Cystic Fibrosis Advance Shows Promise

Inexpensive Treatment Cuts Infections, Boosts Lung Function

By Daniel J. DeNoon
WebMD Health News

Reviewed by Louise Chang, MD

Jan. 18, 2006 -- The newest treatment for cystic fibrosis is a breath of fresh air for patients hoping to reduce flare-ups while saving money.

Unlike some other drugs used to treat this deadly lung disease, the new treatment is inexpensive. It's safe. It improves lung function. Most importantly, it cuts down on the flare-ups of the chronic infections that plague people with cystic fibrosis.

And that's just what it can do today. The treatment -- or an improved version of it -- might one day keep kids with cystic fibrosis from getting germ-filled mucus plugs in their lungs.

The treatment is called inhaled hypertonic saline. It's nothing fancy -- just a concentrated salt solution inhaled through a nebulizer. New clinical trials in Australia and in the U.S. show that the treatment works -- in a totally different way than previously expected. Cystic fibrosis is caused by an inherited genetic defect that allows mucus to collect in the lungs.

Researchers included Peter T.P. Bye, PhD, and Mark Elkins, MHSc, of the University of Sydney and Royal Prince Alfred Hospital, Sydney.

"Hypertonic saline is a new, effective therapy for cystic fibrosis," Elkins tells WebMD. "The benefits that can be anticipated are better lung function, fewer flare-ups of their lung infection, and a better quality of life."

Researchers for the U.S. study included Richard C. Boucher, MD, director of the cystic fibrosis research and treatment center at the University of North Carolina at Chapel Hill, and Scott H. Donaldson, MD, UNC assistant professor of pulmonary and critical care medicine.

"This is the first treatment aimed at a very early stage in cystic fibrosis disease," Donaldson tells WebMD. "It raises the possibility that early use may delay or prevent cystic fibrosis lung disease and add years of life to these patients."

The studies, and an accompanying editorial, appear in the Jan. 19 issue of The New England Journal of Medicine.

New Insights Into Cystic Fibrosis

Hypertonic saline isn't new. It's been used for decades to help people clear their airways. Why? It makes people cough.

That made doctors think hypertonic saline worked only as an irritant. Still, they thought it might help cystic fibrosis patients clear mucus from their lungs.

That idea was met with only partial success. Newer agents -- such as Pulmozyme, which actually thins mucus -- worked better. Hypertonic saline got shelved.

But cystic fibrosis researchers then made a breakthrough in basic science. They found that an extremely thin layer of water -- just a few hundred thousandths of an inch -- covers lung cells. This thin layer of water keeps mucus moving along. But people with cystic fibrosis don't have this layer of water. Instead of moving away, mucus sticks to their lung cells.

That made the U.S. and Australian researchers take a second look at hypertonic saline. The salt solution, they reasoned, might not be an irritant at all. Instead, the salt might draw water to the surface of cystic fibrosis lung cells, loosening up the sticky mucus.

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