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Cystic fibrosis (CF) remains a life-shortening disease, despite improvements in care. To characterize trends in survival among people with CF, investigators analyzed data on more than 34,000 patients in the Cystic Fibrosis Foundation Patient Registry between 2000 and 2010. Results include the following:
The median age of CF patients increased from 14.3 years in 2000 to 16.7 years in 2010.
If mortality were to remain constant at 2010 levels, median survival of children born and diagnosed with CF in 2010 would be 39 years.
If survival were to continue to improve at the rate observed between 2000 and 2010, median survival of children born with CF in 2010 would be 56 years.
Female sex, ethnicities other than non-Hispanic white, and F508del mutation…