Research Summary

Global Disease Burden of Sickle Cell Disease 11 Times Higher Than Original Estimates

As part of the Global Burden of Diseases, Injuries, and Risk Factors Study (GBD) 2021, a team of researchers has found that the number of deaths globally due to sickle cell disease is 11 times higher than what was originally indicated. 

Using standard GBD approaches, the researchers found that between 2000 and 2021, the national incidence rates of sickle cell disease were stable. But the total births of babies with the disease increased globally by 13.7%. The researchers note that the increase may be due to population growth, but continued that the number of people living with sickle cell disease globally increased by 41.4%—5.46 million in 2000 to 7.74 million in 2021.

The researchers estimated the cause-specific all-age deaths globally in 2021 to be 34,400 but found that the total mortality burden was 376,000 deaths—11 times higher than their original estimation.

Further, the researchers found that in children aged 5 years and younger, there were 81,100 total deaths.

The researchers believe their findings highlight a need for further research on sickle cell disease, comprehensive strategies to address the morbidity and mortality associated with the disease, and vast deployment of evidence-based prevention and treatment for patients diagnosed with sickle cell disease.

“As countries strive to reduce child mortality and mortality due to non-communicable diseases, policy makers must consider the growing number of individuals living with sickle cell disease, and the increasing contribution of this disease to all-cause mortality,” the authors concluded. “Progress in improving sickle cell disease health outcomes requires global action, including efficient diagnostic screening, effective case monitoring through population registries, and implementation of high-quality prevention and treatment.”



Thomson AM, McHugh TA, Oron AP, et al. Global, regional, and national prevalence and mortality burden of sickle cell disease, 2000-2021: a systematic analysis from the Global Burden of Disease Study 2021. Lancet Haematol. Published online June 15, 2023. doi:10.1016/S2352-3026(23)00118-7