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Cystic Fibrosis Advance Shows Promise

Inexpensive Treatment Cuts Infections, Boosts Lung Function

New Insights Into Cystic Fibrosis continued...

As it turns out, this seems to be the case.

"These studies show that hydration of the lung surface is the way to go in cystic fibrosis," Boucher tells WebMD. "This was surprisingly effective. It makes sense to make this the base therapy for cystic fibrosis lung disease. If you restore mucus clearance, all the bacteria in the cystic fibrosis lung are in that mucus."

Bacteria are the source of the chronic infections that cause cystic fibrosis flare-ups. Getting rid of some of these germ-filled wads of mucus should mean fewer flare-ups. And that's exactly what happened.

Patients treated with hypertonic saline in the Australian study had 56% fewer flare-ups than those treated with an inactive placebo. Seventy-six percent of the treated patients -- but only 62% of the placebo patients -- were free of flare-ups during the yearlong study.

The patients' lung function improved, too. But that improvement was relatively modest, notes the editorial by Felix Ratjen, MD, PhD, head of respiratory medicine at Toronto's Sick Kids Hospital.

"The effect on lung function isn't all that impressive, but the hypertonic saline improves long-term mucus clearance by making more liquid available on the airway surface," Ratjen tells WebMD. "If there is clearance of mucus from the lower respiratory tract, this may result in less severe infections and lower frequency of infections."

The Real Goal: Treating Infants

What really excites cystic fibrosis researchers is the possibility that very young children might be able to use hypertonic saline. Why? There's the chance that the treatment could keep kids with cystic fibrosis from getting germ-filled mucus plugs in the first place.

"I think younger patients are going to be key," Ratjen says. "If you have established mucus deep in your lungs, in these areas already plugged by mucus there will be no effect of treatment. But if you start treatment in infancy, that would be a different story. You could potentially have a much more pronounced effect on cystic fibrosis lung disease."

Tests are already under way.

"We are already testing the safety and efficacy of inhaled hypertonic saline in 4- and 5-year-olds," Donaldson says. "And we have a plan to start testing in infants, newborns, and 1- to 2-year-olds. The idea is to stop them from getting mucus plugs, rather than have the infectious cascade happen."

Not an Easy Treatment

Hypertonic saline has its limitations. It's certainly no cure for cystic fibrosis. Patients can only inhale the salty mist so far into their lungs, leaving deep mucus plugs untouched.

The saline solution used is usually about 7% salt. Human blood is only 1% salt. The Atlantic Ocean is 4% salt, while the Dead Sea is 12% salt. At first, patients have a hard time getting used to the treatment.

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