S.P.12 Investigating arm function in boys with DMD by means of an international questionnaire

Abstract

Duchenne muscle dystrophy (DMD) is a disorder characterized by progressive muscle weakness, resulting in loss of functions. With increasing life expectancy, the preservation of functional abilities becomes increasingly important. Good knowledge on functional abilities is needed, since this information is important for developing new supportive aids and training strategies. Unfortunately, knowledge on the functional abilities of the upper extremity in boys with DMD is limited. To gain more insight in the arm function of boys with DMD an international questionnaire was distributed. A web-based questionnaire to assess arm function in boys with DMD was developed and distributed around the world (more than 12 countries already participated). Next to questions about the medical history of the patients, arm function was assessed using the Brooke scale (grading scale, 1–6), Capabilities of Upper Extremity questionnaire (CUE) (basic arm functions, 30 items, max score 210, higher score means better function) and ABILHAND (22 ADL items, max score 44, higher score meaning lower function). On 23rd March, 2012, 344 participants had started the questionnaire and 227 participants completed the questionnaire. The mean age of patients was 16.8 years (range: 1–44 years). Scores on the Brooke scale varied between 1 and 6 and seemed to be related with age. The mean CUE total score was 127 (range: 30–210) and the mean ABILHAND score was 22 (range: 0–44), corresponding with a 50% of the maximum score. On this moment, the questionnaire is still ongoing and the results shown are only a small sample of all outcome measures. The outcome variables will be compared and correlations will be calculated. The main focus will be on the pattern of progressive loss of arm/hand function, the effect of medicine use on functional abilities, pain and stiffness and the on the activities that are most important to DMD patients.