Sickle cell disease is 11 times more deadly than previously recorded

A new study suggests the number of deaths due to sickle cell disease is 11 times higher than what is indicated from mortality data sources alone. Sickle cell disease is not just underdiagnosed, but it also increases the risk of infection and of death from conditions like stroke, heart problems, kidney problems, and pregnancy complications.

The study analyzed global health data from 2000 to 2021 and is published today in The Lancet Hematology journal. The research is part of the Global Burden of Disease 2021 study coordinated by the Institute for Health Metrics and Evaluation (IHME) at the University of Washington’s School of Medicine.

When other sources of data on prevalence and birth incidence were combined with mortality data in epidemiological modeling, in 2021, the “total mortality burden” of sickle cell disease was 373,000 deaths, compared to 34,600 sickle-cell-only deaths, or “cause-specific deaths.” The increase was especially pronounced in South Asia and sub-Saharan Africa, where the fatality figures were 67 times higher and nine times higher, respectively.

Researchers concluded that “our research reveals the stark reality that sickle cell disease is far deadlier than its textbook description,” .“The number of babies born with sickle cell disease is rising, which means a very difficult early childhood. Patients are more susceptible to infections and other severe conditions, so early detection is key for treatment.”

Reference: Sickle cell disease is 11 times more deadly than previously recorded, JOURNAL: The Lancet Hematology; DOI: 10.1016/S2352-3026(23)00118-7.