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What you need to know about idiopathic pulmonary fibrosis, a fatal lung disease

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Updated: 1/17/2013 10:07 am

(BPT) - Most people age 50 or older might not be concerned if they experience shortness of breath from walking or doing everyday activities. They may believe this is happening because they are out of shape or have another medical condition. Most do not assume that shortness of breath might be a result of a life-threatening illness like idiopathic pulmonary fibrosis, or IPF.

In fact, most people have probably never even heard of IPF. For this reason, organizations that support patients and Boehringer Ingelheim Pharmaceuticals, Inc. are working to raise awareness of this deadly disease.

IPF is a severely debilitating lung disease that causes swelling and scarring (or fibrosis) of the lungs. Over time, the disease worsens and breathing becomes extremely difficult. The lungs lose the ability to take in and move oxygen throughout the body. Vital organs, like the heart and kidneys, do not get the oxygen they need to work properly. Patients are left breathless and exhausted.

Symptoms, such as a dry cough that won't go away, chest pain or rapid weight loss, are not always present until IPF has worsened, and the damage that IPF causes is permanent. There is no cure for IPF and currently, there are no FDA-approved treatments in the U.S. Sadly, approximately 60 percent of patients with IPF die from the disease within two to five years of diagnosis.

Research shows that IPF may affect as many as 100,000 Americans, though the exact causes of IPF are unknown.

With no FDA-approved medicines for the disease, doctors aim to improve their patients' health-related quality of life and may prescribe oxygen, rehabilitation that can help patients breathe better and lung transplant for appropriate patients.

IPF is considered a rare disease and most doctors in the U.S. are unfamiliar with its signs and how to diagnose it. Doctors must first rule out other cardiovascular and lung conditions, such as chronic obstructive pulmonary disease (COPD) or asthma. More than 50 percent of IPF patients may be initially misdiagnosed and it can take up to a year for a correct diagnosis.

More advanced knowledge of IPF can be found at a select few specialized lung research centers across the country. It is crucial that all doctors understand and become more aware of IPF so they can in turn help patients better manage their disease.

“We have learned that in addition to an urgent need for approved IPF treatment, there is an equally urgent need to educate doctors and support awareness efforts,” said Tunde Otulana, MD, pulmonologist and vice president, Clinical Development and Medical Affairs, Respiratory at Boehringer Ingelheim.

This September, the U.S. observed the first National Pulmonary Fibrosis Awareness Month and Boehringer Ingelheim is committed to supporting the efforts of groups like the Pulmonary Fibrosis Foundation (PFF) and Coalition for Pulmonary Fibrosis to drive greater awareness and understanding of this disease.

To learn more about IPF, please visit http://www.pulmonaryfibrosis.org or http://www.coalitionforpf.org.

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