Cystic fibrosis: survival to adult life: ability to live with disability.

One hundred and twenty-eight patients managed by, or in association with, the Cystic Fibrosis Clinic, Royal Children's Hospital, Melbourne, have reached the age of 16 years; and 83 patients were alive on December 31, 1978, the oldest being 65 years of age. Survival data analysis on this group of patients showed a cumulative survival of 49% to 28 years. Despite the presence of substantial lung disease, most patients continued in full-time employment or education, and enjoyed a normal range of social activities. A review of these adults justifies a positive approach to the management of all patients with cystic fibrosis. With an increasing proportion of patients reaching adult life with minimal lung disease, the outlook can be expected to improve further over the next decade.