title: Health-related quality of life and its relation to disease severity in boys with Duchenne muscular dystrophy: satisfied boys, worrying parents--a case-control study.
abstract: The progression of Duchenne muscular dystrophy is expected to negatively influence the patients' health-related quality of life, but knowledge of the relationship with disease severity is limited. We investigated the relationship between health-related quality of life (KIDSCREEN-52 questionnaire) and disease severity (clinical assessments of body functions and activities) in 40 boys with Duchenne muscular dystrophy (19 ambulant, 21 wheelchair dependent) who were in different phases of the disease and underwent life-limiting events such as the loss of the ability to ambulate and the ability to lift the arms. In addition, we compared boys' health-related quality of life perceptions with that of their parents. The participants' health-related quality of life was similar to healthy peers' and not influenced by disease severity, except for the physical domain. Parents scored much lower than the boys on the KIDSCREEN-52 domains Self Perception, Moods and Emotions, and Bullying. The latter finding needs attention in the management of Duchenne muscular dystrophy.
authors.affiliation: Rehabilitation Centre Groot Klimmendaal, Arnhem, The Netherlands saskiavanopstal@gmail.com.
authors.forename: S L S
authors.initials: SL
authors.lastname: Houwen-van Opstal
authors.affiliation: Radboud University Nijmegen Medical Centre, Nijmegen Centre for Evidence Based Practice, Department of Rehabilitation, Nijmegen, The Netherlands.
authors.forename: M
authors.initials: M
authors.lastname: Jansen
authors.affiliation: Radboud University Nijmegen Medical Centre, Donders Centre for Neuroscience, Department of Neurology and Clinical Neurophysiology, Nijmegen, The Netherlands.
authors.forename: N
authors.initials: N
authors.lastname: van Alfen
authors.affiliation: Radboud University Nijmegen Medical Centre, Nijmegen Centre for Evidence Based Practice, Department of Rehabilitation, Nijmegen, The Netherlands.
authors.forename: I J M
authors.initials: IJ
authors.lastname: de Groot
mesh_terms: D001294:Attitude to Health; D016022:Case-Control Studies; D002648:Child; D006801:Humans; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000503:physiopathology / Q000523:psychology* / Q000534:rehabilitation; D010290:Parents / Q000523:psychology*; D011788:Quality of Life / Q000523:psychology*; D012720:Severity of Illness Index; D011795:Surveys and Questionnaires; D014910:Wheelchairs
keywords: ICF model; KIDSCREEN-52; child report; proxy report