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This is the text from the oral care programme for people with Spinal muscular atrophy (SMA).
Mun-H-Center, a Swedish orofacial resource center for rare diseases and a specialist dental clinic, has gathered knowledge and experience over many years on the clinical care of patients with spinal muscular atrophy (SMA). This document compiles the key insights we have gained regarding the care and treatment of this patient group. People with SMA are often medically fragile. The mouth opening capacity can be reduced and impaired orofacial function can occur. These issues, combined with risks associated with anaesthesia, demand enhanced dental care to prevent oral diseases.
Background
Spinal muscular atrophy is a neuromuscular disease caused by a defect on chromosome 5. SMA types I, II, and III are autosomal recessive inherited conditions. In SMA, the motor neurons in the brainstem, medulla oblongata and spinal cord degenerate, leading to muscle weakness and atrophy – particularly in the chest, back, and neck. Intellectual development is not affected.
SMA I (Werdnig-Hoffmann disease): Symptoms are present from birth or appear before six months of age. Infants with SMA I cannot lift their heads due to weak neck muscles. Breathing function is severely affected, and there is a high risk of infections.
SMA II (so called intermediate form): Symptoms appear between six and 18 months of age. Muscle weakness is usually more pronounced in the legs than in the arms. Scoliosis (curved spine) is common. Breathing function may be affected.
SMA III (Kugelberg-Welander disease): Symptoms appear around two years of age or later. Muscles closest to the torso (proximal muscles) are weakened. Back problems and difficulty walking are common and may worsen over time. Weak throat muscles affect swallowing ability. Tongue muscles may also be weak, showing fasciculations (small twitches). Reduced mouth-opening ability is common among children, adolescents, and adults with SMA. Various types of dental malocclusions can occur.
Dental care for people with SMA
Need for preventive dental care
It is important for children to establish early contact with dental care, preferably with a paediatric dentist. Individuals with SMA require regular and enhanced dental care to maintain good oral health. The opening capacity of the mouth should be monitored yearly. All individuals with SMA, regardless of age, need to regularly exercise jaw movements for preventive purposes. If mouth-opening ability decreases, jaw joint function should be investigated and appropriate treatment initiated. If there are difficulties eating through the mouth and the person uses alternative nutrition methods such as gastrostomy, this also necessitates special dental care to maintain oral health and prevent sensitivity or aversion to activities like tooth brushing and dental examinations.
Risk factors for oral health
Reduced mouth-opening ability can make oral and dental care difficult. Therefore, it is important to plan ahead for potential extractions of molars, including wisdom teeth. If oral motor skills are affected, food can remain in the mouth after meals, increasing the risk of cavities and periodontal disease. Reflux and nausea can lead to erosion damage to the teeth.
Guidance for relatives, assistants, and healthcare professionals
Individuals with SMA may need help from relatives, assistants, and healthcare professionals to perform oral care at home. Practical guidance from dental care providers and access to oral care aids are necessary for them to do this properly.
Use a lift or other aids to transfer the patient from a wheelchair to the treatment chair if needed.
Sometimes it is calmer and safer for the patient to be treated in their specially adapted wheelchair/powered wheelchair.
Use positioning cushions (such as Tumle dental cushions) to create stability in the treatment chair.
Allocate plenty of time for the visit.
Schedule appointments according to the patient’s routines/daily rhythm.
People with SMA often have several healthcare contacts. If possible, allow the person with SMA to participate in choosing the appointment time.
Both dental hygienists and dentists often need assistance during treatment.
Treat the patient in a seated/semi-seated position.
Work in short sessions:
If the patient needs to change their sitting or head position.
If the patient has breathing or phlegm production issues.
If the patient is generally affected and tired.
Equipment that should be available to facilitate dental treatment:
Bite support to aid mouth-opening and resting the jaw.
Mouth angle expander to improve visibility and facilitate mouth-opening.
Oral wipes to clean the mouth when there are swallowing/spitting difficulties.
Examination by a dentist annually or more frequently if needed.
Regular supportive care by a dental hygienist (individually adjusted intervals).
Regular measurement of maximum mouth-opening ability and recording of maximum lateral and forward movement of the lower jaw.
An orthodontist should be involved early on to plan potential interceptive or corrective treatment.
Indications for orthodontics may include reducing severe crowding or moderate crowding to facilitate good oral hygiene.
Enhanced prophylaxis during treatment.
Often, lifelong retention is required, which should be followed.
All individuals with SMA need to regularly exercise jaw movements for preventive purposes.
Oral toys can stimulate young children to activate their mouth and mouth-opening ability.
Older children and adults can exercise jaw movement by opening wide and moving the lower jaw forward and to the sides, repeating each exercise three times daily (see exercise program). Stretching of the jaw muscles:
With fingers
With tools (Therabite or Jaw trainer)
It is important to maintain swallowing and chewing functions. However, the primary concern is that the meal is safe and not too energy consuming.
Time to train your jaw! Exercise program for:
Open your mouth as wide as you can.
Push your lower jaw forward.
Move your jaw to one side...
...and to the other side.
Open your mouth wide again.
Use your hand to stretch...
...or your Therabite...
...or Jaw trainer.
Please feel free to contact us if you have any questions! Best regards, (fill in the practitioner’s name and the clinic’s phone number)
Treat the patient upright or in a semi-seated position with the head turned to one side to reduce the risk of swallowing food or drink the wrong way.
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 if there is a risk of aspiration.
Use an oral wipe if the patient cannot spit or rinse.
It is important to remove supra tartar. Otherwise there is a risk of detachment and aspiration.
It is extra important to maintain good oral hygiene if there is a risk of the patient aspirating their saliva, to avoid the risk of pneumonia.
Oral motor sensory stimulation may be needed in addition to oral and dental care.
Ensure that the patient has a speech-language pathologist contact.
Always contact the responsible physician before any sedation.
Muscle relaxants, such as Midazolam, are contraindicated.
Before anaesthesia:
Inform the anaesthetist about complicating factors such as reduced mouth-opening ability and the need for extra post-operative monitoring.
Assist the patient with applying for dental care support. In Sweden, this is referred to as either F or N dental care.
Have the patient demonstrate how they perform their oral hygiene. Provide additional information and correct the method if needed.
Instruct on an adapted working position and suitable aids for home oral care (electric toothbrush, double action toothbrush, grip enlarger, mouth angle expander, bite support, tools for stretching the jaw muscles, etc.).
An individually designed pictorial support can be used to help remember oral care routines.
It is important to establish a good relationship with assistants, relatives, and healthcare personnel.
Instruct on an adapted working position and suitable aids for home oral care.
Motivate and provide feedback on the assistants’ efforts. Follow up oral health regularly with photos.
About the oral care programme
Authors: Anna Ödman Roussakis, specialist dentist in orthodontics, Annette Carlsson, dental hygienist, Lisa Bengtsson-Stelzer, speech language pathologist, and Åsa Mårtensson, hospital dentist, Mun-H-Center.
Layout, photos, and cover image: Inga Svensson, previous communicator, Mun-H-Center.
Illustrations in the exercise program: Komhit.
Translation: Hanna Samara, communicator, Mun-H-Center
Print: 2024
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.
Islander, G. (2013). Anesthesia and spinal muscle atrophy. Pediatric Anesthesia, 23(9), 804-816.
Logemann, J., Curro, F., Pauloski, B., & Gensler, G. (2013). Aging effects on oropharyngeal swallow and the role of dental care in oropharyngeal dysphagia. Oral Diseases, 19(8), 733-737.
Van Bruggen, H., Van Den Engel-Hoek, L., Van Der Pol, W., De Wijer, A., De Groot, I., & Steenks, M. (2011). Impaired Mandibular Function in Spinal Muscular Atrophy Type II: Need for Early Recognition. Journal of Child Neurology, 26(11), 1392-1396.
Van Bruggen HW. (2015). Mandibular function in neuromuscular disorders. Doctoral thesis, chapter 8, Ipskamp Drukkers, Nijmegen.
Updated: 2024-06-04 14:34
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