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Due to this new statewide effort — the first of its kind for lung diseases in the United States — patients who suffer from this progressive and currently incurable lung disease may soon have better access to care and research.
“Pulmonary Fibrosis is a disease that attacks the air sacs of the lung, causing progressive scarring of the tissue in the lung and, as a result, labored breathing,” explained Gerard J. Criner, director of the Temple Lung Center. “IPF affects about five million people worldwide and typically leads to death within about five years of diagnosis. Most IPF cases are considered to be idiopathic. We simply don’t know what caused it.”
Some known causes of pulmonary fibrosis include:
- Occupational and environmental exposure to asbestos
- Metal dust
- Farming chemicals and mold
- Sarcoidosis, an inflammatory disease
- Radiation
- Drug reactions
- Autoimmune disorders
- Genetic predisposition
“There is currently no proven effective therapy for IPF and most drug interventions are considered experimental,” Criner added.
“This new patient registry may help with patient recruitment in clinical trials in order for us to one day discover what definitively causes IPF and how can we effectively treat it with new medications,” said Gregory Tino, a pulmonologist with the Penn Lung Center, which also is taking part in the effort.
The Pennsylvania Idiopathic Pulmonary Fibrosis State Registry is a collaborative effort of five medical centers in the Commonwealth of Pennsylvania aiming to:
- Increase awareness of IPF
- Provide compassionate care for patients and their families
- Improve access to care and research including the discovery of new therapeutic interventions
Institutions participating in the registry are Temple University, University of Pennsylvania, University of Pittsburgh, Pennsylvania State University and Geisinger Health Systems.
The registry has been coordinated in large part by Naftali Kaminski, M.D., director of the Dorothy P. & Richard P. Simmons Center for Interstitial Lung Disease at the University of Pittsburgh School of Medicine. The initial idea came from Daniel Beren, a Harrisburg attorney and former member of the Pennsylvania General Assembly, R-153rd District, whose wife, Joan, died from IPF. Additional support was provided by the late Sen. Robert J. Thompson, R-19th District, who also died of pulmonary fibrosis; and Rep. James R. Roebuck Jr., D-Philadelphia County, whose wife, Cheryl, received a lung transplant to successfully treat the disease.
The PA-IPF is funded by the Pennsylvania Department of Health and by a donation from the Simmons Family.
For more information about the registry, visit http://www.pa-ipf.org/ or call 1-866-922-4IPF. For more information about the Temple Lung Center, visit http://pulmonary.templehealth.org.
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