Long-Term Survival Rates of a Sickle Cell Disease Cohort in Saudi Arabia: A 2009-2023 Observational Study

Background: Sickle cell disease (SCD) is prevalent in Saudi Arabia. This study evaluates the long-term survival rates of a cohort of SCD patients. Methods: This observational cohort study was conducted at King Saud University Medical City from January 2009 to September 2023. We enrolled 223 SCD patients between 2009 and 2014, collecting comprehensive data at baseline and during follow-up. The primary endpoint was overall survival. Results: The cohort had a median follow-up of 11.5 years, totaling 2,118 patient-years. The recent median age was 28.9 years (12.2-63.8). The survival rates at ages 20, 30, 40, and 50 years were 100%, 98.4%, 95.1%, and 89.0%, respectively, with no mortality observed before the age of 20 years. The incidence of mortality was 0.28 deaths per 100 patient-years. Among the six deaths (2.7%), causes included non-Hodgkin lymphoma, acute chest syndrome, and a sepsis-like condition, with three unknown causes. The median age of death was 36.3 years. The increased use of hydroxyurea, from 47% to 80%, was associated with reduced pain crises and acute chest syndrome, and improved hemoglobin and HbF levels. Of the patients, 43 (19.2%) were lost to follow-up, 16 (7.2%) were referred for stem cell transplant, and 16 (7.2%) were followed at other institutions. Conclusions: This study highlights excellent survival rates for SCD patients in our cohort. Nonetheless, the considerable loss to follow-up highlights the need for strategies to address this issue and larger multicenter studies to confirm our results.