Orofacial function of persons having. Spinal muscular atrophy

Transcription

1 Orofacial function of persons having Report from questionnaires 31 questionnaires Synonym SMA I (Werdnig-Hoffmann disease, SMA II, SMA III (Kugelberg-Welander disease). ICD-10 G12.0 Estimated occurance General symptoms Every year, 4-6 children in Sweden develop the most severe form of spinal muscular atrophy - SMA I. SMA II and III are somewhat less common. Muscular weakness and atrophy are most pronounced in the proximal musculature, including the chest, back and neck muscles. Intellectual development is not affected. SMA I (Werdnig-Hoffmann disease): Symptoms from birth, or prior to the age of 6 months. Respiratory function is severely affected, and these children are at high risk from infections. SMA II (known as the intermediate form): Symptoms present around the age of 6-18 months. Muscular weakness is often more pronounced in the legs than in the arms. Scoliosis (curvature of the spine) is common. Respiratory function may be affected. SMA III (Kugelberg-Welander disease): Presents around the age of 2 years. Weak musculature in the trunk (proximal), back problems, and ambulatory difficulties are common and increase with age. Sometimes, SMA 0 is used for the severe congenital type and SMA IV for the milder type with adult onset. Oral symptoms Infants with SMA I are unable, owing to their weak neck musculature, to lift their heads. Musculature weakness in the throat affects the ability to suck and to swallow. The musculature of the tongue is also weak, and fasciculations (slight trembling) in the tongue may occur. Adolescents and adults with SMA sometimes develop an impaired jaw opening capacity. Some have malocclusion. Oral treatment Early contact with dental services for intensified prophylactic care and oral hygiene information is essential. Regular check-ups of dental and jaw development. Orthodontist should be consulted when needed. When the jaws do not open properly, the function of the jaw joint should be investigated, and appropriate treatment thereafter prescribed. Feeding and swallowing difficulties are investigated and treated by a specialist team at the hospital or multidisciplinary treatment center. Sources The MHC base Rare diseases Dokumentation-Ågrenska 1 (7)

2 Age distribution : 31 Ages: 3-38 (17) (14) Additional diagnoses Medical impairment Yes No Missing Inborn heart defect Other cardiovascular disease Epilepsy Asthma Need of respiratory support Allergy Neuropsychiatric diagnosis Yes No Missing ADHD/ADD Autism (Includes autism, Asperger syndrome and autistic traits) General disability Yes No Missing Intellectual disability Motoric functional impairment Visual impairment Hearing loss Communication difficulties (7)

3 About dental care and oral health Do you feel that you receive the dental care you need? Yes, very much so 23 Yes, somewhat 6 Missing 2 How many times per year do you normally seek dental care? Less than once per year 4 One time per year 8 Two times per year 9 Three or more times per year 8 Missing 2 When were your teeth last X-rayed? During the past two years 15 More than two years ago 3 Never had my teeth X-rayed 9 Missing 4 3 (7)

4 Do you look after your teeth in a good way? Yes, very much so 18 Yes, somewhat 10 No, not really 1 Missing 2 Who brushes your teeth? I always brush myself 6 Someone else always helps me 14 Sometimes I brush myself 11 How often are your teeth brushed? Not everyday 1 Once per day 3 Two times per day 22 Three or more times per day 3 Missing 2 4 (7)

5 About dental care and oral health Yes No Missing Does your mouth hurt? Does your mouth feel dry? Have you ever taken a serious hit to your permanent front teeth? Do you feel that you have a divergent bite? Have you had a brace? Do you feel that you need orthodontics/a brace? Do you grind or press your teeth at night? Never 27 Once or twice per week 3 Missing 1 Do you grind or press your teeth during the day? Never 28 Once or twice per week 3 5 (7)

6 About eating Do you have any problems with eating? Yes, very much so 6 Yes, somewhat 10 No, not really 6 No, not at all 6 Missing 3 Yes No Missing Do you cough daily in connection with meals? Do you gag daily in connection with meals? Do you get acid reflux daily? Do you throw up often (at least twice per week)? Do you have a poor appetite? Does it take a long time before you can swallow a mouthful? Do you press your tongue forward when you swallow so that food ends up outside the mouth? Do you find it difficult to chew, i.e. grind food using your molars? Do you find it difficult to take food from the spoon using your lips? Have you had problems with food and drink leaking out through the corners of your mouth? Does food tend to remain in your mouth after meals? Do you get nutrition in any other way than through your mouth? (7)

7 About drooling Do you drool? Never drool 21 Drool sometimes not every day 5 Drool often every day 1 Missing 3 How much do you drool? Slight drooling, only on the lips 3 Moderate drooling, on lip and chin 2 Missing 1 Total: 6 Is your drooling a problem for you? No, not really 4 No, not at all 1 Missing 1 Total: 6 Is your drooling a problem for your family or people around you? No, not really 1 No, not at all 4 Missing 1 Total: 6 7 (7)