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.

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

Central Clinical School Faculty of Medicine University of Sydney Sydney Australia

CF Center at University Hospital Motol Prague Czech Republic

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

CF Centre at Goethe University Frankfurt Frankfurt am Main Germany

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

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

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

Cork Centre for Cystic Fibrosis Cork University Hospital University College Cork Ireland

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

Cystic Fibrosis Foundation Inova Fairfax Hospital Bethesda Maryland USA Falls Church VA USA

Cystic Fibrosis Reference Center INSERM U1290 Université Claude Bernard Lyon 1 8 avenue Rockefeller 69373 Lyon Cedex 08 France; ERN Lung Cystic Fibrosis Network Frankfurt Germany

Cystic Fibrosis Reference Centre Centro Hospitalar Universitário Lisboa Norte Portugal

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

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

Department of Medical Microbiology University Medical Center Utrecht Utrecht the Netherlands

Department of Medicine Johns Hopkins University Baltimore MD USA

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

Department of Nutrition and Dietetics Alder Hey Children's NHS Trust Liverpool UK

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

Department of Pediatrics Indiana University School of Medicine Indianapolis IN USA

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

Department of Pharmacy Haga Teaching Hospital The Hague the Netherlands

Department of Respiratory Medicine Children's Health Ireland Dublin Ireland

Department of Respiratory Medicine The Alfred Hospital Melbourne Australia

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

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

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

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

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

IRCCS Istituto Giannina Gaslini Via Gerolamo Gaslini 5 Genova 16147 Italy

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

Pediatric Gastroenterology Unit CF Center Hadassah Medical Center Jerusalem Israel

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

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

School of Clinical Medicine Discipline of Paediatrics and Child Health Faculty of Medicine and Health Department of Gastroenterology Sydney Children's Hospital University of New South Wales Sydney NSW Australia

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

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

The Children's Hospital for Wales Cardiff UK

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 Brest Fungal Respiratory Infections Research Unit SFR ICAT University of Angers Angers France

University of Queensland South Brisbane QLD Australia

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

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