Contraception in CF

Multiple studies suggest rates of contraception use among women with CF are comparable to those of the general US population.[1] Rates range from 37% of young women ages 16-24 to 49% in a sample of adults.[2,3] Condoms and oral hormonal contraceptive pills are the most common forms of contraception used among women with CF. This correlates with rates for the general US female population.[4,5] Like others in the general population, women with CF have increased their use of long-acting reversible contraceptives (LARCs), like IUDs and implants, from 8% in 2016 to 27% in 2020.[3,5-7] Two reasons for this increase could be 1) the interaction of Orkambi™ with hormonal contraception and 2) the large number of people with CF who participate in clinical trials that require contraception use, and thus counseling by the research team.[8]

What kinds of contraception exist?

Myths about Contraception

CF Specific Contraception Considerations

Reasons to use contraception beyond pregnancy prevention

Women with CF may benefit from using hormonal contraceptives to treat an underlying medical condition. Females with CF may have amenorrhea or irregular periods, for instance, because of low weight, recurrent lung infections, or stress [20]. Hormonal contraception can help regulate a woman’s period or eliminate it altogether, which may be particularly helpful for women with anemia or low iron. Some women with CF also experience fewer pulmonary exacerbations and a slower rate of lung function decline when using hormonal contraceptives. They may also need fewer courses of antibiotics [21,22] [See Hormones chapter]. 

Contraception Post-Transplant

Transplant patients have unique contraceptive challenges. Post-transplant medications containing mycophenolate, such as CellCeptⓇ, can reduce the effectiveness of hormonal birth control and can cause birth defects in a developing fetus. Because the effects can be so severe, the drug contains a Risk Evaluation and Mitigation Strategy (REMS) and black box warning from the FDA. As part of the REMS, the FDA requires patients and providers to sign a consent form prior to transplant in order to be treated with this class of drugs [23]. Since transplant medications interact with CHC, another method of birth control is recommended to prevent pregnancy throughout the period patient is using mycophenolate and at least six weeks after stopping [24]. If patient wants an IUD, it must be placed before transplant. In the first two years after transplant there is a small risk of infection [25]. Generally, the US-MEC recommends that a woman wait a minimum of 1-3 years post-transplant to consider pregnancy, during which time her contraceptive choices are especially important for her health and well-being [23]. 

CFTR Modulators and Contraception 

While Orkambi ™ interacts with oral contraceptive pills, no other modulator does.

Many women with CF are reporting a “Trikafta baby boom.” Heltshe et al. recorded an increased pregnancy rate among women on KalydecoⓇ [26] While it is too soon to see if this result will hold for women on Trikafta™, there have been many anecdotal reports of women becoming pregnant shortly after initiating treatment. This highlights the need for providers to initiate conversations about contraception use and choices as well as pregnancy plans with their female patients before prescribing modulator therapy. 

Hormonal contraceptives may play a role in rashes that are occuring in women taking Trikafta™ [27]. Should a woman on Trikafta™ experience a rash, it is important to select another form of birth control before stopping the current form to prevent an unplanned pregnancy. Condoms and other barrier methods can be used as a last-minute substitute. In these circumstances, it is best to consult a physician. 

Psychosocial Implications

Many women with CF are often confused about whether they need contraception; they are also often uncertain about which option is best for them. From a young age, they face concerns about the possibility of being infertile. Consequently, many inaccurately believe they do not need to use contraception when they become sexually active [11].

Another concern many have is whether they are healthy enough to have a family. [see Family Building chapter]. It is therefore important for providers to understand that contraceptive use decisions can be fraught for many patients. Women with CF may decide – possibly at a young age – that they do not want to ever become pregnant. Providers should support these women in their decision. They can suggest LARCs over permanent forms of contraception, however, to preserve the woman’s option to make a different choice later in her life. In addition, preserving the sex organs can provide benefits for women with CF as they transition into menopause. [See the Menopause chapter]. 

What we want to know more about

There are major gaps in the research literature on contraception in CF. One way to aid contraception research in this area would be to add a question(s) about contraception use to the CF Foundation Patient Registry. Such a query would help provide long-term data about the efficacy, safety, lung health, and prescription interactions for each type of contraception and how it impacts women with CF. Godfrey et al. completed a pilot registry project that showed women with CF are amenable to CFF tracking such information [5].