Standards for the care of people with cystic fibrosis; establishing and maintaining health

Kevin Southern, Charlotte Addy, Scott Bell, Amanda Bevan, Urzula Borawska, Catherine Brown, Pierre-Régis Burgel, Brenda Button, Carlo Castellani, Audrey Chansard, Mark Chilvers, Gwyneth Davies, Jane Davies, Kris De Boeck, Dimitri Declercq, Michael Doumit, Pavel Drevinek, Isabelle Fajac, Silvia Gartner, Anna GeorgiopoulosSandra Gursli, Carina M. E. Hansen, Andrea Gramegna, Martin Hug, Elise Lammertyn, Edwina (Eddie) C. Landau, Ross Langley, Anna Middleton, Nicole Mayer-Hamblett, Peter Middleton, Monika Mielus, Lisa Morrison, Anne Munck, Barry Plant, Maarten Ploeger, Dominique Pougheon Bertrand, Tacjana Pressler, Bradley S. Quon, Thomas Radtke, Zoe L. Saynor, Ilan Shufer, Alan R. Smyth, Chris Smith, Silke Van Koningsbruggen-Rietschel

Research output: Contribution to journalArticlepeer-review

12 Downloads (Pure)

Abstract

This is the second in a series of four papers updating the European Cystic Fibrosis Society (ECFS) standards for the care of people with CF. This paper focuses on establishing and maintaining health. The guidance is produced using an evidence-based framework and with wide stakeholder engagement, including people from the CF community. Authors provided a narrative description of their topic and statements, which were more directive. These statements were reviewed by a Delphi exercise, achieving good levels of agreement from a wide group for all statements. This guidance reinforces the importance of a multi-disciplinary CF team, but also describes developing models of care including virtual consultations. The framework for health is reinforced, including the need for a physically active lifestyle and the strict avoidance of all recreational inhalations, including e-cigarettes. Progress with cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy is reviewed, including emerging adverse events and advice for dose reduction and interruption. This paper contains guidance that is pertinent to all people with CF regardless of age and eligibility for and access to modulator therapy.
Original languageEnglish
Pages (from-to)12-28
Number of pages17
JournalJournal of Cystic Fibrosis
Volume23
Issue number1
Early online date21 Dec 2023
DOIs
Publication statusPublished - 1 Jan 2024

Keywords

  • cystic fibrosis
  • guidance
  • standards
  • health
  • ECFS

Cite this