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Aggressive Therapy May Stem CF Progression in Infants

Treating cystic fibrosis in first 6 months appears most effective, Australian study finds

FRIDAY, Dec. 5 (HealthDay News) -- It may be possible to control the progression of cystic fibrosis through early detection and aggressive treatment of the disease in infants, according to Australian researchers.

They found that progressive lung damage in CF patients can start as early as infancy, even though lung function shortly after diagnosis is normal. The finding challenges current scientific belief.

The study included 68 infants with CF, ages 6 weeks to 30 months, who were compared to a control group of 49 infants without the disease. At 6 months of age, infants with CF had significantly poorer lung function than those in the control group, and that difference increased with each month of age. This shows that lung function in infants with CF declines sooner than previously thought, the researchers said.

They also found that diminished lung function occurred even when there were no clinical symptoms and irrespective of CF genotype.

The most significant finding was that infants with CF had normal lung function test results shortly after birth and within their first six months of life, the researchers said.

"We might be able to stop some of the lung function reduction we're identifying in the first months of life," study author Dr. Sarath Ranganathan, a consultant respiratory physician at the Royal Children's Hospital in Melbourne, said in an American Thoracic Society news release. "We don't know what's going to work, but we have to target those patients in the first six months of life if we're going to be effective."

The study, published in the second issue for December of the American Journal of Respiratory and Critical Care Medicine, validates the early-screening approach used in Australia and indicates that other nations should adopt similar programs, the researchers said.

"Ninety percent of those with CF can't absorb the fat in their diet, and people with this condition who are better nourished live longer," Ranganathan said. CF patients who can't absorb fat "should take manufactured pancreatic enzymes with every meal that contains fat, so they can have a good high-fat diet with lots of calories."

However, Ranganathan noted, "we can't be certain whether or not fat malabsorption that occurs prior to starting enzymes at diagnosis contributes to diminished lung function later on in early childhood."

More information

The March of Dimes has more about cystic fibrosis.

SOURCE: American Thoracic Society, news release, Dec. 5, 2008

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