title: Health-related quality of life in a cohort of adult patients with mild hemophilia A.
abstract: OBJECTIVE
  To compare the health-related quality of life among adult males affected with mild hemophilia A due to the same mutation (Val2016ala) to that of unaffected age and sex matched controls from the same general population.
  
  
  METHODS
  The Short-Form 36 (SF-36) and Health Assessment Questionnaire (HAQ) were used to measure health-related quality of life and physical function. Other measures included bleeding history, a measure of joint damage, body mass index, age, and viral infection status. Cross-sectional data were collected through research clinics and a retrospective chart audit over a two-year period.
  
  
  CONCLUSIONS
  The study included 47 affected males and 33 controls. The affected males had a higher level of co-morbidity, prior bleeding, and existing joint damage than controls. With the exception of the social function and health transition scales, mean scores for each of the SF-36 domains were worse among affected males. Mean differences were more than a clinically important five points in five of eight domains, with the general health scale showing more than a 10-point difference. Despite the degree of difference noted, only two of the differences were statistically significant (general health and role emotional scales) because of the small sample size and considerable individual variation in SF-36 scale scores. Multiple regression analyses suggested existing joint damage and presence of heart disease as the strongest associates of lower physical health-related quality of life. Joint damage in turn was partly related to prior hemarthroses. Compared to the Canadian population, affected males had lower scores in six out of eight SF-36 domains as well as the physical component summary score. There were no significant differences found in the HAQ scores between the two groups. So-called mild hemophilia A was associated with a negative effect on physical health-related quality of life, contributed to by joint damage as a result of prior bleeding.
authors.affiliation: The Clinical Epidemiology Unit, Department of Medicine, Memorial University of Newfoundland, St John's, NL, Canada. meghanw@mun.ca
authors.forename: M
authors.initials: M
authors.lastname: Walsh
authors.forename: D
authors.initials: D
authors.lastname: Macgregor
authors.forename: S
authors.initials: S
authors.lastname: Stuckless
authors.forename: B
authors.initials: B
authors.lastname: Barrett
authors.forename: M
authors.initials: M
authors.lastname: Kawaja
authors.forename: M-F
authors.initials: MF
authors.lastname: Scully
mesh_terms: D000328:Adult; D016022:Case-Control Studies; D015331:Cohort Studies; D006395:Hemarthrosis; D006467:Hemophilia A / Q000150:complications / Q000523:psychology; D006801:Humans; D008297:Male; D011788:Quality of Life; D011795:Surveys and Questionnaires