The world’s largest private funder of pulmonary fibrosis research and a 20,000-member professional medical association are combining forces to slash the time it takes to diagnose idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases.

Robb Rabito, senior director of program development for the American College of Chest Physicians. Credit: CHEST

On May 17, 2022, the nonprofit Three Lakes Foundation and the American College of Chest Physicians (CHEST), both based in Chicago, Illinois, announced an initiative to educate primary care doctors about IPF, which currently leads to death within 3-5 years of diagnosis.

The “Bridging Specialties” project is of “absolute urgency,” said Robb Rabito, senior director of program development at CHEST, which focuses on pulmonary critical care and sleep medicine.

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“Whenever we have patients who are not getting diagnosed as efficiently as they could be, it’s an urgent issue,” Rabito said. “This is a years-long, open-ended effort. We’re starting with the United States, but at some point, all of our efforts become global.”

Three Lakes and CHEST—which will evaluate, research, and measure the program’s impact on clinical practice and patient care—eventually hope to reach up to 100,000 physicians.

“Our goal is to put a spotlight on this uncommon disease,” said Dana Ball, executive director of Three Lakes, which was established in 2017. “By leveraging other organizations and vested healthcare institutions, we hope to build awareness so they will see the value of educating primary care physicians. If patients don’t respond to current treatments for asthma or [chronic obstructive pulmonary disorder (COPD)], they should be referred to a pulmonologist.”

‘Change Is Coming’

Ball said Three Lakes, the world’s largest private nongovernment funder of pulmonary fibrosis research, has committed to first-year funding and “will reassess at the beginning of the second year, bringing in other funders.” He declined to say how much the initiative will cost.

The foundation, established by an anonymous family in memory of a loved one lost to IPF, selected CHEST as its partner thanks to its large network of pulmonary specialists, Ball said.

Dana Ball, executive director of the Three Lakes Foundation. Credit: Three Lakes Foundation

“We realize that many physicians may never see a patient with IPF because it’s a rare disease,” he told Rare Disease Advisor, noting that many patients are already on supplemental oxygen at the time of diagnosis. “But we think this is worth the investment. Many people sitting at home today have been diagnosed with COPD or another respiratory disease, when in reality they have pulmonary fibrosis.”

The scarring or fibrosis caused by IPF cannot be reversed or repaired, and the disease has no cure. At present, 30,000 to 40,000 patients receive an IPF diagnosis each year in the United States alone, with another 40,000 dying from the disease annually.

Even so, accurately diagnosing IPF is challenging because some of its most common symptoms—such as wheezing, coughing, fatigue, and difficulty breathing—are also hallmarks of other diseases, including bronchitis, asthma, COPD, and COVID-19.

“When I started here 3 years ago, this was a very hopeless situation. What we know is that in every disease where you see dramatic change, there’s a catalyst, a famous person, a rock star who stands up and says ‘we need to address this problem,’” Ball said.

“The great gift this family has given is funding, commitment, and its passion to serve as a catalyst,” he added. “We’ve seen incredible response. Change is coming.”  

Bringing Disciplines Together

Ball said the partnership, in development for half a year, has appointed a 10-member steering committee including patient advocates, pulmonologists, primary care physicians, and a nursing professional. The group will create materials aimed at reducing the time it takes to reach an IPF diagnosis.

Mary Beth Scholand, MD, is an associate professor of pulmonary medicine at the University of Utah in Salt Lake City. Credit: Mary Beth Scholand, MD

Mary Beth Scholand, MD, a professor of internal medicine at the University of Utah in Salt Lake City, is a member of that committee.

“This is a very severe disease with significant morbidity and mortality,” she said, adding that IPF “has no known causes and is largely uncontrollable by the patient.”

Dr. Scholand, who also directs the University of Utah’s Interstitial Lung Program, said that timely, accurate diagnosis of IPF remains a challenge “because primary-care doctors and even pulmonologists think of more common diseases first. Learning the additional signs and symptoms of interstitial lung disease is a key component of this initiative.”

Other committee members are in Alabama, Illinois, Massachusetts, Minnesota, Ohio, and Texas.

“Having experts from both pulmonary and primary care medicine as members of the steering committee is critical,” William Lago, MD, a family physician in Wooster, Ohio, said in a CHEST press release. “Patients first see their family medicine or primary care clinicians and, all too often, the most complex lung diseases present in ways that are indistinguishable from more common conditions like asthma and COPD. Bringing together experts in both fields will yield the best results in creating a path to diagnosis.”

Ball said that despite the pandemic, “there’s a silver lining, in that it’s putting a spotlight on lung disease. Everyone is aware of heart disease and cancer, but lung disease has never had that front seat on the stage. Hopefully, this will increase funding at the government level to better understand the impact of long-haul COVID on lung health, which will bring more money in.”