Prognosis in cystic fibrosis
Cystic fibrosis (CF) was previously considered primarily a paediatric disease; however, most patients are now surviving to adulthood. The condition is now regarded as a life-limiting condition of adulthood. In 2017, around 50% of patients with CF in Australia were older than 18 years.
While there is still no cure for CF, improvements in respiratory and nutritional management continue to increase median survival. Based on data from the United States1, the median predicted survival for patients born between 2014 and 2018 is 44 years of age (ie half of people born with CF between 2014 and 2018 are expected to live beyond 44 years of age). This estimate assumes no further improvements in mortality rate from the current rates.