Goralski Jennifer L, Talati Asha N, Hardisty Emily E, Vora Neeta L
Division of Pulmonary and Critical Care Medicine, the Division of Pediatric Pulmonology, and the Division of Maternal Fetal Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.
Obstet Gynecol. 2025 Jan 1;145(1):47-54. doi: 10.1097/AOG.0000000000005732. Epub 2024 Sep 19.
With improvements in overall health attributable to newly available medications called highly effective modulator therapy, an increasing number of people with cystic fibrosis (CF) are pursuing pregnancy. However, the safety of these medications for pregnant people with CF and the fetus remains largely unknown. Limited data demonstrate a decline in patients' health and well-being after withdrawal of highly effective modulator therapy during pregnancy; however, both animal and human studies suggest an association between highly effective modulator therapy and cataracts in the offspring that requires further investigation. Use of highly effective modulator therapy can also affect the results of newborn screening and may influence fetal outcomes among fetuses affected by CF as a result of transplacental passage of highly effective modulator therapy. An ongoing prospective cohort study will likely provide more information for pregnant people with CF. Until then, multidisciplinary counseling continues to be critical for people with CF who are of reproductive age.
随着一种名为高效调节剂疗法的新型药物使整体健康状况得到改善,越来越多的囊性纤维化(CF)患者正在备孕。然而,这些药物对患有CF的孕妇及其胎儿的安全性在很大程度上仍然未知。有限的数据表明,孕期停用高效调节剂疗法后患者的健康和幸福感会下降;然而,动物和人体研究均表明,高效调节剂疗法与后代白内障之间存在关联,这需要进一步研究。使用高效调节剂疗法还会影响新生儿筛查结果,并且由于高效调节剂疗法可经胎盘传递,可能会影响受CF影响的胎儿的结局。一项正在进行的前瞻性队列研究可能会为患有CF的孕妇提供更多信息。在此之前,多学科咨询对育龄CF患者而言仍然至关重要。