ECFS best practice guidelines: the 2018 revision.

Area of Science:

• Pulmonology and Genetics
• Medical Best Practices
• Public Health

Background:

• Recent advancements in cystic fibrosis (CF) management have significantly improved patient survival rates.
• The expansion of newborn screening programs identifies CF patients with minimal lung disease at diagnosis.
• Emerging mutation-specific therapies and personalized medicine offer improved quality of life and life expectancy for individuals with CF.

Purpose of the Study:

• To revise and update the European consensus on best practice guidelines for cystic fibrosis (CF) care.
• To provide a comprehensive overview of expected standards in CF management.
• To support clinical teams in both developing and established CF centers.

Main Methods:

• Revision of the 2014 ECFS best practice guidelines for CF care.
• Incorporation of current developments in CF management, including newborn screening and personalized therapies.
• Consensus-based approach involving clinical experts and endorsed by relevant European networks.

Main Results:

• Presents updated standards for best practice across key aspects of CF care.
• Covers newborn screening, diagnosis, preventative lung disease treatment, nutrition, complications, transplant/end-of-life care, and psychological support.
• Provides a framework for high-quality CF care delivery throughout Europe.

Conclusions:

• The updated best practice guidelines are essential for optimizing CF care in a rapidly evolving field.
• These standards will assist clinical teams in delivering high-quality care, improving outcomes for individuals with CF.
• The document serves as a vital resource for advancing CF management globally.