title: Upper limb function and activity in people with facioscapulohumeral muscular dystrophy: a web-based survey.
abstract: Purpose To investigate the upper extremity (UE) at the level of impairments and related activity limitations and participation restrictions in people with facioscapulohumeral muscular dystrophy (FSHD). Methods The study was conducted using web-based questionnaires that were distributed amongst people with FSHD in the Netherlands. Eighty-eight respondents started the survey, and 71 completed it. The questionnaires covered the following dimensions: Function, Activity and Participation of the International Classification of Functioning Disability and Health. Results More than 40% of the respondents experienced pain in one arm or both the arms. Increased pain and stiffness scores and longer disease duration were associated with increased limitation scores. For basic activities, lifting the arm above shoulder-level was most frequently reported as most limited, coherent with the clinical picture of FSHD. Among the respondents, 50% indicated restrictions at school, 78% indicated restrictions at work and more than 80% indicated restrictions whilst participating in sports, hobbies, household activities and romantic relationships. Conclusions This study has shown that alongside the well-known problem of lifting the arms above shoulder-level, UE activities below shoulder height during vocational and occupational activities are also problematic in patients with FSHD. Alongside disease duration, pain and stiffness are associated with UE activity limitations. Implications for Rehabilitation Attention is needed for pain and experienced stiffness in the upper extremity as it is frequently present in patients with FSHD. Rehabilitation professionals need to be aware that patients with FSHD not only experience problems with activities above shoulder height, but also with activities below shoulder height. At least 50% of the patients with FSHD experience restrictions in participation as a result of limitations in their UE.
authors.affiliation: a Department of Rehabilitation , Donders Centre for Neuroscience, Radboud University Medical Center , Nijmegen , The Netherlands.
authors.forename: Arjen
authors.initials: A
authors.lastname: Bergsma
authors.affiliation: a Department of Rehabilitation , Donders Centre for Neuroscience, Radboud University Medical Center , Nijmegen , The Netherlands.
authors.forename: Edith H C
authors.initials: EH
authors.lastname: Cup
authors.affiliation: a Department of Rehabilitation , Donders Centre for Neuroscience, Radboud University Medical Center , Nijmegen , The Netherlands.
authors.forename: Mariska M H P
authors.initials: MM
authors.lastname: Janssen
authors.affiliation: a Department of Rehabilitation , Donders Centre for Neuroscience, Radboud University Medical Center , Nijmegen , The Netherlands.
authors.forename: Alexander C H
authors.initials: AC
authors.lastname: Geurts
authors.affiliation: a Department of Rehabilitation , Donders Centre for Neuroscience, Radboud University Medical Center , Nijmegen , The Netherlands.
authors.forename: Imelda J M
authors.initials: IJ
authors.lastname: de Groot
mesh_terms: D000203:Activities of Daily Living; D000328:Adult; D004185:Disability Evaluation; D005260:Female; D006801:Humans; D020407:Internet; D008297:Male; D008875:Middle Aged; D020391:Muscular Dystrophy, Facioscapulohumeral / Q000503:physiopathology*; D010147:Pain Measurement; D011795:Surveys and Questionnaires; D034941:Upper Extremity / Q000503:physiopathology*
keywords: Activities of daily living; facioscapulohumeral dystrophy; pain; stiffness; upper extremity