746 Pages 150 Color & 49 B/W Illustrations
    by CRC Press

    746 Pages 150 Color & 49 B/W Illustrations
    by CRC Press

    Cystic Fibrosis has seen dramatic advances in treatment since the last edition, including targeted cystic fibrosis transmembrane conductance regulator (CFTR) protein modulators for most CFTR gene abnormalities. This new fifth edition is an update and expansion of the rapid clinical and scientific advances in improving prognosis, and the impact of COVID-19, all of which has transformed conventional models of care. It covers basic science, such as how detailed understanding of the biology of the CFTR gene and protein has led to novel and beneficial therapies, as well as all aspects of clinical management in high-, middle- and low-income settings and the voices of individuals with CF from across the world. It will be a useful reference for clinicians, including all levels of trainees, across the whole multidisciplinary team, scientists and students.

    Key Features

    • Follows an appealing organization of chapters, by developing fundamental knowledge of the reader before moving on to more complex or developing topics.
    • Presents a comprehensive, authoritative and up-to-date text, integrating fundamental science and clinical aspects of cystic fibrosis providing an attractive read for clinicians, trainee doctors and scientists.
    • Draws on global expertise and reflects best evidence-based practice from experts conducting cutting-edge clinical and basic science research from around the world.

    Ch 1 Journey from Macromolecular Pathology to Molecular Therapies
    Bonnie W. Ramsey and Christiane (Kris) De Boeck

    Ch 2 Global epidemiology of CF: High income and low/middle income countries (global harmonisation registry countries)
    Ahmad M. Hider and Samya Z. Nasr

    Ch 3 Voices of Patients and Families
    Lorna Allen

    Ch 4 Patient Organisations
    Nettie Burke

    Ch 5 Molecular Biology of CFTR: From the Gene to the Protein
    Deborah M. Cholon, Andrei A. Aleksandrov and Martina Gentzsch

    Ch 6 Biology of the CF airway epithelium
    Tahir Idris, Marc Chanson and Mehdi Badaoui

    Ch 7 The physiology of epithelial ion and fluid transport: beyond CFTR modulators
    Livia Delpiano and Michael A. Gray

    Ch 8 Inflammation in Cystic Fibrosis
    Deepika Polineni and James F. Chmiel

    Ch 9  Model Organisms of Cystic Fibrosis
    Daniel P. Cook and David A. Stoltz

    Ch 10 Systems Biology and the New Omics
    Margarida D. Amaral, Luka A. Clarke, Carlos M. Farinha, Hugo M. Botelho

    Ch 11 Genotype: phenotype correlations
    Karen S. Raraigh and Garry R. Cutting

    Ch 12 Gene Environment Interactions
    Shivanthan Shanthikumar

    Ch 13 Demographic, socioeconomic, and environmental contributions to health in cystic fibrosis
    John B. Palla, Meghan E. McGarry and Susanna A. McColley

    Ch 14 Drug Discovery Platforms for CFTR modulators
    Nicoletta Pedemonte

    Ch 15 CFTR modulator drug discovery and translation into the clinic
    Marlou Bierlaagh, Jeffrey Beekman and Kors van der Ent

    Ch 16 Newborn and Carrier Screening for CF
    John Massie and Juerg Barben

    Ch 17 Diagnostic tests: Sweat testing, epithelial potential differences and genetic testing
    Elke De Wachter and Caroline Raynal

    Ch 18 Immediate Management of The Newly Screened Positive Baby
    Jessica L. Saunders and Clement L. Ren

    Ch 19 Diagnosis of the symptomatic patient
    Dorota Sands and Nicholas J. Simmonds

    Ch 20 Blurred boundaries: CRMS/ CFSPID and CFTR related disorders
    Nicholas J. Simmonds and Jane C. Davies

    Ch 21 Respiratory Disease across the Lifecourse
    Noah Lechtzin and Peter J. Mogayzel, Jr.

    Ch 22 Respiratory effects of the new CFTR modulators
    Jennifer L. Taylor-Cousar, Alex H. Gifford, Dominic Hughes, John A King and Jane C. Davies

    Ch 23 Epidemiology and microbiology of cystic fibrosis pulmonary infections
    Christina Thornton, Ranjani Somayaji and Valerie Waters

    Ch 24 New methods for detecting and identifying bacteria
    Eshwar Mahenthiralingam

    Ch 25 Non-tuberculous Mycobacterial Infections in Cystic Fibrosis 
    Jerry A. Nick and Claire E Wainwright

    Ch 26 Fungal diseases in CF
    Carsten Schwarz

    Ch 27 Molecular microbiology of the CF gut and lung
    Sarath Ranganathan

    Ch 28 Pulmonary Exacerbations in Cystic Fibrosis: Epidemiology, Treatment, Outcomes, and Future Research
    Don B. Sanders and Natalie E. West

    Ch 29 Infection Prevention and Control in Cystic Fibrosis
    Charles S. Haworth and R. Andres Floto

    Ch 30 Technology in Cystic Fibrosis Therapies
    Christina M. Mingora and Patrick A. Flume

    Ch 31 Upper Airway Disease in Cystic Fibrosis
    Cassie Shipp, John Brewington, Daniel Beswick and Raouf Amin

    Ch 32 Gastrointestinal Disease in CF
    Zachary M. Sellers and Michael Wilschanski

    Ch 33 Cystic Fibrosis Liver Disease
    Julia M Boster and Michael R Narkewicz

    Ch 34 Cystic Fibrosis-Related Diabetes
    Melissa S. Putman, Sarah Collins and Kara S Hughan

    Ch 35 Growth in Cystic Fibrosis:  Is chloride transport the key?
    Michael S Stalvey

    Ch 36 Bone Disease in Cystic Fibrosis
    Peter Simm

    Ch 37 Cancer and cystic fibrosis
    Daniel Peckham and Rebecca Birch

    Ch 38 Other cystic fibrosis-related diseases and complications
    Michal Shteinberg, Mirjam Stahl

    Ch 39 Extrapulmonary benefits of the new CFTR modulator drugs
    Nicole Green, Lina Merjaneh, Steven D. Freedman and Margaret Rosenfeld

    Ch 40 Sexual health, fertility, and pregnancy in people with cystic fibrosis
    Raksha Jain, Susan M Sawyer and Malena Cohen-Cymberknoh

    Ch 41 Mental Health Issues in Cystic Fibrosis
    Janice Abbott and Trudy Havermans

    Ch 42 Adherence and Self-Management in Cystic Fibrosis Care
    Gregory Sawicki

    Ch 43 Transplantation
    Siddhartha G. Kapnadak and Kathleen J. Ramos

    Ch 44: Working with cystic fibrosis
    Scott C. Bell, Beth S. Sufian and James A. Passamano

    Ch 45 Growing Old with Cystic Fibrosis
    Andrew M Jones

    Ch 46 Imaging of Cystic Fibrosis Lung Disease
    Harm AWM Tiddens, Marcel van Straten, Mirjam Stahl and Pierluigi Ciet

    Ch 47 Lung function testing including Multiple Breath Washout
    Author: Paul D Robinson

    Ch 48 Infant Pulmonary Function Tests
    Lucy Perrem, Stephanie D Davis

    Ch 49 Exercise testing in cystic fibrosis
    Don Urquhart and Thomas Radtke

    Ch 50 Bronchoscopy and bronchoalveolar lavage in cystic fibrosis
    Isaac Martin and Felix Ratjen

    Ch 51 Patient-derived cell-based models for theratyping and individual CFTR modulator assessment
    Shannon M.A Smits, Lisa W. Rodenburg, Gimano D. Amatngalim and Jeffrey M. Beekman

    Ch 52 Minimally Invasive Investigations for Biomarkers of Airways Disease
    Joshua D. Chandler and Charles R. Esther Jr

    Ch 53 Currrent And Future Interventional Trial Designs To Support The Cf Therapeutic Pipeline
    Nicole Mayer-Hamblett and Bradley Quon

    Ch 54 Gene and RNA-based therapies
    Marie E Egan and Patrick T Harrison

    Ch 55 Cystic fibrosis in limited resource settings
    Silva Filho LVRF, Marco Zampoli2, Malena Cohen-Cymberknoh3, Sushil K Kabra

    Ch 56 Traveling With Cystic Fibrosis
    Meg Dvorak, Lcsw & Kate Yablonsky, Lcsw

    Ch 57 Organisation of Cystic Fibrosis Centre Care
    A Whitney Brown and Bruce C. Marshall

    Ch 58 The role of the CF nurse specialist
    Paula Lomas, Niccola Burke and Jill Fliege

    Ch 59 Physiotherapy
    Eleanor Main, Emma Dixon, Nicky Murray

    Ch 60 The role of the Dietitian
    Inês Asseiceira, Chris Smith and Joaquim Calvo-Lerma

    Ch 61 The role of the pharmacist as part of the multidisciplinary team caring for CF patients
    Martin J. Hug, Ph.D.

    Ch 62 The Role of a Psychologist on the Cystic Fibrosis Care Team
    Christina Jayne Bathgate, Sonia Graziano and Emily Muther

    Ch 63 Adolescent Health and Transition in Cystic Fibrosis
    Ryan Perkins, Gregory S. Sawicki and Traci M. Kazmerski

    Ch 64 Palliative and supportive care
    Stephen J. Bourke and Rachel Quibell

    Ch 65 Using registries & databases to drive up quality
    Oliver J. McElvaney and Christopher H. Goss

    Ch 66 Digital Transformations within CF Healthcare
    Tamara Vagg, Kevin F Deasy and Barry J Plant

    Ch 67 Personalized Medicine for Cystic Fibrosis in the 21st Century
    Kris De Boeck and Margarida D Amaral

    Ch 68 CF Research Priorities for the Future
    Jan-Christoph Thomassen, John Sheridan, Silke van Koningsbruggen-Rietschel, JP Clancy


    Professor 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
    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
    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