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Deadly Lung Disease Sends Patients on ‘Quest for Breath

ents is. Estimates suggest the number in the United States is around 200,000 to 300,000, but many doctors in the field believe the number is much higher, says Kevin R. Flaherty, M.D., assistant professor in the Department of Internal Medicine’s Division of Pulmonary and Critical Care Medicine at the U-M Medical School.

“It’s very hard to diagnose, and the diagnosis is often missed for months and sometimes years before it is recognized,” says Flaherty, noting that early diagnosis is very important in the management of IPF. Patients typically experience shortness of breath, and they are tested for other conditions before it is discovered they have IPF. Chest X-rays and pulmonary function tests can start to detect the fibrosis in the lungs.

He notes that IPF is more common in people 60 years and older, but, as in Galvin’s case, it can develop in younger people as well. It is somewhat more common in men than in women.

Most people believe IPF results from an injury or series of injuries to the lung, followed by an abnormal healing process, Flaherty says. Smoking is probably related in many IPF cases, but the cause and effect is not as direct as it is with lung cancer and other conditions, he says. And genetics almost certainly play a role, as in the case of the Galvin family.

As for treatments, Flaherty says, lung transplantation is an option for some patients in the end stages of IPF. Not all patients are eligible, however, because they can have other diseases that preclude the transplant or they are older than the usual cutoff for lung transplants, which is about 65 years old. And, he says, there simply aren’t enough lungs being donated for everyone who needs a transplant.

At U-M, researchers are exploring various aspects of IPF, both in the laboratory and with patients. For instance, scientists at the U-M Medical School have identified biochemical signals that attract pathogenic cells to damaged lung tissue –
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