Dental Care & Treatment
General Recommendations:
Children should be screened by a pediatric dentist as soon as possible following diagnosis.
Tooth Brushing:
- Minimum frequency 2x/day; an adult should brush the child's teeth from the moment the first tooth appears.
- If mechanical brushes are used, they should be used as one would a normal toothbrush, using up and down movement rather than side to side.
- Parents should check for food pouching in the cheeks and palate.
- If the child resists brushing, parents should take the approach, "I love you too much not to brush your teeth" and do it anyway.
Flossing:
- Flossing is every bit as important as brushing.
- Flossing should begin as soon as teeth erupt.
Acidic beverages are one of the most significant reasons for tooth decay.
- Absolutely no soda, energy drinks or sweetened fruit juice or beverages.
- 100% fruit juice is allowed only in small quantities (4 oz or less/day/30 lbs)
- Desired beverages include milk and water.
- Do not put a child to sleep with a bottle (if you must, please use water)
Diet:
- A well balanced diet limits foods with added sucrose, fructose, sugar or related sweeteners.
- A good diet should contain calcium and vitamin D from natural sources.
Regular dental visits for cleaning, consultations, cavity monitoring and protection such as sealants are a must.
- Poor oral hygiene increases the risk for pneumonia.
- Institute the of prevention of jaw contracture with the therabyte system at the earliest indication of tightness.
- Early referral to an Orthodontist as indicated is important to help utilize palate expanders for narrow high arched palates and teeth crowding.
Relevance of proactive dental hygiene and prevention of jaw contracture:
Significant jaw contractures ultimately develop in a majority of adults with type II SMA. Some children with SMA type II, and virtually all children with type I who survive to later childhood and adulthood also manifest this problem. Jaw contractures can result in inadequate oral hygiene and increase the risk for serious dental complications over time. Because of discomfort experienced during routine dental care due to this issue, many individuals choose to avoid going to the dentist at all rather than address the primary issue. This sets up a vicious cycle of poor dental care and hygiene, and ultimately, tooth decay. Subjects with tooth decay and gingivitis are at an increased risk for pneumonia as well as more serious medical problems such as endocarditis (inflammation and infection on heart valves). Thus, it is critical to avoid these problems in the first place by being proactive from early childhood. We recommend dental visits at least every six months beginning at three years of age for all individuals with SMA. Early consultation with an Orthodontist for consideration of a retainer or palate expander can help prevent jaw and facial deformity and abnormal bit alignment due to decreased use of mandibular muscles.
Anticipatory jaw stretching is one possible solution to help avoid this problem. The Therabyte System is one such solution. In subjects whom significant jaw contracture has already developed, and who require dental or oral surgery or other medical procedures requiring significant mouth opening, a gradual increase in the width of jaw opening over a period of several weeks can increase access. However, this needs to done gently and carefully to avoid damage to the temporomandibular joint and surrounding muscles. If a dental, endodontal or oral surgery is procedure is to be done, and they haven't had adequate ongoing mouth care, standard antibiotic prophylaxis should be considered prior to such a procedure in which those who haven't had adequate preventative mouth care. Most individuals with significant jaw contracture have respiratory impairment which warrants an anesthiology consult if any type of sedation is planned. To avoid serious muscle damage and chronic temporomandibular joint pain, intubation using a laryngeal mask airway (LMA, which doesn't require visualization of vocal cords) or fiber optic intubation via placement of an endotracheal tube over a bronchoscope can ensure adequate ventilation during and after the procedure. Sometimes, use of muscle relaxants or even paralysis are necessary to enable adequate jaw-opening to extract teeth or perform dental or oral surgery procedures. Where such a procedure is performed depends on how fragile their respiratory status is. Most individuals will need to be in a place where they can get appropriate post-procedure care and support until they have recovered to their baseline state with regard to airway protection.
When dental issues have not been addressed in a proactive fashion, individuals who suffer gingivitis, tooth decay, abscesses or severe temporomandibular joint restriction can develop significant and chronic pain. While addressing the primary issue is desirable, sometimes individuals are fearful of aggressive interventions, and may need time to fully consider the risks and benefits of more invasive procedures. It is important not to overlook the importance of adequate pain control or less invasive interim solutions in such patients, including palliative care interventions.
