Living longer with Cystic Fibrosis

146 Pages·2016·1.09 MB·English

European Cystic
Fibrosis Society
Living longer with
Cystic Fibrosis
© 2015 European Cystic Fibrosis Society. All rights reserved. Editors
ISBN 978-3-00-049654-7 Dominique Hubert · Nicholas Simmonds
June 2015
June 2015 Living longer with Cystic Fibrosis
European Cystic
Fibrosis Society
Living longer with
Cystic Fibrosis
Editors
Dominique Hubert · Nicholas Simmonds
June 2015
Living longer with Cystic Fibrosis
ACKNOWLEDGEMENTS
ACKNOWLEDGEMENTS
The editors would like to express their sincere thanks for the relentless support Christine
Dubois has provided throughout the production of this textbook, without which this book
would not have been possible. We would also like to thank Professor Kris De Boeck for her
constant advice and support. Our thanks also to David Debisschop for the translation of the
testimonies in French. Finally, we would like to acknowledge the medical writing team and
K.I.T. Group GmbH for providing such an effcient and helpful service.
© 2015 European Cystic Fibrosis Society. All rights reserved.
No part of this book may be reproduced, transmitted or stored in an information retrieval
system in any form or by any means, graphic, electronic or mechanical, including photo-
copying, taping and recording, without prior written permission from the publisher.
European Cystic Fibrosis Society
Kastanieparken 7
7470 Karup
Denmark
www.ecfs.eu
ISBN 978-3-00-049654-7
2 www.ecfs.eu
Living longer with Cystic Fibrosis
CONTENTS
CONTENTS
Acknowledgements .............................................................................................................................................................................................................2
List of contributors ................................................................................................................................................................................................................4
Preface ........................................................................................................................................................................................................................................................6
Introduction .......................................................................................................................................................................................................................................7
Chapter 1 Changing epidemiology: new challenges in CF ...................................................................................9
Nicholas Simmonds, Barry Plant
Chapter 2 The increasing complexity of CF-related problems:
Pulmonary complications ...........................................................................................................................................................................................27
Christiane Knoop, Carsten Schwarz
Chapter 3 The increasing complexity of CF-related problems:
Emerging infections, non-tuberculous mycobacteria and fungal pathogens .........................39
Anne Prévotat, Marianne Skov, Tacjana Pressler
Chapter 4 The increasing complexity of CF-related problems:
CF-related diabetes and bone disease ..................................................................................................................................................55
Benjamin G. Challis, Amanda I. Adler, Maria Luisa Bianchi
Chapter 5 Increasing prevalence of other medical problems ..........................................................................71
Harry G.M. Heijerman, Scott C. Bell
Chapter 6 Complications of treatment .................................................................................................................................................83
Martin Walshaw, Thomas O.F. Wagner
Chapter 7 End-of-life care for CF patients .......................................................................................................................................95
Dorota Sands, Lieven J. Dupont
Chapter 8 Occupational and social issues .109
Hervé Laborde-Castérot, Anneke Vertommen, Geert Hollemans
Chapter 9 Patients becoming parents: Reproduction and pregnancy .119
Isabelle Durieu, Raphaele Nove-Josserand
Chapter 10 Psychological issues: Dealing with increasing disability .127
Araxie Matossian, Helen Solas
Patient Testimonies .137
European Cystic Fibrosis Society 3
Living longer with Cystic Fibrosis
LIST OF CONTRIBUTORS
LIST OF CONTRIBUTORS
Amanda I. Adler, MD, PhD, FRCP
Cambridge University Hospitals NHS Foundation Trust, Addenbrooke’s Hospital, Cambridge, UK
Scott C. Bell, MD, FRACP
The Prince Charles Hospital, Adult Cystic Fibrosis Centre, Chermside and QIMR Berghofer
Medical Research Institute, Brisbane, Australia
Maria Luisa Bianchi, MD, PhD
Experimental Laboratory for Children’s Bone Metabolism Research, Istituto Auxologico Italiano
IRCCS, Milan, Italy
Benjamin G. Challis, PhD, MRCP
Cambridge University Hospitals NHS Foundation Trust, Addenbrooke’s Hospital, Cambridge, UK
Lieven J. Dupont, MD, PhD
Department of Respiratory Diseases, University Hospital Leuven, Belgium
Isabelle Durieu, MD, PhD
Université Claude Bernard Lyon 1, Cystic Fibrosis Adult Center, Centre Hospitalier Lyon-Sud, Pierre
Bénite, France
Harry G.M. Heijerman, MD, PhD
Department of Pulmonology, Haga Teaching Hospital, The Hague, The Netherlands
Geert Hollemans, MD
CF Centre University Hospital Leuven, Belgium
Dominique Hubert, MD
Medical Director of the Adult CF Centre, Department of Chest Medicine, Cochin University
Hospital, Paris, France
Christiane Knoop, MD, PhD
Medical Director of the Adult CF Centre/Lung Transplant Unit, Department of Chest Medicine,
Erasme Uiversity Hospital, Brussels, Belgium
Hervé Laborde-Castérot, MD
Hôpitaux Universitaires Paris Seine-Saint-Denis, Unité de Pathologies Professionnelles et Envi-
ronnementales, Bobigny, France
Araxie Matossian
CF centre University Hospital Erasme Brussels, Belgium
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Living longer with Cystic Fibrosis
LIST OF CONTRIBUTORS
Raphaele Nove-Josserand, MD
Cystic Fibrosis Adult Center, Centre Hospitalier Lyon-Sud, Pierre Bénite, France
Barry Plant, MD, FRCPI
Cork Adult CF Centre, Department of Respiratory Medicine, Cork University Hospital, University
College Cork, Cork, Ireland
Tacjana Pressler, MD, PhD
CF Center, Copenhagen, National University Hospital Copenhagen, Rigshospitalet, Copenhagen,
Denmark
Anne Prévotat, MD
Cystic Fibrosis Adult Center, Hôpital Calmette, CHRU Lille, France
Dorota Sands, MD, PhD
Institute of Mother and Child, CF Centre, Warsaw, Poland
Carsten Schwarz, MD
Head Adult Cystic Fibrosis Centre, Lung Transplantation and Endoscopy Department of Pediatric
Pneumology and Immunology Charité - Universitätsmedizin Berlin, Germany
Nicholas J Simmonds, MD, FRCP
Department of Cystic Fibrosis, Royal Brompton Hospital / Imperial College, London, UK
Marianne Skov, MD, PhD
CF Center, Copenhagen, National University Hospital Copenhagen, Rigshospitalet, Copenhagen,
Denmark
Helen Solas, Psychologist
University Hospital of Akershus, Lorenskog, Norway
Anneke Vertommen
CF Centre University Hospital Leuven, Belgium
Thomas O.F. Wagner, MD
Christiane Herzog CF Centre for Children, Adolescents, and Adults, Department of Respiratory
Diseases, Goethe University Hospital Frankfurt am Main, Germany
Martin Walshaw, MD, FRCP
Liverpool Adult CF Centre, Liverpool Heart and Chest Hospital / University of Liverpool, UK
European Cystic Fibrosis Society 5
Living longer with Cystic Fibrosis
PREFACE
PREFACE
Living longer with Cystic Fibrosis, the title in itself is already a reason to celebrate. Indeed,
in developed countries the number of adults with CF now outnumber the children with CF.
Many thanks go to Dominique Hubert and Nicholas Simmonds for putting this entire book
together. They did a great job in defning the necessary book chapters. They then identi-
fed the necessary contributors and motivated them to not only provide an overview of the
evidence base in the literature but to also share their personal experience with the readers.
The fnal result has become a highly interesting and easy-to-read book.
We hope that this book will help to attract more adult care physicians to the feld of CF. With
the continuously increasing number of adults with Cystic Fibrosis in every country, there
is great need for training young internal medicine specialists in the complex care of adults
with Cystic Fibrosis.
ECFS is very pleased to offer this book “Living longer with Cystic Fibrosis” to all ECFS
members.
Kris De Boeck MD PhD
ECFS President
6 www.ecfs.eu
Living longer with Cystic Fibrosis
INTRODUCTION
INTRODUCTION
The demographics of Cystic Fibrosis (CF) have changed considerably over the last few
decades with median survival of around 40 years of age in most developed countries. It is
no longer a disease exclusively of childhood as the number of adults living with CF outnum-
bers children in many regions. While this should be celebrated it is also important to recog-
nise that with this improvement in survival comes a new set of challenges for patients and
their caregivers. That is why, after the frst book published in 2012 on “Healthcare Issues
and Challenges in Adolescents with Cystic Fibrosis”, ECFS decided to publish a book dealing
with adult issues “Living Longer with Cystic Fibrosis”.
Our ambition was to cover a wide range of healthcare issues relevant to adults and an
ageing adult population, in order to provide all members of the CF team with a resource to
consult when considering some of the diffcult issues that occur when people with CF grow
older. We also wanted to provide some insight into the emerging issues so that healthcare
services can keep pace and provide effective services in the future.
We thank all the authors from many different countries who worked together to achieve
a comprehensive review of the main issues in adults, and who shared their experiences.
We also thank the adult patients who have contributed to this book by providing invaluable
insight into what it is like to live with CF as an adult.
Dominique Hubert and Nicholas Simmonds
European Cystic Fibrosis Society 7
8 www.ecfs.eu