Surveillance for Sickle Cell Disease - Sickle Cell Data Collection Program, Two States, 2004-2018

Area of Science:

• Public Health
• Epidemiology
• Genetics

Background:

• Sickle cell disease (SCD) is an inherited blood disorder causing severe complications and reduced life expectancy.
• Effective public health surveillance is vital for managing SCD, improving healthcare access, and addressing unmet needs.

Purpose of the Study:

• To describe the establishment, maintenance, and expansion of the CDC's Sickle Cell Data Collection (SCDC) program.
• To characterize the epidemiology of SCD and inform strategies for improving health outcomes.

Main Methods:

• The SCDC program utilizes population-based tracking with comprehensive data linkages across multiple state health systems.
• Data sources include Medicaid, Children's Health Insurance Program databases, death certificates, newborn screening, hospital records, and clinical reports.
• A case definition algorithm classifies confirmed and probable SCD cases based on laboratory confirmation or clinical diagnosis.

Main Results:

• From 2004-2018, cumulative SCD cases in California and Georgia were 9,875 and 14,777, respectively.
• Pediatric SCD patients comprised 27% in California and 40% in Georgia; 63% of genotyped patients had sickle cell anemia.
• Surveillance identified disparities in access to SCD specialty care, with significant travel distances for some patients.

Conclusions:

• The SCDC program's expansion to 11 states demonstrates the feasibility of standardized, rigorous surveillance methods.
• Findings highlight the need for increased access to SCD specialty clinics and inform targeted public health interventions.
• The SCDC program provides a framework for improving care, policy development, and research for individuals with SCD.