Study of sickle cell disease-related death in the United States suggests average age at death is increasing

Sickle cell disease (SCD) is a group of inherited red blood cell disorders. In SCD, 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, stroke, and organ damage.

People with SCD start to have signs of the disease during the first year of life, usually around 5 months of age. Symptoms and complications of SCD are different for each person and can range from mild to serious.   Treatments are available that can prevent health problems or complications and lengthen the lives of those who have this condition.

The Centers for Disease Control and Prevention’s (CDC) National Vital Statistics System collects information on all deaths occurring in the United States, and this information can be used to understand the underlying complications that contributed to a person’s death, watch trends in these complications over time, and help inform public health program planning.

About This Study

Researchers from the CDC conducted a study using death certificate information from the National Vital Statistics System.  Researchers examined deaths in people whose death certificate indicated that they had SCD.  They compared their causes of death to the causes of death among a group of people who had no indication of SCD but were similar to the SCD group in terms of their race, sex, age, and year of death. The study aimed to:

• Analyze trends in SCD-related deaths over time and across various age groups; and
• Compare complications among SCD-related deaths to non-SCD-related deaths.

We invite you to read the study’s abstract here.

Main Findings From This Study

• From 1979 through 2017, there were 25,665 recorded SCD-related deaths among Black people in the United States.
• Persons with SCD may be living longer: From 1979 to 2017, the median age at death increased from 28 years to 43 years.  Over that same time period, SCD-related death rates among Black children younger than 5 years of age declined 158%.
• From 2015–2017, SCD-related deaths were most often related to chronic heart conditions such as high blood pressure (27%).  Acute cardiac events, such as heart attacks, and infections were also common causes of death (24% and 22%, respectively).
• SCD-related deaths were more likely to be caused by infection, stroke, heart disease, liver disease, kidney disease, and lung disease than deaths among a comparison group without SCD who died at the same ages, in the same years, and with the same race and sex distribution as the SCD-related deaths.

Critical Gaps & Future Directions

Gaps

• This study used information from death certificates to identify people with SCD and their cause of death. Cause of death is not always accurately reported on death certificates.
• Trends in SCD-related death may not reflect trends in SCD-related illness or the burden of certain illnesses in people with SCD.

Future Directions

Because of the potential for incorrectly assigned deaths using only death certificate information, future work will look to confirm the trends noted in this study in other datasets that include additional pieces of information.

More Information

View more information about SCD

Paper Reference

Payne AB, Mehal JM, Chapman C, Haberling DL, Richardson LC, Bean CJ, Hooper WC.  Trends in sickle cell disease-related mortality in the United States, 1979-2017.  Annals of Emergency Medicine.  2020; 76 (3S):S28-S36