Chest

Chest

Volume 124, Issue 4, October 2003, Pages 1460-1468
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Clinical Investigations
Cystic Fibrosis
The Effect of Pregnancy on Survival in Women With Cystic Fibrosis

https://doi.org/10.1378/chest.124.4.1460Get rights and content

Study objectives

Patients with cystic fibrosis (CF) are currently living to their fourth decade and are making reproductive decisions. Information concerning the reproductive health of women with CF has been limited to small or single-center studies.

Design

We conducted a matched parallel-cohort study to assess the impact of pregnancy on the survival of women with CF.

Participants

A parallel-cohort study included all women > 12 years of age who were enrolled in the US Cystic Fibrosis Foundation National Patient Registry from 1985 to 1997.

Measurements and results

Six hundred eighty of the 8,136 women in the cohort became pregnant. These 680 women were matched on an index year to 3,327 control women with CF. At the inception of entry into the cohort, women who reported pregnancy were more likely to have had a higher percentage of predicted FEV1 (67.5% predicted vs 61.7% predicted, respectively; p < 0.001) and a higher weight (52.9 vs 46.4 kg, respectively; p < 0.001). Using Kaplan-Meier survival curves, the 10-year survival rate in pregnant women (77%; 95% confidence interval [CI], 71 to 82%) was higher than in those women who did not become pregnant (58%; 95% CI, 55 to 62%). A separate analysis, matching pregnant patients on FEV1 percent predicted, age, Pseudomonas aeruginosa colonization, and pancreatic function, obtained similar results. Using Cox proportional hazard modeling to adjust for baseline age, FEV1 percent predicted, weight, height, and pulmonary exacerbation rate per year, pregnancy was not associated with an increase risk of death. Pregnancy was not harmful in any subgroup including patients with FEV1 < 40% of predicted or diabetes mellitus.

Conclusions

Women with CF who became pregnant were initially healthier and had better 10-year survival rates than women with CF who did not become pregnant. After adjustment for the initial severity of illness, women who became pregnant did not have a significantly shortened survival.

Section snippets

Participants

The US CFF National Patient Registry database contains demographic and clinical data collected at CFF-accredited centers in the United States since 1966, using an annual questionnaire. A full description of the database has been published previously.18 Study subjects were women with CF who had been seen at a CFF-accredited care center in the United States between January 1, 1985, and December 31, 1997. All women who reported pregnancy during this 13-year period were considered for this

Results

There were 8,136 women with CF enrolled in the CFF National Patient Registry who were > 12 years of age. Sixty-three women reported pregnancy in the first year of enrollment, 37 women were diagnosed after the age of 45 years, and 930 had only 1 year of observation during the study period. These patients were excluded from the analyses. Six hundred eighty women (9.6%) became pregnant, 313 of whom (3.9%) had multiple pregnancies (median, two pregnancies; range, one to six pregnancies). A majority

Discussion

This study shows that women with CF who reported pregnancy were older, had higher FEV1 percent predicted values, and had higher BMIs than a randomly selected sample of women with CF who did not report pregnancy. It also shows that after adjusting for these initial demographic differences, women with CF who became pregnant did not have worse survival. Several techniques were utilized to adjust for confounding variables, including selecting specific subsets, stratification, full matching, and

Conclusions

Women with CF who became pregnant were initially healthier and had better 10-year survival rates than did women with CF who did not become pregnant. After adjustment for initial severity of illness, women who became pregnant did not have a significantly shortened survival compared to nonpregnant women with CF. No specific subsets of pregnant patients were identified in this analysis who had worse survival than the comparison group.

ACKNOWLEDGMENTS

We are indebted to Preston Campbell, III, MD, Executive Vice President for Medical Affairs, Cystic Fibrosis Foundation, for his support of this project and for making the CFF National Patient Registry data available to us. We are also indebted to Stacey FitzSimmons for her early work on the CFF National Patient Registry data.

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    This research was supported by the Leroy Matthew Physician Scientist Award from the Cystic Fibrosis Foundation and by Firlands Foundation.

    Reproduction of this article is prohibited without written permission from the American College of Chest Physicians (e-mail:[email protected]).

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    Copyright © 2003 The American College of Chest Physicians. Published by Elsevier Inc. All rights reserved.