Sickle cell disease (SCD) is a genetic disorder affecting approximately 4.5 million people worldwide. Annually, more than 300,000 infants are born with SCD worldwide, and this number is expected to rise to 400,000 by 2050. In the United States (US) African Americans (AA) are disproportionately affected by SCD. SCD is characterized by recurrent episodes of vaso-occlusion (i.e., pain crises, acute chest syndrome) leading to high frequency of acute care utilization (i.e., emergency department and hospital admissions) and progressive organ damage throughout the lifespan.
Tennessee (TN) has an overall population of approximately 6.8 million, with a racial distribution of 17% AA, 74% White, and 6% of people of Hispanic or Latino ethnicity. While TN has a diverse mix of rural and urban residents, racial minorities are disproportionately located in four urban areas: Memphis, Nashville, Knoxville and Chattanooga. TN is at the US epicenter for many health risks with mortality rates ranking: 9th in infant mortality, 7th in cancer, 7th in chronic lower respiratory disease, 6th in heart disease, 5th in stroke, and 3rd in influenza/pneumonia. Our preliminary work suggests a high birth prevalence of SCD and well over 3,000 people living with the disease in TN. However, the true number in TN is unknown, and our lack of understanding of the basic epidemiology of SCD is a barrier to optimal care for affected persons.
Following one year of capacity building in which we laid its foundation, we propose to now implement a statewide SCD surveillance program in TN. There is a critical need in our state where a high prevalence of SCD intersects with high health disparities. The following core activities will be completed during the 3 years of this project, with the aim of implementing the TN SCD surveillance program, analyzing the data, and disseminating the findings. In the first year, we will continue to engage our interdisciplinary team to refine the approach and timeline (Act. 1), construct the core dataset (Act. 2) and complete secondary regulatory approvals (Act. 3). In the second year, we will start Data Analysis and Validation of the core dataset and core results (Act. 4) and report the first phase of results to the CDC and stakeholders in TN (Act. 5). In the final 18 months, we will perform advanced data compilation of healthcare utilization and mortality information (Act. 4b). Finally, a key objective of this project is thorough multi-level dissemination of the findings, which includes information for the public health audience, patient-level resources, enriching patient education programs through creation of SCD Fact Sheets for patients and providers that will be made available through different media channels (Act. 7). Finally, we will leverage our extensive experience with collaborative science, evidenced by the track record of our Multiple Principal Investigators (MPIs) to be active partners in collaborative projects within the CDC Sickle Cell Data Collection (SCDC) group (Act. 6).
Our multidisciplinary team of public health researchers, Tennessee Department of Health directors, epidemiologists, computational scientists, adult and pediatric hematologists, infectious and chronic disease surveillance experts, statisticians, community leaders, and patients, have worked together for more than a decade to care and plan resources for our SCD population. We have team members representing every area in the state, including Memphis, Nashville, Chattanooga, and Knoxville. Through organized and purposeful efforts, we have been able to establish cross-disciplinary and cross state collaborations that have laid the foundation for the current application to establish, for the first time, a surveillance program for SCD in our State. Our work during the current capacity-building phase of the CDC SCDC project has been instrumental in program development, positioning us to successfully establish the first statewide SCD surveillance program in TN.