Sickle cell disease is a growing global problem with mortality rates that are significantly under-reported, according to a major analysis published today.
According to the new estimates, the numbers affected are growing, and nearly 400,000 deaths worldwide could have been linked to the disease in 2021.
The disease is underdiagnosed, meaning that doctors may not know that people who die from complications, such as stroke and pregnancy complications, were affected.
The new analysis is published in The Lancet Haematology.
Researchers estimated that in 2021, 34,600 deaths worldwide could be directly attributed to sickle cell disease. However, the total mortality burden (including deaths caused by other conditions linked to sickle cell) was estimated to be nearly 11-times greater at 376,000. The total mortality burden was especially high in south Asia and sub-Saharan Africa.
The researchers report that half a million babies were born with the disease in 2021, 79% of whom were born in sub-Saharan Africa.
The study was coordinated by the Institute for Health Metrics and Evaluation (IHME) at the University of Washington School of Medicine, Seattle, USA.
The researchers call for universal neonatal screening for the disease.
Researcher Dr Nicholas Kassebaum from IHME said: “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.”
Fellow IHME researcher Azalea Thomson said: “By making use of all available data, we were able to strengthen our understanding of the true burden of sickle cell disease and better contextualise it alongside other leading causes of death. For example, in 2021, in kids under five years in sub-Saharan Africa, total sickle cell disease deaths exceeded those from malnutrition, measles, or syphilis.”
GBD 2021 Sickle Cell Disease Collaborators (2023) “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 Haematology, doi: 10.1016/S2352-3026(23)00118-7
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