Cystic Fibrosis Treatment - New Drug Helps Prevent Onset Of Lung Disease

Recent findings by cystic fibrosis researchers suggest that individuals with normal to mild lung impairment could be helped by a newly tested drug designed to prevent the mucus so identified with the disease. The new drug is called denufosol and is administered during the early stages and seems to hamper the onset of lung disease in the study’s patients.

The results were published on the internet prior to the print edition of the American Thoracic Society’s American Journal of Respiratory and Critical Care Medicine.

“Although the lungs of children with CF are thought to be normal at birth, studies have demonstrated significant lung damage that occurs early in life in children suffering from cystic fibrosis,” said lead investigator Frank Accurso, MD, professor of pediatrics, University of Colorado School of Medicine, Denver. “Many patients continue to suffer progressive loss of lung function despite treatment of complications. Because denufosol can be used early in life, it offers hope for delaying or preventing the progressive changes that lead to irreversible airflow obstruction in CF patients.”

Denufosol is a member of a group of drugs called ion channel regulators. These particular drugs aid in balancing the movement of ions through cell membranes. This regulates the airway surface hydration. Cystic fibrosis sufferers experience a thick, uncomfortable mucus due to this abnormal flow of ion sodium chloride through cell membranes. As well as causing respiratory problems, the mucus enables bacteria to develop acerbating the disease’s affects.

“Abnormal ion transport and defective mucociliary clearance are fundamental defects that contribute to complications of CF lung disease, including mucus plugging, chronic bacterial infection, inflammation and progressive airway damage,” Dr. Accurso noted. “Although currently available drugs target these complications, denufosol was designed to treat the underlying defects that cause the complications, and could potentially modify the course of the disease, particularly when administered early in the disease process.”