Life expectancy for people with sickle cell disease increases by a decade

Key takeaways:

• Life expectancy for people with sickle cell disease increased by 20% between 2008 and 2022.
• The percentage of people with sickle cell disease who died at age 60 years or older also increased.

Life expectancy for individuals with sickle cell disease has increased by a decade since 2008, according to a study conducted by Epic Research.

The percentage of people with sickle cell disease who were aged 60 years or older when they died increased by more than 28% since 2016, findings showed.

“General advancements in medicine and health care can likely be correlated with the increased life expectancy in this population, as well as increased awareness and the availability of new treatment options,” Kersten Bartelt, RN, clinician at Epic Research, told Healio. “What surprised me the most was that we saw the largest increase in life expectancy in the years prior to new treatments being available. Since these treatments were recently approved, there hasn’t been enough time to fully see the effect they are anticipated to have on life expectancy.”

More than 100,000 Americans have sickle cell disease, and one in 13 Black or African American babies is born with sickle cell trait, according to the CDC.

The FDA approved three medications for sickle cell disease treatment in the past decade.

These include voxelotor (Oxbryta, Global Blood Therapeutics), a once-daily oral therapy that inhibits sickle hemoglobin polymerization; L-glutamine oral powder (Endari, Emmaus Medical), developed to reduce severe sickle cell disease-related complications; and crizanlizumab (Adakveo, Novartis), a humanized anti-P-selectin monoclonal antibody intended to reduce frequency of pain crises.

“While there have been many advancements in treatment, sickle cell disease still negatively impacts patients, causing pain and decreasing life expectancy,” Bartelt said. “In this study, we hoped to bring awareness to the improvement we’ve recently seen in life expectancy, while also showing there is still more research that needs to be done to continue closing the gap.”

Researchers used data from Cosmos — a HIPAA-defined limited data set that represents more than 214 million patients from 1,235 hospitals and more than 26,800 clinics that serve patients in all 50 states and Lebanon.

The data set included information about 5,954 people with sickle cell disease and 5.1 million people without sickle cell disease who died between 2008 and 2022.

Investigators determined median age at death for each population. Two teams — each composed of a clinician and research scientists — analyzed the data independently and reached similar conclusions.

Median age at death for people with sickle cell disease rose from 50 years in 2008 to 60 years in 2022, results showed. Median age at death for individuals without sickle cell disease remained consistent at 75 years during that period.

Bartelt and colleagues stratified age at death for people with sickle cell disease each year to assess how proportions of deaths in each decade of life changed over time.

The percentage of people with sickle cell disease who were aged 60 years or older when they died increased from 38.9% in 2016 to 50% in 2022, results showed.

“Our research looked at the age at death for each year included in our study, meaning that there wasn’t much time for patients to be on these newer treatments,” Bartelt told Healio. “As patients are able to take these medications starting at a younger age and for a longer time, we may see the trend of increased life expectancy continue for this population.”

The findings have important implications for the clinical community, Bartelt said.

“As patients are living longer, we need to be prepared to care for patients with sickle cell disease who may have a variety of comorbidities and additional complexities as they age,” Bartelt said.

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