Duchenne Muscular Dystrophy

• Use a lift or other aid if needed to transfer the patient from a wheelchair to the treatment chair.
• Sometimes it may be more relaxed and more reassuring for the patient to be treated in their specially adapted wheelchair.
• Use positioning cushions (such as Tumle dental cushions) to create stability in the treatment chair.

• If the patient has cognitive difficulties, consider using visual support in your appointment information.
• Allocate plenty of time for the visit.
• Adjust the visit time to the patient’s routine/daily rhythm.

• Assistance is often needed for both the dental hygienist and the dentist.
• Treat the patient in a sitting or semi-sitting position:
  • If the patient experiences discomfort lying down, for example, due to reduced respiratory function.
  • If there is a reduced ability to cough and swallow.
• Work in short sessions:
  • If the patient gets tired in the jaw from keeping the mouth open.
  • If the patient needs to change their seated position or head placement.
  • If the patient has breathing or phlegm production issues.
  • If the patient is generally affected and tired.
• Tools to keep on hand, to facilitate dental treatment:
  • Bite support to aid in keeping the mouth open and
    relaxing the jaw.
  • Mouth angle expander for better visibility and to facilitate opening.
  • Oral wipes to clean the mouth if there are difficulties with swallowing or spitting.

• Annual dental check-ups (more frequently if needed).
  • Keep a record of maximum mouth opening ability, as well as maximum lateral and protrusive movements of the lower jaw,
    on a regular basis.
  • Plan ahead for potential extractions of molars if the ability to open the mouth starts to decrease.
• Supportive and individualised treatment by a dental hygienist,
  on a regular basis.

• An orthodontist should be involved early on, to plan potential interceptive or corrective treatment.
• Indications for orthodontic treatment can include reducing severe crowding, or mild crowding, to facilitate good oral hygiene.
• Enhanced prophylaxis during the treatment period.
• Often requires lifelong retention, which should be followed.

• If there are signs of reduced mouth-opening ability, stretching of the jaw muscles should be initiated as soon as possible. Consult a specialist dentist if needed.
• Stretching the jaw muscles:
  • Older children and adults can be encouraged to maximise jaw mobility by opening wide and moving the lower jaw forward and sideways (see exercise programme).
  • Using fingers.
  • Using tools (Therabite or Jaw trainer).
• Note: Stretching/exercising should be carried out without causing
  any pain.

• Treat the patient in a semi-sitting position with the head tilted to the side.
• Be extra attentive during treatment to prevent anything from going down the throat. Consider using two suction devices. Assistance is important.
• Consider using a dental dam to reduce the risk of aspiration.
  Consider drying out the mouth with an oral wipe if the patient cannot spit or rinse their mouth.
• It is important to remove supra tartar, which otherwise risks coming loose and entering the airways, to avoid the risk of pneumonia.
• It is extra important to maintain good oral hygiene if there is a risk of the patient aspirating their saliva.

• Always contact the responsible physician before any sedation.
• Muscle relaxants such as Midazolam are contraindicated.
• Before anaesthesia:
  • Inform the anaesthesiologist about complicating factors such as reduced mouth opening ability and the need for extra postoperative monitoring.

Sensitivity to malignant hyperthermia has been reported and should be considered during anaesthesia.

• Assist the patient with applying for dental care support. In Sweden, this is referred to as N dental care.

• Let the patient show how they manage their oral hygiene.
• Instruct accompanying individuals on how they can assist with oral hygiene.
• Instruct the patient on an adapted working position and suitable aids for oral care at home, such as an electric toothbrush, double action toothbrush, grip enlarger, mouth angle expander and tools for stretching the jaw muscles.
• When respiratory support is used, it can be difficult to perform oral hygiene in the upper front teeth area. It is important to provide thorough instructions as well as lubrication of the lips and dry mucous membranes.

• If needed:
  • Provide practical guidance on oral care to accompanying carers
    and assistans.
  • An individually designed pictorial support can be used to help remember oral care routines.
  • Instruct on an adapted working position and suitable aids for oral care at home.
  • It is important to establish a good relationship with assistants. Motivate and provide feedback on their efforts.
  • One way to motivate can be to document oral health continuously with photos.

Ahlborg, B. (2003). Mouth- and dental care in Duchennes muscular dystrophy. Tandläkartidningen, 95(3), 38-43.

Ahlborg, B., Johansson Cahlin, B., Mårtensson, Å. & Kroksmark, A-K. (2012). Evaluation of a mechanical stretching device, the TheraBite, in patients with restricted maximal mouth opening and neuromuscular disorders: a case series. Journal of Disability and Oral Health, 13(4), 172-173.

Ahlborg, B., Carlsson, A., Kroksmark, A-K., Lundälv, E., Persson, M. & Zellmer, M. Hjälpmedelsutprovning vid mun- och tandvård. Mun-H-Center förlag 2011.

Egli, F., Botteron, S., Morel, C., & Kiliaridis, S. (2018). Growing patients with Duchenne muscular dystrophy: Longitudinal changes in their dentofacial morphology and orofacial functional capacities. European Journal of Orthodontics, 40(2), 140-148.

MHC-basen, Mun-H-Centers databas för sällsynta hälsotillstånd. Retrieved 2020-03-31 from: https://www.mun-h-center.se/forskning-och-fakta/diagnosbeskrivningar/duchennes-muskeldystrofi/

Skandinaviskt konsensusprogram för Duchennes muskeldystrofi (2015) Rehabiliteringscenter for Muskelsvind. Retrieved 2020-03-31, from: https://rcfm.dk/diagnoser/duchennes-muskeldystrofi-dmd/nordisk-konsensusprogram-duchennes-muskeldystrofi/

Socialstyrelsens kunskapsdatabas om sällsynta hälsotillstånd. Retrieved 2020-03-31, from: https://www.socialstyrelsen.se/stod-i-arbetet/sallsynta-halsotillstand/duchennes-muskeldystrofi/

Van Bruggen HW. (2015). Mandibular function in neuromuscular disorders. Doctoral thesis, chapter 8, Ipskamp Drukkers, Nijmegen.