Developments in managing CF continue to drive dramatic improvements in survival. As newborn screening rolls-out across Europe, CF centres are increasingly caring for cohorts of patients who have minimal lung disease on diagnosis. With the introduction of mutation-specific therapies and the prospect of truly personalised medicine, patients have the potential to enjoy good quality of life in adulthood with ever-increasing life expectancy. The landmark Standards of Care published in 2005 set out what high quality CF care is and how it can be delivered throughout Europe. This underwent a fundamental re-write in 2014, resulting in three documents; center framework, quality management and best practice guidelines. This document is a revision of the latter, updating standards for best practice in key aspects of CF care, in the context of a fast-moving and dynamic field. In continuing to give a broad overview of the standards expected for newborn screening, diagnosis, preventative treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support, this consensus on best practice is expected to prove useful to clinical teams both in countries where CF care is developing and those with established CF centres. The document is an ECFS product and endorsed by the CF Network in ERN LUNG and CF Europe.

Adult Cystic Fibrosis Centre The Prince Charles Hospital Brisbane Australia

Cork Adult CF Centre Cork University Hospital University College Cork Republic of Ireland

Cystic Fibrosis Centre Azienda Ospedaliera Universitaria Integrata Verona Italy; Cystic Fibrosis Centre Gaslini Institute Genoa Italy

Cystic Fibrosis Centre Department of Paediatric Medicine Anna Meyer Children's University Hospital Florence Italy

Cystic Fibrosis Centre Royal Brompton Hospital London UK

Department of Clinical Psychology University Hospital Prague Czech Republic

Department of Genetic Epidemiology Federal State Budgetary Institution Research Centre for Medical Genetics Moscow Russia

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

Department of Pediatric Pulmunology CRCM Hôpital Necker Enfants Malades Paris France

Dept of Pulmonology University Medical Center Utrecht Utrecht The Netherlands

Division of Child Health Obstetrics and Gynaecology University of Nottingham Nottingham UK

Division of Pulmonary and Critical Care Medical University of South Carolina USA

Division of Respiratory Medicine Department of Paediatrics The Hospital for Sick Children University of Toronto Canada

Divison of Pediatric Gastroenterology Hepatology and Nutrition University of Minnesota Masonic Children's Hospital Minneapolis MN USA

Frankfurter Referenzzentrum für Seltene Erkrankungen Universitätsklinikum Frankfurt am Main Wolfgang von Goethe Universität Frankfurt am Main Germany

Hopital Robert Debré Assistante publique Hôpitaux de Paris Université Paris 7 Pediatric CF Centre Paris France

Institute of Translational Medicine University of Liverpool Liverpool UK

Manchester Adult Cystic Fibrosis Centre University Hospital of South Manchester NHS Foundation Trust Wythenshawe Hospital Manchester UK

Ostschweizer Kinderspital Sankt Gallen Claudiusstrasse 6 9006 St Gallen Switzerland

Regional Paediatric CF Unit Leeds General Infirmary Leeds UK; Department of Clinical and Health Psychology St James' University Hospital Leeds UK

Regional Paediatric CF Unit The Leeds Children's Hospital Leeds Teaching Hospitals Belmont Grove Leeds UK

Service de Pneumologie et Allergologie Pédiatriques Centre de Ressources et de Compétence de la Mucoviscidose Institut Necker Enfants Malades INSERM U1151 Hôpital Necker Enfants Malades P France

References provided by Crossref.org

Considerations for the use of inhaled antibiotics for Pseudomonas aeruginosa in people with cystic fibrosis receiving CFTR modulator therapy

Age-Related Progression of Microvascular Dysfunction in Cystic Fibrosis: New Detection Ways and Clinical Outcomes

Demographic characteristics, clinical and laboratory features, and the distribution of pathogenic variants in the CFTR gene in the Cypriot cystic fibrosis (CF) population demonstrate the utility of a national CF patient registry

Factors for severe outcomes following SARS-CoV-2 infection in people with cystic fibrosis in Europe

The future of cystic fibrosis care: a global perspective

Identification of 99% of CFTR gene mutations in Bulgarian-, Bulgarian Turk-, and Roma cystic fibrosis patients