Huntington Disease

• Use a lifting aid or other aids, as necessary, to move the patient from his/her wheelchair to a treatment chair.
• Sometimes the patient may feel safer and calmer reciving their treatment in his/her specially adapted wheelchair/Permobil.
• Use supportive cushions (Lasal dental cushions for example) to provide stability, security and better comfort in the treatment chair.
• Adapt the environment with regard to the increased risk of falls due to the patient’s uncontrolled and sudden movements.

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• Consider using images in the invitation if the patient has cognitive difficulties.
• Arrange plenty of time for the appointment.
• Remind the patient of the time of the visit as it approaches.
• Arrange the appointment to suit the patient’s routines/daily rhythm.

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• Both the dental hygienist and the dentist usually need assistance.
• Tools that must be available to reduce the risk of bite injuries and aspiration in connection with treatment: oral support, unbreakable mirror, mouth angle expander, oral wipes and aspirator.
• Consider whether rotating instruments can be used.

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• Treat the patient in a semi-sitting position with the head tilted to one side.
• Be careful to aspirate water (possibly with the use of two aspirators) – assistance is important.
• Consider working with a dental dam if aspiration is associated with risk.
• Consider drying the oral cavity with oral wipes if the patient is unable to spit or rinse his/her mouth.

• Sedation, for example with Dormicum or nitrous oxide, may have a calming effect on involuntary movements. Huntington’s disease is not associated with any risks from sedation, but the patient’s doctor must be contacted.
• If the patient has a PEG tube, medication can be administered through it.

• Recontour sharp teeth.
• Consider administering a soft mouth guard. This may in some cases have to be used around the clock. Inform the patient/assistant that eating or drinking with the guard in the mouth should be avoided due to the risk of caries.

• If the patient has involuntary jaw movements, his/her teeth may be worn down in a short period of time. Protect the patient’s teeth with a mouth guard. The soft variety is usually easier for the patient to accept.
• Remember to save the plaster cast of the patient’s teeth, as soft mouth guards wear out quickly and new ones need to be made regularly.

• If necessary, assist the patient with the application for dental care support.

• It is important to establish a good relationship with carers. Motivate them, give them feedback on their work. Document oral health regularly with photos.
• Encourage assistants to keep an eye on patients’ mouth cavities and to contact dental care services if they notice any sores or bite injuries.
• Give instructions on adapted working positions and useful equipment for oral care at home.

• Let patients show how they manage to carry out their oral hygiene. If necessary, correct them gradually.
• Instruct the patient about adapted positions and good tools for oral care in their home.
• Individually adapted image support and a timetable programme can be used to help remember oral care routines.

• Ahlborg B, Carlsson A, Kroksmark A-K, Lundälv E, Persson M, Zellmer M. Hjälpmedelsutprovning vid mun- och tandvård. Mun-H-Center förlag 2011.
• Manley G, Lane H, Carlsson A, Ahlborg B, Mårtensson Å, Nilsson M B, Simpson SA., Rae D. Guideline for oral healthcare of adults with Huntington´s disease. Neurogen Dis Manage 2012.
• Riksförbundet Huntington sjukdom. Grundutbildning om Huntingtons sjukdom.
• Socialstyrelsens kunskapsdatabas om ovanliga diagnoser.