title: Effects of haemophilic arthropathy on health-related quality of life and socio-economic parameters.
abstract: Although prophylactic treatment is advised for all children with severe haemophilia, the optimal regimen is still under discussion. Should all joint bleeds be prevented, or can a limited amount of arthropathy be tolerated in adulthood without loss of quality of life? To answer this question, the effect of haemophilic arthropathy on health-related quality of life (HRQoL) needs to be quantified. In a retrospective study, the effect of arthropathy on HRQoL and socio-economic parameters was assessed in a single-centre cohort of 96 patients with severe and moderate haemophilia with a minimum age of 13 years. Arthropathy was measured by the radiological Pettersson score of the elbows, knees and ankles (maximum: 78 points). HRQoL was assessed by the Short Form 36 (SF36), measuring eight domains of health. Labourforce participation and medical consumption were assessed using a separate questionnaire. Patients were studied at a mean age of 28.6 years (range: 13-54), the mean time between evaluation and the last Pettersson score was 0.4 years (SD: 1.1). The overall median Pettersson score was 13 (range: 0-78). There was a trend towards lower quality of life with increasing Pettersson scores and age, especially in the physical domains of the SF36. An age-adjusted analysis showed that arthropathy had a small but significant effect on HRQoL in the domain of 'physical function' of the SF36, but not on its other domains, or on labourforce participation and medical consumption. Thus suggesting that the SF36 can be used to assess the effects of haemophilic arthropathy, especially in the domain of 'physical function'.
authors.affiliation: Department of Paediatrics, University Medical Center Utrecht, Utrecht, The Netherlands. k.fischer@azu.nl
authors.forename: K
authors.initials: K
authors.lastname: Fischer
authors.forename: J G
authors.initials: JG
authors.lastname: Bom
authors.forename: E P
authors.initials: EP
authors.lastname: Mauser-Bunschoten
authors.forename: G
authors.initials: G
authors.lastname: Roosendaal
authors.forename: H M
authors.initials: HM
authors.lastname: Berg
mesh_terms: D000293:Adolescent; D000328:Adult; D001175:Arthrography; D004185:Disability Evaluation; D005260:Female; D006467:Hemophilia A / Q000150:complications* / Q000000981:diagnostic imaging / Q000523:psychology; D006801:Humans; D007592:Joint Diseases / Q000000981:diagnostic imaging / Q000209:etiology* / Q000523:psychology; D008297:Male; D008875:Middle Aged; D010342:Patient Acceptance of Health Care; D011788:Quality of Life; D012959:Socioeconomic Factors