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.
Healthcare (Basel, Switzerland)
|December 11, 2025
Summary
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.
More Related Videos
Related Concept Videos
01:24Cystic Fibrosis: Management
443
Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
Sinus disease and chronic...
443
01:23Cystic Fibrosis: Pathogenesis
679
Cystic fibrosis (CF), an autosomal recessive disorder, significantly affects the function of exocrine glands. This genetically inherited disease is characterized by the production of thick and sticky mucus, which can severely affect various organs and systems in the body.
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
679
01:15Methods of Documentation VI: Case Management Model
837
The case management model is a multidisciplinary approach that involves healthcare professionals from diverse disciplines, such as physicians, nurses, therapists, social workers, and pharmacists, working collaboratively to address the various needs of patients. Each healthcare professional brings unique expertise and perspectives, contributing to a more comprehensive understanding of the patient's condition and tailoring treatment plans accordingly.
For example, a patient with a chronic...
For example, a patient with a chronic...
837
01:29Chronic Pancreatitis II: Collaborative Care
286
The management of chronic pancreatitis is multifaceted, involving a comprehensive approach that includes thorough assessment, diagnostic testing, and a variety of management strategies.
Assessment:
Assessment:
286
01:30Chronic Obstructive Pulmonary Disease-V: Nursing Management
5.0K
Nursing management of Chronic Obstructive Pulmonary Disease (COPD) is crucial for providing thorough care and support to patients. Nurses play an integral role in this process through detailed assessment, careful planning, targeted interventions, and ongoing evaluation. Here's an overview of the critical steps in nursing management for COPD.
Assessment
Assessment
5.0K
01:28Chronic Kidney Disease III: Interprofessional Care
320
Chronic kidney disease (CKD) requires collaborative and comprehensive management. CKD progresses through stages and can lead to end-stage kidney disease (ESKD) if untreated. Interprofessional collaboration and patient education are crucial, enabling patients to manage their health and improve their quality of life.Diagnostic approach for chronic kidney diseaseThe diagnosis of CKD primarily focuses on the glomerular filtration rate (GFR), which assesses kidney function by measuring how well...
320