Pregnancy in cystic fibrosis: Review of the literature and expert recommendations

Raksha Jain; Traci M Kazmerski; Lisa C Zuckerwise; Natalie E West; Kristina Montemayor; Moira L Aitken; Edith Cheng; Andrea H Roe; Alexandra Wilson; Caitlin Mann; Sigrid Ladores; Jacqui Sjoberg; Madeline Poranski; Jennifer L Taylor-Cousar

doi:10.1016/j.jcf.2021.07.019

Pregnancy in cystic fibrosis: Review of the literature and expert recommendations

J Cyst Fibros. 2022 May;21(3):387-395. doi: 10.1016/j.jcf.2021.07.019. Epub 2021 Aug 26.

Authors

Affiliations

¹ Department of Medicine, University of Texas Southwestern, 5323 Harry Hines Blvd., Dallas, TX 75390-8558, United States. Electronic address: [email protected].
² Department of Pediatrics, University of Pittsburgh School of Medicine, 120 Lytton Ave. Suite M060 University Center, Pittsburgh, PA 15213, United States.
³ Department of Obstetrics and Gynecology, Division of Maternal-Fetal Medicine, Vanderbilt University Medical Center, 1161 21st Avenue South, B-1100 MCN, Nashville, TN 37232, United States.
⁴ Department of Medicine, Johns Hopkins University, 1830 E. Monument St. 5th Floor, Baltimore, MD 21205, United States.
⁵ Department of Medicine, University of Washington, 1959 NE Pacific Street - Rm BB 1361, Seattle WA 98195-6522, United States.
⁶ Maternal Fetal Medicine, Maternal Fetal Medicine, Medical Genetics, University of Washington, United States.
⁷ Department of Obstetrics and Gynecology, University of Pennsylvania Perelman School of Medicine, 3400 Spruce Street, 1000 Courtyard, Philadelphia, PA 19106, United States.
⁸ Department of Medicine, Cystic Fibrosis Clinical Research, National Jewish Health, 1400 Jackson Street, K333b, Denver, CO 80206, United States.
⁹ School of Nursing, The University of Alabama at Birmingham, NB 470L, 1720 2nd Avenue South, Birmingham, AL 35294-1210, United States.
¹⁰ Adult with Cystic Fibrosis, 130 Johnston Dr, Highland Park, IL 60035, United States.
¹¹ Adult with Cystic Fibrosis, 2700 University Ave. W. Apt. 416, St. Paul, MN 55114, United States.
¹² Departments of Medicine and Pediatrics, National Jewish Health, 1400 Jackson Street; J318, Denver, CO 80206, United States.

PMID: 34456158
DOI: 10.1016/j.jcf.2021.07.019

Abstract

Cystic fibrosis (CF) was historically a disease largely afflicting children. Due to therapeutic advancements, there are now more adults with CF than children. In the past decade, medications including Cystic Fibrosis Transmembrane conductance Regulator (CFTR) modulators became available that treat the underlying cause of CF and are dramatically improving lung function as well as quality and quantity of life for people with CF. As a result, more women with CF are becoming pregnant. We gathered a panel of experts in CF care, family planning, high risk obstetrics, nutrition, genetics and women with CF to review current literature on pregnancies and to provide care recommendations for this unique population.

Keywords: Cystic fibrosis; Family planning; Fertility; Parenthood; Pregnancy.

Publication types

Review
Research Support, Non-U.S. Gov't

MeSH terms

Adult
Child
Cystic Fibrosis Transmembrane Conductance Regulator / genetics
Cystic Fibrosis Transmembrane Conductance Regulator / metabolism
Cystic Fibrosis* / epidemiology
Cystic Fibrosis* / genetics
Cystic Fibrosis* / therapy
Female
Humans
Ion Transport
Pregnancy

Substances

Cystic Fibrosis Transmembrane Conductance Regulator