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Drugs are found to aid breathing in fatal lung disease

  • Article by: DENISE GRADY
  • New York Times
  • May 18, 2014 - 8:51 PM

For the first time, researchers have found drugs that can slow the decline of people with a fatal lung disease, idiopathic pulmonary fibrosis.

Studies of two experimental drugs were published in the New England Journal of Medicine and presented Sunday in San Diego at a meeting of the American Thoracic Society. The drugs did not make patients better, but slowed the rate at which lung function got worse.

The odds of death increase as lung function decreases, so researchers hope that by stabilizing it, the drugs will prolong survival. But it is too soon to tell.

The disease causes scarring of the lungs, making it harder and harder to breathe. At least 80,000 Americans have it. Half of patients die in three to five years.

An editorial accompanying the studies called the results “a major breakthrough for patients,” but also cautioned that questions remain about how long the drugs would work and whether they would help people with severe disease.

Until now, there had been no treatments that could do anything other than relieve symptoms like coughing.

“There’s been nothing,” said Gary Hunninghake, author of the editorial and a lung specialist at Brigham and Women’s Hospital in Boston. “That’s about to change. I think for a lot of patients this is going to be pretty exciting.” Lung transplants save some patients with the disease, but many are too old or too ill for the surgery. What causes the lung scarring is unknown. Smokers and former smokers are at higher risk, but most people who smoke do not develop the disease, and many patients who do get it never smoked.

“Once you’ve been given the diagnosis, people start to plan for how long they’ll be living,” said Kevin Brown, an author of one of the studies and a lung specialist at National Jewish Health, a respiratory hospital in Denver. He added, “Patients hope they can get better, but pray they don’t get any worse.”

Neither of the drugs has been approved by the Food and Drug Administration. The data from the studies will be submitted to the agency as part of the process of seeking approval for the drugs.

One drug, pirfenidone, is made by InterMune, which is based in Brisbane, Calif. The drug already has been approved for use in Europe, Canada and Japan. InterMune paid for the study. The other drug, nintedanib, is made by the German company Boehringer Ingelheim, which also helped pay for the study.

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