Trends in the burden of sickle cell disease in Sierra Leone, 1990-2023: a Global Burden of Disease analysis

Sickle cell disease is common in Sierra Leone, yet its long-term population burden is uncertain. We analyzed Global Burden of Disease 2023 estimates for sickle cell disorders in Sierra Leone from 1990 through 2023. We obtained prevalence, deaths, and disability adjusted life years (DALYs), calculated age standardized rates per 100 000, modeled temporal trends with log linear regression, decomposed mortality changes into effects of population growth and rate change, and assessed age specific and sex specific patterns in 2023. Sickle cell prevalent cases increased from 48 689 (95% UI, 42 588 to 56 140) in 1990 to 90 498 (95% UI, 78 126 to 105 815) in 2023, and deaths rose from 408 (95% UI, 288 to 579) to 635 (95% UI, 438 to 862). DALYs increased from 32 518 (95% UI, 23 446 to 45 336) to 50 608 (95% UI, 36 331 to 66 317). Over the same period, age standardized mortality declined from 10.2 to 7.9 per 100 000 (APC, -0.46%; 95% CI, -0.64 to -0.29) and age standardized DALYs from 811.5 to 631.5 per 100 000 (APC, -0.43%; 95% CI, -0.60 to -0.25). In 2023, children younger than 5 years accounted for 153 deaths (95% UI, 93 to 230; 24.1% of all deaths), and persons younger than 20 years for 314 deaths (95% UI, 203 to 446; 49.5%). Males and females had similar prevalence (45 442 vs. 45 056 cases), but males had 371 deaths and 28 855 DALYs versus 264 deaths and 21 753 DALYs in females. Decomposition indicated that population growth alone would have increased deaths by 179.5%, whereas rate reductions offset this by 79.5%. The burden of sickle cell disease in Sierra Leone has grown in absolute terms, driven mainly by rapid population expansion, while age standardized mortality and DALY rates have declined. Concentrated losses in children and young adults and persistent male excess mortality highlight the need to scale up newborn screening and early comprehensive care, expand access to hydroxyurea and infection prevention, and strengthen resilient sickle cell services.