HEALTH-RELATED QUALITY OF LIFE OF CHILDREN WITH AND WITHOUT SICKLE CELL ANAEMIA AT EDWARD FRANCIS SMALL TEACHING HOSPITAL, THE GAMBIA.

Introduction/background: Children with sickle cell anaemia (SCA) experience recurrent vaso-occlusive crises and complications with possible impact on their health-related quality of life (HRQoL).

Objectives: This study determined HRQoL of 130 children aged 5-15 years with SCA and compared it to age- and sex-matched apparently healthy haemoglobin AA children in The Gambia. It also determined the impact of SCD severity, and sociodemographic and clinical data on HRQoL.

Methods: HRQoL was determined using the Pediatric Quality of Life Inventory. SCD severity was assessed with a validated clinico-laboratory scoring system.

Results: The mean age of the participants was 9.74 ± 2.81 years, with a male: female ratio of 1.1:1. Underweight (p = 0.019) and stunting (p = 0.045) were more prevalent among children with SCA. Majority, 83.1%, had mild SCD, while 13.1% had moderate SCD and 3.8% had severe SCD. The mean HRQoL scores were significantly lower in SCA than HbAA children in the physical, emotional, social, school and overall health domains, p <0.001. Seventy-five (57.7%) of SCA patients had poor HRQoL. SCD severity scores had significant inverse correlations with HRQoL scores in the emotional (r = - 0.2, p = 0.020) and school (r = -0.18, p = 0.039) domains. Significant pain >3 episodes in the preceding 12 months (OR=1.9; 95% CI = 1.392 - 2.201; p = 0.028); late diagnosis (OR = 1.8; 95% CI = 1.697-1.957; p = 0.012); and clinical stroke (OR = 69.3; 95% CI = 1.337-89.36; p = 0.037) were identified as significant independent predictors of poor overall HRQoL among children with SCA.

Conclusion: SCA has a negative impact on all domains of HRQoL. Frequent significant pain crises, late diagnosis and stroke were independent predictors of poor HRQoL in Gambian children with SCA.