Reprinted from The Hill – September 27, 2007

By Rep. Mike Castle (R-Del.)

This week marks National Idiopathic Pulmonary Fibrosis (IPF) Awareness Week, sponsored by the Coalition for Pulmonary Fibrosis (CPF), in Washington, D.C.

Since 2001, CPF has been working to raise awareness of the disease and to directly fund research and has brought much needed attention to this largely unknown disease. Before CPF was founded, there were no resources or support for IPF patients and their families.

IPF is a fatal lung disease that claims the lives of as many Americans as breast cancer each year, but is largely unknown. This disease results from progressive and incurable scarring of the lung tissue and ultimately affects ones ability to breathe and obtain enough oxygen. As the air sacs are replaced with dense fibrotic tissue, the damage done to the lung tissue causes an irreversible loss of lungs’ ability to transfer oxygen into the bloodstream, compromising the ability for vital organs to function normally. Symptoms associated with IPF include: shortness of breath, hacking cough, fatigue and weakness, discomfort in the chest, and rapid weight loss. Current treatments are often unsuccessful in slowing the progress of the disease or in relieving symptoms. The only treatment to improve survival is a lung transplant, yet most patients don’t survive long enough to get one. IPF is one of approximately 200 diseases, which are collectively called interstitial lung diseases (ILDs), in which lung scarring is found.

There is no known cause or cure for this disease and its prevalence is on the rise. IPF affects approximately 128,000 Americans, with 48,000 new cases diagnosed each year, which represents a 156 percent increase in the prevalence of IPF. The disease affects more men than women, and occurs most often between the ages of 50 and 70. The average life expectancy for IPF patients is just three years and every 13 minutes, someone dies from IPF in the United States, which accounts for the death of nearly 40,000 patients each year. However, despite these startling facts, most Americans have never heard of IPF until they or someone they know is given the diagnosis.

While the cause of IPF remains unknown, researchers have begun to identify a number of potential risk factors for developing IPF. Because the cause of IPF remains such a mystery, increased research on cause, treatment, and finding a cure are greatly needed. IPF also receives far less federal funding, only $14 million each year, for research than other similar or even less occurring diseases.

Stem cell research is one area with great potential in treating — or possibly curing — a wide range of diseases, including IPF. Scientists believe that 100 million Americans may one day be cured of or treated for their debilitating disease through discoveries and treatments made possible through embryonic stem cell research. Recent discoveries have shown that IPF is caused by abnormal stem cells. If these cells can be replaced with healthy stem cells, medical researchers may be able to eventually cure this disease. Scientists hope to eventually be able to replace lung tissue damaged by IPF with new stem cell therapies.

Following the death of Rep. Charles Norwood (R-Ga.) from IPF earlier this year, the awareness of this disease in Washington is growing. A resolution has been introduced by Reps. Brian Baird (D-Wash.) and Nathan Deal (R-Ga.), and by Sens. Norm Coleman (R-Minn.) and Dick Durbin (D-Ill.), in recognition of the need for research into the cause of IPF and to raise the importance of identifying viable treatments and an eventual cure for IPF.

IPF is an ultimately debilitating and fatal disease, and one that is very personal to me. Because so much about IPF is unknown, it is my great hope that we act quickly to accelerate research funding at the National Institutes of Health, and that we work to identify the cause and progression of the disease through a registry system, so that new and better approaches to treat and cure IPF will be found. I trust that National IPF Awareness Week will serve to raise the profile of IPF and the 128,000 Americans who suffer from this disease.

Castle is a member of the House Education and Labor, and Financial Services, committees.