Like many people with idiopathic pulmonary fibrosis (IPF), Paul Fogelberg attributed his mounting fatigue and shortness of breath to aging. But when the 50-something could no longer keep up with his teenage girls and their activities, he knew something else had to be wrong.
A visit to his physician revealed crackling sounds in his lungs, and a pulmonologist diagnosed him with IPF in 2004. Fogelberg was given three to five years to live—a common prognosis for those with the progressive disease, which affects about 200,000 people in the United States and 1 million globally.
People with IPF struggle to breathe as scar tissue, or fibrosis, builds up in the air spaces of their lungs. This scar tissue gradually shrinks the size of the lungs and prevents them from effectively moving oxygen into the bloodstream. Ultimately, this fight for breath becomes fatal.
Turning to research
There are currently no treatments to halt the progression of IPF or reverse its effects, except for a lung transplant. Because IPF typically afflicts elderly individuals, age and underlying medical conditions make many of them ineligible for transplants, according to University of Minnesota Health Pulmonologist and Critical Care Physician Craig Henke, MD, who is a member of the University of Minnesota Health Center for Lung Science and Health at University of Minnesota Medical Center. The FDA approved two new drugs in 2014, but they may only slow the progression of the disease—not stop it, Henke said.
When Fogelberg’s physician told him that there was not much he could do about his disease, he got angry—and then took action. He formed Pulmonary Fibrosis Advocates in Wayzata, Minnesota, and started raising awareness and lobbying Congress about the need for more research funding for the disease.
“The money dedicated to pulmonary fibrosis research at the NIH (National Institutes of Health) is embarrassingly tiny,” Fogelberg said. “Just 0.11 percent of research dollars is spent on a disease that kills 40,000 people a year. The prevalence of this disease is up 150 percent since 2001, and as my generation ages, the numbers are going up. I’m tired of going to funerals and writing memorial checks for friends who died from PF.”
Fogelberg was referred to Henke about five years ago. Fortunately for Fogelberg, Henke has devoted the bulk of his career to researching interstitial lung diseases, including IPF.
With his NIH funding, Henke and his team have made several promising discoveries about IPF. They have discovered that the cells that create IPF scar tissue, termed fibroblasts, behave differently than normal fibroblasts. An activated signaling pathway makes the IPF fibroblasts more aggressive, which means they produce more scar tissue.
Building on that work, Henke next led research that identified the origin cell for the disease and its trademark abnormal fibroblasts, findings that were published last year in The American Journal of Pathology.
“Ultimately, we hope that therapeutic strategies targeting the abnormal IPF progenitor cell will stop disease progression,” Henke says.
The Ultimate Goal
Henke researches IPF in hopes of contributing to the discovery of its cause and the eventual creation of effective treatments.
“I see patients in my clinic with IPF, and it’s really hard to watch them suffer and not be able to do anything to help them,” Henke said. “I specifically remember one patient saying, ‘Isn’t there anything you can do to help me?’ As a doctor, that’s what you want to be able to do.”
Fogelberg has been an ardent and financial supporter of Henke’s work. He knows this research won’t help him beat IPF, but he hopes that it ultimately leads to treatment or a cure. Some forms of pulmonary fibrosis have a genetic component, and Fogelberg wants to make sure his daughters don’t suffer from the same illness.
“The research that Dr. Henke and others around the country are doing is going to result in a cure for pulmonary fibrosis,” Fogelberg said. “That’s not going to happen if we don’t fund basic and translational research by doctors like Craig Henke.”