January 9, 2004

Vanderbilt enrolling patients in drug studies for treating Idiopathic Pulmonary Fibrosis

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As part of the surgical advancements at VUMC, doctors use the Da Vinci robotic surgical system for a variety of surgical procedures. Photo by Dana Johnson

Vanderbilt enrolling patients in drug studies for treating Idiopathic Pulmonary Fibrosis

For the more than 83,000 adults with Idiopathic Pulmonary Fibrosis (IPF), treatment options are very limited. Standard therapy, which is effective in a small number of patients, includes the use of steroids and immunosuppressant drugs. In some cases, lung transplant is the only option.

As the prevalence of this disease grows, doctors are searching for more effective treatment options for IPF patients. Vanderbilt University Medical Center is enrolling patients in several drug therapy studies in hopes of discovering a treatment that will halt the progression of the disease.

IPF, a debilitating lung scarring disease that interferes with breathing, has no known cause. Although symptoms of the disease are nonspecific, patients typically present with shortness of breath and a dry cough. Some notice clubbing or rounding of fingertips.

Diagnosis tools include X-rays and ultimately a lung biopsy.

“This is a disease that has no good treatment at the present time,” said Lisa Lancaster, clinical director of the IPF Trials at Vanderbilt University Medical Center. “None of the standard treatments have shown improvement in pulmonary function or survival.

“At Vanderbilt, we have a special interest in IPF because of Dr. Jim Loyd’s research and clinical interest. He helped found our lung transplant program here. And for most transplant centers, emphysema is the main diagnosis for end-stage disease. At Vanderbilt, it’s IPF because of Loyd. We have developed a large referral base.”

Vanderbilt is currently enrolling patients in four, multi-center, international trials. The studies involve FDA- approved drugs including Enbrel, Bosentan, Gamma Interferon and Gleevec.

“We are looking at the treatment process and what is going on at the cellular level,” Lancaster said. “We are hoping to find medicines that can at least stabilize the disease. For these patients, pulmonary function is a gradual decline. We want to be able to find a drug that levels pulmonary function.”

Lancaster said both the pharmaceutical industry and academic physicians on the IPF forefront have worked together to develop research studies to accomplish just that.

Lancaster said the scarring typically begins in the periphery of the lung until it eventually covers the entire organ. The lung volume shrinks, essentially causing suffocation.

John Martin, a patient at the Vanderbilt Pulmonary Clinic, couldn’t be happier with the progress he has made since his participation in the trials.

Martin, who lives in Russellville, Ala., came to Vanderbilt three years ago with an unproductive, hacking cough in hopes of getting a second opinion about his disease and treatment plan. But he got a whole lot more.

“I got involved in the program at Vanderbilt,” said Martin, 77. “The pulmonary rehab in combination with the drug treatment has me really on the mend. I’ve improved since I started the program and feel as if I’m ahead of where I was two years ago.”

Martin, a retired TVA employee and chemistry teacher, who still enjoys raising cattle, said he feels fortunate to be able to participate in the drug trials at VUMC for Gamma Interferon.

“It was a double-blind study,” he said. “But I was one of the lucky ones who got the medicine initially. But the good thing about this particular study, was that everyone eventually received the drug. We all benefited.

“I’m still taking three injections a week of the drug. With no known cure, I would recommend programs like this that offer experimental drugs that could end up being very helpful.”

For those interested in any of the drug trials for IPF at Vanderbilt, call 343-7068 for more information.