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