Survival and lung transplant outcomes for individuals with advanced cystic fibrosis lung disease in the United States and Canada: an analysis of national registries.

Abstract Background Understanding how health outcomes differ for cystic fibrosis (CF) patients with advanced lung disease in the United States (US) compared to Canada has health policy implications. Research Question What are rates of lung transplant (LTx) and rates of death without LTx in the US and Canada among individuals with forced expiratory volume in 1 second (FEV1) Study Design and Methods: Retrospective population-based cohort study, 2005-2016, using the US CF Foundation, United Network for Organ Sharing, and Canadian CF registries. Individuals with CF and ≥2 FEV1 measurements Results There were 5,899 (53% male) and 905 (54% male) CF patients with FEV1 Interpretation There are lower rates of LTx and an increased risk of death without LTx for CF patients with FEV1