Mortality Review of Young Adults with Sickle Cell Disease

Introduction

Sickle cell disease (SCD), a common inherited blood disorder, is associated with increased mortality in young adulthood. The extent to which these deaths result from SCD complications or other causes is not well established.

Methods

This single center retrospective chart review conducted from January 1, 2013, and November, 7, 2023 included adults with SCD identified in our institution’s electronic medical record who died between age 18 and 30 years. Three study team members (CN, PC, EP) determined causes of death using autopsy reports, emergency medical services reports, and clinical documentation.

Discrepancies in data interpretation among the study team were resolved through iterative discussions.

Results

There were 27 deaths, 16 (59%) in men, median age of death of 26 years (range 22-28), and 21 (78%) had hemoglobin SS disease. The most common causes of death were respiratory failure (33%) and infection (26%). Three deaths (11%) were attributed to gun violence and four deaths (14%) to substance use (15%). Half of the study cohort (52%) died in a hospital, divided between the intensive care unit and the emergency department. SCD complications included history of acute chest syndrome (N=25, 93%), hepatopathy (N=13, 48%), central nervous events including stroke (N=13, 48%), nephropathy (N=11, 41%), and one failed bone marrow transplant. SCD therapies included use of hydroxyurea (N=25, 93%) and chronic transfusions (N=18, 67%). Eighteen individuals (67%) had established care at our institution’s SCD center.

Conclusion

Most young adults with SCD in this cohort died with end-organ disease complications, but 26% of deaths are attributable Baltimore’s dual public health crises, gun violence and the opioid epidemic. Individuals in this cohort had a high burden of chronic disease and end organ damage that contributed to a cascade of events leading death. Findings support the need for tailored comprehensive care for young adults with SCD.