Rate of sickle cell disease-related death appears higher during the COVID-19 pandemic
Sickle cell disease (SCD) is a group of inherited red blood cell disorders in which the red blood cells become hard and sticky and look like a C-shaped farm tool called a sickle. The sickle cells die early, which causes a constant shortage of red blood cells. Also, when they travel through small blood vessels, they get stuck and clog the blood flow. This can cause pain and other serious problems such as infection, acute chest syndrome (ACS), stroke, and organ damage.
People with SCD are at greater risk of severe illness and death from respiratory (lung) infections, including COVID-19, than people without SCD. It is critical for eligible people with SCD to receive the COVID-19 vaccine (shot). Also, early recognition of COVID-19 symptoms and appropriate testing is particularly important for people with SCD so that treatment can start immediately.
About this Study
Researchers from CDC examined U.S. death data to better understand whether there were changes in SCD-related deaths during the COVID-19 pandemic. They compared the number and rate of SCD-related deaths for 2020 to the numbers and rates in previous years (2014- 2019).
SCD-related deaths were defined as deaths among people in the U.S. population who had SCD listed as a contributing or underlying cause of death on their death certificate. Rate of SCD-related deaths was the number of SCD-related deaths per 100,000 people in the total U.S. population.
For a summary of the study click here.
Main Findings from this Study
- SCD-related death rates were stable from 2014 through 2019 but increased 12% in 2020.
- In 2020, there were 1,023 SCD-related deaths reported in the United States.
- In comparison to 2019, there was an estimated 113 more SCD-related deaths in 2020 than expected based on the historic SCD-related death rate.
- COVID-19 was listed as a cause of death for 86 (8.4%) of the SCD-related deaths reported in 2020.
- SCD-related deaths occurred most frequently among relatively young people (people 25-59 years old). This was found to be true for both SCD-related deaths associated with COVID-19 and those not associated with COVID-19. This is different from the general U.S. population, where most COVID-19 deaths in 2020 were among people 65 years of age and older.
Critical Gaps and Future Directions
The finding that there was an excess in SCD-related deaths during 2020 compared with what was expected based on the historic SCD-related death rate is consistent with other reports of excess deaths for the total general U.S. population in 2020.
COVID-19 explained some but not all of the excess SCD-related deaths in 2020. Previous studies have shown that in the 10 weeks following the declaration of the COVID-19 national emergency, emergency department visits in the United States for heart attack and stroke declined, suggesting people may have delayed treatment for some life-threatening conditions. It is unknown what proportion of excess SCD-related deaths may have been the result of delayed access to health care.
The findings of this study highlight the importance of COVID-19 vaccination in people with SCD who are at high-risk for COVID-19 illness. Additionally, it is important that people with SCD continue to receive appropriate care for all their medical concerns and complications. Further research is needed to understand the full impact of the pandemic on the health and well-being of persons with SCD.
Paper Reference
Payne AB, Schieve LA, Abe K, Hulihan M, Hooper WC, Hsu LL. COVID-19 and Sickle Cell Disease-Related Deaths Reported in the United States [published online ahead of print, 2022 Jan 21]. Public Health Rep. 2022;333549211063518. doi:10.1177/00333549211063518
