Reproductive health discussions a key need in CF care: Study

Better routine care needed across lifespan, especially for pregnant women

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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A pregnant woman holds a teddy bear with one hand while cradling her belly with the other.

As pregnancy becomes more common among women with cystic fibrosis (CF), and more CF patients are becoming parents, there is a pressing need for CF clinics to incorporate discussions about reproductive health — with a focus on pregnant women — into routine care, a new study highlights.

“As the CF community continues to thrive in the new era of CF care, it is imperative that CF clinics develop a formal infrastructure to support the needs of women with CF across the lifespan, which includes motherhood,” the researchers wrote.

The team noted the “known reproductive issues and potential need for lung transplant in both men and women with CF,” but pointed out that “no … guidelines exist for this population” in current routine care.

“The purpose of this study was to explore CF healthcare providers practices regarding fertility and [fertility preservation] discussions among women with CF,” they wrote.

The study, “Provider Perspectives on Fertility and Fertility Preservation Discussions Among Women With Cystic Fibrosis,” was published in the journal INQUIRY: The Journal of Health Care Organization, Provision, and Financing.

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New treatments have created need for better reproductive health care

CF has historically been considered a disease of childhood. But advances in medical treatments have radically changed CF outcomes in recent years. Nowadays, most people with cystic fibrosis are expected to live until at least their late 40s, and that age keeps advancing.

As people with CF are living longer, many are choosing to become parents.

The journey to parenthood may be more complicated for CF patients, however: CF itself can cause a range of complications related to reproductive health and fertility. Plus, many individuals will eventually undergo a lung transplant, and after a transplant, patients need to take immune-suppressing medicines that can cause damage to a developing fetus if used during pregnancy.

Given these complications, it’s become increasingly important that healthcare providers have frank and open discussions about reproductive health with their CF patients. Among such topics are options for fertility preservation, or storing sperm or egg cells that can be used to make biological children in the future.

Now, a trio of scientists at the University of Alabama at Birmingham set out to investigate whether and how these conversations were being had, specifically focusing on the care of women with CF.

“Despite the known potential need for lung transplantation in the CF population and the desire of women with CF to become mothers, the timing, frequency, and content of conversations between these women and their CF care team related to fertility preservation (FP) or fertility in general is unknown,” the team wrote.

To that end, the team conducted interviews with 20 healthcare providers recruited from adult CF clinics across the U.S.. The interviewed providers included a doctor, as well as nurses, social workers, dietitians, and other specialists.

“A wide range of CF providers were chosen for inclusion in this study to obtain a broad perspective and be representative of the multi-disciplinary approach in delivering CF care,” the scientists wrote.

A major theme across the interviews was that providers are aware that CF care has changed dramatically, especially in the last decade or so with the introduction of CFTR modulators. These highly effective therapies can boost the activity of the faulty CFTR protein in people with certain CF-causing mutations.

As a result of the rapid changes, “what we know about fertility with women and CF is outdated and not accurate anymore,” one provider said. “As women live longer with higher lung function, their experience of pregnancy is gonna be really different than it was 15 years ago.”

Several providers remarked that they have seen an increase in the number of women choosing to get pregnant since the advent CFTR modulators, especially the triple-combination therapy Trikafta (elexacaftor/tezacaftor/ivacaftor).

“We’ve [the clinic] had a pregnancy boom. We’ve also had a few people start Trikafta to get pregnant. We have now a few patients who are pregnant on Trikafta,” one provider said.

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The providers noted that the CF specialty care team usually ends up serving as the main source of healthcare for people with CF. As such, they said it’s important for providers to be able to address the full spectrum of healthcare, including reproductive questions — especially given that other specialties may not be able to address CF-specific issues.

“We’re their primary medical team, and I think it’s prudent for us, because of that, to talk about the whole body,” one provider said.

“We encourage the women to establish care with the women’s health physician, but that’s not something so routine … There’s not a men’s health physician, and not everyone in women’s care is familiar with issues specific to CF,” another said, noting that the CF team are the “medical go-to” for many patients.

What we know about fertility with women and CF is outdated and not accurate anymore. … As women live longer with higher lung function, their experience of pregnancy is gonna be really different than it was 15 years ago.

Despite the acknowledgement that these conversations are important and often fall to the CF care team, several of the providers interviewed felt they themselves were ill-equipped to have these conversations.

“Many providers felt that they personally should not be the ones to do in-depth discussions about fertility or FP options with their patients due to a lack of knowledge,” the researchers wrote.

Providers noted that logistical issues and personal discomfort can be barriers to having these conversations. As such, they highlighted the importance of building trusting relationships and normalizing talking about reproduction as part of routine CF care.

“I think if you standardize it, nobody would [be] taken by surprise. . . we talk about it just like we talk about enzymes, and nutrition, and goin’ to college. . . it just becomes a part of their preventative care,” one provider said.

Providers also emphasized a need for better education about how CF affects reproduction, for both healthcare providers and patients.

“I think that it’s more about getting the staff and the physicians properly educated about what the options are so that we can gauge our patient on what they’re looking for, and then give them the best directions,” one said.

The researchers called for further efforts to integrate reproductive care into CF care, as well as research into how this can be done most effectively.