Abstract
RATIONALE: Patients of Hispanic origin with cystic fibrosis (CF) are the largest growing minority, representing 8.5% of patients with CF in the United States. No national survival analysis of this group has ever been undertaken. OBJECTIVES: We aimed to determine whether Hispanic ethnicity within the CF population is associated with worse outcomes and whether any geographic differences exist. METHODS: Using U.S. Cystic Fibrosis Foundation Patient Registry data from 2010 to 2014, we performed a retrospective cohort analysis comparing survival rates between Hispanics and non-Hispanics using Kaplan-Meier and Cox regression analysis. A subject's residence was categorized into geographic regions based on U.S. Census Bureau data: Northeast, Midwest, West, and South. MEASUREMENTS AND MAIN RESULTS: A total of 29,637 patients were included in the study; 2,493 identified themselves as Hispanic. Hispanics had a lower survival probability overall, with a mean age of death of 22.4 ± 9.9 years compared with non-Hispanics of 28.1 ± 10.0 years (P < 0.0001). Multivariate Cox proportional hazards modeling revealed that Hispanic patients with CF had a 1.27 times higher rate of death compared with non-Hispanics (95% confidence interval, 1.05-1.53) after adjusting for covariates including age, sex, genetic mutations, bacterial cultures, lung function, body mass index, use of CF respiratory therapies, low socioeconomic status, pancreatic enzyme use, and CF-related diabetes. When analyzed by region, Hispanics in the Midwest, Northeast, and West had shorter median survivals compared with non-Hispanics, which was not demonstrated in the South. CONCLUSIONS: Patients with CF of Hispanic origin have a higher mortality rate than non-Hispanic patients with CF. This pattern was seen in the Midwest, Northeast, and West but not in the South.