Are You Talking About Pregnancy with Your Female Cystic Fibrosis Patients?
An increasing number of your female cystic fibrosis (CF) patients have been questioning you about pregnancy and fertility. This is unlikely to change any time soon, as recent data shows that almost 80% of adolescents and young women with CF plan to have children (1). With no formal guidelines on CF and pregnancy, you may lack confidence in answering some of their questions.
As the life expectancy of those living with CF has increased significantly, pregnancy rates in this population have almost doubled in recent years (2,3). Patients are asking their CF providers for more support and resources regarding pregnancy. Despite this, data suggest patients feel they are not provided sufficient and timely knowledge about their reproductive options from their CF teams (4–6).
You want to address this, but it’s overwhelming—and it’s not just you. Feelings of overwhelm and discomfort are common among providers when speaking to patients about CF-specific pregnancy and fertility issues (1,3,7). If you had the information you needed, you could confidently educate your patients, and even proactively initiate conversations about fertility to ensure the best outcomes.
As a nurse or nurse practitioner, you’re the perfect person to have these conversations, since you’re often the primary point of contact for patients (7,8). To confidently guide them, it’s important you are aware of the current literature about pregnancy and CF. This article will focus on three common questions asked by CF patients who are considering pregnancy to help you navigate these important discussions.
Question 1: Am I healthy enough for pregnancy?
Many women with CF report CF-related health issues as one of their primary concerns when considering pregnancy (1,6). In order to guide your patients, you need to know which health metrics are most relevant to assess for pregnancy outcomes. One key metric is baseline lung function, which can be assessed by measuring the forced expiratory volume (FEV1) (4,9,10). Poor pregnancy outcomes have been associated with an FEV1 of less than 40-50% (3,4,11,12). Therefore, sufficient airway management should be discussed prior to conception (4).
Another key metric is pancreatic function. Pancreatic insufficiency is common in CF, affecting 85% of individuals and impacting a patient’s ability to appropriately absorb nutrients (13–15). Thus, it is associated with increased maternal morbidity (13). To mitigate this, nutritional optimization through diet, pancreatic enzyme replacement therapies, or vitamin repletion should all be considered prior to pregnancy (13).
Question 2: How will pregnancy impact my CF treatment?
A major worry that many patients have is whether they will be able to continue their medications, like cystic fibrosis transmembrane receptor (CFTR) modulators, that are necessary to manage their CF (5). As a nurse practitioner, you may feel this is a challenging concern to address since the effects of CF medications in pregnancy have not been studied during clinical trials (2,4,8).
However, some helpful information is available in surveys, case studies, and post-market surveillance data. Overall, the data suggest that CFTR modulators are generally well-tolerated during pregnancy (3,11,13). In some cases, where these modulators were discontinued during pregnancy, patients experienced a significant decline in lung function and had to be put back on the modulators for their safety (11). The data is still somewhat limited, so it is best to carefully consider the benefits versus risks for each patient.
Pregnancy also increases the risk of certain complications, such as CF-related diabetes (CFRD). This means patients may need to prepare for additional glucose monitoring, screening for gestational diabetes, and potentially more hospitalization if CFRD-related complications arise (3,13,14).
Question 3: How will my CF impact my baby’s health?
Women with CF will likely be worried about how their disease will impact the baby. It is reassuring that, in recent years, there have been many successful pregnancies in patients with CF (14). However, there are some CF-related risks to the baby. Studies have shown a ~30% increased risk of delivery via C-section and early delivery [at or before 37 weeks] (3,15). In addition, CF is heritable, so there is a risk of the baby being a carrier of, or affected by, the disease (9).
Given the complexity of this population, knowing which specialists your patient may need to be referred to is important (6). Since pregnancy in a patient with CF is considered high-risk, the input of maternal-fetal medicine specialists may be required, especially if a CF center isn’t nearby (1,10). They can help lead a collaborative approach for the pregnancy, delivery, and postpartum period (4).
To assess the risk of babies having CF, it is also advisable that you refer patients to a genetic counselor (9) There are over 300 CF disease-causing mutations, not all of which can be detected by common genetic tests. This applies especially to non-Caucasian patients, for whom the identification of relevant disease-causing mutations have reportedly been missed in 30% of affected pregnancies (3). Genetic counselors are well-poised to assess the need for more comprehensive testing, such as next-generation sequencing, based on factors like the patient’s family history and clinical presentation (3).
These are just three of the many possible questions your patients may ask, and each situation must be considered independently. But, as you initiate more of these conversations, you will help create a safe space for patients to feel supported and comfortable when seeking professional guidance.
Advances in CF research have been so effective that you’re now facing new questions from your CF patients, but the conversations can carry an optimistic tone. You can have the confidence to help them make informed long-term decisions as they navigate pregnancy and CF. Be proactive and speak to your female patients about their reproductive options.
References
1. Leech MM, Stransky OM, Talabi MB, Borrero S, Roe AH, Kazmerski TM. Exploring the reproductive decision support needs and preferences of women with cystic fibrosis. Contraception. 2021;103(1):32-37. doi:10.1016/j.contraception.2020.10.004
2. Jain R, Magaret A, Vu PT, et al. Prospectively evaluating maternal and fetal outcomes in the era of CFTR modulators: the MAYFLOWERS observational clinical trial study design. BMJ Open Respir Res. 2022;9(1):e001289. doi:10.1136/bmjresp-2022-001289
3. Jain R, Kazmerski TM, Zuckerwise LC, et al. Pregnancy in cystic fibrosis: Review of the literature and expert recommendations. Journal of Cystic Fibrosis. 2022;21(3):387-395. doi:10.1016/j.jcf.2021.07.019
4. Hughan KS, Daley T, Rayas MS, Kelly A, Roe A. Female reproductive health in cystic fibrosis. Journal of Cystic Fibrosis. 2019;18:S95-S104. doi:10.1016/j.jcf.2019.08.024
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7. Corcoran J, Campbell C, Ladores S. Provider Perspectives on Fertility and Fertility Preservation Discussions Among Women With Cystic Fibrosis. Inquiry. 2023;60:00469580231159488. doi:10.1177/00469580231159488
8. Ladores S, Kazmerski TM, Rowe SM. A Case Report of Pregnancy During Use of Targeted Therapeutics for Cystic Fibrosis. Journal of Obstetric, Gynecologic & Neonatal Nursing. 2017;46(1):72-77. doi:10.1016/j.jogn.2016.08.011
9. Kazmerski TM, West NE, Jain R, et al. Family-building and parenting considerations for people with cystic fibrosis. Pediatric Pulmonology. 2022;57(S1):S75-S88. doi:10.1002/ppul.25620
10. Geake J, Tay G, Callaway L, Bell SC. Pregnancy and cystic fibrosis: Approach to contemporary management. Obstet Med. 2014;7(4):147-155. doi:10.1177/1753495X14554022
11. Gur M, Pollak M, Bar-Yoseph R, Bentur L. Pregnancy in Cystic Fibrosis—Past, Present, and Future. J Clin Med. 2023;12(4):1468. doi:10.3390/jcm12041468
12. Shteinberg M, Taylor-Cousar JL, Durieu I, Cohen-Cymberknoh M. Fertility and Pregnancy in Cystic Fibrosis. Chest. 2021;160(6):2051-2060. doi:10.1016/j.chest.2021.07.024
13. Montemayor K, Tullis E, Jain R, Taylor-Cousar JL. Management of pregnancy in cystic fibrosis. Breathe. 2022;18(2). doi:10.1183/20734735.0005-2022
14. Nast D, Paniagua C, Anderson P. Cystic fibrosis: A clinician’s tool for management of care advancing into the adult population. Journal of the American Academy of Nurse Practitioners. 2012;24(11):625-632. doi:10.1111/j.1745-7599.2012.00763.x
15. Ashcroft A, Chapman S, Mackillop L. The outcome of pregnancy in women with cystic fibrosis: a UK population-based descriptive study. BJOG: An International Journal of Obstetrics & Gynaecology. 2020;127(13):1696-1703. doi:10.1111/1471-0528.16423