Cystic Fibrosis - a Disease of Adolescents and Adults?

Summary

Background.
Cystic fibrosis (CF) is no longer a childhood disease. Since the identification of the gene in 1989research has made advances and changed views on the pathogenesis, diagnosis and treatment. The objective of thepresent work is to make doctors treating adult patients familiar with modern therapeutic methods and their value.Methods and Results. In the CF Centre of the Faculty Hospital in Prague Motol 349 patients are followed up ona long-term basis, incl. 95 who died since 1985. Hundred and twenty six (36.1%) patients survived to the age of 18years, of those 41 died and 85 patients live. Comparison of semilongitudinal data of a group of 83 patients bornbefore 1975 whose treatment during childhood and puberty was inadequate and 196 patients born in 1976-90 treatedby modern methods proved the great effect of treatment on the course and prognosis of the disease. The median ageat death increased during from 12.2 years in 1985-90 to 18.8 years in 1991-1998 (p = 0.004). The nutritional statusof adult patients is satisfactory in 40.4%, poor in 33.3% and marginal in 26.3%. A normal pulmonary function wasrecorded in 17.5%, 22.8% are severely affected, the majority of patients (59.7%) has values within 40 to 80% ofnormal levels.Conclusions. Modern intensive treatment improved the prognosis and quality of live in patients with CF. Criticaldeterioration of the clinical condition shifted to the threshold of adult age. It is therefore essential that doctors treatingsuch patients should be familiar with this issue.

Key words:
cystic fibrosis, nutritional status, pulmonary function, mortality, adults.