New Analysis Reveals Sickle Cell Disease Fatality Rate Significantly Underestimated

Recent data analysis reveals that sickle cell disease is a major cause of mortality across various age demographics, with the highest prevalence found in sub-Saharan Africa. It emphasizes the immediate need for newborn screenings and better healthcare access. The increase in mortality rates since the year 2000 is largely due to the population boom in South Asia and sub-Saharan Africa.

The genetic disorder affects around 8 million people globally, with a disproportionate impact on children, adolescents, and young adults.

The latest research suggests that actual deaths attributed to sickle cell disease may be approximately 11 times greater than what existing mortality records indicate. The condition is frequently underdiagnosed and increases susceptibility to a range of complications including infections, strokes, heart-related issues, renal dysfunction, and complications in pregnancy.

Such implications could mean that healthcare providers may fail to recognize or diagnose a patient who dies from a stroke as also having sickle cell disease, thus not including it as a contributory cause of death on the medical record.

By combining prevalence and birth rate data with mortality figures in epidemiological models, the 2021 study estimates the “total mortality burden” from sickle cell disease to be 373,000 deaths as opposed to the 34,600 deaths specifically caused by the disease. These numbers are especially elevated in regions like South Asia and sub-Saharan Africa, showing increases in fatality rates by 67 and nine times, respectively.

The research, which spanned from 2000 to 2021, was recently published in The Lancet Haematology and forms part of the Global Burden of Disease 2021 study. This initiative is coordinated by the Institute for Health Metrics and Evaluation (IHME) at the University of Washington’s School of Medicine.

Dr. Nicholas Kassebaum, the senior author and Adjunct Associate Professor at IHME, states, “Our findings expose the severe underestimation of the deadly nature of sickle cell disease. The birth rate of infants with this genetic disorder is increasing, signifying challenging early years of life. These patients are more prone to severe conditions such as infections, thus making early diagnosis crucial.”

In 2021, approximately half a million infants were born with sickle cell disease, with over 75% of these births occurring in sub-Saharan Africa. When considering the overall mortality burden, including secondary conditions, sickle cell disease emerged as the 12th leading global cause of death for children under five years old. Nevertheless, in nations such as Portugal, Jamaica, Libya, Oman, and San Marino, it was among the top three causes.

Azalea Thomson, the study’s first author and IHME researcher, states, “Comprehensive data collection is vital. We employed a mathematical algorithm incorporating birth rates, prevalence, and survival rates to better gauge the true impact of sickle cell disease, juxtaposing it with other leading causes of death.”

The study highlights the urgent need for both policymakers and public health advocates to address the largely unrecognized burden of sickle cell disease. Strategies such as universal newborn screenings, public registries for case monitoring, and early intervention treatments could benefit approximately 8 million people living with the condition.

Dr. Theresa McHugh, a scientific writer at IHME focused on neonatal and child health, advocates, “Universal newborn screening is pivotal for early detection and management. In many low- to middle-income countries, this process remains fragmented, whereas in the United States it is universal but lacks a national registry. Global awareness and policy changes to enhance screening and treatment accessibility are critical for improved health outcomes.”

The study received funding from the Bill & Melinda Gates Foundation and involved researchers from IHME as well as collaborators from the Global Burden of Disease Study 2021 from across the globe.

Reference: “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” by an international team of researchers, published on 15 June 2023 in The Lancet Haematology. DOI: 10.1016/S2352-3026(23)00118-7.

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• Global Burden of Disease Study 2021
• Institute for Health Metrics and Evaluation
• The Lancet Haematology Journal
• Bill & Melinda Gates Foundation
• World Health Organization on Sickle Cell Disease
• Universal Newborn Screening Programs
• Information on Sickle Cell Complications
• Sickle Cell Disease in Sub-Saharan Africa
• Sickle Cell Disease in South Asia