The Balancing Act’s “Behind the Mystery: Rare and Genetic Diseases” to Feature Idiopathic Pulmonary Fibrosis May 9th/16th

May 8, 2013

Idiopathic pulmonary fibrosis (IPF) is a devastating and ultimately fatal lung disease characterized by excessive scarring of the lungs. Unfortunately, the current prognosis for patients with this disease is they won’t survive. IPF claims nearly 40,000 lives annually – the same as breast cancer.

Given this alarming statistic, why is it that very few people know about IPF? Most likely because it is a rare, orphan disease that affects less than 200,000 Americans. However, IPF most commonly affects people older than 50, so as our population ages, the number of people with IPF will unlikely increase.

Some proportion of IPF is familial or genetic, but the majority is “idiopathic,” meaning we don’t understand its cause. But we do know the course of IPF is always progressive, unpredictable, irreversible and ultimately fatal.

Here are the facts:

– The healthy lung is an elastic structure that easily fills with air during breathing. In IPF, the diseased lung steadily fills with scar tissue and becomes very stiff. This makes it more and more difficult to breath and for the lung to do its job of getting oxygen into the body.

– For the patient, this process results in progressive shortness of breath and the inability to maintain the normal activities of daily living, such as walking.

– The median survival time from diagnosis is 2-5 years, with a 5 year survival rate of approximately 20-40 percent, which makes IPF more rapidly lethal than many malignancies, including breast, ovarian and colorectal cancers.

– IPF is often confused with COPD (chronic obstructive pulmonary disease) or emphysema

– IPF is challenging to treat because of its unpredictability. It is not possible to predict if a patient will progress slowly or rapidly, or when the rate of decline may change.

However, the good news is that understanding of IPF has increased tremendously over the past decade. Today, there is a vibrant community investing in drug discovery and disease awareness. These efforts are close to yielding medicines that will translate into effective therapies for patients that can potentially slow disease progression, which may ultimately prolong survival.

Join Dr. Bill Bradford a senior fellow at InterMune, Inc., a biotechnology firm that has 10+ years invested in IPF research, on Thursday, May 9th and May 16th at 7:00 am (ET/PT) on The Balancing Act’s “Behind the Mystery: Rare and Genetic Diseases” series, as he sheds some light on what’s being accomplished in the field of IPF that gives hope to patients and caregivers.

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