S.P.13 Exploring arm function in FSHD by means of questionnaires

Abstract

<h2>Abstract</h2> Facioscapulohumeral muscular dystrophy (FSHD) is a progressive muscular dystrophy. One of the major problems is limitations in performing daily activities. The natural course of the disease has been described before, and research is done to investigate functional ability of walking. However, little is known about functional abilities of the arms, which are even more important in performing many daily activities. Clear understanding is essential to improve rehabilitation therapies and for development of new arm supports. The aim of this study is to investigate arm function of people with FSHD on activity level, by using questionnaires. A web-based questionnaire to assess arm function was developed. We used questions from the Brooke scale (grading scale, 1–6), the Capabilities of Upper Extremity questionnaire (CUE) (basic arm functions, 30 items, max score 210, higher score means better function) and Abilhand questionnaire (22 ADL items, max score 44, higher score meaning lower function). Also questions about disease duration, age, gender were asked. The Dutch patient organization (VSN) supported with distribution of the questionnaire. Seventy-six people participated (age 13–74y) in the period August 2011 till February 2012. Preliminary results show that from this group, 88% have Brooke 2–4. The CUE total median score was 155 (range 53–210), corresponding with 73.8% of the maximum score. The total Abilhand median score was 38 (range 13–44) corresponding with a 86.4% of the maximum score. Especially activities above shoulder level are problematic. Patterns of limitations in arm function are further analyzed as are correlations with disease course, age, and gender. The project McArm is a national Dutch ‘Pieken in de Delta' project. It is partially funded by: Agentschap.nl, an agency of the Dutch Ministry of Economic Affairs, Agriculture and Innovation, the Province of Noord Brabant and the Province of Limburg.