Prognosis of sickle cell disease in Africa: What do we know and what can we do?

Sickle cell disease is the most common monogenic disease worldwide. The vast majority of patients with sickle cell disease live in sub-Saharan Africa, yet the natural history of sickle cell disease is poorly understood. Mortality is currently impossible to directly calculate, due to the absence of follow-up cohorts of children diagnosed at birth and national death registries. Initial studies indirectly estimated that the majority of homozygous children would die before the age of 5. However, this juvenile mortality has greatly decreased in large cities, where it is now estimated to be between 30 and 50%. Nevertheless, it remains much higher than in wealthy countries. The incidence and risk factors for chronic organ damage are still poorly understood. The mortality and morbidity of sickle cell disease differ significantly across regions of Africa due to genetic, socioeconomic, and geographical reasons. Currently, no simple biomarker guides the monitoring and treatment of sickle cell patients. Simple interventions exist to limit infant mortality, mainly due to infections, but they require neonatal screening, which is not currently broadly organized. Reducing mortality also involves making key disease- modifying treatments available, such as hydroxyurea and blood transfusions. Therefore, it is necessary for African governments to engage in national programs for the organization of sickle cell disease care, including newborn screening. Investments are also needed to conduct robust epidemiological studies to tailor health policies to local needs. (c) 2024 l'Acade<acute accent>mie nationale de me<acute accent>decine. Published by Elsevier Masson SAS. All rights reserved.