The survival of people with cystic fibrosis has improved dramatically over the past 60 years, such that adult patients now outnumber paediatric patients in some countries. ^,  This improvement can be attributed largely to advances in the treatment of complications of cystic fibrosis, but new therapies that target the underlying cause of cystic fibrosis—the absence or impaired functioning of the cystic fibrosis transmembrane conductance regulator (CFTR) protein—promise to transform health outcomes for patients with the disease. Studies of ivacaftor, the first CFTR modulator therapy, have convincingly shown short-term benefits and provided evidence that the drug can slow the progression of lung disease. Implementation during early childhood of such CFTR modulator therapy could offer patients with cystic fibrosis the opportunity of a healthy lifespan. As the outlook for patients with cystic fibrosis changes, the time seems right for all stakeholders to consider what the future might hold. In a Lancet Respiratory Medicine Commission paper, experts in cystic fibrosis from 18 countries, across six continents, present a blueprint for the future of clinical care for children and adults with cystic fibrosis. Their report is comprehensive, summarising future challenges and opportunities in cystic fibrosis care, some of which we believe are worthy of additional comment.

中文翻译：

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囊性纤维化的临床护理：为将来做准备。

在过去的60年中，囊性纤维化患者的生存率已大大提高，因此在某些国家，成年患者现在已超过儿科患者。^， 这种改善在很大程度上归因于囊性纤维化并发症的治疗进展，但是针对囊性纤维化根本原因（囊性纤维化跨膜电导调节剂（CFTR）蛋白的缺乏或功能受损）的新疗法有望改变健康状况。该病患者的预后。对ivacaftor（第一个CFTR调节剂疗法）的研究令人信服地显示出短期益处，并提供了该药物可减慢肺部疾病进展的证据。在儿童早期实施这种CFTR调节剂疗法可为囊性纤维化患者提供健康的寿命。随着囊性纤维化患者的前景发生变化，所有利益相关者似乎都应该考虑未来的时机。在一个柳叶刀呼吸医学委员会的论文，来自六大洲18个国家/地区的囊性纤维化专家介绍了为患有囊性纤维化的儿童和成人提供临床护理的未来蓝图。他们的报告是全面的，总结了囊性纤维化护理的未来挑战和机遇，我们认为其中一些值得进一步评论。