title: Health related quality of life in sickle cell patients: the PiSCES project.
abstract: BACKGROUND
  Sickle cell disease (SCD) is a chronic disease associated with high degrees of morbidity and increased mortality. Health-related quality of life (HRQOL) among adults with sickle cell disease has not been widely reported.
  
  
  METHODS
  We administered the Medical Outcomes Study 36-item Short-Form to 308 patients in the Pain in Sickle Cell Epidemiology Study (PiSCES) to assess HRQOL. Scales included physical function, physical and emotional role function, bodily pain, vitality, social function, mental health, and general health. We compared scores with national norms using t-tests, and with three chronic disease cohorts: asthma, cystic fibrosis and hemodialysis patients using analysis of variance and Dunnett's test for comparison with a control. We also assessed whether SCD specific variables (genotype, pain, crisis and utilization) were independently predictive of SF-36 subscales, controlling for socio-demographic variables using regression.
  
  
  RESULTS
  Patients with SCD scored significantly worse than national norms on all subscales except mental health. Patients with SCD had lower HRQOL than cystic fibrosis patients except for mental health. Scores were similar for physical function, role function and mental health as compared to asthma patients, but worse for bodily pain, vitality, social function and general health subscales. Compared to dialysis patients, sickle cell disease patients scored similarly on physical role and emotional role function, social functioning and mental health, worse on bodily pain, general health and vitality and better on physical functioning. Surprisingly, genotype did not influence HRQOL except for vitality. However, scores significantly decreased as pain levels increased.
  
  
  CONCLUSIONS
  SCD patients experience health related quality of life worse than the general population, and in general, their scores were most similar to patients undergoing hemodialysis. Practitioners should regard their HRQOL as severely compromised. Interventions in SCD should consider improvements in health related quality of life as important outcomes.
authors.affiliation: Department of Biostatistics, Virginia Commonwealth University, Richmond, VA, USA. mcclish@hsc.vcu.edu
authors.forename: Donna K
authors.initials: DK
authors.lastname: McClish
authors.forename: Lynne T
authors.initials: LT
authors.lastname: Penberthy
authors.forename: Viktor E
authors.initials: VE
authors.lastname: Bovbjerg
authors.forename: John D
authors.initials: JD
authors.lastname: Roberts
authors.forename: Imoigele P
authors.initials: IP
authors.lastname: Aisiku
authors.forename: James L
authors.initials: JL
authors.lastname: Levenson
authors.forename: Susan D
authors.initials: SD
authors.lastname: Roseff
authors.forename: Wally R
authors.initials: WR
authors.lastname: Smith
mesh_terms: D000293:Adolescent; D000328:Adult; D000367:Age Factors; D000755:Anemia, Sickle Cell / Q000503:physiopathology* / Q000523:psychology; D002908:Chronic Disease; D005260:Female; D006296:Health Services / Q000706:statistics & numerical data; D006801:Humans; D008297:Male; D008875:Middle Aged; D010146:Pain / Q000209:etiology; D011594:Psychometrics; D011788:Quality of Life; D012647:Self-Assessment; D012737:Sex Factors; D018434:Sickness Impact Profile; D012959:Socioeconomic Factors; D014768:Virginia