Two FDA Approvals on Same Day Renew Hope for Pulmonary Fibrosis Community

CULVER CITY, CALIF., Oct. 16, 2014 (GLOBE NEWSWIRE) -- Patients, families and researchers alike have anticipated for months the U.S. Food and Drug Administration (FDA) decisions on two drugs for Idiopathic Pulmonary Fibrosis (IPF). Expected to come separately by the end of November, the surprise FDA decisions on both drugs came on Wednesday.

The FDA approved InterMune's Esbrriet (pirfenidone) and Boehringer Ingelheim's Ofev (nintedanib) for IPF.

TV, Film and Broadway actress Barbara Barrie, who disclosed recently she has been diagnosed with IPF, is working with non-profit foundation, the Coalition for Pulmonary Fibrosis (CPF), to raise awareness of the little known disease. Barrie's diagnosis three years ago has not slowed the actress down. Like some IPF patients, she is able to carry on a busy, active life without supplemental oxygen or limitation.

"As a patient who has been dealing with this disease," said Barrie, "this news is a huge gift to me and to anyone else fighting Pulmonary Fibrosis."

Sports radio host George Lapides has spoken publicly on his Memphis-based radio show about his diagnosis with IPF and also appeared in a documentary on the disease that aired recently on the Discovery Channel (visit to view). "For those of us with IPF, this is fantastic. We have been hoping this would happen for a long time and now that it has, it gives hope to so many of us."

The drugs are the first FDA approved therapies for the disease that affects 200,000 Americans and claims as many lives each year as breast cancer. IPF is a lethal lung disease characterized by scarring that is irreversible, progressive and deadly in an average of three years

On September 26th, the FDA held the first IPF Workshop to hear directly from patients, families and caregivers about their experiences with the disease. Patients and family members of deceased IPF patients spoke about the hardships of IPF, a disease that debilitates patients early in its course and produces a steady decline in quality of life and gradual worsening of symptoms such as shortness of breath, cough and fatigue as well as issues with anxiety and depression.

IPF survivor, Heather Snyder, who received a lung transplant a few years ago, was one of the eight FDA panelists at the IPF Workshop. Snyder lost her father to the disease when she was a child in the 1980s. "I never thought I'd see this day," she said. "It is historic and hopeful yet frustrating and sad at the same time that it has taken so long."

The research community seems equally hopeful with the news of approvals. "The Food and Drug Administration approval of nintedanib and pirfenidone in the treatment of idiopathic pulmonary fibrosis (IPF) comes as welcome news, certainly for the brave patients who struggle with this deadly respiratory disease every day," said Thomas Ferkol, MD, president of the American Thoracic Society (ATS). "These approvals were the result of continued investments in IPF research, clinical care and increasing awareness among the public. It is truly gratifying news for the respiratory community."

The ATS and CPF have partnered on over a million dollars in research in the last decade in IPF.

About Pulmonary Fibrosis (PF)

Pulmonary Fibrosis (PF) 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 200,000 Americans suffer from PF, and there is currently no known cause or cure. An estimated 48,000 new cases are diagnosed each year. PF is difficult to diagnose and an estimated two-thirds of patients die within five years of diagnosis. Sometimes PF can be linked to a particular cause, such as certain environmental exposures, chemotherapy or radiation therapy, residual infection, or autoimmune diseases such as scleroderma or rheumatoid arthritis. However, in many instances, no known cause can be established. When this is the case, it is called idiopathic pulmonary fibrosis (IPF).

About the CPF

The CPF is a 501C(3) nonprofit organization, founded in 2001 to accelerate research efforts leading to a cure for pulmonary fibrosis (PF), while educating, supporting, and advocating for the community of patients, families, and medical professionals fighting this disease. The CPF funds promising research into new approaches to treat and cure PF; provides patients and families with comprehensive education materials, resources, and hope; serves as a voice for national advocacy of PF issues; and works to improve awareness of PF in the medical community as well as the general public. The CPF's nonprofit partners include many of the most respected medical centers and healthcare organizations in the U.S. With more than 28,000 members nationwide, the CPF is the largest nonprofit organization in the U.S. dedicated to advocating for those with PF. For more information please visit or call (888) 222-8541.

A photo accompanying this release is available at:

CONTACT: For actress Barbara Barrie, patients: Teresa Barnes Coalition for Pulmonary Fibrosis 303-521-4080 For Dr. Tom Ferkol and respiratory experts: Rory Williams American Thoracic Society 415-235-5976 mobile 212-315-8631 office

Source:Coalition for Pulmonary Fibrosis(CPF)