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.

All Wales Adult Cystic Fibrosis Centre University Hospital Llandough Cardiff and Vale University Health Board Cardiff UK

Assistance Publique Hôpitaux de Paris Université Paris Cité Paris France

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

CF Patient Head of Access Off label and Trials Computer Science Architecture Research and Innovation Cystic Fibrosis Foundation of Israel Israel

Copenhagen CF center Rigshospitalet Copenhagen Denmark

Cork Centre for Cystic Fibrosis Cork University Hospital Cork Ireland

Cystic Fibrosis Department Institute of Mother and Child Warsaw Poland; Cystic Fibrosis Centre Pediatric Hospital Dziekanów Leśny Poland

Cystic Fibrosis Europe Brussels Belgium and the Belgian CF Association Brussels 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

Department of Clinical Pharmacy and Pharmacology University Medical Center Groningen University of Groningen Groningen the Netherlands

Department of Medical Microbiology 2nd Faculty of Medicine Motol University Hospital Charles University Prague Czech Republic

Department of Medicine Nursing and Health Sciences Monash University Melbourne Australia 3181 and Department of Respiratory Medicine Alfred Health Melbourne 3004 Australia

Department of Nutrition and Dietetics Royal Alexandra Children's Hospital Brighton UK

Department of Paediatric Respiratory and Sleep Medicine Royal Hospital for Children Glasgow UK

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 Psychiatry Massachusetts General Hospital Harvard Medical School Boston USA

Department of Respiratory Medicine The Children's Hospital at Westmead Sydney NSW Australia

Department of Thoracic Medicine and Faculty of Medicine The University of Queensland The Prince Charles Hospital Brisbane Australia

Department of Women's and Children's Health University of Liverpool Liverpool UK

Division of Respiratory Medicine Department of Medicine St Paul's Hospital and the University of British Columbia Vancouver British Columbia Canada

Divison of Pediatric Respiratroy Medicine BC Childrens Hospital Vancouver Canada

Epidemiology Biostatistics and Prevention Institute University of Zurich Zurich Switzerland

Epigenetics and Cell Fate Centre UMR7216 CNRS Université Paris Cité Paris France and Cystic Fibrosis Europe Brussels Belgium

Haga Teaching Hospital Pharmacy The Hague Netherlands

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

Hospital Universitari Vall d'Hebron Barcelona Spain

Institute of Mother and Child in Warsaw Cystic Fibrosis Department and Dziekanow Lesny Hospital Cystic Fibrosis Center Dziekanow Lesny Poland

IRCCS Istituto Giannina Gaslini Via Gerolamo Gaslini 5 16147 Genova Italy

Laboratoire Educations et Promotion de la santé LEPS UR 3412 University of Sorbonne Paris Nord F 93430 Villetaneuse France

Medical Center University of Freiburg Pharmacy Hugstetter St 55 Freiburg D 79106 Germany

National Heart and Lung Institute Imperial College London; Imperial Biomedical Research Centre; Royal Brompton Hospital Guy's and St Thomas' Trust London UK

National Resource Centre for Cystic Fibrosis Oslo University Hospital Oslo Norway

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

Population Policy and Practice Research and Teaching Department UCL Great Ormond Street Institute of Child Health London UK

Respiratory Medicine and Cystic Fibrosis National Reference Center Cochin Hospital Assistance Publique Hôpitaux de Paris and Université Paris Cité Institut Cochin Inserm U1016 Paris France

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

Seattle Children's Research Institute Seattle WA and Department of Pediatrics University of Washington Seattle Wa USA

Sydney Children's Hospital Australia

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

University Hospital Southampton NHS Foundation Trust Southampton United Kingdom

University of Leuven Leuven Belgium

West Midlands Adult CF Centre Heartlands Hospital Birmingham UK

West of Scotland Adult CF Centre Queen Elizabeth University Hospital Glasgow UK

Westmead Clinical School University of Sydney and CITRICA Dept Respiratory and Sleep Medicine Westmead Hospital Westmead Australia

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