title: Survival of cystic fibrosis patients in South Australia. Evidence that cystic fibrosis centre care leads to better survival.
abstract: Life tables were calculated for 205 South Australians with cystic fibrosis. An improvement in survival was noted between 1948 and 1982. Ninety-three per cent of patients who were diagnosed as having cystic fibrosis after 1973 were alive at 14 years of age, compared with 40% of those who were diagnosed between 1948 and 1973. A Cystic Fibrosis Clinic was established in 1973 and much of this improvement is attributed to the care provided by this centre. Deaths from meconium ileus fell from 58% of infants with this complication between 1948 and 1973 to only 8% between 1973 and 1983, in spite of the increasing incidence of patients who were chronically colonized with Pseudomonas aeruginosa (currently 68% of patients). These figures are similar to those from Victoria and from other cystic fibrosis centres in North America. The improvement in survival means that adults now comprise a quarter of the patients with cystic fibrosis in South Australia, and that adult institutions need to be aware of these patients and their needs.
mesh_terms: D000206:Actuarial Analysis; D001315:Australia; D002648:Child; D002675:Child, Preschool; D003550:Cystic Fibrosis / Q000150:complications / Q000175:diagnosis / Q000401:mortality* / Q000628:therapy; D006776:Hospitals, Pediatric; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007415:Intestinal Obstruction / Q000401:mortality; D008171:Lung Diseases / Q000209:etiology; D010044:Outpatient Clinics, Hospital; D011379:Prognosis; D011552:Pseudomonas Infections / Q000209:etiology