Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues

Pierre-Régis Burgel, Kevin W Southern*, Charlotte Addy, Alberto Battezzati, Claire Berry, Jean-Philippe Bouchara, Edwin Brokaar, Whitney Brown, Pilar Azevedo, Isabelle Durieu, Miquel Ekkelenkamp, Felicity Finlayson, Julian Forton, Johanna Gardecki, Pavla Hodkova, Gina Hong, Jacqueline Lowdon, Su Madge, Clémence Martin, Edward McKoneAnne Munck, Chee Y Ooi, Lucy Perrem, Amanda Piper, Andrew Prayle, Felix Ratjen, Margaret Rosenfeld, Don B Sanders, Carsten Schwarz, Giovanni Taccetti, Claire Wainwright, Natalie E West, Michael Wilschanski, Amanda Bevan, Carlo Castellani, Pavel Drevinek, Silvia Gartner, Andrea Gramegna, Elise Lammertyn, Eddie (Edwina) C Landau, Barry J Plant, Alan R Smyth, Silke van Koningsbruggen-Rietschel, Peter G. Middleton

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

2 Citations (Scopus)
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Abstract

This is the third 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 recognising and addressing CF health issues. The guidance was produced with wide stakeholder engagement, including people from the CF community, using an evidence-based framework. Authors contributed sections, and summary statements which were reviewed by a Delphi consultation.

Monitoring and treating airway infection, inflammation and pulmonary exacerbations remains important, despite the widespread availability of CFTR modulators and their accompanying health improvements. Extrapulmonary CF-specific health issues persist, such as diabetes, liver disease, bone disease, stones and other renal issues, and intestinal obstruction. These health issues require multidisciplinary care with input from the relevant specialists. Cancer is more common in people with CF compared to the general population, and requires regular screening. The CF life journey requires mental and emotional adaptation to psychosocial and physical challenges, with support from the CF team and the CF psychologist. This is particularly important when life gets challenging, with disease progression requiring increased treatments, breathing support and potentially transplantation. Planning for end of life remains a necessary aspect of care and should be discussed openly, honestly, with sensitivity and compassion for the person with CF and their family.

CF teams should proactively recognise and address CF-specific health issues, and support mental and emotional wellbeing while accompanying people with CF and their families on their life journey.

Original languageEnglish
Number of pages16
JournalJournal of Cystic Fibrosis
Early online date16 Jan 2024
DOIs
Publication statusEarly online date - 16 Jan 2024

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