title: Quality of life in patients with sickle cell disease.
abstract: OBJECTIVE
  To evaluate the quality of life in children and adolescents with sickle cell disease attending a blood reference center, and to assess the quality of life of their relatives.
  
  
  METHODS
  Cross-sectional study that included 100 patients with sickle cell disease, which were divided into three subgroups according to age: 5 to 7 (n=18), 8 to 12 (n=32), and 13 to 18 years-old (n=50), and their parents. The Control Group included 50 healthy children and adolescents from a public local school, also divided into the same three age subgroups and their caregivers. The Pediatric Quality of life Inventory (PedsQL), version 4.0, was applied in both groups. The generic questionnaire Medical Outcomes Study 36 - Item Short-Form Health Survey (SF-36) was applied to the relatives. The answers were linearly transformed into a score and compared by non-parametric tests.
  
  
  RESULTS
  The PedsQL scores of patients were significantly lower than those obtained in the Control Group (p<0.0001) in all studied areas (physical, emotional, social skills, and school activities). Similarly, SF-36 scores applied to the patients' parents were lower than those obtained in the Control Group in all studied aspects (p<0.0001).
  
  
  CONCLUSIONS
  Sickle cell disease affects the quality of life of children, adolescents, and their families. Patients sense restrictions in the emotional, social, family and physical aspects, among others.
authors.affiliation: Unifesp.
authors.forename: Adeline Soraya de O da P
authors.initials: AS
authors.lastname: Menezes
authors.forename: Cláudio Arnaldo
authors.initials: CA
authors.lastname: Len
authors.forename: Maria Odete E
authors.initials: MO
authors.lastname: Hilário
authors.forename: Maria Teresa R A
authors.initials: MT
authors.lastname: Terreri
authors.forename: Josefina Aparecida P
authors.initials: JA
authors.lastname: Braga
mesh_terms: D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000175:diagnosis; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D010290:Parents; D011788:Quality of Life; D011795:Surveys and Questionnaires