A recent study published in Blood reveals that the incidence of stroke continues to rise for both adults and children living with sickle cell disease (SCD), despite the implementation of standards of care established by the Stroke Prevention Trial in Sickle Cell Anemia (STOP). Individuals with SCD are at a higher risk for cerebrovascular events (CVEs), such as ischemic or hemorrhagic strokes, due to abnormalities in their red blood cells. The STOP trial, conducted in 1998, demonstrated that chronic transfusions could reduce the occurrence of CVEs in high-risk children with SCD. However, the risk of stroke was found to increase with age, doubling every 20 years.
Research conducted by Dr. Ted Wun and his team using data from California’s Emergency Department Utilization and Patient Discharge Data databases identified 733 SCD patients who had experienced at least one CVE, including strokes and transient ischemic attacks. The study found that the cumulative incidence of hemorrhagic stroke increased significantly with age, with the highest rates occurring in the most recent decade despite a brief decline following the STOP study. These trends indicate that the guidelines established by STOP may not have been effectively implemented, leading to an increase in strokes among SCD patients.
The study also looked at potential reasons for the post-STOP increase in strokes, such as decreased adherence to screening guidelines, technological advancements in stroke diagnosis, and low usage of preventative therapies like blood transfusions or hydroxyurea. Modifiable risk factors for strokes in SCD patients were identified, including high blood pressure, high cholesterol, and prior TIAs. Patients with risk factors like acute chest syndrome or liver failure were more likely to experience hemorrhagic strokes. The findings highlight the importance of screening for and managing these risk factors in adults living with SCD.
Despite the study’s limitations, such as a lack of data on tobacco use and SCD genotype, the researchers emphasize the need for increased focus on preventing strokes in adults with SCD. As individuals with SCD are living longer and facing an elevated risk of stroke, interventions to address modifiable risk factors become critical. The findings underscore the importance of implementing preventive measures and screenings for high-risk individuals to reduce the incidence of strokes in this population.
Moving forward, the researchers hope that this study will prompt further interventional and prospective research on stroke and SCD in adults. By gaining a better understanding of the factors contributing to strokes in SCD patients, healthcare providers can develop more effective strategies for prevention and management. Ultimately, addressing modifiable risk factors and implementing appropriate guidelines can help improve outcomes and reduce the burden of strokes among individuals living with SCD.
