Contraception in CF

Multiple studies suggest that females with CF can now expect to face the same sexual and reproductive health milestones as non-CF US females, including age of onset of sexual activity, number of partners, and sexual activity rate.[1–4] These studies also suggest that rates of contraception use among females with CF are lower than that of the non-CF US female population.[1–4]  Meiss and Kazmerski report, for instance, a 15% lower use of contraception in non-CF females.[3,5] Women with CF commonly use condoms and oral hormonal contraceptive pills as forms of contraception, which corresponds with the same preferences for the general US female population.[6–9] Like others, females with CF have increased their use of long-acting reversible contraceptives (LARCs), like IUDs and implants, from 8% in 2016 to 27% in 2020.[4,7–9] Two reasons for this increase could be: 1) the interaction of Orkambi™ with hormonal contraception and
2) the large number of females with CF who participate in clinical trials that require contraception use and involve counseling by the research team.[10] 

*Male and female condoms protect against STIs.

**To learn more about different forms of birth control, please see the Resource Section at the end of this chapter.

What to know about birth control and modulators

What kinds of contraception exist?

Contraception Counseling

Females with CF receive their contraceptive care from a variety of providers. In a survey of women with CF published in 2018, Kazmerski et al. found that OB/GYNs prescribe or administer 66% of contraceptives to women with CF, while CF clinics prescribe 7%.[11] Primary care providers (PCPs) and others prescribe only a tiny percentage.[11] Women with CF typically view their CF doctor as their primary physician, and 41% report no other primary care provider.[4,11] Researchers have shown that contraception counseling directly impacts contraception use, yet only 9% of young adults with CF have ever received such counseling from any source.[5]   Women with CF make contraceptive choices within the context of their CF and generally prefer to receive reproductive counseling from their CF team due to a perception of inadequate CF-specific knowledge among women’s health providers.[11]  A study conducted in France (2019) found that introducing a gynecological consult into the CF clinic increased rates of contraceptive use – especially of LARCs– among women with CF.[12] Even though American CF clinicians believe contraception counseling is an integral part of CF care, only a minority counsel their patients.[13] Barriers to patients and providers talking about contraception include patient and provider discomfort, lack of time, and lack of training in contraception counseling.[13] Women with CF report feeling that they had been inadequately educated on how different contraceptive methods would interact with their CF and medications and that there was a significant disconnect between their OB/GYNs and their CF teams.[5,11] Women with CF desire coordinated, defragmented reproductive health care and disease-specific reproductive health discussions.[11]   Ideally, providers should give contraception counseling to patients on all medically eligible contraception options to allow them to make the appropriate choices for their lives. The Centers for Disease Control’s (CDC) US Medical Eligibility Criteria for Contraceptive Use (US-MEC) provides “recommendations for using specific contraceptive methods by women…who have certain…medical conditions.” CF was added to this chart in 2016 through the work of Dr. Emily Godfrey.[14]  While safety data is currently limited for CF, the US-MEC table indicates “no risk” for most forms of contraception for CF (level 1). Only combined hormonal contraceptives are rated a Level 2, denoting that the “advantages generally outweigh theoretical or proven risks.”[14] Neither level confers a contraindication to using a method; patient preference can determine the method used.[6] It is important to note that many people with CF have comorbidities such as transplant status, liver disease, and diabetes that are also listed separately in the US-MEC guidelines. Please consult those specific guidelines and discuss which contraception options are safe and effective for each patient.

Myths about Contraception

Sources for this table include Shteinberg, Curtis, Rodriguez, Vertex, and Curtis in Works Cited.

Hormonal Contraception

Reasons to Use Contraception Beyond Pregnancy Prevention

Females with CF may benefit from hormonal contraceptives used to treat an underlying medical condition. Females may have amenorrhea (an absence of menstruation) or irregular periods because of low weight, recurrent lung infections, or illness-related stress.[25] Hormonal contraception can help regulate a female’s period or eliminate it, which may be particularly helpful for females with anemia or low iron. Some females with CF may experience fewer pulmonary exacerbations and a slower rate of lung function decline when they use hormonal contraceptives. They may also need fewer courses of antibiotics.[26,27] 

The use of hormonal contraception has been shown to reduce inflammation.[28] Hormonal contraception use in adolescents with CF has no adverse effect on FEV₁and may improve lung function if used for more than three years.[26]  [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 severe, the drug insert contains a risk evaluation and mitigation strategy (REMS) and a black box warning from the FDA. As part of the REMS, the FDA requires patients and providers to sign a consent form before transplant to be treated with this class of drugs.[29] Since transplant medications interact with combined hormonal contraception, it is recommended to use another method of birth control to prevent pregnancy during the period in which the patient is using mycophenolate and at least six weeks after stopping.[30] If a patient wants an IUD, it must be placed before transplant. In the first two years after a transplant, there is a small risk of infection.[31] Generally, the US-MEC recommends a female wait a minimum of 1-3 years post-transplant to consider pregnancy. During this time, her contraceptive choices are crucial for her health and well-being.[29] 

CFTR Modulators and Contraception 

While OrkambiTM interacts with oral contraceptive pills, no other modulator does!

Orkambi ™ interacts with oral contraceptive pills, but no other modulator does. Many females with CF are getting pregnant after starting Trikafta™, resulting in a “Trikafta baby boom.”[18,31]  Heltshe et al.reported that females on KalydecoⓇ also have an increased pregnancy rate.[32] These pregnancies highlight the need for providers to initiate conversations with their female patients about contraception choices and pregnancy plans before prescribing modulator therapy. 

Besides increased rates of pregnancy, females on Trikafta™ may also have rashes if on hormonal contraceptives.[33] If a female on Trikafta™ experiences a rash, it is important to select another form of birth control before she/they stop their current contraceptive method 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 females 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 incorrectly assume they do not need to use contraception when they become sexually active.[13] However, a recent paper reports that up to 38.9% of pregnancies are unplanned in the CF community.[2]  In the current political climate, a female with CF who unintentionally becomes pregnant may be unable to access termination easily.[34] Conversations around contraception are, therefore, critically important for persons with chronic illness. 

Another concern many females with CF have is whether they are healthy enough to have a family. [see Family Building chapter]. Providers need to understand that decisions about contraceptive use can be difficult for many patients. 

Females with CF may decide – possibly at a young age – that they do not want ever to become pregnant. Providers should support these females in their decisions. They may suggest LARCs over permanent forms of contraception to preserve a female’s choice to make a different decision later in her/their life. The provider can refer the patient to a gynecologist who is comfortable with providing permanent contraception to those who do not wish to have children naturally. Of note, preserving patients’ sex organs can offer benefits to females with CF as they transition into menopause. [See the Menopause chapter].

What Female with CF Want to Know More About

There needs to be more research on contraception in CF; one way to aid contraception research would be to add a question or questions 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 the CFF tracking such information.[7] Future research on contraception and CF should also focus on the comparative safety, efficacy, and side effects of various types of contraceptive options in a rapidly aging CF population. Examples could be a study of the effects of transdermal vs. oral CHCs on bone health or between low- and high-dose oral CHCs. 

Personal Narratives