Diagnosis and management of cystic fibrosis: summary of NICE guidance

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What you need to know

  • Some people with cystic fibrosis (including adults) are undiagnosed because they missed the newborn screening process; they carry less common cystic fibrosis mutations; or have atypical manifestations of cystic fibrosis
  • Common complications associated with cystic fibrosis that might be identified in primary care include malnutrition, liver disease, infertility, and reduced bone mineral density
  • Forced expiratory volume in 1 second (FEV1) is the typical measure of lung function and a key predictor of life expectancy in people with cystic fibrosis
  • Optimising lung function is a major goal in cystic fibrosis care
  • Exercise is advised in cystic fibrosis to help maintain and slow the decline in respiratory function, facilitate airway clearance techniques, help improve bone mineral density, and to increase…