Journal of Cystic Fibrosis

, volume 23 , issue 2 , pages 187-202

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

Anne Stephenson ¹

K. Southern ²

Charlotte Addy ³

ALBERTO BATTEZZATI ⁴

Claire Berry ⁵

Jean-Philippe Bouchara ⁶

J. P. Bouchara ⁶

Edwin J Brokaar ⁷

A. Whitney Brown ⁸

Pilar Azevedo ⁹

Isabelle Durieu ^{10, 11}

Miquel B. Ekkelenkamp ¹²

Felicity Finlayson ¹³

Julian T Forton ¹⁴

Johanna Gardecki ¹⁵

Pavla Hodkova ¹⁶

Gina Hong ¹⁷

Jacqueline Lowdon ¹⁸

Su Madge ¹⁹

Clémence Martin ²⁰

Edward F. McKone ²¹

Anne Munck ²²

Chee Y Ooi ²³

Lucy Perrem ²⁴

Amanda J Piper ²⁵

Andrew Prayle ²⁶

Felix Ratjen ²⁷

Margaret Rosenfeld ²⁸

Don B. Sanders ²⁹

Carsten Schwarz ³⁰

Giovanni Taccetti ³¹

Claire Wainwright ³²

Natalie E. West ³³

M. Wilschanski ³⁴

Amanda Bevan ³⁵

Carlo Castellani ³⁶

Pavel Dřevínek ³⁷

Silvia Gartner ³⁸

Andrea Gramegna ³⁹

Elise Lammertyn ⁴⁰

Edwina C. Landau ⁴¹

Barry J. Plant ⁴²

Alan Smyth ⁴³

Silke van Koningsbruggen-Rietschel ⁴⁴

Peter Hugh Middleton ⁴⁵

Hide authors affiliations Show authors affiliations: 45 affiliations

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

Paris Cité University

French Institute of Health and Medical Research

Institut Cochin

Hôpital Cochin

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 |

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

Istituti di Ricovero e Cura a Carattere Scientifico

University of Milan

Italian Institute for Auxology

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

University of Western Brittany

University of Angers

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

French Institute of Health and Medical Research

Université Claude Bernard Lyon 1

Hôpital Lyon Sud

¹¹

ERN-Lung Cystic Fibrosis Network, Frankfurt, Germany |

¹²

Department of Medical Microbiology, University Medical Center Utrecht, Utrecht, the Netherlands |

¹³

Department of Respiratory Medicine, The Alfred Hospital, Melbourne, Australia |

¹⁴

The Children's Hospital for Wales, Cardiff, UK |

¹⁵

CF Centre at Goethe University Frankfurt, Frankfurt am Main, Germany |

¹⁶

CF Center at University Hospital Motol, Prague, Czech Republic |

¹⁷

Department of Medicine, University of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA |

¹⁸

Clinical Specialist Paediatric Cystic Fibrosis Dietitian, Leeds Children's Hospital, UK |

¹⁹

Royal Brompton Hospital, Part of Guys and StThomas's Hospital, London, UK |

²⁰

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

Paris Cité University

French Institute of Health and Medical Research

Institut Cochin

Hôpital Cochin

²¹

St.Vincent's University Hospital and University College Dublin School of Medicine, Dublin, Ireland

University College Dublin

St. Vincent's University Hospital

²²

Hospital Necker Enfants-Malades, AP-HP, CF centre, Université Paris Descartes, Paris, France |

²³

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 |

²⁴

Department of Respiratory Medicine, Children's Health Ireland, Dublin, Ireland |

²⁵

Central Clinical School, Faculty of Medicine, University of Sydney, Sydney, Australia |

²⁶

Child Health, Lifespan and Population Health & Nottingham Biomedical Research Centre, School of Medicine, University of Nottingham, Nottingham, UK

University of Nottingham

Nottingham Biomedical Research Centre

²⁷

Division of Respiratory Medicine, Department of Pediatrics and Translational Medicine, Research Institute, Hospital for Sick Children, Toronto, Canada |

²⁸

Department of Pediatrics, Seattle Children's Research Institute, University of Washington School of Medicine, Seattle, WA, USA

University of Washington

Seattle Children's Hospital

²⁹

Department of Pediatrics, Indiana University School of Medicine, Indianapolis, IN, USA |

³⁰

Division Cystic Fibrosis, CF Center, Clinic Westbrandenburg, HMU-Health and Medical University, Potsdam, Germany |

³¹

Meyer Children's Hospital IRCCS, Cystic Fibrosis Regional Reference Centre, Italy |

³²

University of Queensland, South Brisbane, QLD, Australia |

³³

Department of Medicine, Johns Hopkins University, Baltimore, MD, USA |

³⁴

Pediatric Gastroenterology Unit, CF Center, Hadassah Medical Center, Jerusalem, Israel |

³⁵

University Hospital Southampton NHS foundation Trust, Southampton, United Kingdom |

³⁶

IRCCS Istituto Giannina Gaslini, Via Gerolamo Gaslini 5, Genova 16147, Italy

Istituti di Ricovero e Cura a Carattere Scientifico

Istituto Giannina Gaslini

³⁷

Department of Medical Microbiology, Second Faculty of Medicine, Motol University Hospital, Charles University, Prague, Czech Republic

Charles University

Motol University Hospital

³⁸

Cystic Fibrosis Unit and Pediatric Pulmonology, Hospital Universitari Vall d'Hebron, Barcelona, Spain |

³⁹

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

Istituti di Ricovero e Cura a Carattere Scientifico

University of Milan

⁴⁰

Cystic Fibrosis Europe, Brussels, Belgium and the Belgian CF Association, Brussels, Belgium |

⁴¹

The Graub CF Center, Pulmonary Institute, Schneider Children's Medical Center, Petah Tikva, Israel |

⁴²

Cork Centre for Cystic Fibrosis (3CF), Cork University Hospital, University College Cork, Ireland

University College Cork (National University of Ireland, Cork)

Cork University Hospital

⁴³

School of Medicine, Dentistry and Biomedical Sciences, Belfast and NIHR Nottingham Biomedical Research Centre, Queens University Belfast, Nottingham, UK

Queen's University Belfast

Nottingham Biomedical Research Centre

⁴⁴

CF Center Cologne, Children´s Hospital, Faculty of Medicine, University Hospital Cologne, Cologne, Germany |

⁴⁵

Westmead Clinical School, Department Respiratory & Sleep Medicine, Westmead Hospital, University of Sydney and CITRICA, Westmead, Australia

University of Sydney

Westmead Hospital

Publication type: Journal Article

Publication date: 2024-03-01

Elsevier

Journal of Cystic Fibrosis

scimago Q1

wos Q1

SJR: 1.772

CiteScore: 10.0

Impact factor: 6.0

ISSN: 15691993, 18735010

DOI: 10.1016/j.jcf.2024.01.005

Copy DOI

PubMed ID: 38233247

Pulmonary and Respiratory Medicine

Pediatrics, Perinatology and Child Health

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.

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Cite this

GOST |

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GOST Copy

Stephenson A. et al. Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues // Journal of Cystic Fibrosis. 2024. Vol. 23. No. 2. pp. 187-202.

GOST all authors (up to 50) Copy

Stephenson A. et al. Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues // Journal of Cystic Fibrosis. 2024. Vol. 23. No. 2. pp. 187-202.

RIS |

Cite this

RIS Copy

TY - JOUR

DO - 10.1016/j.jcf.2024.01.005

UR - https://linkinghub.elsevier.com/retrieve/pii/S1569199324000055

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

T2 - Journal of Cystic Fibrosis

AU - Stephenson, Anne

AU - Southern, K.

AU - Addy, Charlotte

AU - BATTEZZATI, ALBERTO

AU - Berry, Claire

AU - Bouchara, Jean-Philippe

AU - Bouchara, J. P.

AU - Brokaar, Edwin J

AU - Brown, A. Whitney

AU - Azevedo, Pilar

AU - Durieu, Isabelle

AU - Ekkelenkamp, Miquel B.

AU - Finlayson, Felicity

AU - Forton, Julian T

AU - Gardecki, Johanna

AU - Hodkova, Pavla

AU - Hong, Gina

AU - Lowdon, Jacqueline

AU - Madge, Su

AU - Martin, Clémence

AU - McKone, Edward F.

AU - Munck, Anne

AU - Ooi, Chee Y

AU - Perrem, Lucy

AU - Piper, Amanda J

AU - Prayle, Andrew

AU - Ratjen, Felix

AU - Rosenfeld, Margaret

AU - Sanders, Don B.

AU - Schwarz, Carsten

AU - Taccetti, Giovanni

AU - Wainwright, Claire

AU - West, Natalie E.

AU - Wilschanski, M.

AU - Bevan, Amanda

AU - Castellani, Carlo

AU - Dřevínek, Pavel

AU - Gartner, Silvia

AU - Gramegna, Andrea

AU - Lammertyn, Elise

AU - Landau, Edwina C.

AU - Plant, Barry J.

AU - Smyth, Alan

AU - van Koningsbruggen-Rietschel, Silke

AU - Middleton, Peter Hugh

PY - 2024

DA - 2024/03/01

PB - Elsevier

SP - 187-202

IS - 2

VL - 23

PMID - 38233247

SN - 1569-1993

SN - 1873-5010

ER -

BibTex |

Cite this

BibTex (up to 50 authors) Copy

@article{2024_Stephenson,

author = {Anne Stephenson and K. Southern and Charlotte Addy and ALBERTO BATTEZZATI and Claire Berry and Jean-Philippe Bouchara and J. P. Bouchara and Edwin J Brokaar and A. Whitney Brown and Pilar Azevedo and Isabelle Durieu and Miquel B. Ekkelenkamp and Felicity Finlayson and Julian T Forton and Johanna Gardecki and Pavla Hodkova and Gina Hong and Jacqueline Lowdon and Su Madge and Clémence Martin and Edward F. McKone and Anne Munck and Chee Y Ooi and Lucy Perrem and Amanda J Piper and Andrew Prayle and Felix Ratjen and Margaret Rosenfeld and Don B. Sanders and Carsten Schwarz and Giovanni Taccetti and Claire Wainwright and Natalie E. West and M. Wilschanski and Amanda Bevan and Carlo Castellani and Pavel Dřevínek and Silvia Gartner and Andrea Gramegna and Elise Lammertyn and Edwina C. Landau and Barry J. Plant and Alan Smyth and Silke van Koningsbruggen-Rietschel and Peter Hugh Middleton and others},

title = {Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues},

journal = {Journal of Cystic Fibrosis},

year = {2024},

volume = {23},

publisher = {Elsevier},

month = {mar},

url = {https://linkinghub.elsevier.com/retrieve/pii/S1569199324000055},

number = {2},

pages = {187--202},

doi = {10.1016/j.jcf.2024.01.005}

}

MLA

Cite this

MLA Copy

Stephenson, Anne, et al. “Standards for the care of people with cystic fibrosis (CF); recognising and addressing CF health issues.” Journal of Cystic Fibrosis, vol. 23, no. 2, Mar. 2024, pp. 187-202. https://linkinghub.elsevier.com/retrieve/pii/S1569199324000055.

Publisher

Elsevier

Journal

Journal of Cystic Fibrosis

scimago Q1

wos Q1

SJR

1.772

CiteScore

10.0

Impact factor

6.0

ISSN

15691993 (Print)

18735010 (Electronic)

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