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

•This is the second of four papers updating standards for the care of pwCF.•This paper focuses on establishing and maintaining health.•Guidance is evidence-based with wide engagement from across the CF community.•The guidance reinforces the importance of a multi-disciplinary CF team.•An active and h...

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Bibliographic Details
Published in:	Journal of cystic fibrosis Vol. 23; no. 1; pp. 12 - 28
Main Authors:	Southern, Kevin W, Addy, Charlotte, Bell, Scott C, Bevan, Amanda, Borawska, Urzula, Brown, Catherine, Burgel, Pierre-Régis, Button, Brenda, Castellani, Carlo, Chansard, Audrey, Chilvers, Mark A, Davies, Gwyneth, Davies, Jane C, De Boeck, Kris, Declercq, Dimitri, Doumit, Michael, Drevinek, Pavel, Fajac, Isabelle, Gartner, Silvia, Georgiopoulos, Anna M, Gursli, Sandra, Gramegna, Andrea, Hansen, Carina ME, Hug, Martin J, Lammertyn, Elise, Landau, Edwina (Eddie) C., Langley, Ross, Mayer-Hamblett, Nicole, Middleton, Anna, Middleton, Peter G, 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, van Koningsbruggen-Rietschel, Silke
Format:	Journal Article
Language:	English
Published:	Netherlands Elsevier B.V 01-01-2024
Subjects:	Cystic fibrosis Cystic Fibrosis - drug therapy Cystic Fibrosis Transmembrane Conductance Regulator - genetics ECFS Electronic Nicotine Delivery Systems Guidance Health Humans Mutation Respiratory System Agents - therapeutic use Guidance Cystic fibrosis ECFS Health Standards
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Summary:	•This is the second of four papers updating standards for the care of pwCF.•This paper focuses on establishing and maintaining health.•Guidance is evidence-based with wide engagement from across the CF community.•The guidance reinforces the importance of a multi-disciplinary CF team.•An active and healthy lifestyle is key, which includes avoiding e-cigarettes. 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.
Bibliography:	ObjectType-Article-2 SourceType-Scholarly Journals-1 ObjectType-Feature-3 content type line 23 ObjectType-Review-1
ISSN:	1569-1993 1873-5010
DOI:	10.1016/j.jcf.2023.12.002