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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

FAQ’s: Enzyme Therapy, Hereditary IPF, Progression of IPF & Oxygen

January 25, 2017 By Dr. Jeremy Feldman

Your Questions Answered

Is here a role for “systemic enzyme therapy” in fibrotic lung disease?

pulmonary fibrosis supplementsThere is no high quality medical study or real science that supports the over the counter enzyme replacement products in the treatment of any lung disease including Pulmonary Fibrosis (IPF or non-specific interstitial pneumonitis).  I have written about these “supplements” and they continue to be a great source of revenue for the manufacturers but offer little by way of proven benefits for patients.  On this very topic, the national news recently announced that the makers of Prevagen, another supplement were under investigation for unsupported claims about health benefits.  As always, if it sounds too good to be true then it almost certainly is not true.  Buyer beware!  The same goes for outrageous claims about stem cell therapy.

Is IPF hereditary (Can it be passed from parent to child)?

The vast majority of patients with IPF do not have familial or heritable disease and their children are not at increased risk.  There are cases of familial IPF where many family members are affected.  These patients typically have disease onset earlier than the more common sporadic IPF.  If you are the only person in your family with IPF then it is very unlikely that you have the type of IPF that you can pass from parent to child.

How fast does IPF progress?

Many years ago we thought that patients with IPF had a slow and gradual decline in lung function.  We now know that for many patients their course may be more complex.  For example, many patients are stable for a period of time and then experience an abrupt decline.  We describe these episodes of decline as an exacerbation or flare of IPF.  Patients may then stabilize but often at a lower level of lung function.  Other patients may have periods of stability initially and then many years into their disease course experience progressive decline over a period of months.  There is no one answer to this question.  Your lung doctor will measure your lung function, your exercise capacity (walk distance) and periodically image your lungs (CT scans) to determine how fast your disease is progressing.

Do I have to have to use my oxygen?

oxygen tanks for IPFThis is a very common question.  Perhaps all patients worry about this even if they don’t ask the question.  As IPF progresses, most patients develop low oxygen levels.  This is caused by progressive scar formation in the lungs that does not participate in taking oxygen from the air we breathe and moving it into the blood flowing through the lungs.  Some patients feel very short of breath when their oxygen levels are low and other patients are not symptomatic.  Conventional medical advice is that when your oxygen level drops below 88-89% you should use your oxygen.  There have not been large high quality research studies to test the benefit of oxygen (we have also not done any high quality studies that prove that driving on flat tires is a bad idea).  Nonetheless standard practice is to prescribe oxygen when the saturations are lower than 88-89%.  Medicare and other insurers will pay for oxygen under these circumstances.  In the real world, many patients find that using their oxygen all the time leads to nasal congestion and a sore nose.  Taking brief breaks is probably ok.  The best rule is that if you feel short of breath, use your oxygen.  Don’t let vanity or denial be a barrier to feeling better and being more active.  Many of my patients are reluctant to use oxygen initially.  Over time they come to realize that breathing more easily and being less short of breath are priorities.

Filed Under: FAQ's

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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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