Transforming Care Models in Cystic Fibrosis: A Review
Barry Lawrence Diener1, Maria Berdella2, Joan DeCelie-Germana3
1Division of Pediatric Pulmonary, Stony Brook Children's Hospital, Renaissance School of Medicine, Stony Brook University, Stony Brook, NY 11794, USA.
Insights
Cystic fibrosis (CF) care has transformed due to demographic shifts and new therapies like CFTR modulators. The multidisciplinary CF team model has evolved to meet changing patient needs, incorporating telehealth and shared decision-making.
Area of Science:
- Pulmonology
- Genetics
- Healthcare Management
Background:
- Cystic fibrosis (CF) is a chronic, multisystem disease requiring extensive multidisciplinary care.
- Historically viewed as a pediatric condition, CF now significantly affects adults, with a rising median patient age.
- Evolving patient demographics and medical advancements necessitate changes in CF care delivery.
Purpose of the Study:
- To review the transformation of cystic fibrosis care in the United States.
- To highlight key factors driving changes in CF care models and team structures.
- To examine adaptations in response to demographic shifts and therapeutic breakthroughs.
Main Methods:
- This is a narrative review focusing on the US experience.
- Analysis of demographic trends in cystic fibrosis patients.
- Examination of the impact of CFTR modulator therapies and the COVID-19 pandemic on care.
Main Results:
- CF care has shifted from pediatric to a combined pediatric and adult model.
- CFTR modulator therapies, telehealth, and remote monitoring are reshaping care.
- Increased patient and family involvement, including shared decision-making and coproduction, is evident.
Conclusions:
- The cystic fibrosis care model and team structure have significantly evolved.
- Adaptations include incorporating new technologies and patient-centered approaches.
- Future CF care will likely continue to integrate these advancements to meet the needs of an aging patient population.
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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