Skip to main content
Log in

Unplanned pregnancies following the introduction of elexacaftor/tezacaftor/ivacaftor therapy in women with cystic fibrosis

  • Opinion
  • Published:
Archives of Gynecology and Obstetrics Aims and scope Submit manuscript

Abstract

Cystic fibrosis (CF) causes infertility and subfertility due to various factors, including altered cervical mucus, delayed puberty, and hormonal imbalances. With the introduction of the CFTR modulator therapy elexacaftor-tezacaftor-ivacaftor, we have observed an increase in unplanned pregnancies among women undergoing ETI treatment in our CF center, despite repeated recommendations for strict fertility monitoring. It appears that these pregnancies are more likely attributed to reduced attention to the possibility of conception rather than contraceptive failure. The perception of subfertility developed by women with CF over time, before the era of modulators, can influence their long-term habits and lead to the underuse of contraceptive methods. While further research is needed to fully understand the effects of ETI on fertility, healthcare providers should be attentive to the fertility concerns of women with CF, particularly those treated with modulators in adulthood.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Data availability

Since these are very confidential personal data, it is not applicable.

References

  1. Hughan KS, Daley T, Rayas MS, Kelly A, Roe A (2019) Female reproductive health in cystic fibrosis. J Cyst Fibros. https://doi.org/10.1016/j.jcf.2019.08.024

    Article  PubMed  Google Scholar 

  2. Shteinberg M, Lulu AB, Downey DG, Blumenfeld Z, Rousset-Jablonski C, Perceval M, Colombo A, Stein N, Livnat G, Gur M, Bentur L, Mussaffi H, Blau H, Sarouk I, Dagan A, Kerem E, Aviram M, Picard E, Aliberti S, Álvarez A, Miranda JP, Polverino E, Durieu I, Stuart Elborn J, Cohen-Cymberknoh M (2018) Failure to conceive in women with CF is associated with pancreatic insufficiency and advancing age. J Cyst Fibros 18(4):525–529. https://doi.org/10.1016/j.jcf.2018.10.009

    Article  PubMed  Google Scholar 

  3. Goldsweig B, Kaminski B, Sidhaye A, Blackman SM, Kelly A (2019) Puberty in cystic fibrosis. J Cyst Fibros. https://doi.org/10.1016/j.jcf.2019.08.013

    Article  PubMed  Google Scholar 

  4. Schram CA, Stephenson AL, Hannam TG, Tullis E (2015) Cystic fibrosis and ovarian reserve: a cross-sectional study examining serum anti-mullerian hormone (amh) in young women. J Cyst Fibros 14(3):398–402. https://doi.org/10.1016/j.jcf.2014.09.008

    Article  CAS  PubMed  Google Scholar 

  5. Bower JK, Volkova N, Ahluwalia N, Sahota G, Xuan F, Chin A, Weinstock TG, Ostrenga J, Elbert A (2023) Real-world safety and effectiveness of elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis: Interim results of a long-term registry-based study. J Cyst Fibros. https://doi.org/10.1016/j.jcf.2023.03.002

    Article  PubMed  Google Scholar 

  6. Roe AH, Traxler S, Schreiber CA (2016) Contraception in women with cystic fibrosis: a systematic review of the literature. Contraception. https://doi.org/10.1016/j.contraception.2015.07.007

    Article  PubMed  Google Scholar 

  7. Jain R, Taylor-Cousar JL (2021) Fertility, pregnancy and lactation considerations for women with CF in the CFTR modulator Era. J Pers Med 11(5):418. https://doi.org/10.3390/jpm11050418

    Article  PubMed  PubMed Central  Google Scholar 

  8. Shteinberg M, Taylor-Cousar JL, Durieu I, Cohen-Cymberknoh M (2021) Fertility and pregnancy in cystic fibrosis. Chest 160(6):2051–2060. https://doi.org/10.1016/j.chest.2021.07.024

    Article  PubMed  Google Scholar 

  9. Nash EF, Middleton PG, Taylor-Cousar JL (2020) Outcomes of pregnancy in women with cystic fibrosis (CF) taking CFTR modulators - an international survey. J Cyst Fibros 19(4):521–526. https://doi.org/10.1016/j.jcf.2020.02.018

    Article  PubMed  Google Scholar 

  10. O’Connor KE, Goodwin DL, NeSmith A, Garcia B, Mingora C, Ladores SL, Rowe SM, Krick S, Solomon GM (2021) Elexacafator/tezacaftor/ivacaftor resolves subfertility in females with CF: a two center case series. J Cyst Fibros. https://doi.org/10.1016/j.jcf.2020.12.011

    Article  PubMed  PubMed Central  Google Scholar 

  11. Peng G, Taylor-Cousar JL, Lee M, Keller A, West NE, Kazmerski TM, Goralski JL, Aitken ML, Roe AH, Hadjiliadis D, Uluer A, Flume PA, Mody S, Bray LA, Jain R (2023) Association between unplanned pregnancies and maternal exacerbations in cystic fibrosis. J Cyst Fibros. https://doi.org/10.1016/j.jcf.2023.03.020

    Article  PubMed  Google Scholar 

Download references

Funding

This research received no financial support. The content of this article is solely the responsibility of the authors.

Author information

Authors and Affiliations

Authors

Contributions

Conceptualization: VD; Methodology: VD; Writing—original draft preparation: VD; Writing—review and editing: VD, GA, AG, FAB, LT; Supervision: VD.

Corresponding author

Correspondence to Valeria Daccò.

Ethics declarations

Conflict of Interest

The authors declare that they have no conflict of interest.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Daccò, V., Alicandro, G., Trespidi, L. et al. Unplanned pregnancies following the introduction of elexacaftor/tezacaftor/ivacaftor therapy in women with cystic fibrosis. Arch Gynecol Obstet 308, 1657–1659 (2023). https://doi.org/10.1007/s00404-023-07153-y

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00404-023-07153-y

Keywords

Navigation