As medicine progresses, people with cystic fibrosis (CF) live longer and healthier lives. Life goals evolve accordingly and for many, the future includes parenthood. It is estimated that almost 80% of young women with CF want to become pregnant.
However, guidelines to assist these women are scarce currently. “Don’t get me wrong, the CF and Ob/Gyn [obstetrician-gynecologist] care teams supported me through both pregnancies and I appreciate all of their hard work. But without research and data, my clinicians were forced to make their best guess with limited evidence to guide my care,” said a mother with CF.
To fill this gap, Jain et al compiled care recommendations from prepregnancy to postpartum in a review article published in the Journal of Cystic Fibrosis.
The ability of women with CF to conceive naturally has increased since the approval of CF transmembrane conductance regulator (CTFR) modulator therapies. Hence, health care providers (HCPs) should advise patients of the possibility of unplanned pregnancies and provide information about contraceptive options if pregnancy is not desired.
For those who are unable to conceive naturally, in vitro fertilization and intrauterine insemination of sperm are the most commonly used assisted reproductive technologies. In addition, surrogacy and adoption are options for those who are not able to become pregnant or whose health is a concern. All the available options should be communicated to patients.
Ideally, preconception consultation should be performed by a multidisciplinary team. Maternal health status should be evaluated in detail at preconception, as some aspects are known to contribute to pregnancy outcome (eg, weight, pulmonary and cardiac function, pancreatic insufficiency, CF-related diabetes control, and bacterial burden). The risks of the pregnancy should be carefully weighed.
“If prepregnancy obstetrics consultation did not occur, early referral to an obstetric team specializing in high‐risk pregnancies including those in people with CF, and regular communication between the CF and obstetrics teams is essential,” Kazmerski and colleagues write in Pediatric Pulmonology.
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Genetic counseling should be part of pregnancy planning. Reproductive partners should be offered CF carrier screening during preconception. If a partner is identified as a CF carrier, the couple should be referred for additional genetic counseling to discuss reproductive options and prenatal diagnostic testing.
Moreover, it is relevant to discuss the importance of strict follow-up care and the eventual need for additional clinic visits and antibiotics use during pregnancy.
Pregnancy and Delivery
Most commonly used CF-related medications can be used during pregnancy under appropriate guidance. The risks and benefits have to be carefully calculated for each specific scenario.
Women with CF must be screened for gestational diabetes. Insulin can be used for glycemic control in pregnant women with CF, but they may have higher insulin requirements than women without CF. Data supporting the use of oral alternatives to insulin is scarce.
Anesthesiologists should be involved in delivery planning to discuss the benefits of early regional analgesia and other relevant issues. Cesarean delivery should be reserved for the usual obstetric indications.
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At delivery, HCPs must ensure adequate hemodynamic monitoring, especially for patients with cardiac and pulmonary disease, due to the expected increase in maternal cardiac output.
Women with CF seem to be more likely to have cardiac conduction disorders, diabetes, asthma, thrombophilia, and anemia following vaginal delivery than do women without CF. They may also require longer hospital stays. The occurrence of deaths and the need for mechanical ventilation, although rare, are more likely to occur in women with CF than in women without CF.
It is essential to motivate women with CF for early mobilization in the immediate postpartum period due to the risks of thromboembolism, infectious morbidity, and deconditioning. Jain et al also recommend early chest physiotherapy and eventual resumption of medications that may have been paused during pregnancy if appropriate and safe for lactation.
Regarding infant outcomes, congenital anomalies, in particular, cardiac anomalies, seem to be more frequent in infants born to women with CF than in those to women without CF. Rates of neonatal and infant deaths were similar in both populations.
In addition, among the women with CF, those with a percent predicted forced expiratory volume in 1 second less than 60% may have a higher chance of delivering premature, lower birth weight babies.
Despite the difficulty of maintaining the recommended number of clinical visits in the immediate postpartum period, it is important to ensure maternal health stability. If needed, HCPs should consider alternative approaches for follow-up. Also, postpartum consultations should be performed by a multidisciplinary team.
Again, most commonly used CF-related medications are considered safe to use during lactation, although data is still limited. Moreover, all contraceptive methods seem to be effective and safe for women with CF, although lumacaftor/ivacaftor (Orkambi®) reduces the effectiveness of hormonal contraception. Relevant comorbidities must be considered when discussing contraception for women with CF.
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Women with CF should beware of their nutritional status postpartum. A lactating woman with CF may require more than 500 kcal extra per day. Regimens aiming at energy restriction and/or calorie intake are discouraged.
Special Considerations for Transplanted Women
There are cases of successful pregnancies in women with CF who have undergone lung transplantion. However, these are considered high-risk pregnancies and should be monitored accordingly.
“There are some unique challenges to consider in women with CF who have undergone lung transplantion such as maternal rejection of the transplanted lung (graft rejection) and infant complications secondary to status of maternal health or from immunosuppressive medications,” Jain et al explained.
Hence, transplantation centers usually recommend that women in these circumstances either avoid pregnancy or wait at least 2 years after transplantation to decrease the impact of the use of immunosuppressive medicines prior to pregnancy. Such medication should be used with caution during pregnancy. Moreover, as many immunosuppressant medications pass through breast milk, they are not recommended during lactation.
Jain R, Kazmerski TM, Zuckerwise LC, et al. Pregnancy in cystic fibrosis: review of the literature and expert recommendations. J Cyst Fibros. 2022;21(3):387-395. doi:10.1016/j.jcf.2021.07.019
Kazmerski TM, West NE, Jain R, et al. Family-building and parenting considerations for people with cystic fibrosis. Pediatr Pulmonol. 2022;57(Suppl 1):S75-S88. doi:10.1002/ppul.25620
Bray LA, Campbell CM, Brown J, Ladores S. Paths to motherhood for women with cystic fibrosis. MCN Am J Matern Child Nurs. 2022;47(3):147-153. doi:10.1097/NMC.0000000000000812