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

Pierre-Régis Burgel¹, Kevin W Southern², Charlotte Addy³, Alberto Battezzati⁴, Claire Berry⁵, Jean-Philippe Bouchara⁶, Edwin Brokaar⁷, Whitney Brown⁸, Pilar Azevedo⁹, Isabelle Durieu¹⁰, Miquel Ekkelenkamp¹¹, Felicity Finlayson¹², Julian Forton¹³, Johanna Gardecki¹⁴, Pavla Hodkova¹⁵, Gina Hong¹⁶, Jacqueline Lowdon¹⁷, Su Madge¹⁸, Clémence Martin¹⁹, Edward McKone²⁰, Anne Munck²¹, Chee Y Ooi²², Lucy Perrem²³, Amanda Piper²⁴, Andrew Prayle²⁵, Felix Ratjen²⁶, Margaret Rosenfeld²⁷, Don B Sanders²⁸, Carsten Schwarz²⁹, Giovanni Taccetti³⁰, Claire Wainwright³¹, Natalie E West³², Michael Wilschanski³³, Amanda Bevan³⁴, Carlo Castellani³⁵, Pavel Drevinek³⁶, Silvia Gartner³⁷, Andrea Gramegna³⁸, Elise Lammertyn³⁹, Eddie Edwina C Landau⁴⁰, Barry J Plant⁴¹, Alan R Smyth⁴², Silke van Koningsbruggen-Rietschel⁴³, Peter G Middleton⁴⁴

Affiliations

¹ Respiratory Medicine and Cystic Fibrosis National Reference Center, Cochin Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), Institut Cochin, Inserm U1016, Université Paris-Cité, Paris, France.
² Department of Women's and Children's Health, Institute in the Park, Alder Hey Children's Hospital, University of Liverpool, Eaton Road, Liverpool L12 2AP, UK. Electronic address: kwsouth@liv.ac.uk.
³ All Wales Adult Cystic Fibrosis Centre, University Hospital Llandough, Cardiff and Vale University Health Board, Cardiff, UK.
⁴ Clinical Nutrition Unit, Department of Endocrine and Metabolic Medicine, IRCCS Istituto Auxologico Italiano, and ICANS-DIS, Department of Food Environmental and Nutritional Sciences, University of Milan, Milan, Italy.
⁵ Department of Nutrition and Dietetics, Alder Hey Children's NHS Trust, Liverpool, UK.
⁶ University of Brest, Fungal Respiratory Infections Research Unit, SFR ICAT, University of Angers, Angers, France.
⁷ Department of Pharmacy, Haga Teaching Hospital, The Hague, the Netherlands.
⁸ Cystic Fibrosis Foundation, Inova Fairfax Hospital, Bethesda, Maryland, USA, Falls Church, VA, USA.
⁹ Cystic Fibrosis Reference Centre-Centro, Hospitalar Universitário Lisboa Norte, Portugal.
¹⁰ Cystic Fibrosis Reference Center (Constitutif), Service de médecine interne et de pathologie vasculaire, Hospices Civils de Lyon, Hôpital Lyon Sud, RESearch on HealthcAre PErformance (RESHAPE), INSERM U1290, Université Claude Bernard Lyon 1, 8 avenue Rockefeller, 69373 Lyon Cedex 08, France; ERN-Lung Cystic Fibrosis Network, Frankfurt, Germany.
¹¹ Department of Medical Microbiology, University Medical Center Utrecht, Utrecht, the Netherlands.
¹² Department of Respiratory Medicine, The Alfred Hospital, Melbourne, Australia.
¹³ The Children's Hospital for Wales, Cardiff, UK.
¹⁴ CF Centre at Goethe University Frankfurt, Frankfurt am Main, Germany.
¹⁵ CF Center at University Hospital Motol, Prague, Czech Republic.
¹⁶ Department of Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.
¹⁷ Clinical Specialist Paediatric Cystic Fibrosis Dietitian, Leeds Children's Hospital, UK.
¹⁸ Royal Brompton Hospital, Part of Guys and StThomas's Hospital, London, UK.
¹⁹ Institut Cochin, Inserm U1016, Université Paris-Cité and National Reference Center for Cystic Fibrosis, Hôpital Cochin AP-HP, ERN-Lung CF Network, Paris 75014, France.
²⁰ St.Vincent's University Hospital and University College Dublin School of Medicine, Dublin, Ireland.
²¹ Hospital Necker Enfants-Malades, AP-HP, CF Centre, Université Paris Descartes, Paris, France.
²² School of Clinical Medicine, Discipline of Paediatrics and Child Health, Faculty of Medicine & Health, Department of Gastroenterology, Sydney Children's Hospital, University of New South Wales, Sydney, NSW, Australia.
²³ Department of Respiratory Medicine, Children's Health Ireland, Dublin, Ireland.
²⁴ Central Clinical School, Faculty of Medicine, University of Sydney, Sydney, Australia.
²⁵ Child Health, Lifespan and Population Health & Nottingham Biomedical Research Centre, School of Medicine, University of Nottingham, Nottingham, UK.
²⁶ Division of Respiratory Medicine, Department of Pediatrics and Translational Medicine, Research Institute, Hospital for Sick Children, Toronto, Canada.
²⁷ Department of Pediatrics, Seattle Children's Research Institute, University of Washington School of Medicine, Seattle, WA, USA.
²⁸ Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, USA.
²⁹ Division Cystic Fibrosis, CF Center, Clinic Westbrandenburg, HMU-Health and Medical University, Potsdam, Germany.
³⁰ Meyer Children's Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Italy.
³¹ University of Queensland, South Brisbane, QLD, Australia.
³² Department of Medicine, Johns Hopkins University, Baltimore, MD, USA.
³³ Pediatric Gastroenterology Unit, CF Center, Hadassah Medical Center, Jerusalem, Israel.
³⁴ University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom.
³⁵ IRCCS Istituto Giannina Gaslini, Via Gerolamo Gaslini 5, Genova 16147, Italy.
³⁶ Department of Medical Microbiology, Second Faculty of Medicine, Motol University Hospital, Charles University, Prague, Czech Republic.
³⁷ Cystic Fibrosis Unit and Pediatric Pulmonology, Hospital Universitari Vall d'Hebron, Barcelona, Spain.
³⁸ Department of Pathophysiology and Transplantation, Respiratory Unit and Adult Cystic Fibrosis Center, Università degli Studi di Milano, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milano, Italy.
³⁹ Cystic Fibrosis Europe, Brussels, Belgium and the Belgian CF Association, Brussels, Belgium.
⁴⁰ The Graub CF Center, Pulmonary Institute, Schneider Children's Medical Center, Petah Tikva, Israel.
⁴¹ Cork Centre for Cystic Fibrosis (3CF), Cork University Hospital, University College Cork, Ireland.
⁴² School of Medicine, Dentistry and Biomedical Sciences, Belfast and NIHR Nottingham Biomedical Research Centre, Queens University Belfast, Nottingham, UK.
⁴³ CF Center Cologne, Children´s Hospital, Faculty of Medicine, University Hospital Cologne, Cologne, Germany.
⁴⁴ Westmead Clinical School, Department Respiratory & Sleep Medicine, Westmead Hospital, University of Sydney and CITRICA, Westmead, Australia.

PMID: 38233247
DOI: 10.1016/j.jcf.2024.01.005

Free article

Review

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

Pierre-Régis Burgel et al. J Cyst Fibros. 2024 Mar.

Free article

. 2024 Mar;23(2):187-202.

doi: 10.1016/j.jcf.2024.01.005. Epub 2024 Jan 16.

Authors

Affiliations

¹ Respiratory Medicine and Cystic Fibrosis National Reference Center, Cochin Hospital, Assistance Publique Hôpitaux de Paris (AP-HP), Institut Cochin, Inserm U1016, Université Paris-Cité, Paris, France.
² Department of Women's and Children's Health, Institute in the Park, Alder Hey Children's Hospital, University of Liverpool, Eaton Road, Liverpool L12 2AP, UK. Electronic address: kwsouth@liv.ac.uk.
³ All Wales Adult Cystic Fibrosis Centre, University Hospital Llandough, Cardiff and Vale University Health Board, Cardiff, UK.
⁴ Clinical Nutrition Unit, Department of Endocrine and Metabolic Medicine, IRCCS Istituto Auxologico Italiano, and ICANS-DIS, Department of Food Environmental and Nutritional Sciences, University of Milan, Milan, Italy.
⁵ Department of Nutrition and Dietetics, Alder Hey Children's NHS Trust, Liverpool, UK.
⁶ University of Brest, Fungal Respiratory Infections Research Unit, SFR ICAT, University of Angers, Angers, France.
⁷ Department of Pharmacy, Haga Teaching Hospital, The Hague, the Netherlands.
⁸ Cystic Fibrosis Foundation, Inova Fairfax Hospital, Bethesda, Maryland, USA, Falls Church, VA, USA.
⁹ Cystic Fibrosis Reference Centre-Centro, Hospitalar Universitário Lisboa Norte, Portugal.
¹⁰ Cystic Fibrosis Reference Center (Constitutif), Service de médecine interne et de pathologie vasculaire, Hospices Civils de Lyon, Hôpital Lyon Sud, RESearch on HealthcAre PErformance (RESHAPE), INSERM U1290, Université Claude Bernard Lyon 1, 8 avenue Rockefeller, 69373 Lyon Cedex 08, France; ERN-Lung Cystic Fibrosis Network, Frankfurt, Germany.
¹¹ Department of Medical Microbiology, University Medical Center Utrecht, Utrecht, the Netherlands.
¹² Department of Respiratory Medicine, The Alfred Hospital, Melbourne, Australia.
¹³ The Children's Hospital for Wales, Cardiff, UK.
¹⁴ CF Centre at Goethe University Frankfurt, Frankfurt am Main, Germany.
¹⁵ CF Center at University Hospital Motol, Prague, Czech Republic.
¹⁶ Department of Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA.
¹⁷ Clinical Specialist Paediatric Cystic Fibrosis Dietitian, Leeds Children's Hospital, UK.
¹⁸ Royal Brompton Hospital, Part of Guys and StThomas's Hospital, London, UK.
¹⁹ Institut Cochin, Inserm U1016, Université Paris-Cité and National Reference Center for Cystic Fibrosis, Hôpital Cochin AP-HP, ERN-Lung CF Network, Paris 75014, France.
²⁰ St.Vincent's University Hospital and University College Dublin School of Medicine, Dublin, Ireland.
²¹ Hospital Necker Enfants-Malades, AP-HP, CF Centre, Université Paris Descartes, Paris, France.
²² School of Clinical Medicine, Discipline of Paediatrics and Child Health, Faculty of Medicine & Health, Department of Gastroenterology, Sydney Children's Hospital, University of New South Wales, Sydney, NSW, Australia.
²³ Department of Respiratory Medicine, Children's Health Ireland, Dublin, Ireland.
²⁴ Central Clinical School, Faculty of Medicine, University of Sydney, Sydney, Australia.
²⁵ Child Health, Lifespan and Population Health & Nottingham Biomedical Research Centre, School of Medicine, University of Nottingham, Nottingham, UK.
²⁶ Division of Respiratory Medicine, Department of Pediatrics and Translational Medicine, Research Institute, Hospital for Sick Children, Toronto, Canada.
²⁷ Department of Pediatrics, Seattle Children's Research Institute, University of Washington School of Medicine, Seattle, WA, USA.
²⁸ Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, USA.
²⁹ Division Cystic Fibrosis, CF Center, Clinic Westbrandenburg, HMU-Health and Medical University, Potsdam, Germany.
³⁰ Meyer Children's Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Italy.
³¹ University of Queensland, South Brisbane, QLD, Australia.
³² Department of Medicine, Johns Hopkins University, Baltimore, MD, USA.
³³ Pediatric Gastroenterology Unit, CF Center, Hadassah Medical Center, Jerusalem, Israel.
³⁴ University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom.
³⁵ IRCCS Istituto Giannina Gaslini, Via Gerolamo Gaslini 5, Genova 16147, Italy.
³⁶ Department of Medical Microbiology, Second Faculty of Medicine, Motol University Hospital, Charles University, Prague, Czech Republic.
³⁷ Cystic Fibrosis Unit and Pediatric Pulmonology, Hospital Universitari Vall d'Hebron, Barcelona, Spain.
³⁸ Department of Pathophysiology and Transplantation, Respiratory Unit and Adult Cystic Fibrosis Center, Università degli Studi di Milano, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milano, Italy.
³⁹ Cystic Fibrosis Europe, Brussels, Belgium and the Belgian CF Association, Brussels, Belgium.
⁴⁰ The Graub CF Center, Pulmonary Institute, Schneider Children's Medical Center, Petah Tikva, Israel.
⁴¹ Cork Centre for Cystic Fibrosis (3CF), Cork University Hospital, University College Cork, Ireland.
⁴² School of Medicine, Dentistry and Biomedical Sciences, Belfast and NIHR Nottingham Biomedical Research Centre, Queens University Belfast, Nottingham, UK.
⁴³ CF Center Cologne, Children´s Hospital, Faculty of Medicine, University Hospital Cologne, Cologne, Germany.
⁴⁴ Westmead Clinical School, Department Respiratory & Sleep Medicine, Westmead Hospital, University of Sydney and CITRICA, Westmead, Australia.

PMID: 38233247
DOI: 10.1016/j.jcf.2024.01.005

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.

Keywords: Breathing; CFRD; Complications; Cystic fibrosis; End of life; Liver; Lung infection; Mental health; Pulmonary; Transplant.

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Conflict of interest statement

Declaration of competing interest The authors had no declarations of interest in relation to the current work. Declarations of interest for each author outside the current work are summarised in Supplementary Table 4.

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

Affiliations

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

Authors

Affiliations

Abstract

Conflict of interest statement

Publication types

MeSH terms

LinkOut - more resources

Full Text Sources

Medical