The Prevalence of Sickle Cell Disease in Colorado and Methodologies of the Colorado Sickle Cell Data Collection Program: Public Health Surveillance Study

Abstract

Abstract BackgroundSickle cell disease (SCD) is a genetic blood disorder that affects approximately 100,000 individuals in the United States, with the highest prevalence among Black or African American populations. While advances in care have improved survival, comprehensive state-level data on the prevalence of SCD remain limited, which hampers efforts to optimize health care services. To address this gap, the Colorado Sickle Cell Data Collection (CO-SCDC) program was established in 2021 as part of the Centers for Disease Control and Prevention’s initiative to enhance surveillance and public health efforts for SCD. ObjectiveThe objectives of this study were to describe the establishment of the CO-SCDC program and to provide updated estimates of the prevalence and birth prevalence of SCD in Colorado, including geographic dispersion. Additional objectives include evaluating the accuracy of case identification methods and leveraging surveillance activities to inform public health initiatives. MethodsData were collected from Health Data Compass (a multi-institutional data warehouse) containing electronic health records from the University of Colorado Health and Children’s Hospital Colorado for the years 2012‐2020. Colorado newborn screening program data were included for confirmed SCD diagnoses from 2001 to 2020. Records were linked using the Colorado University Record Linkage tool and deidentified for analysis. Case definitions, adapted from the Centers for Disease Control and Prevention’s Registry and Surveillance System for Hemoglobinopathies project, classified cases as possible, probable, or definite SCD. Clinical validation by hematologists was performed to ensure accuracy, and prevalence rates were calculated using 2020 US Census population estimates. ResultsIn 2019, 435 individuals were identified as living with SCD in Colorado, an increase of 16%‐40% over previous estimates, with the majority (n=349, 80.2%) identifying as Black or African American. The median age of individuals was 19 years. The prevalence of SCD was highest in urban counties, with concentrations in Arapahoe, Denver, and El Paso counties. Birth prevalence of SCD increased from 11.9 per 100,000 live births between 2010 and 2014 to 20.1 per 100,000 live births between 2015 and 2019 with 58.5% (n=38) of cases being hemoglobin (Hb) SS or HbSβ0 ConclusionsThe CO-SCDC program successfully established the capacity to perform SCD surveillance and, in doing so, identified baseline prevalence estimates for SCD in Colorado. The findings highlight geographic dispersion across Colorado counties, highlighting the need for equitable access to specialty care, particularly for rural populations. The combination of automated data linkage and clinical validation improved case identification accuracy. Future efforts will expand surveillance to include claims data to better capture health care use and address potential underreporting. These results will guide public health interventions aimed at improving care for individuals with SCD in Colorado.