Contraceptive use in women with cystic fibrosis


A recent study examined the contraception preferences of women with cystic fibrosis and also revealed startling data about the unintended pregnancy rate in this patient population.


Nearly half of women of child-bearing age with cystic fibrosis use estrogen-containing oral contraceptive pills (OCPs), followed by condoms and long-acting reversible contraceptive (LARC) methods, according to a pilot study in the journal Contraception. The study, which linked reproductive health questions to the Cystic Fibrosis Foundation National Patient Registry (CFFNPR), also revealed that 33% of women with CF reported ever being pregnant, half of whom reported having at least one unintended pregnancy. 

“This rate is as high as the unintended rate of women nationally,” said principal investigator Emily Godfrey, MD, MPH, an associate professor of family medicine and ob/gyn at the University of Washington School of Medicine in Seattle.

Dr. Godfrey, who is an Expert Working Group Member for the Centers for Disease Control and Prevention’s (CDC) United States Medical Eligibility Criteria for Contraceptive Use (MEC), proposed many years ago that CF be added to the list “because persons with CF now live long into their reproductive years, thanks to recent scientific advances with their medications,” she said. “However, because persons with CF typically take seven or more different medications each day, it is uncertain whether there are drug interactions or other considerations when prescribing hormonal contraception.”

Recent evidence indicates that some of these recently approved CF drugs do indeed interact with hormonal contraception, according to Dr. Godfrey. “Very few studies have been conducted evaluating the safety of contraception in women with CF,” shetold Contemporary OB/GYN. “Thus I pursued this study so that people with CF and their healthcare providers could have better answers about the safety of contraception in CF.”

Atotal of 150 women with CF aged 18 to 49 who were also registrants of CFFNPR completed an online survey in which they reported their contraceptive use between 2012 and 2016. Overall, 42% reported used estrogen-containing OCPs, 34% relied on condoms and 27% on LARC methods such as intrauterine devices (IUDs) and implants. 

Dr. Godfrey noted that Pseudomonas aeruginosa phenotype is important in CF because the mucoid phenotype, as opposed to the non-mucoid phenotype, is associated with worse clinical outcomes. 

“While endogenous estrogen has been shown to be associated with an increase in mucoid pseudomonas infections, we did not find a significant association for mucoid Pseudomonas aeruginosa infection among progestin-only and estrogen-containing hormonal contraceptive users,” she said.

In contrast, risk of osteoporosis was significantly elevated among women with CF who used depot-medroxyprogesterone acetate compared to non-users: odds ratio (OR) = 5.36; 95% confidence interval (CI): 1.00 to 29.12. 

The most surprising finding from the survey was that 50% of women with CF who were ever pregnant reported having had an unintended pregnancy. “This is important because according to the U.S. MEC, CF represents a medical condition associated with increased risk for adverse health events associated with pregnancy,” she said. “Therefore it is imperative that contraceptive use among women with CF be optimized in order to reduce the risks of unintended pregnancy in this population.”

Based on the findings of the study, hormonal contraception, whether it contains estrogen or progestin only, is safe to use in CF. However, because CF is associated with increased risk for adverse health events in pregnancy, “women with CF should adopt the most highly effective methods of contraception if they do not plan to become pregnant,” Dr. Godfrey said. “Also, women who take lumacaftor (Orkambi) should avoid taking oral contraceptive pills if they wish to avoid unintended pregnancy.”


Dr. Godfrey said further research is needed for definitive answers to whether hormonal contraception adversely affects lung and bone health and nutritional status. 


Dr. Godfrey is a paid instructor for Merck and has received grants from Bayer and Cooper Surgical.

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