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

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Burgel, Pierre-Régis, Southern, Kevin W ORCID: 0000-0001-6516-9083, Addy, Charlotte, Battezzati, Alberto, Berry, Claire, Bouchara, Jean-Philippe, Brokaar, Edwin, Brown, Whitney, Azevedo, Pilar, Durieu, Isabelle et al (show 34 more authors) , Ekkelenkamp, Miquel, Finlayson, Felicity, Forton, Julian, Gardecki, Johanna, Hodkova, Pavla, Hong, Gina, Lowdon, Jacqueline, Madge, Su, Martin, Clémence, McKone, Edward, Munck, Anne, Ooi, Chee Y, Perrem, Lucy, Piper, Amanda, Prayle, Andrew, Ratjen, Felix, Rosenfeld, Margaret, Sanders, Don B, Schwarz, Carsten, Taccetti, Giovanni, Wainwright, Claire, West, Natalie E, Wilschanski, Michael, Bevan, Amanda, Castellani, Carlo, Drevinek, Pavel, Gartner, Silvia, Gramegna, Andrea, Lammertyn, Elise, Landau, Eddie Edwina C, Plant, Barry J, Smyth, Alan R, van Koningsbruggen-Rietschel, Silke and Middleton, Peter G (2024) Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. S1569-1993(24)00005-5-S1569-1993(24)00005-5.

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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.

Item Type:	Article
Uncontrolled Keywords:	Breathing, CFRD, Complications, Cystic fibrosis, End of life, Liver, Lung infection, Mental health, Pulmonary, Transplant
Divisions:	Faculty of Health and Life Sciences Faculty of Health and Life Sciences > Institute of Life Courses and Medical Sciences
Depositing User:	Symplectic Admin
Date Deposited:	25 Jan 2024 09:46
Last Modified:	25 Jan 2024 09:47
DOI:	10.1016/j.jcf.2024.01.005
Open Access URL:	https://doi.org/10.1016/j.jcf.2024.01.005
Related URLs:	Author Publisher
URI:	https://livrepository.liverpool.ac.uk/id/eprint/3178013