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Aug. 9, 2001 — Mention Viagra, the popular drug used to treat erectile dysfunction, and people tend to snicker. But as researchers at Boston Children’s Hospital and other centers have found, Viagra could be a life-saver for kids with a rare but potentially fatal lung disease called pulmonary hypertension.

According to the National Heart, Lung, and Blood Institute, about 300 cases of pulmonary hypertension are diagnosed in the U.S. each year. In the disease, blood pressure within a certain vessel in the lungs rises sharply for no known reason. The increase in blood pressure, or hypertension, leads to stiffening of the blood vessels in the lungs. This causes increasing shortness of breath and puts a severe, often fatal, strain on the heart.

Children with the disease are typically treated with nitric oxide delivered through a ; the gas helps the blood vessels in the lungs relax and allows more blood to flow through them. Yet when doctors try to wean them off the gas, their blood pressure can skyrocket again, putting them back in danger.

But as David Wessel, MD, and Andrew Atz, MD, reported in the journal in 1999, the same mechanism that helps men with erectile dysfunction achieve erection after taking Viagra may also help children be eased off nitric oxide. The drug works by preventing the release of nitric oxide from blood vessels, allowing the vessels to stay relaxed and blood to flow through them.

Marshall L. Summar, MD, who commented on the Viagra treatment, tells WebMD that a child’s inability to produce enough nitric oxide appears to be a factor in them developing the disease.

“Anything you can do to bolster nitric oxide production is probably going to be of some help,” he says. “There are probably some more direct ways to get at it that are a lot cheaper than Viagra that we’re looking at, but that’s for the future.” Summar is associate professor of pediatrics in the division of medical genetics and molecular physiology and biophysics at Vanderbilt University Medical Center in Nashville.

To date, Wessel and colleagues at Children’s Hospital have successfully weaned 13 of 14 children off of nitric oxide, in some cases using Viagra pills they crushed and delivered through feeding tubes. An additional 15 to 20 children have been treated at other hospitals with similar results, Wessel said in a Boston Globe interview last week.

Wessel recently returned from London, where he and nine other doctors attempted to persuade Pfizer, the maker’s of Viagra, to develop and test the drug in an formulation that would specifically treat children with pulmonary hypertension.

But because the demand for drugs to treat this disease is very small compared to the multibillion dollar market for erectile dysfunction drugs, it’s not certain whether Pfizer would be willing to commit to the estimated $4-10 million it would cost to test a drug in clinical trials.

“Our focus right now is on the medical possibilities,” Pfizer spokesman Geoff Cook tells WebMD. “There has been data out there that seems to show Viagra potentially could be a very important treatment for a condition that really has no treatments. It’s a crying medical need and that’s our focus.”