Methods: Two audio-taped, semi-structured interviews were conducted with the woman with CF. Interviews were transcribed and reviewed for accuracy prior to analysis. Narrative analysis was conducted to identify key experiences and gaps in clinical care and research.
Results: Ana (pseudonym) is a 29-year-old woman with CF who was diagnosed with CF at four months old. She has homozygous DF508del, the most common CF genetic variant that affects approximately half of those with CF (CFF, 2017). She has multiple CF-related co-morbidities including: pancreatic insufficiency, recurrent sinusitis, and gastrointestinal obstructions. She began IVA/LUMA therapy in August 2015 shortly after FDA approval, and two weeks later found out that she was pregnant after trying unsuccessfully to conceive with her husband for two years. The only change in her regimen was IVA/LUMA. She reported that she always had thick cervical mucus, a classic clinical presentation of CF in women, which blocks sperm transport (McArdle, 2011). Within two weeks of IVA/LUMA, her pulmonary function tests increased by six percentage points, her energy levels rose, and her sinus congestion improved. When she happily notified her CF care team about her pregnancy, which she directly linked to IVA/LUMA, she was counseled to discontinue the drug because it has no specific safety profile available for pregnant women. She advocated to stay on the drug because of her improved quality of life. Her son was born slightly premature at 34 weeks gestation, and stayed in the neonatal intensive care for three weeks due to breathing difficulties. He is now a healthy toddler who is developmentally on track for his age. When he was seven months old, Ana found out that she was pregnant again. She was on IVA/LUMA throughout the first pregnancy and afterwards. She reported that with IVA/LUMA, her mucus, both from her lungs and cervix, were thinner. She continued to be on IVA/LUMA with her second pregnancy that has been largely smooth and uneventful. At the time of this abstract development, Ana is 38 weeks gestation and awaiting the arrival of another son.
Conclusion: There are a few anecdotal reports of women becoming pregnant on IVA/LUMA; however, there are no official numbers of these pregnancies being tracked by the CF Foundation’s Data Registry. Post-marketing research needs to be conducted in order to develop the safety profiles of these targeted therapeutics in order to facilitate an informed dialogue between CF providers and patients regarding the appropriate initiation and use of these drugs in women of childbearing age. CF providers must engage patients in regular and sensitive discussions related to reproductive and sexual health, including the potential for improved fertility due to CF drugs like IVA/LUMA, and the need for effective contraception if they do not wish to become pregnant. In a seminal study of women with CF, only 33% understood how CF affects fertility, and 84% reported not using any form of contraception while sexually active (Korzeniewska et al., 2009). Moreover, in another study of women with CF who became pregnant, 22% of these pregnancies were terminated due to either unplanned pregnancy or deteriorating health status (Roe et al., 2015). The vast implications for fertility, sexual, and reproductive health in this vulnerable group of women in the era of personalized medicine must be investigated. Pregnancy and motherhood are especially complex events for women with CF who have heavy treatment burdens related to their own health maintenance. An unexpected pregnancy has the potential to result in catastrophic outcomes for the woman with CF and/or her fetus. Therefore, it is critical to monitor any pregnancies that occur after initiation of CF targeted therapeutics like IVA/LUMA, and additional research is warranted to ensure their safe use in pregnant women.