NICE has published NICE Guideline (NG) 78 on Cystic fibrosis: diagnosis and management.

Cystic fibrosis (CF) is an inherited condition which affects the lungs, pancreas, liver, and intestines. Cystic fibrosis has a significant effect on on quality of life and reduces life expectancy, with a median predicted survival of 45 years. People with CF are also more vulnerable to several other problems, especially pulmonary infection (such as Staphylococcus aureus or Pseudomonas aeruginosa), which can demand long-term antibiotic therapy.

The recommendations made by NG78 aim to support:

  • diagnosis
  • service delivery, including how to organise services and multidisciplinary teams
  • annual and routine reviews
  • monitoring, assessment and management, including for lung disease, pulmonary infection, distal intestinal obstruction syndrome, liver disease, and CF-related diabetes
  • preventing cross-infection.