"We are dealing here with one of the few diseases left on the planet for which there are no proven causes and no treatments," he said.
Idiopathic pulmonary fibrosis occurs spontaneously, although certain factors -- such as smoking or exposure to airborne toxins -- do raise risks for the illness. "IPF is a progressive scarring process in the lungs that gradually robs a person of the ability to breathe," Shreve explained. Some sort of signaling seems to go awry at the cellular level, he said, converting normal, expansive lung tissue into stiff, fibrotic scar tissue.
"Once it starts in patients with IPF, your body just never sends a signal to stop that scar tissue from being produced," Shreve said. "This scar tissue is obviously not lung tissue that is able to process oxygen."
There have so far been very few promising leads in discovering the root causes of IPF, said Dr. Jesse Roman, one of the country's leading researchers in the disease and a professor of medicine at Emory University in Atlanta.
"Studies do suggest very specific [cellular] pathways, and there's a number of molecules that everybody is tuned into," he said. "But how you block them and how they relate to what happens in humans, that's less clear."
So, scientists are turning to creative new ways of looking at IPF.
Cross-talk between scientists worldwide led to the first-ever summit on the disease that included both veterinary and human medical researchers. The meeting was held in October on the campus of Purdue University in West Lafayette, Ind., and was attended by Corcoran, Roman and others. It was sponsored by the Westie Foundation of America and the American Kennel Club (AKC) Canine Health Foundation.
Westies, which grow to just under a foot in length, are described by the AKC as "courageous and self-reliant, but friendly."
"They're a very popular pet because of their size and their nature," Corcoran said.
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