title: Health-related quality of life in children and adolescents with sickle cell disease.
abstract: OBJECTIVE
  To assess health-related quality of life (HRQOL) in children and adolescents with sickle cell disease (SCD).
  
  
  METHODS
  The PedsQL 4.0 Generic Scales, a multidimensional self-report instrument that has been shown to be valid and reliable for use in children and adolescents with chronic illness, consists of 23 items that assess physical, emotional, social, and school functioning. Questionnaires were administered to 124 children and adolescents (ages 8 to 18 years, child self-report) with SCD (100 sickle cell anemia, 24 sickle β zero thalassemia) and their parents (parent-proxy report). Summary scores for children's and parents' ratings of overall HRQOL and psychosocial health and subscale scores for physical, emotional, social, and school functioning were compared with published data for healthy children. Both summary and subscale scores for children with SCD also were compared with those of their parents.
  
  
  RESULTS
  Children with SCD and their parents rated overall HRQOL and all subdomains of HRQOL lower than did healthy children and their parents (P < .001). Children with SCD rated their own HRQOL significantly better than their parents did for overall HRQOL and all subdomains (P < .001) except emotional functioning (P = .06).
  
  
  CONCLUSIONS
  Children with SCD and their parents perceived overall HRQOL and all HRQOL subdomains to be lower than scores reported in healthy children. Therefore, successful therapeutic efforts to improve HRQOL could represent important advances in the health of children with SCD.
authors.affiliation: Children’sMedical Center, Dallas, TX 75235, USA. juanita.dale@childrens.com
authors.forename: Juanita Conkin
authors.initials: JC
authors.lastname: Dale
authors.forename: Cindy J
authors.initials: CJ
authors.lastname: Cochran
authors.forename: Lonnie
authors.initials: L
authors.lastname: Roy
authors.forename: Ethel
authors.initials: E
authors.lastname: Jernigan
authors.forename: George R
authors.initials: GR
authors.lastname: Buchanan
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000503:physiopathology* / Q000523:psychology; D002648:Child; D004644:Emotions; D005260:Female; D006296:Health Services / Q000706:statistics & numerical data; D006801:Humans; D008297:Male; D011788:Quality of Life; D015203:Reproducibility of Results