Reproductive health has become a major part of the conversation in cystic fibrosis (CF) care, thanks to the introduction of cystic fibrosis transmembrane conductance regulator (CFTR) modulators. These medications have transformed the quality of life for many patients with CF, allowing them to live longer, healthier lives and consider having biological children.
One of the most striking changes has been in fertility among women with CF. After the approval of the triple-combination modulator elexacaftor/tezacaftor/ivacaftor (Trikafta) in 2019 in the U.S. and in 2020 in the U.K., pregnancy rates nearly doubled in those countries. Women who once faced fertility challenges are beginning to conceive naturally, often to their surprise.
Expanded Reproductive Care Guidance
In response to these changes, the Cystic Fibrosis Foundation convened a multidisciplinary group of specialists to develop the first clinical set of guidance for reproductive health care throughout the lifespan. The guide, published in August 2026, recommends providing age-appropriate education early and discussing reproductive health at least annually starting in the teen years.
Women with CF are becoming pregnant more often and, overall, doing better during pregnancy than expected. CFTR modulators play an important role in this, as they can improve the cervical environment and increase fertility. However, women who do not want to become pregnant should not assume that having CF will prevent pregnancy, and they should discuss contraceptive options with their CF specialist and Ob/Gyn.
A preconception visit with a CF specialist can help patients optimize their health before trying to conceive. This visit may include assessments to manage or enhance lung function, respiratory health, and other CF-related health conditions. Patients should also meet with a genetic counselor to discuss carrier screening and reproductive genetic counseling options.
Historical Gaps, Contraception, and Medication Interactions
For much of CF care history, reproductive health received little attention, and many patients assumed they could not have biological children. Routine appointments typically prioritized lung function, weight stability, and daily symptom impact over fertility discussions. A common misunderstanding persists that women with CF do not require contraception, yet they have access to the full range of birth-control methods. The older modulator Lumacraftor/ivacaftor may lower hormonal contraceptive effectiveness, while newer CFTR modulators generally do not; patients should coordinate with both CF and obstetric specialists.
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CFTR modulators and many CF medicines cross the placenta, exposing the fetus; discontinuing an effective modulator without medical guidance can jeopardize the patient’s health. The disease can impair pancreatic function, leading 30-40% of adult patients to develop CF-related diabetes and increasing the risk of gestational diabetes during pregnancy. Men with CF who desire biological children usually require sperm retrieval combined with assisted reproductive technologies such as IVF. Ongoing research offers a glimpse of future options that may expand fertility possibilities for men with CF.
Pregnancy and Reproductive Health
Pregnancy for a woman with CF is still considered higher risk, with an raised risk of premature birth and gestational diabetes. Patients will need more frequent visits with their CF team and obstetric team, and their care team will closely monitor lung function, nutrition, weight gain, and medications. The new guidance recommends an oral glucose tolerance test before pregnancy and extra testing during pregnancy to manage blood sugar levels and reduce diabetes-related risks.
After delivery, breastfeeding is increasingly an option for women with CF, as their nutrition and weight are better managed. However, CFTR modulators do pass into breast milk, so patients should discuss breastfeeding with their CF care team and the baby’s pediatrician. Early research and case reports suggest that because CFTR modulators cross the placenta, babies with cystic fibrosis who are exposed to the drugs before birth may not be born with pronounced signs of CF.
Some of the common signs include high levels of immunoreactive trypsinogen (IRT) in the blood, severe jaundice, salty skin, or severe neonatal constipation. Ironically, this promising development creates a new challenge: Traditional newborn screening may miss CF in a baby who is exposed to CFTR mod.
Men with CF still face significant fertility challenges, as more than 95% are born without vas deferens. While CFTR modulators cannot restore a vas deferens that did not develop before birth, research suggests that prenatal exposure to these medications may preserve fertility later in life. The pace of change in cystic fibrosis care has been extraordinary, and reproductive health should no longer be an afterthought. The conversation is no longer just about managing cystic fibrosis today, but about helping patients plan for the life they want to build tomorrow.