Pulmonary function disparities exist and persist in Hispanic patients with cystic fibrosis: A longitudinal analysis

Pediatr Pulmonol. 2017 Dec;52(12):1550-1557. doi: 10.1002/ppul.23884. Epub 2017 Oct 30.

Abstract

Background: Hispanic patients with cystic fibrosis (CF) have decreased life expectancy compared to non-Hispanic white patients. Pulmonary function is a main predictor of life expectancy in CF. Ethnic differences in pulmonary function in CF have been understudied. The objective was to compare longitudinal pulmonary function between Hispanic and non-Hispanic white patients with CF.

Methods: This cohort study of 15 018 6-25 years old patients in the CF Foundation Patient Registry from 2008 to 2013 compared FEV1 percent predicted and longitudinal change in FEV1 percent predicted in Hispanic to non-Hispanic white patients. We used linear mixed effects models with patient-specific slopes and intercepts, adjusting for 14 demographic and clinical variables. We did sub-analyses by CFTR class, F508del copies, and PERT use.

Results: Hispanic patients had lower FEV1 percent predicted (79.9%) compared with non-Hispanic white patients (85.6%); (-5.8%, 95%CI -6.7% to -4.8%, P < 0.001), however, there was no difference in FEV1 decline over time. Patients on PERT had a larger difference between Hispanic and non-Hispanic white patients in FEV1 percent predicted than patients not on PERT (-6.0% vs -4.1%, P = 0.02). The ethnic difference in FEV1 percent predicted was not statistically significant between CFTR classes (Class I-III: -6.1%, Class IV-V: -5.9%, Unclassified: -5.7%, P > 0.05) or between F508del copies (None: -7.6%, Heterozygotes: -5.6%, Homozygotes: -5.3%, P > 0.05).

Conclusions: Disparities in pulmonary function exist in Hispanic patients with CF early in life and then persist without improving or worsening over time. It is valuable to investigate the factors contributing to pulmonary function in Hispanic patients with CF.

Keywords: cystic fibrosis (CF); epidemiology; healthcare disparities; hispanic latino; pulmonary function testing (PFT); social dimensions of pulmonary medicine.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Cohort Studies
  • Cystic Fibrosis / genetics
  • Cystic Fibrosis / physiopathology*
  • Cystic Fibrosis Transmembrane Conductance Regulator / genetics
  • Female
  • Forced Expiratory Volume
  • Heterozygote
  • Hispanic or Latino / statistics & numerical data*
  • Homozygote
  • Humans
  • Lung / physiopathology
  • Male
  • Young Adult

Substances

  • Cystic Fibrosis Transmembrane Conductance Regulator