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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: N-Acetyl Cyteine, Air Travel & GI Side Effects with IPF

June 16, 2016 By Dr. Jeremy Feldman

Answering Your Questions

Why does this site not endorse the use of N-Acetyl Cyteine (NAC) for IPF?

The PANTHER study conclusively showed that there is no benefit to the use of this medication.  The risks of NAC are minimal but we are not in a position to endorse medications that are shown not to be beneficial in high quality studies.  NAC has a long track record of being safe so if I had a patient that had a strong desire to use the medication I would just emphasize that they are not treating their IPF with the supplement as best we can tell.  Our recommendations are in line with the American Thoracic Society’s guidelines as well.

How can patients with IPF that require oxygen travel by plane?

Traveling with IPFI have a variety of posts on this topic on this site and on pulmonaryhypertensionRN.com.  The following link has more details—Traveling with IPF.  The short answer is that the first step is to discuss the safety of traveling by air with your lung doctor.  If your doctor thinks it is safe, then you will need to coordinate oxygen.

You will need a portable concentrator to travel by plane.  If you don’t have one then you will need to speak with your oxygen provider.  You should plan to have enough battery power for your portable concentrator for about 2-3 times the estimated duration of your flight.  Your oxygen provider should furnish extra batteries (which are quite heavy).  Make sure that your batteries are fully charged prior to getting to the airport.  When you arrive at the airport, plug your portable concentrator into a power outlet so that you are not wasting battery power before you takeoff.  Be sure to leave extra time to get through security.  Plan on having a wheelchair assist through the airport.  It is easy to walk more than a half a mile through many airports and there is no reason to start your travels exhausted and short-of breath.

What can be done to help with gastrointestinal side effects from OFEV and Esbriet?

GER may contribute to IPFThis is a very important question.  Both FDA approved therapies for IPF have GI side effects (diarrhea) as one of the most common challenges.  Both medications should be taken with food.  Adding fiber is a good first step.  Bran or Metamucil are good sources of fiber. This serves to bulk up the stool.  Next step is Imodium, an over the counter anti-diarrheal.  My practice is to limit Imodium to no more than 5 tabs per day.

For OFEV, if the symptoms persist, I would stop the medicine for a few days and resume at the 100mg once daily dose for a week, then if tolerated increase to 100mg twice daily.  If severe diarrhea persists then I would stop the medication and consider trying Esbriet.  For Esbriet, I would reduce the number of pills per dose by one and see if that helps the diarrhea.  If severe diarrhea persists despite the above interventions then I would stop the medication and consider trying OFEV.  Occasionally we will use a medication called diphenoxylate-atropine (Lomotil) to help with difficult to control diarrhea.

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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