Chris Galvin has found a decidedly poetic way to describe a disease that is the furthest thing from beautiful. The condition took his father's life and, Galvin always knew, threatened him as well.
"It's kind of like the storm that's sitting out on the horizon that you know one day will come to shore on your beach," says Galvin, 37. "It's something I've thought about my whole life and have always had an adequate amount of fear about."
His fears were realized last year, when he was diagnosed with idiopathic pulmonary fibrosis (IPF), a progressive scarring of the lungs in which patients' lung function declines and they develop shortness of breath with minimal activity, and even at rest during the late stages of the disease. There is no cure; IPF took the lives of Galvin's father (at age 45), an uncle, an aunt and a grandmother, and about 40,000 lives a year in the United States. His own diagnosis in January 2005, Galvin says, was devastating.
Yet he and his family maintain some hope for the future. They have started an initiative called Quest for Breath to help support IPF research, which includes an annual event to raise money for the fund named for their father, the Martin Edward Galvin Fund for Idiopathic Pulmonary Fibrosis Research at the University of Michigan Health System.
"Because this disease has no cure, it's very important, especially when the government is funding less research, to pour efforts into raising money to jumpstart this research," says Galvin, of Troy, Mich. "My family and I decided to become very involved with the University of Michigan in helping to raise funds for research, but also to help develop a community where awareness could be spread about the disease."
He notes that the University of Michigan is doing important research about the disease that he and his family hope will lead to major breakthroughs in the future.
Nobody knows just how big the community of IPF patients 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 - one of the first steps in a chain of events leading to IPF.
Bethany B. Moore, Ph.D., Galen B. Toews, M.D., and other Medical School researchers hope their work will help to unravel the mysteries surrounding IPF and will lead to therapeutic drugs to block progressive lung damage or diagnostic tests to make early detection possible.
That kind of discovery won't help Chris Galvin, though he remains hopeful about the future. His IPF progressed rapidly in the first year but has reached a plateau more recently. He had 60 to 70 percent lung capacity when he was first diagnosed, and a year and a half later was down to 33 percent capacity. He now can't run or lift heavy items without taking oxygen.
While there is no cure for IPF, he has taken part in treatments, starting with a high dose of steroids last summer. Later, a pill-variety of chemotherapy was added. Along with over-the-counter supplements, those medications seem to be somewhat effective at stemming the progression of scarring, Galvin's doctors have told him.
In spite of the prognosis, Galvin has found a way to keep a positive attitude with the help of meditation and his decision not to view himself as sick.
"I still work full-time, I still maintain all my friendships and social activities pretty much the same as before," he says. "I choose to think positively, and it makes all the difference in the world."