Epidemiology

The US Centers for Disease Control and Prevention estimate that sickle cell disease occurs in about 1 in every 365 black or African-American births, and that sickle cell disease may affect approximately 100,000 Americans (over 90% non-Hispanic black or African-American and about 3% to 9% Hispanic or Latino).[2]

Sickle cell trait or disease offers a protective effect against malaria in endemic regions and this has led to positive selection for the gene mutation. Sickle cell disease is particularly common in sub-Saharan Africa; South America, Central America and the Caribbean; India; Saudi Arabia and the Middle East; and the Mediterranean region; although it is seen throughout the world due to migration and the trans-Atlantic slave trade.[3][4]​​ Estimates suggest that over 75% of babies with sickle cell disease are born in sub-Saharan Africa.[4]

The global burden of sickle cell anemia is increasing.[3][5]​ The total number of babies born with sickle cell disease were estimated to have increased globally by 13.7% (to 515,000) between 2000 and 2021, mostly due to population growth in the Caribbean and western and central sub-Saharan Africa. The number of people living with sickle cell disease increased by approximately 41.4% to 7.74 million in 2021.[5]​ Specific-cause mortality was estimated to be 34,400 in 2021. However, the total sickle cell disease mortality burden was estimated to be 376,000, with the burden highest in children under 5 years (81,100 deaths).[5]

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