Department of Respiratory and Sleep Medicine, Royal Children's Hospital.
Murdoch Childrens Research Institute.
Curr Opin Pulm Med. 2020 Nov;26(6):685-695. doi: 10.1097/MCP.0000000000000731.
With improving life expectancy and quality of life, sexual and reproductive health (SRH) has become an increasingly important aspect of patient-centered cystic fibrosis care. This review aims to describe advances in cystic fibrosis-related SRH and highlight optimal practices.
Recent publications suggest that people with cystic fibrosis follow a similar trajectory of sexual development and activity as their noncystic fibrosis peers, although contraception use is lower. Although fertility is reduced in patients with cystic fibrosis, improved survival and assisted reproductive technologies have led to an increasing pursuit and incidence of pregnancy. Cystic fibrosis transmembrane regulator modulators that correct the underlying cystic fibrosis defect might improve fertility and thus far appear safe in pregnancy, though data are limited.Despite medical knowledge of SRH in cystic fibrosis, patients continue to report they lack sufficient education about these aspects of their healthcare, and cystic fibrosis multidisciplinary teams are ill prepared to counsel their patients.
Understanding of the effects of cystic fibrosis on SRH continues to improve, although many questions remain regarding optimal care from the choice of contraception to the safety of cystic fibrosis-specific medications in pregnancy. Further development of cystic fibrosis-informed interdisciplinary specialist networks and a wider framework of practice would both enhance health outcomes and better support patients.
随着预期寿命和生活质量的提高，性与生殖健康（SRH）已成为以患者为中心的囊性纤维化护理中日益重要的方面。本文旨在描述囊性纤维化相关 SRH 的进展，并强调最佳实践。
最近的研究表明，囊性纤维化患者的性发育和性行为轨迹与非囊性纤维化患者相似，尽管避孕措施的使用率较低。尽管囊性纤维化患者的生育能力下降，但生存的改善和辅助生殖技术的应用导致了越来越多的妊娠追求和发生率。囊性纤维化跨膜转导调节因子（CFTR）调节剂可纠正潜在的囊性纤维化缺陷，可能改善生育能力，并且到目前为止，在妊娠期间似乎是安全的，尽管数据有限。尽管囊性纤维化患者对 SRH 的医学知识有所了解，但患者仍报告称，他们缺乏足够的医疗保健方面的教育，囊性纤维化多学科团队在为患者提供咨询方面准备不足。
尽管关于囊性纤维化对 SRH 的影响的理解在不断提高，但仍有许多问题有待解决，例如避孕方法的选择、囊性纤维化特定药物在妊娠期间的安全性等。进一步发展囊性纤维化相关的跨学科专业网络和更广泛的实践框架，将提高健康结果，并更好地支持患者。
