Due to the lack of national surveillance data, the precise incidence and prevalence of sickle cell disease (SCD) in the United States are unknown. There is an urgent need for state-based surveillance programs to have more accurate incidence and prevalence counts of SCD and to have a better understanding of the geographic distribution and health care needs of the affected population. This North Carolina (NC) application has Core and Optional components over a three-year performance period. The purposes of the Core Component are to implement a high-quality state-wide, population-based SCD surveillance program in NC and disseminate aggregate-level reports on surveillance findings to stakeholders. We will curate and link multiple state-wide data sources, including NC Medicaid claims, newborn screening, death records, NC emergency department and hospital discharge records, and clinical databases to achieve the following outcomes:
1. Improved accuracy in the description of the epidemiology and geographic distribution of SCD in NC
2. Improved accuracy in the description of healthcare utilization patterns of SCD in NC
3. Improve capacity for stakeholders to make informed decisions based on SCD surveillance findings.
The purpose of the Optional Component is to conduct individual-level data analyses and disseminate results that will inform stakeholders and improve the care of people with SCD. We aim to achieve the following outcomes:
1. Improved description of adherence to SCD Evidence-Based guidelines (hydroxyurea, penicillin prophylaxis, and transcranial Doppler screening)
2. Improved description of the use and cost of newer disease modifying therapies
3. Improved description of opioid use patterns
4. Improved description of how social determinants of health (e.g. neighborhood characteristics, access to SCD healthcare) contribute to early mortality and poor health outcomes in SCD in NC
5. Improved description of health outcomes and access to healthcare for vulnerable subpopulations in NC (youth transitioning to adult care, pregnant women, and the aging population with SCD)
6. Improved description of the epidemiology of confirmed COVID-19 in SCD
7. Improved capacity for stakeholders to make informed decisions to improve adherence to SCD Evidence-Based guidelines and mitigate modifiable factors contributing to early mortality and poor health outcome among vulnerable subpopulations with SCD in NC
NC is well poised to implement a SCD surveillance program because of a relatively large SCD population, existing state SCD infrastructure including state-level databases, our team’s expertise in SCD surveillance, and strong collaborations among stakeholders. A state-wide SCD surveillance program in NC allows for the collection and analysis of health data to accurately describe the epidemiology of SCD, identify population healthcare needs, and inform healthcare practices and health policy for SCD in the state. Implementing a surveillance system also allows NC to connect with other states participating in SCD surveillance for the collective purpose of data sharing and advancing our understanding of SCD-related healthcare, health outcomes, and health disparities across states and nationally, ultimately leading to a better understanding of the epidemiology and healthcare needs of the SCD population in the U.S.