Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2023 Jul 12;13(7):1545.
doi: 10.3390/life13071545.

Reproductive Counseling and Care in Cystic Fibrosis: A Multidisciplinary Approach for a New Therapeutic Era

Affiliations
Review

Reproductive Counseling and Care in Cystic Fibrosis: A Multidisciplinary Approach for a New Therapeutic Era

Julie McGlynn et al. Life (Basel). .

Abstract

With the advent of highly effective modulator therapies, many people with cystic fibrosis (CF) are living longer, healthier lives. Pregnancy rates for women with CF more than doubled between 2019 and 2021, reflecting increases in both planned and unplanned pregnancies. For men with CF, CF-associated infertility can be mitigated with assistive reproductive technology, yet patient knowledge of these challenges and options is variable. Preconception and prenatal counseling for individuals with CF and for parents of children with CF who wish to expand their families requires nuanced discussions to promote informed reproductive decisions, drawing from a combination of standard practice recommendations and CF-specific assessments. This review article synthesizes the current literature and practice recommendations regarding reproductive counseling and care in CF, outlining the role of genetic counseling, carrier screening, teratogen counseling, in vitro fertilization and pre-implantation genetic diagnosis, and careful assessment and management of cystic fibrosis-related diabetes when present. Via a multidisciplinary, patient-centered approach, clinicians can support adults with CF and parents of children with CF as they make informed reproductive decisions and embark on family planning.

Keywords: cystic fibrosis; family planning; fertility; genetic counseling; highly effective modulators; pregnancy.

PubMed Disclaimer

Conflict of interest statement

The authors declare no conflict of interest.

Figures

Figure 1
Figure 1
From the 2021 Cystic Fibrosis Foundation Patient Registry, reproduced with permission. In 2021, there were 32,100 individuals with CF in the Registry. Adults comprised 58.3 percent of the CF population (n = 18,714).
Figure 2
Figure 2
From the 2021 Cystic Fibrosis Foundation Patient Registry, reproduced with permission.
Figure 3
Figure 3
Clinical support and care team for a pregnant woman with CF. Pregnancy support system from CF team members and related specialties. Intersecting lines and arrows reflect the varying multidisciplinary care and communication needs.

References

    1. Rowe S.M., Miller S., Sorscher E.J. Cystic fibrosis. N. Engl. J. Med. 2005;352:1992–2001. doi: 10.1056/NEJMra043184. - DOI - PubMed
    1. The Clinical and Functional Translation of CFTR (CFTR2) Project. [(accessed on 2 May 2023)]. Available online: www.CFTR2.org.
    1. Cystic Fibrosis Foundation . Cystic Fibrosis Foundation Patient Registry 2021 Annual Data Report. Cystic Fibrosis Foundation; Bethesda, MD, USA: 2022.
    1. Heijerman H.G.M., McKone E.F., Downey D.G., Van Braeckel E., Rowe S.M., Tullis E., Mall M.A., Welter J.J., Ramsey B.W., McKee C.M., et al. Efficacy and safety of the elexacaftor plus tezacaftor plus ivacaftor combination regimen in people with cystic fibrosis homozygous for the F508del mutation: A double-blind, randomised, phase 3 trial. Lancet. 2019;394:1940–1948. doi: 10.1016/S0140-6736(19)32597-8. - DOI - PMC - PubMed
    1. Taylor-Cousar J.L., Shteinberg M., Cohen-Cymberknoh M., Jain R. The Impact of Highly Effective Cystic Fibrosis Transmembrane Conductance Regulator Modulators on the Health of Female Subjects with Cystic Fibrosis. Clin. Ther. 2023;45:278–289. doi: 10.1016/j.clinthera.2023.01.016. - DOI - PubMed

LinkOut - more resources