Transforming Care Models in Cystic Fibrosis: A Review

囊性纤维化护理模式转型:综述

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Abstract

Cystic fibrosis (CF) is a multisystemic, chronic disease that requires a large multidisciplinary team for effective treatment. Over the past 20 years, the landscape of cystic fibrosis care has evolved from an almost exclusively pediatric disease to both a pediatric and adult condition. The median age of cystic fibrosis patients is rising, and the number of adults with CF is also increasing. With new developments in cystic fibrosis care, patients' health and needs have changed, and therefore the care model of the cystic fibrosis team has also changed. The introduction of highly effective CFTR modulator therapy, the COVID-19 pandemic, and the partnership of people with CF (PwCF) and their families have catalyzed the transformation of the CF care model, which includes the growth and evolution of the CF care team given the changes in the demographics of CF patients and the incorporation of telehealth and remote patient monitoring, shared decision-making, and coproduction of care. This narrative review, focusing on the United States (US) experience, explores the transformation of CF care, highlighting demographic changes, medical breakthroughs, and systemic adaptations.

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