Rare diseases: matching wheelchair users with rare metabolic, neuromuscular or neurological disorders to Electric Powered Indoor/Outdoor Wheelchairs (EPIOCs).

Abstract

Copyright © 2015 The Author(s). Introduction: Little is written about the rehabilitation of those with rare diseases and their use of powered wheelchairs. Purpose: The aim of this study is to describe the clinical features of electric powered indoor/outdoor wheelchairs (EPIOC) users with rare diseases (RD) that impact on EPIOC provision and seating needs. Method: Retrospective review of electronic and case note records of EPIOC recipients attending a specialist wheelchair service between June 2007 and September 2008. Records were reviewed by a consultant in rehabilitation medicine, data systematically extracted and entered into a computer database. Further data were entered from clinical records and extracted under three themes; demographic, diagnostic, clinical (including comorbidity and associated features of the illness/disability (ACFs)) and wheelchair factors. Records were reviewed a mean of 64 (range 0-131) months after receiving their wheelchair. Results: Fifty four (27 male) EPIOC users, mean age 37.3 (sd 18.6, range 11-70) met the inclusion criteria. Diagnoses included Friedreich’s ataxia (n=10), motorneurone disease (n=6), osteogenesis imperfecta (n=4), arthrogryposis (n=4), cerebellar syndromes (n=4) and others (n=26). Nineteen users had between them 36 comorbidities and 30 users had 44 ACFs likely to influence the prescription. Tilt-in-space was provided to 34 (63%) users and specialised seating to 17 (31%). Those provided with SS were significantly younger than those who had standard equipment (p<0.004). Four users had between them complex control or interfacing issues. Two users required support for oxygen cylinders. Conclusions: This study contributes to the limited understanding of the rehabilitation needs of severely mobility impaired individuals with rare diseases from the perspective of a powered wheelchair service. Rehabilitation is complicated by comorbidity and the complex clinical findings in this group of wheelchair users.