Ontario’s Firestone Institute for Respiratory Health (FIRH) Improves Treatment Options for Pulmonary Fibrosis

30,000 Canadians suffer from pulmonary fibrosis, a chronic respiratory disease that creates scarring in lung tissue and, over time, results in shortness of breath and deprives vital organs of necessary oxygen. While there is currently no known cure, ground-breaking research completed at the Firestone Institute for Respiratory Health (FIRH), part of St. Joseph’s Healthcare Hamilton, is providing new hope for patients with pulmonary fibrosis.

Driving the future of pulmonary fibrosis care through clinical trials

FIRH is driving the future of pulmonary fibrosis care through its participation in an international study that trialed a pharmaceutical treatment for patients with pulmonary fibrosis. The results of this clinical trial led by Dr. Martin Kolb, Respirologist at St. Joseph’s and Professor of Medicine at McMaster University, were published in the New England Journal of Medicine in October 2019.

There are many different types of pulmonary fibrosis with different etiologies, treatments and prognoses. For example, Nintedanib is a pharmaceutical treatment approved for use in patients with Idiopathic Pulmonary Fibrosis (IPF). However, only about a quarter of all pulmonary fibrosis cases are IPF, and the other three quarters of patients do not have an approved pharmaceutical treatment. This clinical trial tested the use of Nintedanib for patients with progressive non-idiopathic pulmonary fibrosis.

The trial had positive results and found that the medication worked to slow down the progression of the disease. “The data clearly shows the medication from this trial works,” remarked Dr. Kolb. For the first time, patients with non-idiopathic pulmonary fibrosis have hope that the scarring of their lungs can be slowed down. “It is a profoundly positive trial,” Dr. Kolb stated.

Dr. Kolb believes the results of this trial are “triggering a fundamental change in how pulmonary fibrosis is managed.” Medicine in the field of pulmonary fibrosis traditionally put extreme emphasis on making a distinct diagnosis to “give a name” to the specific disease that the patient has, and neglected to look at how the disease behaves. And therefore drug approval for pulmonary fibrosis has to date been tied to disease name, instead of behavior.

Dr. Kolb used cancer treatment as a comparison, which is based on behaviour. “Cancer, regardless of whether it is breast, lung or colon, are all treated with drugs that kill cells that proliferate quickly, because this is a common denominator of all cancers,” said Dr. Kolb. “The results of this trial show that pulmonary fibrosis can also be treated based on disease behavior”, said Dr. Kolb.

Making an impact in patient’s lives

“This study addressed a huge medical need for patients with pulmonary fibrosis who didn’t have many treatment options,” said Dr. Kolb. One of the participants in this trial was Melissa Sulpher, a mother of four who was referred to FIRH during pregnancy. “I’m not over exaggerating when I say the opportunity to participate in research like this has changed my life,” remarked Melissa. “It’s not a miracle cure, but it still feels a bit like a miracle if this can stop the progression, so being in the trial really has made a big difference for me and my family.” Read more about Melissa’s experience participating in this trial in our #TalkClinicalTrials blog series .

Partnering with industry to make it happen

FIRH usually has three or four clinical trials for pulmonary fibrosis occurring at once. These trials are labour and resource intensive. “For a disease group like this we usually recruit between 3 and 6 patients per trial. And these trials are pretty labour intensive. Study visits are often 3 to 4 hours,” said Dr. Kolb.

This labour intensive pulmonary fibrosis trial was made possible by Boehringer Ingelheim (BI), a global pharmaceutical company that has its Canadian headquarters in Burlington, Ontario. Following the positive results of the clinical trial, BI applied for Health Canada approval, which is now under priority review. According to Dr. Kolb, Ontario is a “great performer in the clinical trials field,” which leads to industry investment from companies like BI.

Firestone Institute for Respiratory Health

This study for pulmonary fibrosis is the type of research that makes the Firestone Institute for Respiratory Health a world leader in the understanding and treatment of life-threatening lung diseases. FIRH conducts research to increase understanding of respiratory health and disease through collaborative basic and clinical investigations with the expectation of improving patient care. Beyond pulmonary fibrosis, FIRH is known internationally for research on complex airway diseases such as asthma, COPD, and others.

For over 40 years, collaboration between St. Joseph’s Healthcare Hamilton and McMaster University through the FIRH has led to research discoveries that have translated into new treatments for patients around the world. In June 2018, FIRH had four publications in the New England Journal of Medicine. Other major accomplishments of FIRH include the invention of the Aerochamber, a device that has revolutionized the use of inhalers, and the development of the only conclusive non-invasive test for asthma, called the sputum test.


Learn more about the Firestone Institute for Respiratory Health here. Interested in conducting your next clinical trial in Ontario? Visit www.ctontario.ca.

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