Sickle Cell Disease Is 11 Times More Deadly Than Previously Recorded, Suggests Study

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 risk of infection and of death from conditions like stroke, heart problems, kidney problems, and pregnancy complications.

This means that the doctor taking care of a sickle cell disease patient who died from stroke may not even know that person had sickle cell disease or may not know that sickle cell disease can cause stroke, both of which could lead the doctor to not list sickle cell disease as a cause of death for that person.

When other sources of data on prevalence and birth incidence were combined with mortality data in epidemiological modelling, 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 9 times higher, respectively.

The study analysed global health data from 2000 to 2021 and was recently published in The Lancet Haematology 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.

In 2021, half a million babies were born with sickle cell disease, and more than three-quarters of these births were in sub-Saharan Africa. Under the analysis of total mortality burden (including secondary conditions), sickle cell disease was the 12th leading cause of death globally for children under the age of 5 years. However, total sickle cell disease mortality burden was among the top three causes of death in Portugal, Jamaica, Libya, Oman, and San Marino.

“Our research reveals the stark reality that sickle cell disease is far deadlier than its textbook description,” says senior author Dr. Nicholas Kassebaum, Adjunct Associate Professor at IHME. “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.”

Overall, study findings “highlight the need for a coalescence of efforts to address the large and under-recognised burden of sickle cell disease,” the researchers added.

They emphasized that policymakers and public health advocates need to work toward policies supporting an early SCD diagnosis and start of treatment, particularly in high burden areas that face substantial health inequities.

One way to do so would be through the adoption of universal newborn screening for SCD. “Universal newborn screening combined with preventive treatment is feasible and effective even in low-resource locations,” the scientists wrote. However, newborn screening in lower income countries “is fragmented,” said Theresa McHugh, PhD, a scientific writer at IHME and a study author. Meanwhile the US, which has a mandated SCD screening program, lacks a national registry to house the data.

Improving health outcomes “requires global action,” the scientists wrote. This includes “efficient diagnostic screening, effective case monitoring through population registries, and implementation of high-quality prevention and treatment.”