PITTSBURGH, September 24, 2007 Patients who live in Pennsylvania and suffer from the progressive and incurable lung disease idiopathic pulmonary fibrosis (IPF) may soon have access to better research and care, thanks to an innovative patient registry effort coordinated in large part by Naftali Kaminski, M.D., director of the Dorothy P. & Richard P. Simmons Center for Interstitial Lung Disease in the division of pulmonary, allergy and critical care medicine at the University of Pittsburgh School of Medicine. This IPF registry is the first such effort in the United States for lung diseases.
In progressive disease with no proven therapy, access to clinical trials is sometimes the only hope for patients, said Dr. Kaminski, whose research focuses on fundamental mechanisms of pulmonary fibrosis and other advanced lung diseases. We believe this registry will improve access to care and research for patients with IPF in Pennsylvania.
Partner institutions in the statewide effort are medical schools affiliated with the Pennsylvania State University, Hershey, Pa.; Temple University and the University of Pennsylvania, both in Philadelphia; and the Geisinger Health System, based in Danville, Pa.
IPF is a degenerative illness distinguished by progressive lung scarring and diminished breathing capacity that typically leads to death within about five years of diagnosis. It is estimated that 5 million people worldwide and 130,000 in the United States are affected by pulmonary fibrosis. About 30,000 people die from the disease every year. According to the American Lung Association, some known causes of the disease include occupational and environmental exposure to asbestos, metal dust, farming chemicals and mold, an inflammatory disease called sarcoidosis, radiation, drug reactions, autoimmune disorders and possibly a genetic predisposition.
Most cases are considered to be idiopathic, or of unknown origin. There currently is no proven effective therapy for IPF and most drug interventions are considered experimental. Long-term benefit may be possible with lung transplantation, a radical approach dependent upon a limited number of donated organs.
One limiting factor in finding a cure for IPF has been the notion that this is a rare disease that makes recruiting patients for drug studies difficult, said Kevin Gibson, M.D., Simmons Center medical director, who oversees the development and testing of new therapies. At the Simmons Center, we follow about 400 patients with IPF, and other centers in Pennsylvania also follow significant numbers. With a statewide registry, we may be able to reach many beyond the ones who come to our clinic and greatly increase the testing and approval of new medications.
Although Dr. Kaminski has been instrumental in getting the registry started, the initial idea came from Daniel Beren, a Harrisburg attorney and former member of the Pennsylvania General Assembly, R-153rd District, whose wife, Joan Cranmer, died from IPF in 2006. Additional support was provided by the late Sen. Robert J. Thompson, R-19th District, who also died of pulmonary fibrosis in 2006; and Rep. James R. Roebuck Jr., D-Philadelphia County, whose wife, Cheryl, received a lung transplant to successfully treat the disease.
The whole focus is to find out what causes the disease and how it can be treated, said Beren. You suffer terribly with this, as my wife did. If I can help in some way so that people can find some relief, I'd like to do that.
The registry includes a Web site that contains information for patients, their families and health professionals and an Internet-based data bank that will store information concerning IPF patients to better provide them with guidance about care, research and support groups close to the areas in which they live. A quarterly newsletter also will be sent to all registry participants.
For more information on the registry, call 1-866-922-4473 or visit http://paipf.medicine.pitt.edu/.
Other investigators participating in the Pennsylvania IPF registry are Kathleen Lindell, R.N., M.S.N., Simmons Center; Rebecca Bascom, M.D., Pennsylvania State University; Gerald Criner, M.D., Temple University School of Medicine; Gregory Tino, M.D., University of Pennsylvania Health System; and David Fisk, M.D., Geisinger Health System.
For more information on the Simmons Center, call 412-802-3275 or visit http://simmonscenterild.upmc.com.