Health-related quality of life of adolescents with sickle cell disease in sub-Saharan Africa: a cross-sectional study.

Background: Sickle cell disease (SCD) is a chronic hematologic disease associated with increased morbidity and mortality. Hemoglobinopathies are the most prevalent genetic disease globally, and SCD is estimated to affect 0.7% of Ugandan. The disease may adversely impact on the quality of life of sickle cell patients. This study aimed to evaluate the health related quality of life (HRoL) of adolescents with SCD.

Methods: This was a mixed-methods study of adolescents with sickle cell disease and their caretakers living in Kampala city, Uganda. All children aged 8-17 years with homozygous sickle cell disease attending the sickle cell clinic at Mulago Hospital during the study period were included in this study. Participants completed the PedsQL™ generic core scales parent-proxy and child self-report questionnaire during a routine clinic visit. HRQoL was the primary outcome measured. Socio-demographics and disease related data were obtained through personal interview with caretakers and reviewing patients' medical records. Mean scores were used for HRQoL and linear regression for associated factors.

Results: Of the 140 adolescents with SCD included in the study, 40% were male. A total of 95 adolescents (68%) were between the age of 8-12 years with a mean age of 14.25 years. The physical function was assessed slightly higher by adolescents with a mean score of57.5 ± 20.3 compare to caretakers with 52.8 ± 22.1(p < 0.001). As assessed by caretakers, physical HRQoL scores were negatively associated with pain about-10.02 CI [- 19.22, - 0.81](p = 0.033), whereas it was positively associated with Pneumococcal vaccine with the score of 28.43 CI [16.78,40.09](p < 0.001) as assessed by adolescents and 31.37CI [22.22,40.51](p < 0.001) by caretakers. Pneumococcal vaccination impacted positively the psychosocial functioning with a score of 8.67CI [1.51,15.84] (p = 0.018) as assessed by children and 15.94 CI [5.50,26.38](p = 0.003) as assessed by the caretakers.

Conclusions: This study highlighted that pain was negatively associated with both physical and psychosocial functioning; whereas getting Pneumococcal vaccine was positively associated with both physical and psychosocial functioning as reported by children and caretakers.