Trends in the burden of sickle cell disorders in Sierra Leone, 1990–2023: An analysis of Global Burden of Disease Study 2023 estimates

Abstract

Background Sickle cell disease is a major cause of childhood mortality in sub-Saharan Africa, yet country-specific burden estimates for high-prevalence settings in West Africa remain limited. Objective To describe Global Burden of Disease (GBD) 2023 modeled estimates of sickle cell disorders burden in Sierra Leone from 1990 through 2023, including temporal trends, age and sex patterns, and demographic contributors to mortality change. Methods We analyzed GBD 2023 modeled estimates for sickle cell disorders in Sierra Leone, including prevalence, deaths, years lived with disability (YLDs), years of life lost (YLLs), and disability-adjusted life-years (DALYs) as absolute counts and age-standardized rates. Temporal trends in age-standardized rates were assessed using log-linear regression. The Kitagawa-Das Gupta decomposition partitioned the change in estimated deaths into population growth, age-structure change, and age-specific mortality-rate changes. Results Estimated prevalent cases increased from 48,689 (95% UI, 42,588−56,140) in 1990–90,498 (78,126−105,815) in 2023. Estimated deaths increased from 408 (288−579) to 635 (438−862), while the estimated age-standardized mortality rate declined from 10.2 to 7.9 per 100,000 (APC, −0.46%; 95% CI, −0.64 to −0.29). Decomposition attributed 159.6% of the net increase in deaths to population growth, −7.8% to age-structure change, and −51.8% to lower modeled age-specific rates. In 2023, an estimated 49.5% of deaths occurred before age 20. Point estimates suggested possible higher male mortality, but uncertainty intervals were wide and compatible with no clear sex difference. Conclusions GBD estimates suggest that Sierra Leone’s absolute burden of sickle cell disorders increased substantially between 1990 and 2023, while modeled rates declined. These modeled estimates highlight a growing absolute burden and persistent early-life mortality, supporting the need for improved surveillance, newborn screening, infection prophylaxis, hydroxyurea access, and longitudinal care systems.