What do Marlon Brando and Evel Knievel have in common? Both well-known Americans died of pulmonary fibrosis, or PF, perhaps the deadliest disease that no one knows about. In fact, PF kills just as many Americans annually as breast cancer and, like breast cancer, lacks a cure. But unlike breast cancer research dollars for PF are virtually nil.
PF impacts about 128,000 Americans, including me. It is a progressive and fatal disease characterized by scarring of the lungs, which causes an irreversible loss of the lung tissue's ability to transport oxygen — literally robbing victims of the ability to breathe. And the prevalence of this devastating disease (or, at least, our ability to detect it) is on the rise. Reports of the disease are up more than 150 percent since 2001, and roughly 48,000 new cases are diagnosed annually.
Despite its growing prevalence, patients diagnosed with PF face demoralizing statistics. The median survival rate is just three years, and more than two-thirds of patients will die within five years. In total, the disease claims 40,000 lives every year, which translates to one death from PF every 13 minutes. The idiopathic form of PF is in fact more rapidly lethal than many cancers, including breast, ovarian and colorectal.
The federal government needs to review its funding of PF research, as diseases that lose a quarter of the number of patients each year can receive as much as five times the funding. There are other rare diseases with more recognizable names but far fewer patients that draw more attention and funding. Given the increased incidence of PF throughout the community, and the growing number of younger patients, it behooves the government to increase its investment in finding answers. The billions spent on caring for tens of thousands of patients with PF over the next decade will be reduced dramatically with the advent of serious treatments for the disease.
Increased funding will help inform people about PF, the severity of the diagnosis and the urgent need for treatments. There are promising treatments on the horizon that could slow disease progression — and we need to give those treatments a fighting chance.
As PF Awareness Month — September — comes to a close, this is an excellent time to refocus our efforts around this disease. An important step is the Pulmonary Fibrosis Research Enhancement Act, a bill to increase National Institute of Health funding and grants for research into PF. The bill currently has 71 co-sponsors in the U.S. House of Representatives and 13 in the U.S. Senate, but, to date, not a single Utah representative has signed on as a co-sponsor. I urge the Utah delegation to support what could be life-saving legislation.
Indeed, I am lucky to still be here — and to be able to be a voice for hundreds of thousands of people around the world who are literally struggling to breathe because of this disease. By increasing awareness of this disease, we can extend the time for research to find a cure. There is a cure for PF; we just haven't found it yet.
Olene Walker is the former governor of Utah. She was diagnosed with pulmonary fibrosis in 2002.
Copyright 2016, Deseret News Publishing Company