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Updated: Feb 1, 2026

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Published on: April 19, 2024
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Cystic Fibrosis: Advancing Along the Continuum
Summary
Cystic fibrosis (CF) is a genetic disorder impacting multiple body systems. Improved treatments mean CF patients live longer, requiring advanced care coordination by nurses.
Area of Science:
- Genetics and Molecular Biology
- Physiology
- Clinical Medicine
Background:
- Cystic Fibrosis (CF) is an autosomal recessive genetic disorder caused by mutations in the CF transmembrane conductance regulator (CFTR) gene.
- CFTR mutations lead to dysfunctional cellular protein channels, resulting in multisystemic dysfunction.
- Manifestations include obstructive lung disease, pancreatic insufficiency, CF-related diabetes, malabsorption, liver disease, and infertility.
Purpose of the Study:
- To highlight the evolving nature of Cystic Fibrosis (CF) care.
- To emphasize the critical role of Advanced Practice Nurses (APNs) in managing CF across the lifespan.
- To underscore the need for interprofessional collaboration and care transition in CF management.
Main Methods:
- Literature review on CF pathophysiology and treatment advancements.
- Analysis of current trends in pediatric and adult CF care models.
- Examination of the role and responsibilities of APNs in CF patient management.
Main Results:
- CF is no longer solely a pediatric disorder, with patients now living into adulthood.
- APNs face increasing complexity in managing "maturing-life" issues for CF patients.
- Effective CF care necessitates collaboration between pediatric APNs, adult APNs, nurse midwives, and medical specialists.
Conclusions:
- The management of Cystic Fibrosis (CF) requires a lifespan approach.
- Advanced Practice Nurses (APNs) are pivotal in coordinating care and facilitating transitions for CF patients.
- Open communication among patients, families, APNs, and CF centers is essential for optimal outcomes.
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