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NLM Director’s Comments Transcript
IPF Treatment Progress: 06/30/2014

Picture of Dr. Lindberg
Abstract outline of the lungs inside a lab test bottle
Photo: Courtesy of
the National Library of Medicine.

Greetings from the National Library of Medicine and MedlinePlus.gov

Regards to all our listeners!

I'm Rob Logan, Ph.D. senior staff National Library of Medicine for Donald Lindberg, M.D, the Director of the U.S. National Library of Medicine.

Here is what's new this week in MedlinePlus.listen

I’m Rob Logan, Ph.D., senior staff, U.S. National Library of Medicine, for Donald Lindberg, M.D, the Director of the National Library of Medicine.

Here is what’s new this week in MedlinePlus.

New research suggests progress as well as questions about treating a fatal lung disease that impacts about 100,000 Americans, finds a commentary recently published in the New England Journal of Medicine.

Gary Hunninghake M.D., writes the results of recently reported clinical trials suggest two medications may slow the advance of idiopathic pulmonary fibrosis (IPF). Hunninghake, a lung disease specialist at Brigham and Women’s Hospital in Boston, explains until now newly diagnosed IPF patients had a discouraging three year survival rate of only 50 percent.

In fact, Hunninghake’s commentary begins with a description of the disappointment he formerly experienced when he discussed IFP with new patients. Hunninghake writes (and we quote): ‘I suspect that many of my patients have picked up on more than a hint of frustration in my voice when I tell them the cause of their shortness of breath is idiopathic pulmonary fibrosis’ (end of quote).

Until now, Hunninghake reports the only options for patients were careful monitoring, a lung transplant, or palliative care. He writes: ‘there has been little to offer in the way of treatment’ (end of quote) for a condition that robs patients of their breath by scarring the lungs, which causes them to stiffen.

Unlike similar diseases that inflame or scar the lungs, MedlinePlus.gov’s pulmonary fibrosis health topic page explains the cause of lung damage cannot be determined in IPF patients.

More positively, Hunninghake explains new clinical trials suggest two medications may deter lung function decline among patients diagnosed at an early stage of IPF. One of the medications, pirfenidone, improved patient’s walking distance (which suggests their prior shortness of breath improved). Other trial results suggest the drug reduced mortality rates among the participating IPF patients.

Hunninghake explains one of the trials also suggests IPF may be caused by an aberrant healing of wounds rather than previously suspected chronic inflammation. Hunninghake notes the latter findings may provide a more streamlined approach to future research efforts to prevent IFP’s progression.

The two drug trials (published in the New England Journal of Medicine with Hunninghake’s commentary) compared medications to a placebo on about 555 and 1050 IPF patients.

Since the number of participants is not high, Hunninghake infers the generalizeability of the findings may not be robust and the results additionally may be limited to the narrow recruitment criteria within both trials. Hunninghake adds (and we quote) ‘the studies provide little insights into the use of these drugs in patients with more severe disease or with an acute disease exacerbation’ (end of quote).

Hunninghake emphasizes the trials’ aggregate findings also raise important questions including (and we quote): ‘Are these effects durable beyond one year? How do these drugs work when used together….’ (end of quote).

However, Hunninghake finds the combined results provide progress to treat IPF patients in the early stages of the disease - and identify a clearer research pathway that may boost future IFP prevention.

Meanwhile, a helpful overview of IPF is provided by the National Heart, Lung, and Blood Institute (NHLBI) in the ‘start here’ section of MedlinePlus.gov’s pulmonary fibrosis health topic page. NHLBI also provides a website about the treatment of IPF in the ‘treatment’ section of MedlinePlus.gov’s pulmonary fibrosis health topic page.

MedlinePlus.gov’s pulmonary fibrosis health topic page additionally provides links to the latest pertinent journal research articles, which are available in the ‘journal articles’ section. Links to clinical trials that may be occurring in your area are available in the ‘clinical trials’ section. You can sign up to receive updates about pulmonary fibrosis as they become available on MedlinePlus.gov.

To find MedlinePlus.gov’s pulmonary fibrosis health topic page type ‘pulmonary fibrosis’ in the search box on MedlinePlus.gov’s home page, then, click on ‘pulmonary fibrosis (National Library of Medicine).’ MedlinePlus.gov also has comprehensive health topic pages on lungs and breathing as well as pulmonary rehabilitation.

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Please email Dr. Lindberg anytime at: NLMDirector@nlm.nih.gov

That's NLMDirector (one word) @nlm.nih.gov

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A disclaimer — the information presented in this program should not replace the medical advice of your physician. You should not use this information to diagnose or treat any disease without first consulting with your physician or other health care provider.

It was nice to be with you. I look forward to meeting you here next week.