University of Texas Southwestern Medical Center, Dallas, Texas.
Department of Pediatrics, University of Pittsburgh School of Medicine.
Curr Opin Pulm Med. 2023 Nov 1;29(6):595-602. doi: 10.1097/MCP.0000000000001009. Epub 2023 Sep 1.
With improved long-term survival and the expanding availability of cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapies that treat the underlying genetic defect in cystic fibrosis, more people are interested in parenthood. Cystic fibrosis care centers and people with cystic fibrosis need more information to guide decisions related to parenting.
Here we present currently available data on fertility, pregnancy, and parenthood in the modern era of cystic fibrosis care. Fertility may be improving in female individuals with cystic fibrosis with the use of CFTR modulator therapies, and there is an associated increase in annual pregnancies. Infertility in male individuals with cystic fibrosis remains approximately 97-98% and is unchanged with CFTR modulators in those already born with cystic fibrosis. As more female individuals with cystic fibrosis experience pregnancy, questions remain about the impact of pregnancy on their health and that of their child. Fortunately, there are multiple routes to becoming a parent; however, more work is needed to understand the impact of pregnancy and parenthood in the context of CF as some previous data suggests potential challenges to the health of parents with cystic fibrosis.
We encourage cystic fibrosis care teams to have knowledge and resources available to support the reproductive goals of all individuals with cystic fibrosis.
目的综述:随着囊性纤维化跨膜电导调节因子(CFTR)调节剂治疗囊性纤维化的潜在遗传缺陷的长期生存和应用范围的扩大,越来越多的人对生育感兴趣。囊性纤维化护理中心和囊性纤维化患者需要更多信息来指导与生育相关的决策。
最新发现:在这里,我们介绍了在现代囊性纤维化护理时代与生育、妊娠和生育相关的现有数据。CFTR 调节剂的应用可能会改善女性囊性纤维化患者的生育能力,且每年妊娠的数量也有所增加。男性囊性纤维化患者的生育能力仍约为 97-98%,而在已经患有囊性纤维化的患者中,CFTR 调节剂对其没有影响。随着越来越多的女性囊性纤维化患者经历妊娠,关于妊娠对其自身和子女健康的影响的问题仍然存在。幸运的是,有多种途径可以成为父母;然而,需要进一步的研究来了解在囊性纤维化背景下妊娠和生育的影响,因为一些先前的数据表明囊性纤维化父母的健康可能存在潜在挑战。
总结:我们鼓励囊性纤维化护理团队了解并提供相关资源,以支持所有囊性纤维化患者的生育目标。