San Jose, Calif. (March 28, 2007) – Based on a new Johns Hopkins study published in the March 29, 2007 issue of the New England Journal of Medicine, thousands of people at risk for a deadly lung disease have new hope in a step toward discovering effective treatments. Scientists at Johns Hopkins have identified the genetic culprits that trigger familial pulmonary fibrosis, or the genetic form of pulmonary fibrosis, and may hold promise also in understanding the non-genetic form of the disease.

“This important discovery gives hope to families who are losing so many of their loved ones to this devastating disease,” said Mark Shreve, chief executive officer of the CPF. “These findings may provide the fuel that is needed to help move the important research in a positive direction.”

Until the creation of the CPF in 2001, there were no national resources or support services for IPF patients, and little focus was placed on the disease. The CPF now serves as the definitive resource for information and support for IPF patients and the healthcare professionals who treat them.

Prevalence and Incidence

Prevalence and incidence of the disease have increased an alarming 150 percent since 2001. Pulmonary fibrosis affects 128,000 people in the U.S. and has no FDA-approved treatments and no cure. It is believed that as many as 20 percent of pulmonary fibrosis cases have a genetic origin.

Scar Tissue Consumes Patients’ Lungs

Very few Americans have heard of pulmonary fibrosis, but the deadly lung disease, characterized by progressive and massive scarring in the lungs, affects several times more people than cystic fibrosis and Lou Gehrig’s Disease, yet receives a small fraction of the federal research funding.

Fighting for Family, Others

A member of the CPF staff, Teresa Geiger, vice president of Patient Outreach & Advocacy, has lost five members of her family to pulmonary fibrosis in the last several years, and 100 percent of the generation preceding her. Like other families fighting this disease, she is concerned for herself and her three siblings as well as future generations of her family.

“It is really difficult to see people you love suffer and die from this disease,” said Geiger. “The situation for patients is desperate because there is no treatment that will save them. Imagine hearing someone you care about is suffering from a disease that has, until recently, been all but forgotten and ignored. This research gives me and my family hope for a future without pulmonary fibrosis.”

Deirdre Roney, a CPF board member, has lost seven people in her family to pulmonary fibrosis. The latest death came in November 2006, with the loss of a 37-year-old cousin.

“I am concerned for myself and my remaining family members,” said Roney. “But we are not backing down. We must join together to increase research for pulmonary fibrosis; it is severely under-funded. We must raise the level of awareness and education, even in the physician community, about this deadly disease. My children are at risk; and as of now, there is no treatment or cure between them and pulmonary fibrosis."