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Review
. 2021 May 15;11(5):418.
doi: 10.3390/jpm11050418.

Fertility, Pregnancy and Lactation Considerations for Women with CF in the CFTR Modulator Era

Raksha Jain  1 , Jennifer L Taylor-Cousar  2
Affiliations
Review

Fertility, Pregnancy and Lactation Considerations for Women with CF in the CFTR Modulator Era

Raksha Jain et al. J Pers Med. .

Abstract

Cystic fibrosis (CF) is an autosomal recessive genetic disorder impacting approximately 80,000 people of all races and ethnicities world-wide. CF is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene which encodes a protein of the same name. Protein dysfunction results in abnormal chloride and bicarbonate transport in mucus membranes, including those in the respiratory, gastrointestinal and reproductive tracts. Abnormal anion transport causes viscous secretions at the site of involvement. The majority of people with CF succumb to respiratory failure following recurrent cycles of infection and inflammation in the airways. Historically, providers treated the signs and symptoms of CF, but since 2012, have been able to impact the basic defect for the subset of people with CF who have mutations that respond to the new class of drugs, CFTR protein modulators. With the improved health and longevity afforded by CFTR modulators, more women are interested in parenthood and are becoming pregnant. Furthermore, this class of drugs likely increases fertility in women with CF. However, the safety of CFTR modulators in pregnancy and lactation is only beginning to be established. We summarize available data on the impact of CFTR modulators on fertility, pregnancy and lactation in women with CF.

Keywords: CFTR modulator; contraception; fertility; lactation; pregnancy.

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Conflict of interest statement

J.L.T.-C: In the last 3 years, she reports grants to her institution from the Cystic Fibrosis Foundation, grants and personal fees (advisory boards/clinical trial design consultation/non-branded speaking) from Gilead, Vertex, Celtaxys and Proteostasis, grants from N30 and Bayer, and personal fees (advisory boards/clinical trial design consultation) from Novartis, Genentech, Protalix, Santhera, 4DMT, Polarean Imaging, Insmed, and AbbVie, and Service on the CF TDN Clinical Research Executive Committee and as Chair of the Women’s Health Research Working Group, and as the Chair of the American Thoracic Society’s Clinical Problems Assembly Program Committee. R.J.: In the last 3 years, she reports grants to her institution from the Cystic Fibrosis Foundation, grants and personal fees (advisory boards/consulting) from Boeringer Ingelheim and Vertex, grants from Genentech, Sound Pharma, Corbus, Armata, and service on the CF TDN Women’s Health Research Working Group (Vice-Chair).

Figures

Figure 1
Figure 1
(A) Hysteroscopic image of the uterus of a woman with CF prior to initiation of CFTR modulator. (B) Image of the uterus in the same woman 3 months after initiation of IVA.
Figure 2
Figure 2
The number of pregnancies reported in the U.S. CFFPR [3] The number of pregnancies in women with CF has been increasing over time.
See this image and copyright information in PMC

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References

    1. Gan K.H., Geus W.P., Bakker W., Lamers C.B., Heijerman H.G. Genetic and clinical features of patients with cystic fibrosis diagnosed after the age of 16 years. Thorax. 1995;50:1301–1304. doi: 10.1136/thx.50.12.1301. - DOI - PMC - PubMed
    1. McCloskey M., Redmond A., Hill A., Elborn J. Clinical features associated with a delayed diagnosis of cystic fibrosis. Respiration. 2000;67:402–407. doi: 10.1159/000029538. - DOI - PubMed
    1. Registry, Cystic Fibrosis Foundation. 2019 Annual Data Report. [(accessed on 18 April 2021)]; Available online: https://www.cff.org/Research/Researcher-Resources/Patient-Registry/2018-....
    1. Cystic Fibrosis Mutation Database. [(accessed on 3 May 2021)]; Available online: http://www.genet.sickkids.on.ca/Home.html.
    1. UK Cystic Fibrosis Registry Annual Data Repot 2019; UK Trust: 2021. [(accessed on 18 April 2021)]; Available online: https://www.cysticfibrosis.org.uk/sites/default/files/202012/2019%20Regi....

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