10/21/2005 - In the eight years since Vanderbilt created its Idiopathic Pulmonary Fibrosis (IPF) Center, it has grown to become one of the largest IPF referral centers in the nation.
This year the center joined a network of academic medical centers funded by a five-year National Institutes of Health (NIH) grant to develop effective therapies for IPF.
IPF is a debilitating, progressive, scarring disease of the lung that results in respiratory failure. Very little is understood about the cause and there is presently no effective medical therapy.
IPF is not incredibly common, said Lisa Lancaster, M.D., assistant professor of Medicine and clinical director of the IPF program and IPF Clinical Trials Center. But we are seeing many more cases. We think the epidemiological studies underestimate the true prevalence of the disease.
Standard treatment has included potent immune suppressing medications, but none of the prior studies have shown that these medications are effective for this disease.
Currently the No. 1 treatment option for patients is enrolling in a clinical trial. The other option for a patient who qualifies is lung transplantation the only definitive therapeutic treatment available, but one which is associated with its own unique problems.
The average life expectancy for IPF patients is five to six years after diagnosis. The progressive scarring restricts the lungs ability to exchange oxygen and carbon dioxide, leading to shortness of breath and increased risk of infections. Even a modest infection may have serious consequences, whether viral or bacterial, and could be life threatening.
Our goals are to improve health care for IPF patients by offering therapeutic trials, evaluating patients for lung transplantation and providing patient and family education, said Lancaster. We've also formed a monthly support group for IPF patients and their families.
Vanderbilt evaluated more than 170 new IPF patients last year and the number of new referrals continues to grow. More than 80 patients have been enrolled in IPF clinical trials at Vanderbilt.
Vanderbilt currently has five active trials of IPF drugs under way, with a sixth slated for early 2006.
Typically, IPF patients are age 50 and above, but clinicians have diagnosed patients as young as 20 to 30 years old. The clinic often sees patients from surrounding states, including Arkansas, Mississippi, Alabama, Georgia, North Carolina, Indiana, Missouri and Kentucky.
The IPF clinical center team includes Lancaster; James Loyd, M.D., leader in this effort with the help of Cheryl Markin, coordinator of familial IP studies; Aaron Milstone, M.D.; Bonnie Slovis, M.D.; Paula Watson, M.D.; Wendi Mason, ACNP; Rhonda Greer, interstitial lung disease coordinator; and research analyst Laura Treese. Completing the team are Joyce Johnson, M.D., and John Worrell, M.D., with their expertise in interstitial lung disease pathology and radiology, respectively.
We are developing a large IPF and interstitial lung database and repository for clinical, epidemiologic and bench research, said Lancaster. We actively screen patients for a history of more than one family member with pulmonary fibrosis. We are trying to identify a genetic link.
Despite IPF being a serious and progressive disease, Lancaster said there is now more hope than ever for new understanding and effective therapy, and Vanderbilt investigators are making major contributions to these efforts.©2013 Vanderbilt University Medical Center