Meet the editors

Prashant Mohite is trained as a cardiac surgeon in India and works as a Locum Consultant in Cardiac Retrieval at Golden Jubilee National Hospital. He was involved in the development of several programs at Harefield Hospital, England, including Organ Care Systems for heart and lungs, ex vivo lung perfusion, awake extracorporeal life support (ECLS), ECLS as a bridge to lung transplant, and donation after cardiac death (DCD) heart

transplantation. He is an avid researcher having published in well-known journals and presented at multiple national and international meetings as an invited speaker as well as through voluntary abstract submissions.

Dr. Anna Reed is a consultant in respiratory and transplant medicine, based at Harefield Hospital, England. She graduated from Leeds University in 1998 and obtained a Ph.D. in Pulmonary Vascular Pharmacology from Imperial College London in 2011. She maintains an active research interest in areas such as surfactant flux in primary graft dysfunction, ECMO bridging to lung transplant, pharmacokinetic and pharmacodynamic alter-

ations of commonly used anti-infectives on extracorporeal life support, Aspergillus disease and the immunological impact on the transplanted lung, and more. She has authored multiple manuscripts in high-profile journals and has reviewed numerous journal articles.

André R. Simon is a consultant cardiac surgeon and director of heart and lung transplantation and ventricular assist devices at Harefield Hospital, England. He is also an honorary senior lecturer at Imperial College London. He has written more than 100 peer-reviewed papers, reviews, and editorials. He has also contributed chapters to several textbooks. He has raised more than £2 million in funding for research into transplantation im-

munology, cardiac imaging, and ventricular assist devices. Dr. Simon is the founder and president of HeartHelp, a charitable non-government organization.

Contents

**Preface XI**

**Chapter 1 1**

**Chapter 2 9**

**Chapter 3 25**

**Chapter 4 51**

**Chapter 5 67**

**Chapter 6 91**

Introductory Chapter: Basics of Cystic Fibrosis *by Prashant N. Mohite and Vicky Gerovasili*

Detection and Management of Early Glucose Abnormalities

*by Katerina Theocharous, Bernadette Prentice, Charles F. Verge,* 

*by Waleed Mohamed Abdulkhair and Mousa Abdullah Alghuthaymi*

*by Prashant N. Mohite, Kavita Dave, Anna Reed and André R. Simon*

Recent Advances in Targeted Genetic Medicines for Cystic Fibrosis *by Salsabil Elboraie, Konstantinos N. Kafetzis, Rajeev Shrivastava* 

Lung Transplantation in Patients with Cystic Fibrosis

Cystic Fibrosis-Related Diabetes (CFRD)

*by Manfred Ballmann*

in Cystic Fibrosis

*Adam Jaffé and Shihab Hameed*

Microbial Cystic Fibrosis

*and Aristides D. Tagalakis*

## Contents


Fibrosis by Salsabil Elboraie, Konstantinos N. Kafetzis, Rajeev Shrivastava and Aristides D. Tagalakis

Preface

Cystic fibrosis is a genetic disorder affecting multiple systems in the body with various levels of severity. Over the last few decades, the patterns of presentation, aspects of diagnosis, and approaches to its management have changed substantially. Rather than an acute disease in children, cystic fibrosis is also now a chronic condition in adults. In the developed world there are more adults than children affected with the disease. As more affected children are reaching adulthood, the burden on healthcare is ever increasing given the natural history of cystic fibrosis, which is riddled with exacerbations and complications requiring constant clinical input, healthcare professional visits, and frequent hospital admissions. The treatment of cystic fibrosis has also changed greatly over the years. Gene therapy that remained on the horizon for many years is now a reality in clinical practice. Current management is focused

on preventing disease complications rather than treating them.

authors who have contributed to this work.

Today, the medical community is facing new challenges in the management of cystic fibrosis. The disease exhibits as a chronic affair with slow-deteriorating stable phases interspersed with acute exacerbations. These aspects of the disease mandate close observation and timely intervention at early signs of exacerbations to prevent catastrophic complications. Cystic fibrosis transforms the lungs into a hotbed for a variety of microbial flora that slowly develops resistance to multiple antibiotics. Advances in lung transplantation, immunosuppression, and post-operative management have led to increased survival rates for lung transplant recipients, qualifying more and more recipients as candidates for re-transplantation.

Since cystic fibrosis affects multiple bodily systems, its management naturally involves multiple specialties including genetics, microbiology, neonatology, pediatrics, diabetology, gastroenterology, infertility, psychiatry, respiratory, thoracic surgery, and transplant surgery. This volume serves as a handbook as well as a reference for clinicians, medical professionals, healthcare assistants and trainees in these various specialties. This covers the basics of the included as well as examines recent advances in the field. As "a picture is worth a thousand words," we have included in this book appropriate clinical photographs, explanatory cartoons, flowcharts, and diagrams. The book offers a comprehensive view of cystic fibrosis for students, professionals, and patients alike. We congratulate and thank all the

> **Prashant Mohite, Anna Reed and André R. Simon**  Royal Brompton and Harefield NHS Foundation Trust,

> > United Kingdom
