Study: Mortality rate disparity between sickle cell, cystic fibrosis
Researchers call for policy reform to address rising SCD mortality rates
Mortality rates related to sickle cell disease (SCD) have increased in the U.S. in recent decades, while mortality for people with cystic fibrosis (CF) has declined over the same time period, a study shows.
“Several factors contribute to this stagnation, including under-resourced care systems, limited access to first-line therapies and challenges with new therapies, such as cost and access,” Hiluf Abraha, a doctoral student at the University of South Carolina and co-author of the study, said in a university news story.
The study, “Mortality Rate Trends for Sickle Cell Disease and Cystic Fibrosis in the US,” was published as a research letter in JAMA Pediatrics. Based on the findings, the scientists are calling for federal action to help address these differences.
SCD is a blood disorder that primarily affects people of African descent, while CF is a condition marked by abnormal mucus production that predominantly affects people of European descent. Both genetic disorders can lead to life-threatening complications, and in recent years, new therapies have been approved for both in the U.S.
Life expectancy gains, losses
Using data from the National Center for Health Statistics, Abraha and his colleagues compared mortality rates from 2008 to 2023, when there were 15,242 deaths attributable to SCD and 7,451 to CF.
The overall age-adjusted mortality rate for SCD was significantly higher than that for CF — 0.29 vs. 0.15 per 100,000 people.
Moreover, they found mortality rates for CF declined across patients of all ages, apart from those 65 and older. But in sickle cell, mortality rates increased over the 2010s and into the 2020s in people of all ages, except in those 24 and younger. These gains for CF patients are “attributed to advances in therapeutics and improved care.”
The increasing mortality rates seen in sickle cell patients, however, reflect “limited progress in treatment and research,” wrote the researchers, who said “several factors contribute to this stagnation, including under-resourced care systems, limited access to first-line therapies, such as hydroxyurea, and challenges with new therapies.” The cost of new cutting-edge therapies likely also plays a role, according to the scientists.
They called for the U.S. federal government to help improve outcomes for SCD patients by expanding Medicaid, which offers health insurance to low-income Americans, and by allowing Medicaid to cover new, expensive treatments.
“Decoupling financial incentives from patient costs through federally funded programs for underfunded diseases could facilitate the development of effective treatments,” the researchers wrote. “Without action, mortality disparities will persist, highlighting the need for policy reform and funding to ensure equitable SCD care.”
About the Author
