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Sickle cell disease (SCD) is associated with complications that increase the risk of early death. In recent decades of SCD research, multiple interventions have proven successful in reducing premature mortality in infants and children with SCD. However, adults with SCD still have a high risk of early mortality, with an average life expectancy of 54 years [1]. 

This study, published in Annals of Emergency Medicine, evaluated the trends in SCD mortality among Black Americans using 1979 to 2017 U.S. mortality data to determine why this disparity in adult mortality remains. 

It was found that the average annual SCD-related death rate decreased in children but increased in adults. Moreover, the median age at death increased from 28 to 43 years. Trends also showed that acute causes of death, including acute infections and cardiac, pulmonary, and cerebrovascular complications, were more common in younger age groups. Meanwhile, adults with SCD were more likely to die from chronic cardiac and pulmonary complications and renal disorders. 

Several explanations were proposed regarding the still high risk of early mortality in adults with SCD. First, treatment options for SCD, like hydroxyurea, may not reach their full potential in adults due to late intervention in childhood. Second, there is a low uptake of interventions for SCD by physicians, and third, additional barriers prevent complete intervention implementation. These findings imply that while treatment options are available, patients with SCD may not be able to access them. 

The researchers concluded that life expectancy for SCD is increasing overall, and SCD-related deaths are more likely to be due to chronic complications than acute complications. A call to action is made to better connect all patients with SCD with appropriate treatment for the disease and appropriate prevention and treatment of chronic complications to optimize life expectancy [2]. 

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[1] Lubeck, D., Agodoa, I., Bhakta, N., Danese, M., Pappu, K., Howard, R., Gleeson, M., Halperin, M., & Lanzkron, S. (2019). Estimated Life Expectancy and Income of Patients With Sickle Cell Disease Compared With Those Without Sickle Cell Disease. JAMA Network Open, 2(11), e1915374. https://doi.org/10.1001/jamanetworkopen.2019.15374

 

[2] Payne, A. B., Mehal, J. M., Chapman, C., Haberling, D. L., Richardson, L. C., Bean, C. J., & Hooper, W. C. (2020). Trends in Sickle Cell Disease–Related Mortality in the United States, 1979 to 2017. Annals of Emergency Medicine, 76(3), S28–S36. https://doi.org/10.1016/j.annemergmed.2020.08.009

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