Poster Presentation - SOMANZ ASM Society of Obstetric Medicine of Australia and New Zealand ASM 2023

Considerations in managing the growing population of pregnant patients with cystic fibrosis (#52)

Anna Hunt 1 , Lisa Ward 2
  1. Obstetric Medicine, Gold Coast University Hospital, Gold Coast, QLD
  2. Endocrinology, Gold Coast University Hospital, Gold Coast, QLD

Trikafta, a triple combination cystic fibrosis transmembrane conductance regulator (CFTR) modulator, was approved by the TGA and listed on PBS in 2022. Treatment with Trikafta improves fertility in women with cystic fibrosis (CF) through its impact on cervical mucus viscosity alongside improving overall health and nutritional status.1,2 Pregnancy rates in the US doubled from 140 to 280 from 1998 to 2018.3 With the availability of this treatment, pregnancy rates in women with CF are likely to rise. 

CFTR modulators have been shown to cross the placenta, it is found in cord blood and at lower levels in breast milk.2.4 Teratogenicity has not been seen and there is limited long-term data hence Trikafta is TGA category 3B.4

We reviewed the cased of a 23-year-old patient with spontaneous pregnancy one month after commencing Trikafta, this was continued throughout the pregnancy. Preconception lung function was good with FEV1 3.45L (94.8% predicted) which remained stable throughout pregnancy with no requirement for hospitalisation. CF related diabetes (CFRD) required new insulin therapy. CF patients often have had a high fat high calorie diet that may require review when managing diabetes in pregnancy. Induction of labour occurred at 37 weeks, with delivery via emergency caesarean section under general anaesthetic for a fetal indication. Postpartum discharge was delayed due to cough and difficult airway clearance postoperatively. Pregnancy has not been shown to accelerate lung function loss but linked to more pulmonary exacerbations.

This case highlights the need for multidisciplinary management of such patients, with respiratory, maternal fetal medicine, obstetric medicine, neonatology, pharmacy, physiotherapy, diabetic educator and dietetics involved. Preconception counselling should call attention to nutrition, appropriate gestational weight gain, CFRD and its management in pregnancy, the risks and benefits of continuing Trikafta during pregnancy for both mother and child, and the importance of respiratory physiotherapy in late pregnancy and postpartum.

 

  1. 1. Tümmler B. (2023). Post-approval studies with the CFTR modulators Elexacaftor-Tezacaftor-Ivacaftor. Frontiers in pharmacology, 14, 1158207. https://doi.org/10.3389/fphar.2023.1158207
  2. 2. Bacalhau, M., Camargo, M., Magalhães-Ghiotto, G. A. V., Drumond, S., Castelletti, C. H. M., & Lopes-Pacheco, M. (2023). Elexacaftor-Tezacaftor-Ivacaftor: A Life-Changing Triple Combination of CFTR Modulator Drugs for Cystic Fibrosis. Pharmaceuticals (Basel, Switzerland), 16(3), 410. https://doi.org/10.3390/ph16030410
  3. 3. Taylor-Cousar J. L. (2020). CFTR Modulators: Impact on Fertility, Pregnancy, and Lactation in Women with Cystic Fibrosis. Journal of clinical medicine, 9(9), 2706. https://doi.org/10.3390/jcm9092706
  4. 4. Nash, E. F., Middleton, P. G., & Taylor-Cousar, J. L. (2020). Outcomes of pregnancy in women with cystic fibrosis (CF) taking CFTR modulators - an international survey. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 19(4), 521–526. https://doi.org/10.1016/j.jcf.2020.02.018