Birth Prevalence of Sickle Cell Disease and County-Level Social Vulnerability - Sickle Cell Data Collection Program, 11 States, 2016-2020.

Sickle cell disease (SCD) remains a public health priority in the United States because of its association with complex health needs, reduced life expectancy, lifelong disabilities, and high cost of care. A cross-sectional analysis was conducted to calculate the crude and race-specific birth prevalence for SCD using state newborn screening program records during 2016-2020 from 11 Sickle Cell Data Collection program states. The percentage distribution of birth mother residence within Social Vulnerability Index quartiles was derived. Among 3,305 newborns with confirmed SCD (including 57% with homozygous hemoglobin S or sickle ß-null thalassemia across 11 states, 90% of whom were Black or African American [Black], and 4% of whom were Hispanic or Latino), the crude SCD birth prevalence was 4.83 per 10,000 (one in every 2,070) live births and 28.54 per 10,000 (one in every 350) non-Hispanic Black newborns. Approximately two thirds (67%) of mothers of newborns with SCD lived in counties with high or very high levels of social vulnerability; most mothers lived in counties with high or very high levels of vulnerability for racial and ethnic minority status (89%) and housing type and transportation (64%) themes. These findings can guide public health, health care systems, and community program planning and implementation that address social determinants of health for infants with SCD. Implementation of tailored interventions, including increasing access to transportation, improving housing, and advancing equity in high vulnerability areas, could facilitate care and improve health outcomes for children with SCD.

Emergency department utilization before and during the COVID-19 pandemic among individuals with sickle cell disease.

BACKGROUND: The emergency department (ED) is a vital source of healthcare for individuals living with sickle cell disease (SCD). Prior research indicates that during the COVID-19 pandemic some individuals with SCD avoided the ED for fear of acquiring COVID-19 or delayed visiting the ED by self-management of symptoms or pain crisis at home. The purpose of the current study was to understand ED utilization rates before and during the pandemic among individuals living with SCD. METHODS: We conducted a retrospective cohort study using population-based SCD surveillance systems in California, Georgia, Michigan, and Tennessee to assess the impact of the pandemic on ED utilization among people with SCD by (1) analyzing trends in monthly ED utilization from January 2019 - December 2020, with specific attention given to immediate changes at the onset of the pandemic; and (2) calculating changes in the volume of utilization by comparing the total ED visits made from March - December 2020 to the same period in 2019, both overall and by demographic characteristics. RESULTS: Across all states, a decline in ED utilization during the onset of the pandemic was seen, with the largest decline seen in those under age 10. By December 2020, utilization rates were higher than their lowest observed month of April 2020, but had not fully returned to pre-COVID levels. During the pandemic, ED visits in each state decreased by as much as 25%, and the number of people with any ED utilization decreased by as much as 26%. CONCLUSIONS: This study confirms and extends the existing literature related to the impact of the pandemic on healthcare utilization patterns in the US, in a unique population with increased healthcare needs.

Assessing Patterns of Telehealth Use Among People with Sickle Cell Disease Enrolled in Medicaid During the Start of the COVID-19 Pandemic.

Background: Telehealth can be defined as using remote technologies to provide health care. It may increase access to care among people with sickle cell disease (SCD). This study examined (1) telehealth use, (2) characteristics of telehealth use, and (3) differences between telehealth users and nonusers among people with SCD during the COVID-19 pandemic. Methods: This was a retrospective analysis of Medicaid claims among four states [California (CA), Georgia (GA), Michigan (MI), Tennessee (TN)] participating in the Sickle Cell Data Collection program. Study participants were individuals ≥1 year old with SCD enrolled in Medicaid September 2019-December 2020. Telehealth encounters during the pandemic were characterized by provider specialty. Health care utilization was compared between those who did (users) and did not (nonusers) use telehealth, stratified by before and during the pandemic. Results: A total of 8,681 individuals with SCD (1,638 CA; 3,612 GA; 1,880 MI; and 1,551 TN) were included. The proportion of individuals with SCD that accessed telehealth during the pandemic varied across states from 29% in TN to 80% in CA. During the pandemic, there was a total of 21,632 telehealth encounters across 3,647 users. In two states (MI and GA), over a third of telehealth encounters were with behavioral health providers. Telehealth users had a higher average number of health care encounters during the pandemic: emergency department (pooled mean = 2.6 for users vs. 1.5 for nonusers), inpatient (1.2 for users vs. 0.6 for nonusers), and outpatient encounters (6.0 for users vs. 3.3 for nonusers). Conclusions: Telehealth was frequently used at the beginning of the COVID-19 pandemic by people with SCD. Future research should focus on the context, facilitators, and barriers of its implementation in this population.

Comparative 60-day effectiveness of bivalent versus monovalent mRNA vaccines in Shelby County: a population-level analysis.

Background: Two monovalent mRNA vaccines, available in December 2020, were demonstrated to have high efficacy against both the original SARS-CoV-2 strain and variants circulating through the summer and into the fall of 2021. In the context of the Omicron/BA.1 variant, which was predominant from late fall 2021 into winter of 2022 in the United States, and subsequent Omicron subvariants that have been predominant thereafter, vaccine effectiveness of the monovalent mRNA vaccine option is attenuated. Objectives: We aim to investigate the relative effectiveness of the bivalent booster compared to the monovalent booster against SARS-CoV-2 infection in the 60 days following administration in Shelby County, TN. Design: This observational population-based cohort study utilizes COVID-19 surveillance data to identify adults who were vaccinated with a monovalent booster dose between August 1, 2022 and August 30, 2022 or a bivalent booster dose between September 1, 2022 and September 30, 2022. Both groups were followed for COVID-19 status for 60 days from their administration date. Methods: We calculated incidence rates with 95% confidence intervals and propensity-adjusted hazard ratios with 95% confidence intervals of COVID-19 diagnosis in the 60 days following administration of the booster dose between the bivalent group and the monovalent group. Stratified analysis was conducted by age group (18-34, 35-64, and 65+ years old). Results: The incidence of reported SARS-CoV-2 infection was substantially higher for those who received the monovalent booster, across age groups. Overall, we observed a 51% lower hazard of infection during the study period among those who received the bivalent booster, compared to the monovalent booster. Conclusion: These results support and extend prior findings that the bivalent booster dose may be more effective in preventing infection against the Omicron sub-variants of SARS-CoV-2.

Association Between mRNA Vaccination and Infection From SARS-CoV-2 During the Delta and Omicron BA.1 Waves: A Population-Level Analysis.

Introduction: COVID-19 mRNA vaccine protection against the Omicron variant of SARS-CoV-2 has been shown to be attenuated. Previous research in Shelby County, Tennessee found that vaccine effectiveness might differ by age in the Omicron surge, a finding not reported for other variants. To assess whether patterns in vaccine effectiveness by age group differed on the basis of the predominant strain of SARS-CoV2, we evaluated vaccine effectiveness in Shelby County, Tennessee by age group in the Delta wave and Omicron BA.1 (Omicron) wave. Methods: Case and vaccination statuses of residents were assessed using COVID-19 surveillance data. Age was stratified as 18-34, 35-64, and ≥65 years. Vaccination groups included unvaccinated, fully vaccinated, and fully vaccinated + booster. Person time was counted in each wave by vaccination status until the time of a positive reported COVID-19 test or until the end of the study period. Results: Incidence of COVID-19 was much higher during the Omicron wave than during the Delta wave across all vaccination groups. During the Delta wave, among adults, 79.2% fewer cases were identified in those fully vaccinated and 94.8% fewer in those fully vaccinated + booster, compared with 40.2% and 66.7%, respectively, in the Omicron wave, compared with those who were unvaccinated. Conclusions: This study found evidence that vaccine effectiveness differed by age group during the Omicron wave, where the same pattern was not prominent in the Delta wave. Further analysis investigating the influence of behavior patterns and other potential confounders on vaccine effectiveness would be useful in further understanding the relationship between age and vaccine effectiveness.

Common data model for sickle cell disease surveillance: considerations and implications.

Objective: Population-level data on sickle cell disease (SCD) are sparse in the United States. The Centers for Disease Control and Prevention (CDC) is addressing the need for SCD surveillance through state-level Sickle Cell Data Collection Programs (SCDC). The SCDC developed a pilot common informatics infrastructure to standardize processes across states. Materials and Methods: We describe the process for establishing and maintaining the proposed common informatics infrastructure for a rare disease, starting with a common data model and identify key data elements for public health SCD reporting. Results: The proposed model is constructed to allow pooling of table shells across states for comparison. Core Surveillance Data reports are compiled based on aggregate data provided by states to CDC annually. Discussion and Conclusion: We successfully implemented a pilot SCDC common informatics infrastructure to strengthen our distributed data network and provide a blueprint for similar initiatives in other rare diseases.

Real-World Association Between mRNA Vaccination and Infection From the Omicron Strain of SARS-CoV-2: A Population-Level Analysis.

Introduction: Two mRNA vaccines approved in the U.S. have high efficacy against COVID-19 disease from the original strain of SARS-CoV-2. We evaluated the population-level association between vaccination status and COVID-19 infection by age group during the initial wave of the Omicron variant in a diverse population in the Mid-South U.S. Methods: In this observational population-based cohort study, vaccination information and positive COVID-19 cases in Shelby County, Tennessee, from December 12, 2021 through January 22, 2022 were collected from surveillance data at the Shelby County Health Department (Memphis, Tennessee). Exposure groups included individuals who were unvaccinated, were fully vaccinated, and were fully vaccinated + booster. We calculated incidence rates of COVID-19 diagnosis per person-year among county adult (aged 18+ years) residents in crude form and stratified by age group. Results: In this population-based study, we identified 64.56% fewer COVID-19 infections in the fully vaccinated + booster group and 41.08% fewer in the fully vaccinated group than in the unvaccinated group. Conclusions: These results confirm and extend the findings of recent immunologic and epidemiologic studies in a racially diverse region of the Mid-South U.S. In stratified analysis, we also found evidence suggesting that vaccine protection against Omicron may increase with age.

Medicaid Coverage in Early Childhood for Children With Sickle Cell Disease.

This cohort study examines patterns of Medicaid coverage in the first 3 years of life among children with sickle cell disease across 5 states.