Key points

  • Newborn screening for cystic fibrosis leads to a reduction in prevalence, as couples are empowered to make better-informed reproductive choices.
  • Adult patients as well as children should be cared for by multidisciplinary teams in specialist cystic fibrosis centres, equipped to understand and manage all aspects of the disease.
  • ‘CF lung attacks’, i.e. periods of acute worsening of respiratory symptoms, negatively affect quality of life, accelerate decline in lung function and worsen prognosis.
  • Median life expectancy is now in the mid-30s and it is likely this will increase.
  • Although most of the morbidity and mortality caused by cystic fibrosis are still due to respiratory disease, complications affecting other bodily systems are becoming more prominent as life expectancy increases.

See the entire Cystic Fibrosis Chapter