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Home » Lung Health » How a New Therapy May Improve Quality of Life for Patients with Pulmonary Fibrosis

People living with pulmonary fibrosis, a disease where lung tissue is damaged, have a hard time breathing and getting oxygen into their blood. But a new therapy currently studied in clinical trials may help.

Treating pulmonary fibrosis

More than 200,000 Americans are estimated to be living with pulmonary fibrosis today. Each year, 50,000 new cases are diagnosed. There is no cure for the condition which is characterized by scarred lungs.

As patients with pulmonary fibrosis progress, there is an increased need for oxygen, which puts a lot of stress on a patient’s heart.

“We see patients get more short of breath, need more oxygen, and slow down in their activities over time,” says Dr. Lisa Lancaster, professor of medicine and director of the interstitial lung disease program at Vanderbilt University Medical Center.

Researchers and doctors want to both slow the disease progression and improve symptoms. Dr. Lancaster says while current fibrotic lung disease treatments slow disease progression, they don’t necessarily help patients feel better, improve their symptom of shortness of breath, or improve their endurance.

Quality of life

People with pulmonary fibrosis often need oxygen therapy to help them breathe better, stay active, and maintain their lifestyle. It is important for them to try to stay active, eat well, and reduce stress. They may benefit from breathing exercises to help their lungs stay strong and improve efficiency.

According to the American Lung Association, people with pulmonary fibrosis have an average survival rate of three to five years after diagnosis, and their quality of life is often poor.

“We’re hoping to change that and give patients the power to decide what activities they want to be engaged in and improve their quality of life — and hopefully prolong their life,” says Dr. Wassim Fares, the chief medical officer for Bellerophon Therapeutics.

An investigational therapy with nitric oxide provides hope

For years, nitric oxide has helped newborns who need oxygen in the hospital. Nitric oxide relaxes the muscles in the walls of blood vessels, resulting in increased oxygen, and blood flow in the lungs. Now, for patients with pulmonary fibrosis, INOpulse (by Bellerophon Therapeutics) is a drug-device combination therapy that’s currently being studied in clinical trials.

INOpulse utilizes a proprietary delivery system that ensures accurate nitric oxide dosing delivery independent of a patient’s breath rate. It’s lightweight, portable, and able to be used for treatment outside of the hospital. The long-term goal of the INOpulse therapy is to help improve patients’ endurance and survival, and potentially lessen the risk of heart failure, which is a complication of pulmonary disease.

Results from clinical trials to date showed that patients treated with INOpulse had a 20 percent improvement in their moderate to vigorous physical activity as compared to the placebo group. Moderate to vigorous physical activities including walking, housework, climbing stairs, and jogging, to name a few.  

INOpulse is also under evaluation in clinical trials for treating COVID-19, as well as other pulmonary conditions, such as sarcoidosis and COPD.

More information can be found at

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