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Original Research: Genetic and Developmental Disorders |

Assessing Differences in Mortality Rates and Risk Factors Between Hispanic and Non-Hispanic Patients With Cystic Fibrosis in California

MyMy C. Buu, MD; Lee M. Sanders, MD, MPH; Jonathan A. Mayo, MPH; Carlos E. Milla, MD; Paul H. Wise, MD, MPH
Author and Funding Information

FUNDING/SUPPORT: This study was supported by the Cystic Fibrosis Foundation, 2nd year Clinical Fellowship [Grant BUU10B0]; the Ernest and Amelia Gallo Endowed Postdoctoral Fellowship; the Lucile Packard Foundation for Children’s Health; Stanford National Institutes of Health-National Center for Advancing Translational Sciences-Clinical and Translational Science Award (NIH-NCATS-CTSA [Grant UL1 TR001085]); and the Child Health Research Institute of Stanford University.

CORRESPONDENCE TO: MyMy C. Buu, MD, Center for Excellence in Pulmonary Biology, Stanford University School of Medicine, 770 Welch Rd, Ste 350, Palo Alto, CA 94304


Copyright 2016, American College of Chest Physicians. All Rights Reserved.


Chest. 2016;149(2):380-389. doi:10.1378/chest.14-2189
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Background  Over the past 30 years, therapeutic advances have extended the median lifespan of patients with cystic fibrosis (CF). Hispanic patients are a vulnerable subpopulation with a high prevalence of risk factors for worse health outcomes. The consequences of these differences on health outcomes have not been well described. The objective of this study was to characterize the difference in health outcomes, including mortality rate, between Hispanic and non-Hispanic patients with CF.

Methods  This study is a retrospective analysis of CF Foundation Patient Registry data of California residents with CF, diagnosed during or after 1991, from 1991 to 2010. Ethnicity was self-reported. The primary outcome was mortality. Hazard ratios were estimated from a Cox regression model, stratified by sex, and adjusted for socioeconomic status, clinical risk factors, and year of diagnosis.

Results  Of 1,719 patients, 485 (28.2%) self-identified as Hispanic. Eighty-five deaths occurred, with an overall mortality rate of 4.9%. The unadjusted mortality rate was higher among Hispanic patients than among non-Hispanic patients (9.1% vs 3.3%, P < .0001). Compared with non-Hispanic patients, Hispanic patients had a lower survival rate 18 years after diagnosis (75.9% vs 91.5%, P < .0001). Adjusted for socioeconomic status and clinical risk factors, Hispanic patients had an increased rate of death compared with non-Hispanic patients (hazard ratio, 2.81; 95% CI, 1.70-4.63).

Conclusions  Hispanic patients with CF have a higher mortality rate than do non-Hispanic patients, even after adjusting for socioeconomic status and clinical severity. Further investigation into the mechanism for the measured difference in lung function will help inform interventions and improve the health of all patients with CF.

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