This is improving with most children reaching early adulthood.
The current median age of those who have died is 28 years and the median predicted survival is 45.1 years (1).
FEV1 is a key predictor of life expectancy in people with cystic fibrosis, and optimising lung function is a major goal of care (1).
The improvement in life expectancy is likely to be a result of improved nutritional status, physiotherapy and antibiotic treatment.
The variability in survival with this disease may reflect a variable expression of the condition and an environmental contribution to the development of clinical manifestations.
Patients may be a candidate for a heart–lung transplant operation.
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