Research Funding Needed to Stop Deadly Lung Disease

May 03, 2012 at 09:43 am by steve

Victor Thannickal, MD, director of UAB Division of Pulmonary, Allergy and Critical Care

A UAB physician was on Capitol Hill earlier this year urging lawmakers to support research funding to study idiopathic pulmonary fibrosis (IPF), a deadly disease with no known cure that appears to be affecting more people every year. HR2505, the Pulmonary Fibrosis Research Enhancement Act, would expand the research, prevention and awareness activities of the Centers for Disease Control (CDC) and the National Institutes of Health (NIH).

Victor Thannickal, MD, director of the Division of Pulmonary, Allergy and Critical Care at UAB, addressed Congress as an expert in this serious disease. He explained the importance of learning to accurately diagnose and treat IPF, which causes scarring of the lungs. "The scarring prevents the lungs from properly moving oxygen into the blood stream and breathing becomes difficult until the lungs are no longer capable of sustaining life," he says.

Finding a cure will not be easy because the cause of the disease is still unknown. Lung transplantation is often the only option for some patients, and UAB is one of the few lung transplant centers in the South. For most patients, however, IPF is a death sentence. Survival after diagnosis is typically three to five years. “IPF likely results from the lung’s inability to regenerate and repair itself after injury” Thannickal says. “It is a progressive and fatal disease that literally sucks the air out of the alveoli and suffocates the patient. It is a terrible way to die, and currently there is no treatment for it.”

IPF patient Gary Godwin of Fairhope, Ala., is fortunate to have received a lung transplant as his disease had progressed to the point that he carried an oxygen taken with him at all times. His journey began in 2006 when symptoms began. After several years of various treatments, he came to UAB in 2010.

At first, Godwin was told that because of his age - he was 65 at the time - his diabetes, and blockage in arteries in his heart, he was not a candidate. However, after receiving four stents in the left side of his heart, he was reevaluated and eventually placed on the transplant list for a lung on May 5, 2010.

That same month, Godwin and his wife, Susan, temporarily relocated to Birmingham to be close to the hospital. On May 29th he got the first call to come for the surgery. But because he had fluid in his lungs, the surgeons couldn't operate. He later received four more false alarm calls before receiving the transplant on Sept. 28, 2010. "That's my new birthday, I am told," he says.

Only one in every 1,000 people who need a lung transplant gets one, so Godwin feels blessed to be alive. "I am thankful to Victor (Thannickal) and Louise Hecker, a research scientist for helping me and for continuing to search for answers to the IPF puzzle," he says. "It is incredible how quickly this disease kills. I've asked God “why me,” and I think the mission He has given me is to share my story." Godwin is working on a book about his journey with IPF that he hopes will increase awareness of the disease and help other patients.

Awareness of this deadly disease is vital to developing the resources to understand it. A number of patient advocate groups throughout the United States are trying to increase funding for research. “IPF research is underfunded based on its comparison with other rare diseases like cystic fibrosis (CF) and amyotrophic lateral sclerosis (ALS), both of which are uncommon. Each year, about 75 percent more people are diagnosed with IPF than with CF or ALS, but both of these diseases receive a higher level of research funding,” Thannickal says. “Also, IPF kills about 40,000 people in the U.S. each year – the same number killed by breast cancer. Breast cancer gets more than $600 million in funding every year, while IPF gets less than $30 million.”

UAB is involved in most of the current clinical trials for IPF and is a member of the NIH-sponsored IPF network. Thannickal says that promising new research has revealed biomarkers and new therapeutic targets for the disease. Researchers also have discovered problems with current treatments.  “As part of the clinical trials, we have found that treatments given to IPF patients over the past 30 years have had negative effects,” he says. “Drugs were ineffective and, in some cases, harmful. A recent NIH press release said placebo patients actually did better than those on the standard treatment regimen. It’s important that we know if a therapy is ineffective.”

No specific cause for IPF has been identified, but Thannickal says that current thinking points to multiple factors that include environmental exposures such as cigarette smoke or other environmental pollutants. Aging and genetic susceptibility also are believed to play a role in development of the disease.

“The prevalence of IPF is increasing,” Thannickal says. “More patients are diagnosed every day because we have improved recognition of the disease and diagnoses are more accurate. Also, the U.S. population is aging, and the numbers are expected to rise for the next two decades. It also may be that the true incidence of the disease is increasing because of environmental exposures, some of which we still don’t understand.”

Thannickal says that UAB is partnered with another participating facility in current clinical trials, “and that organization has led to insights into what may or may not work,” he says. “UAB is involved in new treatments and we are working on new paradigms of how IPF occurs in the first place.”

In the past, for example, Thannickal says physicians were treating the inflammation that occurs in the lungs of IPF patients. “With the new therapies, we treat the fibrotic component instead,” he says. “It could be the most effective way to treat IPF patients.”

Thannickal and others around the country will continue their quest for research funds to find the answers and the best treatment to fight IPF. “We are leading the effort in Alabama, and we need to continue to move forward. We must keep trying to improve the lives of these patients.”




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