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Idiopathic Pulmonary Fibrosis

Jeremy Feldman, MD

  • What is Idiopathic Pulmonary Fibrosis?
    • Idiopathic Pulmonary Fibrosis Definition
    • Symptoms of Idiopathic Pulmonary Fibrosis
    • Causes of Idiopathic Pulmonary Fibrosis
    • Who Gets Idiopathic Pulmonary Fibrosis
    • Gastro-Esophageal Reflux (GER) and Idiopathic Pulmonary Fibrosis
    • Interstitial Lung Disease vs. Idiopathic Pulmonary Fibrosis
    • Is Pulmonary Fibrosis Hereditary?
    • Research, Life Expectancy and Prognosis for Idiopathic Pulmonary Fibrosis
  • Diagnosing & Monitoring PF
    • Monitoring Progress of IPF
    • Idiopathic Pulmonary Fibrosis Diagnosis
    • Chest X-rays Used in IPF
    • CPET used in IPF
    • HRCT Scans Used to Evaluate IPF
    • Lung Biopsy To Diagnose IPF
    • Pulmonary Function Tests (PFTS)
    • Six Minute Walk Test To Monitor IPF
    • Stages of Idiopathic Pulmonary Fibrosis
    • Complications of Idiopathic Pulmonary Fibrosis
  • PF Treatment Options
    • Esbriet (pirfenidone) Used to Treat IPF
    • OFEV (nintedanib) Used to Treat IPF
    • Pulmonary Rehab for IPF Patients
    • Lung Transplantation for Pulmonary Fibrosis
    • Oxygen Used to Treat IPF
      • Update on Oxygen Use
    • Clinical Trials in Idiopathic Pulmonary Fibrosis
    • IPF & Leg Swelling
    • Pulmonary Hypertension and Idiopathic Pulmonary Fibrosis
    • IPF Exacerbation
  • PF Advocacy & News
  • FAQ’s
    • Respiratory failure, NSIP, diet
    • Leg swelling, lung biopsy, side effects & pets
    • FAQ’s Enzyme Therapy, Progression of IPF & Oxygen
    • FAQ: Diarrhea, Fibrosis Reversal, IPF Symptoms
    • FAQ: Oxygen Flow, Anesthesia & Genetics
    • FAQ: Contagious Cough? Stem Cells, Familial IPF, Flying
    • FAQ: GERD, Fatigue & Environmental Exposure
    • FAQ: How to Travel to Your Doctor’s Office with Oxygen
    • FAQ: Lung Biopsy with Pulmonary Fibrosis
    • FAQ’s: Alcohol, Clinical Trials, Pulmonary Function Testing
    • FAQs: Emphysema, Exercise & Lung Biopsy

Inhaled Nitric Oxide and Pulmonary Fibrosis

January 28, 2020 By Dr. Jeremy Feldman

Bellerophon Phase 2 Study

Research for IPF

In November, Bellerophon announced that their phase 2 study of portable inhaled nitric oxide for pulmonary fibrosis led to clinically important improvements for patients.  This is great news for the pulmonary fibrosis community.  

Patients with many different types of pulmonary fibrosis were included in the study.  All had to be using supplemental oxygen at baseline.  The study measured amount of physical activity and symptoms of breathlessness.  Patients were randomized to receive either inhaled nitric oxide by INOpulse (a two and a half-pound small device) or placebo by the device.  All patients had to use the device and their oxygen at least 12 hours per day.  

Why Are These Results Important?  

Until now, no medication has been shown to improve exercise capacity.  The 2 approved therapies (OFEV and Esbriet) were shown to slow the progression of scarring in patients with idiopathic pulmonary fibrosis.  What sets Inhaled Nitric Oxide apart is that patients were actually able to improve their amount of physical activity.  Patients felt better and could do more.  

Why might inhaled nitric oxide improve exercise capacity?  Nitric oxide is normally made by the blood vessels of the lungs.  It acts locally within the lung blood vessels to improve the matching of blood flow to areas of the lung that are getting the most oxygen.  During activity this improved blood flow matching should blunt the increased pressure within the pulmonary arteries that occurs in patients with pulmonary fibrosis during exercise.  Nitric oxide may also increase the amount of oxygen that can move from the lungs into the blood.  

The main disadvantage of the INOpulse system (nitric oxide delivery system) is that it is yet another device that needs to be carried around.  Patients already are carrying their oxygen which might weigh from two to ten pounds.  This novel device adds two and a half pounds.  On a brighter note, despite the added weight, patients in the phase 2 study still showed marked improvement in their activity levels.  Perhaps as the technology improves the device will get smaller and lighter (much as cell phones did—remember the original mobile phones that looked like a giant brick).  

Bellerophon is gearing up for a phase 3 study (large study that would hopefully lead to approval by the Food and Drug Agency.  We are excited about this study and look forward to its initiation in 2020.  

Filed Under: Research

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Idiopathic Pulmonary Fibrosis Treatment Options

What is IPF?

  • What is Idiopathic Pulmonary Fibrosis?
    • Symptoms of Idiopathic Pulmonary Fibrosis
    • Causes of Idiopathic Pulmonary Fibrosis
    • Who Gets Idiopathic Pulmonary Fibrosis
    • Gastro-Esophageal Reflux (GER) and IPF
    • Interstitial Lung Disease vs. Idiopathic Pulmonary Fibrosis
    • Is Pulmonary Fibrosis Hereditary?
    • Research, Life Expectancy & Prognosis for Pulmonary Fibrosis

Diagnosing IPF

  • Idiopathic Pulmonary Fibrosis Diagnosis
    • Chest X-rays Used in IPF
    • CPET used in IPF
    • HRCT Scans Used to Evaluate IPF
    • Lung Biopsy To Diagnose IPF
    • Pulmonary Function Tests (PFTS)
    • Six Minute Walk Test To Monitor IPF
    • Stages of Idiopathic Pulmonary Fibrosis
    • Complications of Idiopathic Pulmonary Fibrosis

Treating IPF

  • Pulmonary Fibrosis Treatment Options
    • Esbriet (pirfenidone) Used to Treat IPF
    • OFEV (nintedanib) Used to Treat IPF
    • Pulmonary Rehab for IPF Patients
    • Lung Transplantation for Pulmonary Fibrosis
    • Oxygen Used to Treat IPF
    • Clinical Trials in Idiopathic Pulmonary Fibrosis
    • IPF & Leg Swelling
    • Pulmonary Hypertension and Idiopathic Pulmonary Fibrosis
    • IPF Exacerbation

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