Stars aligning for U.S. Patients Suffering from Idiopathic Pulmonary Fibrosis

Two Investigational Drugs Receive Breakthrough Designation, First FDA Workshop on IPF Announced, Bringing Attention to Little Known Disease


CULVER CITY, CALIF., July 18, 2014 (GLOBE NEWSWIRE) -- The stars are seemingly aligning for patients with Idiopathic Pulmonary Fibrosis (IPF) and patient groups like the Coalition for Pulmonary Fibrosis (CPF), who are preparing for good news for a change.

This week alone, the U.S. Food and Drug Administration (FDA) granted breakthrough therapy designation for two investigational treatments for the disease: Boehringer Ingelheim's nintedanib and InterMune's pirfenidone. Just a couple of weeks ago, the FDA announced it will hold the first FDA Workshop on IPF on September 26th and hear directly from patients regarding their experiences with IPF and their needs and willingness to except risk relative to drug development efforts. FDA IPF workshop information can be found at: http://www.eventbrite.com/e/public-meeting-on-idiopathic-pulmonary-fibrosis-patient-focused-drug-development-registration-11505211397?aff=es2&rank=1

The positive news is welcome to patients, researchers and advocates as it comes after more than a decade of multiple failed clinical trials in the disease and nearly a half a million deaths to it in the U.S. in the same time span.

"The CPF is so pleased to see the FDA has prioritized the review of these drugs and is preparing for direct testimony from the people who suffer from IPF," said Mishka Michon, Chief Executive Officer of the CPF, a non-profit foundation and patient advocacy group for patients and families suffering from the disease. "It has been a long time coming for patients and families who have witnessed many failed clinical trials to date. We are hopeful that patients will finally have access to a treatment here in the U.S."

Idiopathic Pulmonary Fibrosis causes progressive, irreversible and lethal scarring in the lungs and the cause is unknown. The average life expectancy post diagnosis is 2.8 years. 

Positive Drug Data

In May, both Boehringer Ingelheim and InterMune, Inc., announced positive data from their Phase 3 clinical trials for nintedanib and pirfenidone, respectively, at the American Thoracic Society (ATS) international medical conference.  If the FDA approves them, the drugs would be the first approved therapies for IPF in the U.S.

National Pulmonary Fibrosis Awareness Month in September

September promises to further increase the attention to IPF as the CPF holds national awareness efforts for the 12th year as part of National Pulmonary Fibrosis Awareness Month in September. The CPF and the ATS will co-host an awareness week for the disease during PF month, the same week as the FDA hearing (September 21-27) and the CPF will bring patients and advocates to Capitol Hill in September, as well.

Report to the FDA

The CPF is currently gathering patient experiences via a software platform survey system with the Genetic Alliance called PEER.  The program allows the CPF to query its patients, their family members and caregivers as well as the families of deceased patients, about what they have gone through with the disease. The CPF will analyze the data and present it to the FDA in a report in time for the FDA IPF Workshop.  The survey can be found on the CPF's website at: http://www.coalitionforpf.org/peer-portal/

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 501(c)(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 www.coalitionforpf.org or call (888) 222-8541.


            

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