Incidence and survival curves of cystic fibrosis in Sweden

Abstract

A registry of all patients with cystic fibrosis (CF) in Sweden has been kept since 1968. During the 10-year period 1968-1977 the number of CF patients in Sweden increased by about 8-9 per year, i.e. from 116 to 200, the median age of patients increased from 5 to 11 years, the percentage of patients above 18 years of age increased from 3 to 9 of the total CF population, and the median survival age increased from 7.5 to 16 years. For CF patients born without meconium ileus the median survival age in 1973-1977 was 17.8 years. From these figures the incidence of CF in Sweden is estimated to be 1 : 2200 to 1 : 4500. Several factors may have contributed to the improved outcome for CF patients, such as better medical treatment with more intensive physical therapy, the use of inhalation therapy and new antibiotics, improved social care and intensified information.