Standards for the care of people with cystic fibrosis; establishing and maintaining health
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.
Author
Southern, Kevin
Addy, Charlotte
Bell, Scott
Bevan, Amanda
Borawska, Urzula
Brown, Catherine
Burgel, Pierre-Régis
Button, Brenda
Castellani, Carlo
Chansard, Audrey
Chilvers, Mark
Davies, Gwyneth
Davies, Jane
De Boeck, Kris
Declercq, Dimitri
Doumit, Michael
Drevinek, Pavel
Fajac, Isabelle
Gartner, Silvia
Georgiopoulos, Anna
Gursli, Sandra
Gramegna, Andrea
Hansen, Carina Me
Hug, Martin
Lammertyn, Elise
Landau, Edwina Eddie C
Langley, Ross
Mayer-Hamblett, Nicole
Middleton, Anna
Middleton, Peter
Mielus, Monika
Morrison, Lisa
Munck, Anne
Plant, Barry
Ploeger, Maarten
Bertrand, Dominique Pougheon
Pressler, Tacjana
Quon, Bradley S
Radtke, Thomas
Saynor, Zoe L
Shufer, Ilan
Smyth, Alan R
Smith, Chris
West, Natalie E
van Koningsbruggen-Rietschel, Silke
Addy, Charlotte
Bell, Scott
Bevan, Amanda
Borawska, Urzula
Brown, Catherine
Burgel, Pierre-Régis
Button, Brenda
Castellani, Carlo
Chansard, Audrey
Chilvers, Mark
Davies, Gwyneth
Davies, Jane
De Boeck, Kris
Declercq, Dimitri
Doumit, Michael
Drevinek, Pavel
Fajac, Isabelle
Gartner, Silvia
Georgiopoulos, Anna
Gursli, Sandra
Gramegna, Andrea
Hansen, Carina Me
Hug, Martin
Lammertyn, Elise
Landau, Edwina Eddie C
Langley, Ross
Mayer-Hamblett, Nicole
Middleton, Anna
Middleton, Peter
Mielus, Monika
Morrison, Lisa
Munck, Anne
Plant, Barry
Ploeger, Maarten
Bertrand, Dominique Pougheon
Pressler, Tacjana
Quon, Bradley S
Radtke, Thomas
Saynor, Zoe L
Shufer, Ilan
Smyth, Alan R
Smith, Chris
West, Natalie E
van Koningsbruggen-Rietschel, Silke
Citations
Altmetric:
Date
2023-12-21
Type
Article
Subject
Respiratory medicine, Patients. Primary care. Medical profession. Forensic medicine, Health services. Management
Collections
Citation
Southern K, Addy C, Bell S, Bevan A, Borawska U, Brown C, Burgel PR, Button B, Castellani C, Chansard A, Chilvers M, Davies G, Davies J, De Boeck K, Declercq D, Doumit M, Drevinek P, Fajac I, Gartner S, Georgiopoulos A, Gursli S, Gramegna A, Hansen CM, Hug M, Lammertyn E, Landau EEC, Langley R, Mayer-Hamblett N, Middleton A, Middleton P, Mielus M, Morrison L, Munck A, Plant B, Ploeger M, Bertrand DP, Pressler T, Quon BS, Radtke T, Saynor ZL, Shufer I, Smyth AR, Smith C, West NE, van Koningsbruggen-Rietschel S. Standards for the care of people with cystic fibrosis; establishing and maintaining health. J Cyst Fibros. 2023 Dec 20:S1569-1993(23)01727-7. doi: 10.1016/j.jcf.2023.12.002. Epub ahead of print.
Journal / Source Title
Journal of Cystic Fibrosis
DOI
10.1016/j.jcf.2023.12.002
PMID
38129255
Publisher
Elsevier
Publisher’s URL
http://www.sciencedirect.com/science/journal/15691993