Journal
CHEST
Volume 151, Issue 6, Pages 1320-1328Publisher
ELSEVIER SCIENCE BV
DOI: 10.1016/j.chest.2017.01.019
Keywords
cystic fibrosis; FEV1; lung function; lung transplantation; survival
Categories
Funding
- Cystic Fibrosis Foundation
- National Institutes of Health [R01HL103965, R01 AI101307, P30 DK089507, F32 HL131246-01]
- US Food and Drug Administration [R01 FD003704]
- Cystic Fibrosis Foundation Third Year Clinical Fellowship Award [RAMOS16A0]
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BACKGROUND: Lung transplantation (LTx) is frequently considered for patients with cystic fibrosis (CF) when the FEV1 reaches < 30%. This study estimated transplant-free survival for patients with CF and an FEV1 < 30% and identified predictors of death without LTx. METHODS: We conducted a retrospective cohort study using the CF Foundation Patient Registry from January 1, 2003 to December 31, 2013. Adult patients (>= 18 years) with FEV1 < 30% prior to LTx were included. We performed Kaplan-Meier survival estimates censored at LTx. Multivariable Cox proportional hazard regression identified predictors of mortality. RESULTS: There were 3,340 patients with an FEV1 < 30%. Death without LTx occurred in 1,250 patients (37.4%); 951 patients (28.5%) underwent LTx; 918 patients (27.5%) remained alive without LTx at the end of follow-up; and 221 patients (6.6%) were lost to follow-up. Median transplant-free survival after FEV1 < 30% was 6.6 years (95% CI, 5.9-7.0). Adjusted predictors of death without LTx included supplemental oxygen use (hazard ratio [HR], 2.1; 95% CI, 1.7-2.6), Burkholderia cepacia infection (HR, 1.8; 95% CI, 1.3-2.6), BMI <= 18 (HR, 1.6; 95% CI, 1.3-1.9), female sex (HR, 1.6; 95% CI, 1.2-2.0), CF-related diabetes in patients receiving insulin (HR, 1.4; 95% CI, 1.2-1.8), and >= one exacerbation per year (HR, 1.7; 95% CI, 1.3-2.2 vs. 0 exacerbations). CONCLUSIONS: Median survival was > 6.5 years for patients with CF and an FEV1 < 30%, exceeding prior survival estimates. There was substantial heterogeneity in survival, with some patients with CF dying soon after reaching this lung function threshold and others living for many years. For this reason, we conclude that FEV1 < 30% remains an important marker of disease severity for patients with CF. Patients with a supplemental oxygen requirement or frequent exacerbations should have prompt referral because of their increased risk of death.
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