Reviewed by Jerry A. Nick, MD

Although there is not yet a cure for CF, significant advances in the treatment of the disease have occurred. With new therapies there has been a remarkable increase in the expected survival of CF patients. Early discoveries of the ability of pancreatic enzyme replacement to permit digestion of proteins and fats allowed CF patients to survive childhood. More recently, advances in techniques to clear the airways of mucous, as well as improved antibiotics, have propelled median survival to over 37 years.

Many of the therapies available or under development for the treatment of CF are medications that are administered by inhalation of an aerosol or mist (nebulization). However, with each new medication and therapy the complexity of the treatment plan increases. Perhaps more than any other chronic disease, successful management of CF requires an enormous commitment by the patient. Especially in the setting of advanced disease, individuals with CF may be required to spend hours each days to keep up with airway clearance and a variety of inhaled, injected, and orally administered medications to combat airway infections, chronic sinusitis, diabetes, and pancreatic insufficiency.

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