Cystic Fibrosis

Explores cystic fibrosis: from how it affects various systems of the body; through to the conventional treatments for CF lung and digestive disease. Finally, the genetics, the molecular basis of the disease, and the potential for gene therapy are examined.

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Cystic fibrosis is one of the most common genetic diseases in Caucasian populations. One in 2500 babies are born with the disease, usually to parents who have no idea that they each carry one defective CF gene. CF affects the respiratory and digestive systems and despite major improvements in treatment over the last 20 years the disease is usually lethal before the third decade of life. Treatment is lifelong and time-consuming, necessitating eating enzyme capsules with every meal to help digest food, daily physiotherapy to keep the lungs clear of mucus, and frequent courses of antibiotics to control lung infections. The book explores all aspects of the disease from basic clinical information on what is going wrong in the organs affected by CF to the conventional treatments for CF lung and digestive disease. Since the second edition of Cystic Fibrosis: The Facts, published just after the CF gene was identified, major advances have occured in our understanding of the genetic basis of CF. The third edition includes this new information and also has a new section on the potential for gene therapy being used in the future for the long-term treatment of this disease.