RESUMO
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. 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. 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]. Treatment adherence and early intervention in SCD improved HRQL outcomes