Survival of cystic fibrosis patients in South Australia. Evidence that cystic fibrosis centre care leads to better survival

Med J Aust. 1985 Sep 16;143(6):230-2.

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

  • Actuarial Analysis
  • Australia
  • Child
  • Child, Preschool
  • Cystic Fibrosis / complications
  • Cystic Fibrosis / diagnosis
  • Cystic Fibrosis / mortality*
  • Cystic Fibrosis / therapy
  • Hospitals, Pediatric
  • Humans
  • Infant
  • Infant, Newborn
  • Intestinal Obstruction / mortality
  • Lung Diseases / etiology
  • Outpatient Clinics, Hospital*
  • Prognosis
  • Pseudomonas Infections / etiology