Reviewed by Sara Brayshaw, MS, RN Jerry A. Nick, MD

How is Cystic Fibrosis Treated?

Although there is not yet a cure for cystic fibrosis, significant advances in the treatment of cystic fibrosis have occurred. With new therapies, there has been a remarkable increase in the expected survival of people with cystic fibrosis. Early discoveries of the ability of pancreatic enzyme replacement to permit digestion of proteins and fats allowed people with CF survive childhood. These were followed by advances in techniques and medications to clear the airways of mucus, as well as inhaled antibiotics. More recently, agents which partially restore CFTR function have become available for people with specific CFTR mutations, including those with two copies of the F508del mutation.

A complete list of approved CF medication and those under development is available.

Together, these advances in treatment, combined with the model of care provided by the CF Foundation-sponsored Care Center Network, have propelled median projected survival to about 40 years. Accredited Cystic Fibrosis Care Centers can be identified through their website.

In addition to sponsoring CF Care Centers and much of the research and new drug development, the CF Foundation has also assembled experts to form treatment guideline for all aspects of CF care, which have helped to standardize medical treatment for CF across the US and worldwide.

 

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