title: Health-related quality of life in hemophilia: results of the Hemophilia-Specific Quality of Life Index (Haem-a-Qol) at a Brazilian blood center.
abstract: BACKGROUND
  Studies on health-related quality of life are based on the increasingly evident need for medical care not to be limited to preventing death, but to focus instead on the value of health.
  
  
  OBJECTIVE
  This study aimed to measure the health-related quality of life in hemophilia, using the Hemophilia- Specific Quality of Life (Haem-A-QoL) questionnaire and describe the socioeconomic characteristics and health conditions of these patients.
  
  
  METHODS
  The Brazilian version of the Hemophilia-Specific Quality of Life questionnaire was administered to hemophiliac adults, treated in an on-demand regime at the Juiz de Fora Regional Blood Center - HEMOMINAS Foundation. The patients were interviewed about demographic and socioeconomic data and their understanding of the questionnaire. Clinical data were collected from medical records. The Mann-Whitney U test was used for statistical analysis. The level of significance was set for p-values < 0.05. Statistical analysis was performed using the Statistical Package for the Social Sciences (SPSS, version 15.0).
  
  
  RESULTS
  Thirty-nine patients were evaluated. The mean age was 36.8 years. 84.6% had hemophilia A; 20.5% of the patients had hemophilia classified as mild, 41% as moderate and 38.5% as severe. The records of 10.5% of the patients registered seropositivity for anti-HIV and 57.9% for anti-HCV. Target joints were detected in 69.2%. The mean total Hemophilia-Specific Quality of Life score was 35.55. 'Sports and leisure'and 'Physical health'were the most impaired dimensions and the dimension 'Relationship and partners'was the least impaired. The Hemophilia-Specific Quality of Life scores showed good discriminant validity for hemophilia severity (p-value = 0.001), HIV-infection (p-value = 0.02), HCV-infection (p-value = 0.01) and the presence of target joints (p-value < 0.001).
  
  
  CONCLUSIONS
  Health-related quality of life in hemophilia, measured by the Hemophilia-Specific Quality of Life questionnaire, was influenced by the presence of arthropathy and infectious diseases transmitted by blood products. Rehabilitation measures should be encouraged in order to improve the quality of life of these patients.
authors.affiliation: Hemocentro Regional de Juiz de Fora - Fundação Hemominas, Juiz de Fora, MG, Brazil ; Universidade Federal de Juiz de Fora - UFJF, Juiz de Fora, MG, Brazil.
authors.forename: Adriana Aparecida
authors.initials: AA
authors.lastname: Ferreira
authors.forename: Isabel Cristina Gonçalves
authors.initials: IC
authors.lastname: Leite
authors.forename: Maria Teresa
authors.initials: MT
authors.lastname: Bustamante-Teixeira
authors.forename: Camila Soares Lima
authors.initials: CS
authors.lastname: Corrêa
authors.forename: Danielle Teles
authors.initials: DT
authors.lastname: da Cruz
authors.forename: Daniela de Oliveira Werneck
authors.initials: Dde O
authors.lastname: Rodrigues
authors.forename: Monica Calil Borges
authors.initials: MC
authors.lastname: Ferreira
keywords: Hemophilia A; Hemophilia B; Joint diseases; Musculoskeletal diseases/etiology; Quality of life; Questionnaires