S73 Epidemiology of cystic fibrosis-related deaths in the united states, 1999–2023: a CDC WONDER-based study

Abstract

<h3>Background</h3> Cystic fibrosis (CF) is a hereditary multisystem disorder predominantly affecting the respiratory system, contributing significantly to morbidity and mortality in the developed nations, despite advancement in treatments. Characterizing mortality patterns across demographic and geographic populations is essential for developing targeted prevention and management strategies. Therefore, we analyzed temporal mortality trends in cystic fibrosis across diverse populations in the United States from 1999–2023. <h3>Methods</h3> Data were extracted from the CDC WONDER database (1999–2023) to identify mortality rates among individuals aged ≥1 year with CF listed as the underlying cause of death (ICD-10: E84.0, E84.1, E84.8, E84.9). Age-adjusted mortality rates (AAMRs) per 100,000 population were calculated and stratified by sex, age group, race/ethnicity, geographic region, and place of death. Temporal trends were analyzed using Joinpoint regression to estimate average annual percent change (AAPC). <h3>Results</h3> From 1999–2023, 11,997 deaths were reported among individuals with CF across all age groups (&lt;1 to ≥65 years). The AAMR decreased significantly from 0.19 in 1999 to 0.09 in 2023 (AAPC: -3.1%; p&lt;0.000001), with accelerated decline in recent years. Both sexes demonstrated comparable average AAMRs (0.15), though with different rates of decline: men (AAPC: -2.7%; 95% CI: -4.17 to -1.29; p=0.0002) and women (AAPC: -2.9%; 95% CI: -4.19 to -1.66; p=0.000008). By race/ethnicity, non-Hispanic populations exhibited higher overall AAMRs compared to Hispanics (0.18 vs. 0.06), with incongruent trends observed among non-white races and Hispanic populations. Regionally, the Midwest recorded the highest AAMR (0.17), followed by the South (0.16), Northeast (0.15), and West (0.13). Age-stratified analysis revealed peak mortality in the 15–34 years group (0.32/100,000), followed by the 35–64 years group (0.12/100,000). Most deaths occurred in inpatient medical facilities (8,260 deaths; 68.8%). <h3>Conclusion</h3> CF mortality rates declined significantly over two decades, with comparable reductions in both sexes. However, substantial disparities persist, with young adults (15–34 years) experiencing high mortality rates and notable racial/ethnic differences. Regional disparities were evident across geographic areas. While these findings suggest improved CF management and care, they underscore the critical need for targeted interventions addressing persistent demographic and geographic disparities to ensure equitable outcomes across all populations.