New Developments in Pulmonary Fibrosis

Fibrosis refers to the tough scar tissue that many of our organs produce as a response to injury and inflammation. Scientists estimate that fibrosis may be the direct cause behind 45 percent of all deaths in developed nations; it is implicated in multiple diseases, from lung-related diseases to kidney failure.

A new study conducted in mice by Monica Cassandras and colleagues at the UC San Francisco Cardiovascular Research Institute (CVRI), published Oct.12 2020 in Nature Cell Biology, highlights the potential of a, “novel, inhalable regenerative therapy for the treatment of idiopathic pulmonary fibrosis (IPF).” One of the main characteristics of IPF is progressive scarring of the lung tissue responsible for exchanging blood CO2 for oxygen without an identifiable cause. IPF affects 1 in every 200 adults over the age of 60 in the United States, meaning that over 200,000 people currently live with this condition. Unfortunately, there are no current cures for IPF, and there are about 50,000 new cases being diagnosed annually.

“Current therapy and pharmacologic strategies target the scarring process in IPF, but little attention has been paid to how scarring alters the natural regenerative capacity of the lung,” said Tien Peng, MD, an assistant professor in residence in the UCSF Division of Pulmonary, Allergy, and Critical Care and the principal investigator of the new study.

By examining the signaling between scar-forming cells and lung stem cells, the researchers at UCSF have identified the specific signaling proteins that mutate in the fibrotic lung. Their research has shown that if mice with lung fibrosis, “inhaled a lab-grown version of a key protein, called bone morphogen protein 4 (BMP4) it could reverse the misbehavior of their stem cells, restore normal lung regeneration, and ultimately improve lung function.”

Our own PF NOW! Virtual Support Group

With the shift into virtual communication this year due to COVID-19, it has been difficult for many individuals to be able to remain connected with their loved ones, friends, and communities. In November 2020, the Pulmonary Fibrosis Now! organization decided to start a Virtual Support Group for our followers and subscribers to create a space for them to meet and connect with others living with or caring for someone with pulmonary fibrosis. We have been meeting via Zoom on a monthly basis and have had participants from various countries join the conversation! In 2021, we hope to continue on with these groups and give our community more opportunities to share their experiences and learn from others. We would like you to know that you are not alone and we are here to support you!

Our own research study on lung fibrosis

We have much anecdotal evidence on the effect of systemic enzymes in reducing PF symptoms including cough and breathlessness and also decreasing the need for supplemental oxygen. The past year, we conducted a small study as proof of concept to collect scientific data on the effect of systemic enzyme supplements on symptoms and quality of life in people with PF. With encouraging results from this study, we have initiated a larger, long-term study to test if the supplements can reverse the fibrin built up in the lungs of people with IPF and reduce symptoms. The study is expected to be completed before the end of 2021. If successful, this would be the first of its kind natural and safe treatment for reversing lung fibrosis in IPF patients.

To connect with others who have or are impacted by PF, join our online community forum, and attend our virtual support groups on Zoom.