Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2017 Jun;151(6):1320-1328.
doi: 10.1016/j.chest.2017.01.019. Epub 2017 Jan 20.

Heterogeneity in Survival in Adult Patients With Cystic Fibrosis With FEV1 < 30% of Predicted in the United States

Kathleen J Ramos  1 , Bradley S Quon  2 , Sonya L Heltshe  3 , Nicole Mayer-Hamblett  4 , Erika D Lease  5 , Moira L Aitken  5 , Noel S Weiss  6 , Christopher H Goss  7
Affiliations

Heterogeneity in Survival in Adult Patients With Cystic Fibrosis With FEV1 < 30% of Predicted in the United States

Kathleen J Ramos et al. Chest. 2017 Jun.

Abstract

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.

Keywords: FEV(1); cystic fibrosis; lung function; lung transplantation; survival.

PubMed Disclaimer

Figures

Figure 1
Figure 1
Kaplan Meier estimates for survival after (A) FEV1 < 30% predicted and (B) time to lung transplantation after FEV1 < 30% among patients who did not die.
Figure 2
Figure 2
Number of lung transplantations and deaths each year after FEV1 < 30%. The Figure represents the proportion of eligible patients remaining each year who died or underwent lung transplantation.
See this image and copyright information in PMC

References

    1. O'Sullivan B.P., Freedman S.D. Cystic fibrosis. Lancet. 2009;373(9678):1891–1904. - PubMed
    1. Cystic Fibrosis Foundation Patient Registry, 2014 Annual Data Report. Bethesda, Maryland, Cystic Fibrosis Foundation, 2015.
    1. Weill D., Benden C., Corris P.A. A consensus document for the selection of lung transplant candidates: 2014—an update from the Pulmonary Transplantation Council of the International Society for Heart and Lung Transplantation. J Heart Lung Transplant. 2015;34(1):1–15. - PubMed
    1. Kerem E., Reisman J., Corey M., Canny G.J., Levison H. Prediction of mortality in patients with cystic fibrosis. N Engl J Med. 1992;326(18):1187–1191. - PubMed
    1. Milla C.E., Warwick W.J. Risk of death in cystic fibrosis patients with severely compromised lung function. Chest. 1998;113(5):1230–1234. - PubMed

MeSH terms