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.

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 authors who have contributed to this work.

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

Dedication

*Dedicated to Smita, Christian, and Kirsten who sacrificed our company during the* 


*creation of this book.*
