Care of the adolescent with cystic fibrosis

Abstract

Cystic fibrosis is an inherited condition which affects the exocrine glands. The lungs and the pancreas are the organs primarily involved. It is the most common and one of the most serious genetic disorders in the United Kingdom. It was first recognized in the 1930s when 90% of affected children died within the first year of life. Although cystic fibrosis can still be fatal, improvements in treatment have resulted in a steady increase in life expectancy. At present, 65% of affected children in England and Wales will survive until adulthood (18 years). The median age of survival has risen from 2 years in 1940 to approximately 30 years. The quality of life for those affected has also improved with at least 75% of adults with cystic fibrosis beng active and in full-time employment.