Jaws Author Dies From Devastating, Terminal Disease

Jaws Author Dies From Devastating, Terminal Disease

Idiopathic Pulmonary Fibrosis Affects Thousands, No Effective Treatments, No

Cure

SAN JOSE, Calif., Feb. 13 -- "Jaws" author, Peter Benchley, died Saturday from idiopathic pulmonary fibrosis (IPF), according to the AP. Very few Americans have heard of IPF, but the deadly lung disease characterized by progressive and massive scarring in the lungs affects 83,000 Americans and there are no FDA treatments and no cure. The Coalition for Pulmonary Fibrosis (CPF) http://www.coalitionforpf.org/ is a resource for media and can arrange timely interviews with highly regarded experts on the subject.

Most people who have IPF live only 3-5 years. Lung transplant is currently the only treatment option proven to prolong life, yet most don't live long enough to get one. Although there are clinical trials underway investigating novel treatments for IPF, there remains no effective treatment to extend life; even though there are four clinical trials, none of the trials represent a cure.

The state of research in IPF includes studies underway that may impact the way the disease is treated, such as the largest familial study being done at Duke University that promises to learn more about the genetic link to the disease and the associated defective genes. The National Insitutes of Health (NIH) has developed the IPF Research Network that is a collaborative study with 11 study centers and one data collection center; it will begin recruiting patients in June 2006.

For comparative purposes, IPF affects 2/3 more people than Cystic Fibrosis (CF) and 1/3 more than ALS (Lou Gehrig's disease). However, both CF and ALS receive upwards of $100 million a year in federal research dollars and IPF will receive $3.4 million in the 2005 fiscal year from the NIH.

"We are grateful for the work that is being done in IPF research," said Mark Shreve, CPF CEO. "But it is clearly not enough. We need more funding for education, awareness and research. Researchers are doing the best they can with the limited resources they have."

In Congress, Congressman Charlie Norwood (GA) has IPF and was transplanted successfully a year ago. He helped pass a House Resolution (H. R. 178) in the fall recognizing IPF and the need for awareness, education and research. The Senate version of the bill (S.R. 236) is currently seeking Senate support.

Until the creation of the CPF, there were no resources or outlets for patients and little focus was placed on the disease that affects a known 83,000 people (the number is likely much higher due to misdiagnosis and underdiagnosis.

The CPF is governed by the nation's leading pulmonologists, individuals affected by pulmonary fibrosis, medical research professionals and advocacy organizations. The CPF has 9,500 members, and is the largest organization in the U.S. dedicated to pulmonary fibrosis. The organization has funded research in the area of IPF at various centers across the country including Duke University, University of Chicago and University of Pittsburgh as well as UCLA.

About Idiopathic Pulmonary Fibrosis

IPF is a lung disorder characterized by a progressive scarring -- known as fibrosis -- and deterioration of the lungs, which slowly robs its victims of their ability to breathe. Approximately 83,000 Americans suffer from IPF, and there is currently no known cause or cure. An estimated 31,000 new cases are diagnosed each year. IPF is difficult to diagnose, and an estimated two-thirds of patients die within five years of diagnosis.

About the Coalition for Pulmonary Fibrosis

The Coalition for Pulmonary Fibrosis (CPF) is a 501(C)(3) nonprofit organization, founded in 2001 to further education, patient support and research efforts for interstitial lung disease, and specifically pulmonary fibrosis. The CPF is governed by the nation's leading pulmonologists, individuals affected by pulmonary fibrosis, medical research professionals and advocacy organizations. With more than 9,000 members nationwide, the CPF is the largest nonprofit organization in the country dedicated to helping those with pulmonary fibrosis. The CPF's nonprofit partners include the Caring Voice Coalition, the Genetic Alliance, the Mary D. Harris Memorial Foundation, the National Coalition of Autoimmune Patient Groups, the National Organization for Rare Disorders (NORD), The Pulmonary Paper, the Second Wind Lung Transplant Association, and more than 30 leading medical and research centers nationwide. For more information please visit www.coalitionforpf.org.

Contact:
Teresa Geiger
Coalition for Pulmonary Fibrosis
303-521-4080
tgeiger@coalitionforpf.org

PRNewswire -- Feb. 13

Source: Coalition for Pulmonary Fibrosis

Web site: http://www.coalitionforpf.org/

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