tablet with Idiopathic pulmonary fibrosis (1500 x 1000)

Doctors see a lot of sick people and disease. That’s the job. Observe, diagnose and prescribe a treatment. Let’s imagine that John, an active middle-aged man, sees his doctor, complaining of shortness of breath while hiking. During the visit, a doctor asks John about his symptoms and looks, listens, and feels for physical signs that can help lead to a diagnosis. Shortness of breath is a symptom of very common diseases like asthma and COPD, which affect millions of Americans—18.7 million adults have asthma and 6.8 million live with COPD. It’s likely those could be behind John’s shortness of breath. But there is another, lesser-known disease that proved to be true for John—a lung disease called idiopathic pulmonary fibrosis (IPF).


What is IPF?

lung disease

IPF is a lung disease that has no known cause and is unpredictable. It more commonly affects men over the age of 50 and is accompanied by shortness of breath and a dry cough. Underlying these symptoms is progressive scarring (fibrosis) of the lung tissue. The scarring of the lungs makes it difficult for the lungs to deliver oxygen to the blood. This means that the muscles, heart, and other organs do not receive enough oxygen to function properly. Eventually, the lungs become hardened and stop working.

So why haven’t you heard of this devastating disease? Because IPF is rare, which means it affects less than 200,000 people in the United States at any given time. More than 100,000 people are currently living with IPF, and about half that many are newly diagnosed each year. Although IPF affects far fewer people than diseases like asthma and COPD, it’s estimated that 40,000 people die from IPF each year—that’s as many as breast cancer.

Since IPF has symptoms similar to other respiratory diseases, many people don’t know that they have it—and neither do their doctors. IPF is also under- or misdiagnosed, which can delay proper treatment. IPF usually can’t be seen on a normal chest x-ray or CT scan, so high-resolution computer tomography (HRCT) scans may be needed for proper diagnosis.


Medical treatment

For many years, progress in treating IPF has been slow, but it is quickly changing for the better. There are a growing number of ways doctors can help people manage IPF even though the disease can vary among people. Therapies like supplemental oxygen and pulmonary rehabilitation are historical treatment mainstays effective in supporting lung function. There are also medicines now available that were approved last year by the FDA. Research in IPF continues. At the annual conference of the American Thoracic Society (ATS) last month in Denver, there were several studies that give us more information on these medicines.


Other treatments

It’s also important to think beyond just the medicines and therapies. Sometimes emotional support can make all the difference to people with difficult diseases. Patient advocacy groups such as the Pulmonary Fibrosis Foundation and the Coalition For Pulmonary Fibrosis help people stay connected and access important information. Online communities such as PatientsLikeMe and Inspire connect people and their families. is another resource for information.

Now doctors can do more for IPF than ever before, and we cannot let a lack of awareness stand in the way of helping John and others. Awareness of IPF signs and symptoms can help people get a proper diagnosis and early treatment, so they can continue to live their lives. With new treatment options and emerging research, this is an exciting time for IPF. Now, more than ever, we have a chance to both improve the quality of life of people with IPF, as well as attempt to stem the tide of IPF progression.

Ben Kramer
Ben Kramer, M.D., is a Vice President, Medical Affairs at Genentech, responsible for Rheumatology, Respiratory and Ophthalmology. Prior to joining the pharmaceutical industry, Ben was an investigator, private practice physician and Clinical Instructor of Medicine in California and New York. He is an author of many publications and abstracts in the areas of allergy, asthma and COPD.



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