone M. Comparison of oral Gerodontology 23(Suppl. 1), 5–32 (2006).
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23(12), 1788 (2008).
Presents various treatment modalities for the
Case study of restorative care for an adult
22 British National Formulary (Volume 57). BMJ care of people with Huntingtons disease.
Group, London, UK, and RPS Publishing, with Huntingtons disease.
Lane H. The oral health and oral health care of London, UK, (2009).
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Presents a study examining the oral health
toothbrushes to remove dental plaque. J. Adv. and describing the dental care carried out in
Nurs. 39(5) 480–489 (2002).
38 Nagele P, Hammerle AF. Sevoflurane and the study for people with Huntington's
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management of a patient with Huntington's 11 Alvarez JO, Navia JM. Nutritional status, Neurodegen. Dis. Manage. (2012) 2(1)
future science group Guideline for oral healthcare of adults with Huntington's disease SPecial rePort
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101 European Huntington's Disease Network. using intranasal and intravenous sedation
for people with challenging behavior. A very
102 Huntington's disease association fact sheet 7: Peter van Splunter valuable technique in the treatment of
Huntington's disease and diet. people with Huntington's disease.
44 da Fonseca MA, Walker PO. Dental management of a child with Huntington's 103 Australian Huntington's Disease Association disease: case report. Spec. Care Dentist. 13(2), 71–73 (1993).
45 Starck WJ, Morrissette MP, Chewning LC. Treating a mandibular condylar fracture in a 104 Huntington's Disease association fact sheet 8: patient with Huntington's disease. J. Am. the importance of dental care. Dent. Assoc. 123(9), 52–58 (1992).
46 Kieser J, Jones G, Borlase G, MacFadyen E. Dental treatment of patients with 105 Sunstar Gum. neurodegenerative disease. NZ Dent. J. 95(422), 130–134 (1999).
106 Mun-H-Center. future science group
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