CYSTIC FIBROSIS AND PREGNANCY: A National Survey - ScienceDirect

The Lancet

Volume 316, Issue 8199, 18 October 1980, Pages 842-844

The Lancet

https://doi.org/10.1016/S0140-6736(80)90183-X Get rights and content

Abstract

In a survey of 119 cystic fibrosis (CF) centres in the U.S.A. and Canada, information was obtained on 129 pregnancies in 100 patients with CF. 97 pregnancies were completed resulting in 86 viable infants, only one of whom had CF. This is close to the expected incidence and attests to the recessive nature of the transmission of CF, the commonest lethal genetic disorder of Caucasians. Shortened gestation and increased maternal and perinatal mortality were related to severe maternal pulmonary infection. Increased respiratory and cardiac work, and especially hypervolæmia, are hazards for pregnant women with CF, and when combined with advanced pulmonary disease may lead to increased perinatal complications. Maternal hypoxia and transplacental passage of antibiotics may affect the fetus; however, there were no congenital anomalies in this series in spite of frequent use of antibiotics by these mothers. Unless the clinical score is high, pregnancy should be avoided in CF patients.

References (41)

Ej Plotz et al.
Pregnancy in a patient with cystic fibrosis (mucoviscidosis) and diabetes mellitus
Am J Obstet Gynec
(1967)
Wj Warwick et al.
Survival patterns in cystic fibrosis
J Chron Dis
(1975)
Hg Herrod et al.
Mother and daughter with cystic fibrosis
J Pediatr
(1977)
Pa di Sant'Agnese et al.
Cystic fibrosis in adults: 75 cases and a review of 232 cases in the literature
Am J Med
(1979)
Rm Goldring et al.
Pulmonary hypertension and cor pulmonale in cystic fibrosis of the pancreas
J Pediatr
(1964)
Lm Taussig et al.
A new prognostic score and clinical evaluation system for cystic fibrosis
J Pediatr
(1973)
L. Kopito et al.
Water and electrolytes in cervical mucus from patients with cystic fibrosis
Fertil Steril
(1973)
Eh Oppenheimer et al.
Observation on cystic fibrosis of the pancreas: VI. the uterine cervix
J Pediatr
(1970)
Cystic Fibrosis Foundation
1974 Report on survival studies of patients with cystic fibrosis
(March, 1976)
B. Siegel et al.
Pregnancy and delivery in a patient with cystic fibrosis of the pancreas: report of a case
Obstet Gynec
(1960)

Re Ball et al.

Mucoviscidosis in young adults: report of a case in a twenty year old female

N Engl J Med

(1961)

G. Polgar et al.

Cystic fibrosis in adults: studies of pulmonary function and some physical properties of bronchial mucus

Am Rev Resp Dis

(1972)

Mj Novy et al.

Cystic fibrosis and pregnancy: report of a case, with a study of a pulmonary function and arterial blood gases

Obstet Gynec

(1967)

Ec Rosenow et al.

Cystic fibrosis and pregnancy

JAMA

(1968)

Ma Ayers

Pregnancy and cystic fibrosis of the lungs and pancreas: a case report

Ohw State Med J

(1970)

Jw Larsen

Cystic fibrosis and pregnancy

Obstet Gynec

(1972)

Rj Grand et al.

Pregnancy in cystic fibrosis of the pancreas

JAMA

(1966)

Lo Lubchenco et al.

Intrauterine growth in length and head circumference as estimated from live births at gestational ages from 26 to 42 weeks

Pediatrics

(1967)

Vital and Health Statistics Analytical Studies

(1965)

(1975)

Cited by (84)

Pulmonary disorders in pregnancy: Bronchiectasis, cystic fibrosis, sarcoidosis and interstitial diseases
2022, Best Practice and Research: Clinical Obstetrics and Gynaecology
This chapter aims to provide expert guidance to obstetricians, general practitioners, allied health staff and women with lung disease about the interactions between pregnancy and different lung diseases. This chapter will cover other airway diseases such as bronchiectasis and cystic fibrosis (CF) together with sarcoidosis and interstitial lung disease (ILD), noting that another chapter covers asthma. The physiological changes which occur in pregnancy, such as the changes in airway physiology, resting ventilation and sleep, are summarised in another chapter.
This chapter extends the evidence-based approach and clinical expertise of the recent European Respiratory Society/Thoracic Society of Australia and New Zealand (ERS/TSANZ) taskforce. The papers selected were based on the population (pregnant women with lung disease other than asthma) and the effects of these diseases on risks of pregnancy-associated complications and miscarriages, breastfeeding, nutritional considerations, lung function, long-term maternal outcomes and management considerations during pregnancy.
As there are very few randomised control trials in the area, the majority of the literature consists of observational studies (prospective or retrospective), cross-sectional surveys and case series. Other guidelines have also recently been published, which may be helpful to the reader.
Challenges Faced by Women with Cystic Fibrosis
2021, Clinics in Chest Medicine
Pregnancy in women with Cystic Fibrosis in the 21<sup>st</sup> century
2021, Journal of Cystic Fibrosis
Baseline Cystic fibrosis disease severity has an adverse impact on pregnancy and infant outcomes, but does not impact disease progression
2021, Journal of Cystic Fibrosis
Citation Excerpt :
In the past, patients with severe CF were advised to avoid pregnancies due to rapid respiratory deterioration, a high spontaneous abortion rate and other pregnancy complications [4]. Furthermore, due to concerns for a reduction in pulmonary function during pregnancy, even in healthy women [5,6], patients with a forced expiratory volume in one second (FEV1) percent predicted under 50% were formally advised to avoid or terminate pregnancies in the 1970s [7]. Newer guidelines acknowledge the inconsistency in pregnancy outcomes, avoiding such recommendations [4,8].
With increasing longevity and quality of life in adults with Cystic fibrosis (CF), growing maternity rates are reported. Women with severe CF are becoming pregnant, with unpredictable maternal and fetal outcomes.
To determine how baseline disease severity, pancreatic insufficiency (PI) and Pseudomonas aeruginosa (PA) infection affect fertility, the pregnancy course, delivery, neonatal outcome, and subsequent disease progression.
A multicenter-retrospective cohort study. Data on patients that had been pregnant between 1986-2018 was collected from ten CF centers worldwide. Disease severity [mild or moderate-severe (mod-sev)] was defined according to forced expiratory volume % predicted in 1 second (FEV₁) and body mass index (BMI). Three time periods were compared, 12 months prior to conception, the pregnancy itself and the 12 months thereafter.
Data was available on 171 pregnancies in 128 patients aged 18-45 years; 55.1% with mod-sev disease, 43.1% with PI and 40.3% with PA. Women with mod-sev disease had more CF-related complications during and after pregnancy and delivered more preterm newborns. However, FEV₁ and BMI decline were no different between the mild and mod-sev groups. A more rapid decline in FEV₁ was observed during pregnancy in PI and PA infected patients, though stabilizing thereafter. PI was associated with increased risk for small for gestational age infants.
Baseline disease severity, PA infection and PI have an adverse impact on infant outcomes, but do not impact significantly on disease progression during and after pregnancy. Consequently, pregnancies in severe CF patients can have a good prognosis.
A case-control study on pregnancy in Italian Cystic Fibrosis women. Data from the Italian Registry
2018, Respiratory Medicine
Citation Excerpt :
Fertility, impaired in 95–98% of male subjects [6], is not in women, and cases of pregnancies in CF women have begun to be reported in the scientific literature since 1960 as single case reports [7,8]. In the following years pregnancy in CF patients became a more common occurrence as a result of improving survival rates in females, as documented in several paper since 1980 [9,10]. Women with CF have a longer life expectancy and therefore, since adolescence, they may pose questions about their fertility and the possibilities of motherhood.
Pregnancy in CF women is no longer an exceptional event, but few information on its effect on clinical status in European CF patients are available. This study describes Italian CF pregnant population, to investigate its effect on CF woman.
Data were collected (2010–2015) by the Italian CF Registry. A case-control study was performed between pregnant and never pregnant women.
A total of 81 pregnant women (aged 18-45ys) were identified with a mean age at pregnancy of 31ys. Median age at diagnosis resulted higher in cases (P = 0.010). A decline from 6 to 12 months before pregnancy to one year after delivery in BMI (P = 0.034), in FEV₁ (P < 0.001) and FVC (P < 0.001) was registered among cases. Difference in change between cases and controls was found for the BMI (P = 0.006); weak evidence of difference in FEV₁ (p = 0.080) and FVC (p = 0.056) were noted between the groups.
Data from ICFR show that CF women can become mother with only slight adverse effect on their nutritional status. Further study should be carried out to investigate long term effect of pregnancy on lung function.
Obstetric care in women with genetic disorders
2017, Best Practice and Research: Clinical Obstetrics and Gynaecology
Citation Excerpt :
Severely compromised maternal lung function can lead to iatrogenic prematurity if preterm delivery is necessary for maternal health. Frequent assessment of fetal growth should be performed in addition to fetal surveillance in the third trimester [30,33,35,36]. Limitations of the labor and delivery process will likely be determined by the underlying pulmonary function.
The management of pregnant women who are themselves affected with genetic diseases is an increasingly relevant and important issue. Improvements in early diagnosis and management of genetic disease, as well as advances in assisted reproductive technology have impacted pregnancy rates in a cohort of women who may not have otherwise been able to conceive. A multidisciplinary approach is key to the management of pregnant women with complex health conditions, including genetic diseases. Pertinent issues should be addressed in the preconception, antepartum, intrapartum and postpartum periods to optimize maternal and fetal health. Additionally, counseling regarding risk of inheritance in offspring and options for prenatal diagnosis should be reviewed if available. This reviews aims to help provide background and insight into the management strategies for various commonly encountered and complex genetic conditions in the setting of pregnancy

View all citing articles on Scopus

Copyright © 1980 Published by Elsevier Ltd.