Survival and Lung Transplant Outcomes for Individuals With Advanced Cystic Fibrosis Lung Disease Living in the United States and Canada: An Analysis of National Registries

Chest. 2021 Sep;160(3):843-853. doi: 10.1016/j.chest.2021.04.010. Epub 2021 Apr 17.

Abstract

Background: Understanding how health outcomes differ for patients with advanced cystic fibrosis (CF) lung disease living in the United States compared with Canada has health policy implications.

Research question: What are rates of lung transplant (LTx) and rates of death without LTx in the United States and Canada among individuals with FEV1 < 40% predicted?

Study design and methods: This was a retrospective population-based cohort study, 2005 to 2016, using the US CF Foundation, United Network for Organ Sharing, and Canadian CF registries. Individuals with CF and at least two FEV1 measurements < 40% predicted within a 5-year period, age ≥ 6 years, without prior LTx were included. Multivariable competing risk regression for time to death without LTx (LTx as a competing risk) and time to LTx (death as a competing risk) was performed.

Results: There were 5,899 patients (53% male) and 905 patients (54% male) with CF with FEV1 < 40% predicted living in the United States and Canada, respectively. Multivariable competing risk regression models identified an increased risk of death without LTx (hazard ratio [HR], 1.79; 95% CI, 1.52-2.1) and decreased LTx (HR, 0.66; 95% CI, 0.58-0.74) among individuals in the United States compared with Canada. More pronounced differences were seen in the patients in the United States with Medicaid/Medicare insurance compared with Canadians (multivariable HR for death without LTx, 2.24 [95% CI, 1.89-2.64]; multivariable HR for LTx, 0.54 [95% CI, 0.47-0.61]). Patients of nonwhite race were also disadvantaged (multivariable HR for death without LTx, 1.56 [95% CI, 1.32-1.84]; multivariable HR for LTx, 0.47 [95% CI, 0.36-0.62]).

Interpretation: There are lower rates of LTx and an increased risk of death without LTx for US patients with CF with FEV1 < 40% predicted compared with Canadian patients. Findings are more striking among US patients with CF with Medicaid/Medicare health insurance, and nonwhite patients in both countries, raising concerns about underuse of LTx among vulnerable populations.

Keywords: access to transplantation; advanced lung disease; cystic fibrosis; lung transplantation.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adult
  • Canada / epidemiology
  • Child
  • Cystic Fibrosis* / diagnosis
  • Cystic Fibrosis* / ethnology
  • Cystic Fibrosis* / mortality
  • Cystic Fibrosis* / surgery
  • Disease Progression
  • Female
  • Health Policy
  • Health Services Misuse
  • Humans
  • Insurance Claim Review
  • Lung Transplantation* / methods
  • Lung Transplantation* / statistics & numerical data
  • Male
  • Mortality
  • Needs Assessment
  • Registries / statistics & numerical data
  • Respiratory Function Tests* / methods
  • Respiratory Function Tests* / statistics & numerical data
  • Risk Assessment / methods
  • Risk Assessment / statistics & numerical data
  • United States / epidemiology
  • Vulnerable Populations