This guideline covers diagnosing and managing cystic fibrosis. It specifies how to monitor the condition and manage the symptoms to improve quality of life. There are also detailed recommendations on treating the most common infections in people with cystic fibrosis.
Cystic fibrosis is a multi-system genetic disorder affecting the lungs, pancreas, liver and intestine. It can have a significant impact on life expectancy and quality of life. The current median age of those who have died is 28 years and the median predicted survival is 45.1 years.
Diagnosis is primarily made during newborn screening. The median age at diagnosis is 2 months, and 1 in every 2500 babies born in the UK has cystic fibrosis. Approximately 60% of people on the UK cystic fibrosis registry are aged over 16 years.
Many different mutations are responsible for cystic fibrosis. The UK registry shows that 90.8% of people with cystic fibrosis have one known genotype; however 8.9% of people have at least one unknown genotype.