Abstract
Background: Sickle Cell Disease (SCD) remains a major public health concern in the United States, with substantial geographic and demographic disparities. Despite medical advances, disease burden persists across age, sex, and state lines. The Global Burden of Disease (GBD) 2021 dataset provides a comprehensive platform to assess national SCD trends. This study aimed to quantify current SCD burden and evaluate temporal trends using the Estimated Annual Percentage Change (EAPC) in prevalence, mortality, and disability-adjusted life years (DALYs).
Methods: Data were obtained using the Institute for Health Metrics and Evaluation (IHME) Global Burden of Disease (GBD) 2021 Results Tool. Age-standardized prevalence, death, and DALY rates were analyzed by state and sex. EAPCs were calculated to assess temporal trends. Age-stratified crude mortality and DALY numbers identified high-burden subgroups. Descriptive statistics were performed using Microsoft Excel, with focus on sex- and age-specific burden and geographic variation.
Results: The age-standardized prevalence rate for SCD showed a declining trend with an EAPC of –0.64% (95% CI: –0.83% to –0.44%). The age-standardized death rate also declined with an EAPC of –0.82% (95% CI: –0.95% to –0.24%). The most pronounced decrease was seen in the DALY rate, with an EAPC of –1.06% (95% CI: –1.15% to –0.97%), indicating gradual improvement in disease-related morbidity and mortality over time.
Geographically, the highest prevalence and incidence rates were observed in the District of Columbia, Maryland, and Delaware. The lowest rates were seen in North Dakota, Montana, and Maine. Age-standardized mortality was highest in Mississippi, South Carolina, and Louisiana, while DALY rates peaked in Mississippi, South Carolina, and Alabama. Idaho, Hawaii, and Montana consistently reported the lowest burden.
Across most states, females had higher prevalence and incidence rates, though a few northern and western states showed male predominance. Age-specific prevalence peaked in the 15–19 age group for both sexes, with 3,201.53 cases in females and 2,881.27 in males. DALY burden was highest in males aged 35–39 years (1,815.37) and in females aged 30–34 years (1,962.98). Crude deaths were concentrated in the 30–44 age range for both sexes. Infants and neonates showed the highest incidence, mortality, and DALY rates, while children aged 5–14 and adults aged 75+ had the lowest.
Sex-specific analysis revealed a clear female predominance in prevalence and DALY counts across nearly all age groups. Death rates showed mixed trends, with male predominance in early adulthood and female predominance in older age groups. Incidence also showed consistent female predominance.
Conclusion: This analysis highlights the persistent and unequal burden of SCD across the U.S., with higher prevalence and mortality concentrated in southern and mid-Atlantic states. While female patients exhibit higher prevalence and DALY rates overall, early adult males face disproportionately high mortality. The highest burden in early life underscores the lifelong impact of SCD and the need for robust neonatal and pediatric services.
The declining EAPC trends in prevalence, mortality, and DALY rates suggest slow but meaningful improvements in disease outcomes. To further reduce disparities and disease burden, targeted interventions should include: expansion of universal newborn screening programs; improved access to hydroxyurea and curative therapies such as stem cell transplantation; increased funding for comprehensive sickle cell centers; community-based education campaigns to improve health literacy and treatment adherence; and enhanced surveillance systems to better monitor outcomes and resource allocation. These strategies are essential to achieving equity and optimizing care for individuals living with SCD.
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