Rates of strokes in Californians with sickle cell disease in the post-STOP era

Area of Science:

• Neurology
• Hematology
• Public Health

Background:

• Neurovascular complications, including stroke and transient ischemic attacks (TIAs), are significant causes of morbidity in sickle cell disease (SCD).
• The Stroke Prevention Trial in Sickle Cell Anemia (STOP) trial established chronic transfusions as the standard of care for high-risk children with SCD.
• Understanding stroke incidence and trends in SCD patients before and after the STOP trial is essential for refining prevention strategies.

Purpose of the Study:

• To determine the cumulative incidence (CMI) and rates of primary and recurrent strokes and TIAs in individuals with SCD.
• To analyze trends in stroke and TIA incidence in SCD patients before and after the 1998 STOP trial.
• To identify risk factors associated with cerebrovascular events in the SCD population.

Main Methods:

• Utilized statewide data from the California Department of Health Care Access and Innovation (1991-2019).
• Included 7636 patients diagnosed with SCD.
• Calculated cumulative incidence and incidence rates for ischemic stroke, intracranial hemorrhage (ICH), and TIAs, comparing pre- and post-STOP trial periods.

Main Results:

• The cumulative incidence of ischemic stroke by age 60 was 13.5%, and for intracranial hemorrhage (ICH) was 6.8%.
• Ischemic stroke rates increased in children (<18 years) and adults (31-50 years) from 2010-2019 compared to the previous decade.
• Increased rates of ICH in 18-30 year olds and TIAs in children (<18 years) were observed in the later decade; hypertension and hyperlipidemia were identified as risk factors.

Conclusions:

• Stroke and TIA incidence in SCD patients shows concerning trends, with increasing rates in recent years.
• The findings highlight the ongoing need for robust stroke prevention strategies in SCD, particularly in adults.
• Emphasis on managing modifiable cerebrovascular risk factors, such as hypertension and hyperlipidemia, is critical for reducing stroke burden in SCD.