#NACFC2021 – Large Rise in Pregnancies With Trikafta Seen at Nationwide Children’s

Marisa Wexler, MS avatar

by Marisa Wexler, MS |

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Since the approval of Trikafta in the U.S., Nationwide Children’s Hospital in Ohio has seen a steep increase in the number of women with cystic fibrosis (CF) choosing to become pregnant.

“Due to the large pregnancy volume we saw, we learned a lot of lessons,” said Susan Shiyuan Li, MD, a CF clinician at Nationwide, who spoke at the 2021 North American Cystic Fibrosis Conference (NACFC).

Li particularly stressed the importance of multidisciplinary care for CF patients during pregnancy, and of educating them in what to expect so they can better manage their own health. Li’s presentation was titled, “Pregnancy and outcomes in the era of CFTR modulators and COVID-19 pandemic.” 

Trikafta, by Vertex Pharmaceuticals, is a combination therapy containing three CFTR modulators — medications that work to correct the activity of the CFTR protein (which is defective in CF) in people with specific disease-causing mutations.

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The medication was first approved in the U.S. in late 2019. That approval has since been expanded to include more mutations and children as young as age 6

In the first year Trikafta was widely available, between March 2020 and March 2021, 18 women with CF getting care at Nationwide became pregnant. To put this in perspective, that’s a pregnancy rate of 16% among the women of childbearing age with CF at the center — by comparison, the national rate of CF pregnancies in 2014 was about 2.5%.

Nearly all who became pregnant were using Trikafta, and remained on the medication throughout their pregnancies. 

This indicates that pregnancy may be easier to achieve for people on Trikafta — which, Li noted, is a point doctors should discuss with patients starting on the medication. However, people with CF are known to be at a higher risk for complications related to pregnancy.

“Due to the rapidly growing population, we quickly realized that we needed to develop a uniform and multidisciplinary approach” to caring for people with CF during pregnancy, Li said.

Li and other clinicians at Nationwide formed a “pregnancy taskforce” that included CF specialists as well as endocrinologists, dietitians, and a mental health coordinator. The team met every other week to discuss the pregnant patients, learning from their experiences.

“At the core of what we learned is the need to empower the patient to take care of her own health by providing her with the knowledge and education about her pregnancy,” Li said.

She noted the importance of trying to identify patients who are thinking about getting pregnant, but haven’t yet done so. Such women were scheduled for “pre-conception visits,” and could be given a crash course on what to expect while being pregnant with CF.

“We would help [the patients] understand all their risk factors going into pregnancy, and help them to understand that, because of this, they are going to be experiencing perhaps the highest burden of care in their lifetime in the upcoming months,” Li said.

It’s important for clinicians to be upfront about how much medical monitoring is involved, particularly in the age of Trikafta, because patients may be getting used to needing medical help less frequently, Li said.

Another noteworthy topic discussed in these early meetings is that certain antibiotics which patients may be used to taking to manage CF exacerbations — such as tetracyclines — are not safe for use in pregnancy.

Women who became pregnant were referred to a variety of supportive medical services depending on their particular situation — for example, special protocols were put in place for people with advanced lung disease, CF-related diabetes (CFRD), and/or mental health concerns.

Of the 18 pregnancies, one was electively terminated, and three ended in miscarriage — representing a “very similar miscarriage rate to that of the general population,” Li said.

The remaining 14 women went on to give birth to 15 babies, including one set of twins.

In general, not many major health problems marked these pregnancies, including among women with advanced lung disease or CFRD. Four women were admitted to emergency rooms, with “only one” due to a CF exacerbation. Other reasons included COVID-19 infection, and difficulty breathing due to carrying twins.

“Given the high complexity level of these patients, a multidisciplinary approach is definitely needed … and helps to improve outcomes,” Li concluded.

Li also noted areas where care could be improved, particularly with regard to the sheer amount of medical care needed — Li described some patients as having to decide about whether to prioritize certain parts of their care over others during and after pregnancy, on top of navigating life as a new parent.

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.