Long-term outcomes for patients with cystic fibrosis in Australia

Registries have an important role in monitoring outcomes of complex diseases

In the 1950s, a diagnosis of cystic fibrosis (CF) brought with it a high likelihood of dying from lung disease and a low expectation of surviving beyond 10 years of age.1 By 2011, expectations of survival for people living with CF have been transformed; mean age at death now approaches 27 years,2 and over 90% of patients survive beyond 17 years of age.3 Two studies reported in this issue of the Journal provide a broad overview of recent outcomes achieved in the management of Australian patients with CF.2,3 Both of these Australian studies recognised improved survival among people with CF, but reasons for this improvement remain unclear. Many factors probably contribute, including improvements in medical care, such as anti-pseudomonas antibiotics and better chest therapy in general, and the proven benefit of a high-fat diet for maintaining normal growth and nutritional status.4 Improved socioeconomic status may be a factor: a recent analysis of mortality rates among people with CF in the United Kingdom emphasised that lower socioeconomic status (based on two UK government classifications) contributes to higher mortality rates.5 In addition, a fall in the number of early deaths from CF with the advent of newborn screening has been clearly demonstrated.6 Newborn screening was commenced in New South Wales in 1981 and is of particular significance in the Australian setting. Recent data demonstrate a significant survival advantage for a cohort of babies who were diagnosed with CF during the first 3 years of screening compared with a non-screened cohort. Mortality among the non-screened population after 25 years was nearly twice that among the screened population (66% v 32%).7