A review of U.S. mortality statistics found the sickle cell disease (SCD) mortality rate has significantly decreased over the past 40 years as treatments have improved, but the mortality rate has increased for young adults.

SCD is a blood disorder that can cause a variety of symptoms in patients, including stroke and cognitive impairment. It is one of the most common inherited disorders in the U.S. that primarily affects people of African ancestry, with 10% of African Americans having sickle cell trait and 1 in 365 having SCD. 

The University of Cincinnati’s Kristine Karkoska, MD, said the most significant improvement to SCD treatment in recent decades was the introduction of the medication hydroxyurea. Other improvements in care include the implementation of universal newborn screening in 2006 that allows for early diagnosis and establishment of care and the establishment of comprehensive SCD care centers, particularly in pediatric settings.

From 2010-2020, the overall SCD crude mortality rate was 1.1 per 100,000 Black individuals, a decrease from 1.2 per 100,000 from 1999-2009. Of the 5,272 SCD-related deaths from 2010-20, 61% listed SCD as the primary cause of death, with cardiovascular disease (28%), accidents and cerebrovascular disease (both 7%), malignancy (6%), septicemia (4.8%) and renal disease (3.8%) being the next most common primary causes of death.

“The decrease in overall mortality was what we expected and had hoped to see,” said Karkoska, assistant professor of internal medicine in UC’s College of Medicine. “I was most struck by the degree of non-SCD related chronic diseases that were included in mortality data.”

The crude mortality rate for patients aged 15-19 increased from 0.9 per 100,000 in 1999-2009 to 1.4 per 100,000 in 2010-20, which Karkoska said highlights the need for better care as patients transition from pediatric to adult care. While there are no easy fixes and research is ongoing to improve the transition of care for many chronic diseases that begin in childhood, Karkoska said communication between pediatric and adult programs is vital.

“In addition, the pediatric program needs to treat the young person as an adult, while the adult program needs to recognize that newly-transitioned young adults often require additional oversight initially,” she said. “I have been uniquely positioned to see this as a pediatric hematologist who now cares for young adults.”