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

Department of Women's and Children's Health, University of Liverpool, Liverpool, UK. Electronic address: kwsouth@liv.ac.uk. All Wales Adult Cystic Fibrosis Centre, University Hospital Llandough, Cardiff and Vale University Health Board, Cardiff, UK. Department of Thoracic Medicine and Faculty of Medicine, The University of Queensland, The Prince Charles Hospital, Brisbane, Australia. University Hospital Southampton NHS Foundation Trust, Southampton, United Kingdom. Institute of Mother and Child in Warsaw, Cystic Fibrosis Department and Dziekanow Lesny Hospital, Cystic Fibrosis Center, Dziekanow Lesny, Poland. West Midlands Adult CF Centre, Heartlands Hospital Birmingham, UK. Respiratory Medicine and Cystic Fibrosis National Reference Center, Cochin Hospital, Assistance Publique Hôpitaux de Paris (AP-HP) and Université Paris-Cité, Institut Cochin, Inserm U1016, Paris, France. Department of Medicine, Nursing and Health Sciences, Monash University, Melbourne, Australia 3181, and Department of Respiratory Medicine, Alfred Health, Melbourne 3004, Australia. IRCCS Istituto Giannina Gaslini, Via Gerolamo Gaslini 5, 16147 Genova, Italy. Epigenetics and Cell Fate Centre, UMR7216 CNRS, Université Paris Cité, Paris, France, and Cystic Fibrosis Europe, Brussels, Belgium. Divison of Pediatric Respiratroy Medicine, BC Childrens Hospital, Vancouver, Canada. Population, Policy and Practice Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, London, UK. National Heart & Lung Institute, Imperial College London; Imperial Biomedical Research Centre; Royal Brompton Hospital, Guy's & St Thomas' Trust, London, UK. University of Leuven, Leuven, Belgium. Cystic Fibrosis Reference Center, Department of Pediatrics, Ghent University Hospital, Ghent, Belgium; Department of Pediatrics, Center for children and adolescents with diabetes, Ghent University Hospital, Ghent, Belgium; Department of Internal Medicine and Paediatrics, Faculty of Medicine and Health Sciences, Ghent University, Ghent, Belgium. Sydney Children's Hospital, Australia. Department of Medical Microbiology, Second Faculty of Medicine, Motol University Hospital, Charles University, Prague, Czech Republic. Assistance Publique - Hôpitaux de Paris, Université Paris Cité, Paris, France. Hospital Universitari Vall d'Hebron, Barcelona, Spain. Department of Psychiatry, Massachusetts General Hospital, Harvard Medical School, Boston, USA. National Resource Centre for Cystic Fibrosis, Oslo University Hospital, Oslo, Norway. Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Respiratory Unit and Adult Cystic Fibrosis Center, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milano, Italy. Department of Clinical Pharmacy & Pharmacology, University Medical Center Groningen (UMCG), University of Groningen, Groningen, the Netherlands. Medical Center - University of Freiburg, Pharmacy, Hugstetter St. 55, Freiburg, D-79106, Germany. Cystic Fibrosis Europe, Brussels, Belgium and the Belgian CF Association, Brussels, Belgium. The Graub CF Center, Pulmonary Institute, Schneider Children's Medical Center, Petah Tikva, Israel. Department of Paediatric Respiratory & Sleep Medicine, Royal Hospital for Children, Glasgow, UK. Seattle Children's Research Institute, Seattle, WA and Department of Pediatrics, University of Washington, Seattle, Wa, USA. Department of Respiratory Medicine, The Children's Hospital at Westmead, Sydney, NSW, Australia. Westmead Clinical School, University of Sydney and CITRICA, Dept Respiratory & Sleep Medicine, Westmead Hospital, Westmead, Australia. Cystic Fibrosis Department, Institute of Mother and Child, Warsaw, Poland; Cystic Fibrosis Centre, Pediatric Hospital, Dziekanów Leśny, Poland. West of Scotland Adult CF Centre, Queen Elizabeth University Hospital, Glasgow, UK. Hospital Necker Enfants-Malades, AP-HP, CF centre, Université Paris Descartes, Paris, France. Cork Centre for Cystic Fibrosis (3CF), Cork University Hospital, Cork, Ireland. Haga Teaching Hospital, Pharmacy, The Hague, Netherlands. Laboratoire Educations et Promotion de la santé, LEPS, UR 3412, University of Sorbonne Paris Nord, F-93430, Villetaneuse, France. Copenhagen CF center, Rigshospitalet, Copenhagen, Denmark. Division of Respiratory Medicine, Department of Medicine. St. Paul's Hospital and the University of British Columbia. Vancouver, British Columbia, Canada. Epidemiology, Biostatistics and Prevention Institute, University of Zurich, Zurich, Switzerland. Physical Activity, Health and Rehabilitation Thematic Research Group, School of Sport, Health and Exercise Science, Faculty of Science and Health, University of Portsmouth, UK and Wessex Cystic Fibrosis Unit, University Hospitals Southampton NHS Foundation Trust, UK. CF Patient, Head of Access, Off label and Trials, Computer Science Architecture, Research and Innovation, Cystic Fibrosis Foundation of Israel, Israel. School of Medicine, Dentistry and Biomedical Sciences, Queens University Belfast, Belfast and NIHR Nottingham Biomedical Research Centre, Nottingham, UK. Department of Nutrition and Dietetics, Royal Alexandra Children's Hospital, Brighton, UK. CF Center Cologne, Children´s Hospital, Faculty of Medicine, University Hospital Cologne, Cologne, Germany.

Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society. 2024;(1):12-28
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Abstract

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.

Methodological quality

Publication Type : Review

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