Hodson and Geddes' Cystic Fibrosis

Andrew Bush MD FHEA FRCP FRCPCH FERS FAPSR ATSFProfessor of Paediatrics and Paediatric Respirology, National Heart and Lung Institute, and Imperial Centre for Paediatrics and Child Health, Imperial College Consultant Paediatric Chest Physician, Royal Brompton & Harefield NHS Foundation Trust NIHR Senior Investigator Emeritus Margarida D. Amaral Professor BioISI – Biosystems & Integrative Sciences Institute Faculty of Sciences, University of Lisboa, Portugal Jane C. Davies Professor in Paediatric Respirology & Experimental Medicine. Imperial College London Honorary Consultant in Paediatric Respiratory Medicine Royal Brompton Hospital, part of Guy’s & St Thomas’ NHS Foundation Trust London United Kingdom Nicholas Simmonds MD(Res) FRCP Associate Director | Consultant Respiratory Physician Adult Cystic Fibrosis Centre, Royal Brompton Hospital Professor of Practice (Respiratory Medicine) National Heart and Lung Institute, Imperial College London Adult Cystic Fibrosis Centre, Royal Brompton Hospital Sydney Street, London United Kingdom Jennifer L. Taylor-Cousar, MD, MSCS, ATSF President, Medical Staff Medical Director, Clinical Research Services Co-Director and CF TDC Director, Adult CF Program Professor, Departments of Medicine and Pediatrics, Divisions of Pulmonary, Critical Care and Sleep Medicine and Pediatric Pulmonary Medicine Divisions of Pulmonary Sciences and Critical Care Medicine and Pediatric Pulmonology University of Colorado Anschutz Medical Campus Sarath Ranganathan MBChB MRCP FRCPH FRACP PhD FThorSoc ATSF Stevenson Chair and Head, Department of Paediatrics | Academic Director of Clinical Education Strategy and Risk University of Melbourne Consultant, Respiratory and Sleep Medicine | Royal Children’s Hospital Head, Respiratory Diseases Research Group | Infection and Immunity | Murdoch Children’s Research Institute

Ch 1 Journey from Macromolecular Pathology to Molecular Therapies

Ch 2 Global Epidemiology of CF: High-Income and Low-/Middle-Income Countries (Global Harmonization Registry Countries)

Ch 3 Voices of Patients and Families

Ch 4 Patient Organisations

Ch 5 Molecular Biology of CFTR: From the Gene to the Protein

Ch 6 Biology of the CF Airway Epithelium

Ch 7 The Physiology of Epithelial Ion and Fluid Transport: Beyond CFTR Modulators

Ch 8 Inflammation in Cystic Fibrosis

Ch 9 Model Organisms of Cystic Fibrosis

Ch 10 Systems Biology and the New Omics

Ch 11 Genotype: Phenotype Correlations

Ch 12 Gene Environment Interactions

Ch 13 Demographic, Socioeconomic, and Environmental Contributions to Health in Cystic Fibrosis

Ch 14 Drug Discovery Platforms for CFTR Modulators

Ch 15 CFTR Modulator Drug Discovery and Translation into the Clinic

Ch 16 Newborn and Carrier Screening for CF

Ch 17 Diagnostic Tests: Sweat Testing, Epithelial Potential Differences and Genetic Testing

Ch 18 Immediate Management of the Newly Screened Positive Baby

Ch 19 Diagnosis of the Symptomatic Patient

Ch 20 Blurred Boundaries: CRMS/CFSPID and CFTR Related Disorders

Ch 21 Respiratory Disease across the Lifecourse

Ch 22 Respiratory Effects of the New CFTR Modulators

Ch 23 Epidemiology and Microbiology of Cystic Fibrosis Pulmonary Infections

Ch 24 New Methods for Detecting and Identifying Bacteria

Ch 25 Non-Tuberculous Mycobacterial Infections in Cystic Fibrosis

Ch 26 Fungal Diseases in CF

Ch 27 Molecular Microbiology of the CF Gut and Lung

Ch 28 Pulmonary Exacerbations in Cystic Fibrosis: Epidemiology, Treatment, Outcomes, and Future Research

Ch 29 Infection Prevention and Control in Cystic Fibrosis

Ch 30 Technology in Cystic Fibrosis Therapies

Ch 31 Upper Airway Disease in Cystic Fibrosis

Ch 32 Gastrointestinal Disease in CF

Ch 33 Cystic Fibrosis Liver Disease

Ch 34 Cystic Fibrosis-Related Diabetes

Ch 35 Growth in Cystic Fibrosis: Is Chloride Transport the Key?

Ch 36 Bone Disease in Cystic Fibrosis

Ch 37 Cancer and Cystic Fibrosis

Ch 38 Other Cystic Fibrosis-Related Diseases and Complications

Ch 39 Extrapulmonary Benefits of the New CFTR Modulator Drugs

Ch 40 Sexual Health, Fertility, and Pregnancy in People with Cystic Fibrosis

Ch 41 Mental Health Issues in Cystic Fibrosis

Ch 42 Adherence and Self-Management in Cystic Fibrosis Care

Ch 43 Transplantation

Ch 44: Working with Cystic Fibrosis

Ch 45 Growing Old with Cystic Fibrosis

Ch 46 Imaging of Cystic Fibrosis Lung Disease

Ch 47 Lung Function Testing Including Multiple Breath Washout

Ch 48 Infant Pulmonary Function Tests

Ch 49 Exercise Testing in Cystic Fibrosis

Ch 50 Bronchoscopy and Bronchoalveolar Lavage in Cystic Fibrosis

Ch 51 Patient-Derived Cell-Based Models for Theratyping and Individual CFTR Modulator Assessment

Ch 52 Minimally Invasive Investigations for Biomarkers of Airways Disease

Ch 53 Currrent and Future Interventional Trial Designs to Support the CF Therapeutic Pipeline

Ch 54 Gene and RNA-Bbased Therapies

Ch 55 Cystic Fibrosis in Limited Resource Settings

Ch 56 Traveling with Cystic Fibrosis

Ch 57 Organisation of Cystic Fibrosis Centre Care

Ch 58 The Role of the CF Nurse Specialist

Ch 60 The Role of the Dietitian

Ch 61 The Role of the Pharmacist as Part of the Multidisciplinary Team Caring for CF Patients

Ch 62 The Role of a Psychologist on the Cystic Fibrosis Care Team

Ch 63 Adolescent Health and Transition in Cystic Fibrosis

Ch 64 Palliative and Supportive Care

Ch 65 Using Registries and Databases to Drive up Quality

Ch 66 Digital Transformations within CF Healthcare

Ch 67 Personalized Medicine for Cystic Fibrosis in the 21st Century

Ch 68 CF Research Priorities for the Future