About the book
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, the aspects of diagnosis and approaches to its management have changed substantially. Rather an acute disease in the children it is now a chronic condition in the adults. In the developed world there are more adults than children affected with the disease now. As more affected children are reaching adulthood fighting the early encroachments of the disease, the burden on the healthcare is ever increasing given the natural history of cystic fibrosis that is riddled with exacerbations and complications requiring constant clinical input, healthcare professional visits, and frequent hospital admissions.
The treatment of cystic fibrosis has also displayed a monumental change over the years. The gene therapy that remained on the horizon for some good years is now a reality in clinical practice. The current management is focused on preventing disease complications rather than treating them.Today the medical fraternity is facing new challenges in the management of cystic fibrosis. The disease is exhibiting as a chronic affair with slow-deteriorating stable phases interspersed with acute exacerbations. This mandates a close observation and timely intervention at early signs of exacerbations to prevent catastrophic complications. The cystic fibrosis transforms lungs into a thriving bed for the variety of microbial flora that slowly develops resistance to multiple antibiotics. Advances in the lung transplantation technique, immunosuppression as well as post-operative management have led to an increase in the survival of lung transplant recipients qualifying more and more recipients as a candidate for re-transplantation.
Cystic fibrosis affecting multiple systems naturally involves multiple specialties in its management - genetics, microbiology, neonatology, pediatrics, diabetology, gastroenterology, infertility, psychiatry, respiratory, thoracic surgery, and transplant surgery are some to be named. This book is intended for the clinicians, medical professionals, healthcare assistants and trainees in these specialties. With few available books covering this subject, this book aims to be different. Chapters should cover the topics in entirety without missing onto the basics, at the same time reaching out to the recent advances in the field. Chapters should show uniformity, consistency and similar standards in their presentation and material and therefore authors are urged to follow the general guidelines of authorship.