The Effect of Pregnancy on Survival in Women With Cystic Fibrosis

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The Effect of Pregnancy on Survival in Women With Cystic Fibrosis*

H. Goss, MD, MS; Gordon D. feld, MD, MS, FCCP;

Otto, MS and Moira L. Aitken, MD, FCCP

* From the Division of Pulmonary and Critical Care Medicine (Drs.

Goss, feld, and Aitken), Department of Medicine, University of

Washington Medical Center, Seattle, WA; and Statistical Consulting

(Ms. Otto), Seattle, WA.

Correspondence to: H. Goss, MD, MS, Assistant Professor

of Medicine, University of Washington Medical Center, Campus Box

356522, 1959 NE Pacific, Seattle, WA 98195; e-mail:

goss@...

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 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.

Key Words: cystic fibrosis • outcome • pregnancy • survival