#NACFC2021 – Care Teams Should Discuss Pregnancy ‘Early and Often’

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by Marisa Wexler MS |

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Clinicians who are caring for people with cystic fibrosis (CF) should talk to their patients about pregnancy and family planning “early and often,” according to researchers.

In a presentation at the 2021 North American Cystic Fibrosis Conference (NACFC), Kristina Montemayor, MD, an assistant professor at Johns Hopkins University, also highlighted the need for greater expertise in caring for people with CF during and after pregnancy.

Her talk was titled, “Current Attitudes and Practices with Pre-Pregnancy Anticipatory Guidance and Pregnancy Care in Cystic Fibrosis: A National Survey of CF Providers.”

With the advent of new, highly effective therapies like CFTR modulators, people with CF are living longer and healthier lives. Along with this increase, more and more women with the disease are choosing to get pregnant.

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People with CF are at increased risk of pregnancy-related complications. Recently, a team of specialists and women with CF published a set of recommendations for how best to manage pregnancy — from planning for conception through care after birth — with CF.

“Despite the increasing number of pregnancies in women with CF, there are limited studies evaluating real-world pregnancy care in CF, and identifying any differences in the management of pregnancy and pregnancy-related complications and cystic fibrosis remains important to understand,” Montemayor said.

“Thus, the objective of our study was to assess current attitudes and practices among CF clinicians regarding pre-pregnancy anticipatory guidance and pregnancy care in CF,” she added.

Montemayor and colleagues conducted a survey of 93 CF clinicians in the U.S. Their research was funded by the CF Foundation.

About three-quarters of the respondents were physicians; the rest were nurses, physician’s assistants, and other providers. Roughly two-thirds of the respondents identified as female.

“An overall message [of the survey] is to discuss pregnancy early and often,” Montemayor said.

Specifically, 69% of the clinicians reported discussing pregnancy and family planning at routine visits. Most clinicians favored having these conversations early: 86% thought such conversations should start before patients reach the age of 21, and 67% supported initiating these talks prior to age 18.

The vast majority of clinicians were comfortable recommending mucus-clearing treatments, vitamin supplements, and pancreatic enzyme replacement therapies (PERT) for patients throughout their pregnancies — all of these CF therapies are generally established as safe for pregnant patients.

Most clinicians were also comfortable recommending CFTR modulators throughout pregnancies. Markedly more clinicians were comfortable recommending Kalydeco or the triple-combination therapy Trikafta, compared with the two dual-combination therapies Orkambi and Symdeko.

“We anticipate that this is related to the known efficacy of highly effective [CFTR] modulator therapy, and continuation of therapy provides an increased benefit to mothers with CF,” Montemayor said.

Antibiotic approaches endorsed by most clinicians for managing pulmonary exacerbations (times when lung symptoms suddenly worsen) were largely consistent with those given to patients who are not pregnant, Montemayor noted. However, overall confidence levels were generally low, highlighting a need for more research into best practices.

“Future prospective studies evaluating pulmonary exacerbation management during pregnancy would be of great importance to the CF community,” Montemayor said.

Notably, 1 in 5 clinicians reported being unaware of whether or not their patients were breastfeeding after giving birth. This is important to assess because it can affect the nutritional needs of the breastfeeding mother, and some medications may be excreted in breast milk, Montemayor said. Clinicians generally recommended against breastfeeding for people who are underweight.

“Clinician practices and comfort related to CF-specific aspects of pregnancy care vary,” Montemayor concluded. “We need to expand peri-pregnancy [around the time of pregnancy] expertise in the CF community, and future prospective studies are needed to understand the maternal-fetal effects of CF therapies.”

Editor’s note: The Cystic Fibrosis News Today team is providing coverage of the virtual 2021 North American Cystic Fibrosis Conference (NACFC) Nov. 2–5. Go here to see the latest stories from the conference.


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