title: Treatment adherence and quality of life outcomes in patients with sickle cell disease.
abstract: OBJECTIVE
  To assess the health-related quality of life (HRQL) of patients with sickle cell disease (SCD) and to measure the impact of treatment adherence on disease complication, severity, crisis, and outcome.
  
  
  METHODS
  This was a cross-sectional study on patients with SCD who attended the Hematology Clinic at King Abdulaziz University Hospital from January 2009 to December 2011. We measured the primary outcome of health-related quality of life (HRQL) using the World Health Organization quality of life assessment instrument (WHOQOL-BREF). Data were collected and analyzed using the Statistical Package for Social Sciences. Analysis of HRQL was carried out along the scoring of WHOQOL-BREF.
  
  
  RESULTS
  One hundred fifteen patients completed the questionnaire. Eighty-seven patients (75.7%) had severe SCD, while 28 (24.3%) had mild disease. Patients with severe disease had a low HRQL (p=0.002). Pain episodes were the main cause of hospitalization (n=59; 51.3%). Thirty-six of patients (31.3%) who had pain episodes were on regular narcotics and had low HRQL scores (p=0.0001). The HRQL scores significantly decreased as pain levels increased. Patients with delayed treatment or those who were not adherent to treatment showed worse HRQL scores (p=0.001).
  
  
  CONCLUSIONS
  Treatment adherence and early intervention in SCD improved HRQL outcomes.
authors.affiliation: Hematology Department, Faculty of Medicine, King Abdulaziz University Hospital, Jeddah, Kingdom of Saudi Arabia. dr.jaouni@gmail.com
authors.forename: Soad K
authors.initials: SK
authors.lastname: Al Jaouni
authors.forename: Mohammad S
authors.initials: MS
authors.lastname: Al Muhayawi
authors.forename: Taher F
authors.initials: TF
authors.lastname: Halawa
authors.forename: Mutasem S
authors.initials: MS
authors.lastname: Al Mehayawi
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000188:drug therapy* / Q000503:physiopathology; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010349:Patient Compliance; D011788:Quality of Life; D012529:Saudi Arabia; D011795:Surveys and Questionnaires; D055815:Young Adult