Chest
Volume 118, Issue 1, July 2000, Pages 85-91
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Clinical Investigations: Cystic Fibrosis
Pregnancy in Cystic Fibrosis: Fetal and Maternal Outcome

https://doi.org/10.1378/chest.118.1.85Get rights and content

Objective

To assess the effect of pregnancy on pulmonary function and survival in women with cystic fibrosis (CF) and to assess the fetal outcome.

Design

Cohort study. The data analyzed were collected from the Toronto CF database, chart review, and patient questionnaire.

Setting

Tertiary-care center.

Patients

All women with CF who, at the time of diagnosis or pregnancy, attended the Toronto Cystic Fibrosis Clinics between 1961 and 1998.

Results

From 1963 to 1998, there were 92 pregnancies in 54 women. There were 11 miscarriages and 7 therapeutic abortions. Forty-nine women gave birth to 74 children. The mean follow-up time was 11 ± 8 years. One patient was lost to follow-up shortly after delivery, and one was lost after 12 years. The overall mortality rate was 19% (9 of 48 patients). Absence of Burkholderia cepacia (p < 0.001), pancreatic sufficiency (p = 0.01), and prepregnancy FEV1 > 50% predicted (p = 0.03) were associated with better survival rates. When adjusted for the same parameters, pregnancy did not affect survival compared to the entire adult female CF population. The decline in FEV1 was comparable to that in the total CF population. Three women had diabetes mellitus, and seven developed gestational diabetes. There were six preterm infants and one neonatal death. CF was diagnosed in two children.

Conclusions

The maternal and fetal outcome is good for most women with CF. Risk factors for mortality are similar to those for the nonpregnant CF population. Pregnancies should be planned so that there is opportunity for counseling and optimization of the medical condition. Good communication between the CF team and the obstetrician is important.

Section snippets

Materials and Methods

All women with CF who attended the CF program from 1960 to 1998 at the Hospital for Sick Children or at St. Michael's/Wellesley Hospital (from 1992) were eligible for the study. Demographic and clinical follow-up data were abstracted from the patient database. In general, patients were seen one to four times per year, and the following pertinent data were recorded: height; weight; pancreatic function; and pulmonary function, as assessed by spirometry.11 Information on gestational age, birth

Statistical Analysis

Pregnancies with gestational lengths of > 26 weeks were included in the statistical analysis.

Data were presented as mean ± SD or median with range, as appropriate. Paired data (pre- and postpregnancy) were analyzed using a two-tailed t test. Subgroups were compared using a two-tailed t test or the Mann-Whitney test. The significance level was set at p ≤ 0.05.

Kaplan-Meier estimates of survival were computed, using data from the first completed pregnancy for each woman. Survival was determined

Outcomes of Pregnancies

For prepregnancy characteristics see Table 1.

From 1963 to 1998, there were 92 pregnancies in 54 women, with 74 births in 49 women (range, 1 to 3 births [no multiple births]). There were 11 miscarriages and 7 medical terminations. Terminations were for psychosocial reasons in three patients and poor health in four patients (PS, 1 patient; PI, 3 patients). In the latter group, one patient carried a pregnancy to term 4 years later, but she deteriorated during and after the pregnancy and died 4

Discussion

The fetal outcome was good for this population, with a mean gestational age and birth weight comparable to data for Ontario from 1973 to 1993.12 A higher percentage of pregnancies resulting in live births in women < 25 years of age was seen compared with the whole Ontario population (42% vs 32%, respectively).12 The number of infants who were small for gestational age did not substantially differ from the numbers reported for Canada.16 Hilman et al7 reported a higher percentage of preterm

Conclusion

Pregnancy was well-tolerated in our population of CF women. Most patients carrying a pregnancy to term had mild to moderate disease. The risk factors for deteriorating health and early death after pregnancy are the same as for the whole adult female CF population, with the presence of B cepacia being the most important risk factor for early death. Pregnancy did not affect the rate of yearly decline in FEV1. Follow-up studies of pregnant women with poor lung function and of women with diabetes

ACKNOWLEDGMENT

The authors thank Anna Tsang and Sandi Peroff for their assistance in contacting patients, and the Ottawa CF clinics for follow-up information.

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    Supported in part by the Canadian Cystic Fibrosis Foundation (M.G.).

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