mmatory disease called sarcoidosis, radiation, drug reactions, autoimmune disorders and possibly a genetic predisposition, according to the American Lung Association. By far, most cases are considered to be idiopathic, or of unknown origin. Treatment options include corticosteroids and supplemental oxygen. There is no cure, although long-term benefit is possible with lung transplantation. According to the United Network for Organ Sharing, slightly fewer than 1,200 lung transplants were done in the U.S. in 2004 for all causes, including IPF. As of the same year, more than 3,500 people were still waiting for donor lungs to become available.
"Why a patient with IPF dies is not completely understood," said Dr. Kaminski. "We have funding from the National Institutes of Health to do some reverse engineering on IPF using genomics in order to better understand the disease mechanisms to look at all the genes expressed and track them as the disease progresses."
Since 2003, Dr. Kaminski and his colleagues have examined donated lungs from 12 patients, beginning with tissue from a retired firefighter who suggested the idea after participating in support group sessions. "We had never had patients wanting to donate their lungs before and had not even discussed the possibility," said Ms. Lindell, adding that research into the proposal revealed there were no such programs in the country for lung disease.
"The tissue that is available from lungs is rare and primarily from cancer patients," explained Dr. Kaminski. "Most IPF patients will have one biopsy for diagnostic purposes, and because of surgical risks and their fragile health, some may not get a biopsy at all."
But thanks to warm autopsy donors, tissue studied soon after death can be compared to tissue collected at biopsy usually years earlier. Preliminary analysis has revealed some promising paths for further research.
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