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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

What if My Pulmonary Fibrosis is Getting Worse?

September 16, 2016 By Dr. Jeremy Feldman

IPF symptomsThe first and most important point is that regular evaluations of your lung function and exercise capacity are important to identify if your IPF is getting worse.  Patients may feel worse for many reasons—some lung related and others in no way related to lung function.  As a result, I am a believer in regular pulmonary function testing and six minute walks.

In the patient that has not yet started treatment with one of the two FDA approved therapies (OFEV and Esbriet) I start one or the other agent.  I generally allow three to six months on one therapy to determine if the medicine is working.  Unfortunately as these medicines do not result in improvement in lung function, “working” means stability in your lung function.  At the three-month mark, I repeat a spirometry to measure the forced vital capacity (FVC) and a six-minute walk test.

If there is clear evidence of progression and the patient has been able to tolerate the full dose of the chosen medicine for the full three-month period, then we discuss changing to the other medication.  I will usually schedule a short interval follow up and repeat testing to verify that the change is sustained.  If it has taken a month or so to gradually increase the dose then I will be patient for another six weeks and repeat testing.  If at that time, there continues to be evidence of progression then I would encourage switching to the other medicine.  If all is going well then I do a full pulmonary function test and six minute walk at the six-month mark.

Combination Therapy??

Currently, there is no data supporting the use of both OFEV and Esbriet at the same time.  That does not mean that it might not be useful, just that no one has studied it.  As a result, your insurance is not likely to pay for both drugs together.  I have not yet been successful in persuading a payer to approve both drugs at once.  I hope that over the next few years we are able to study this question to better guide our treatment decisions.

Pulmonary Hypertension and IPF

In many patients with IPF, the pressure in their pulmonary arteries increases.  This can lead to increased stress on the right side of the heart.  Over time, some patients develop progressive impairment in their right heart function that leads to right heart failure.  The treatment of pulmonary hypertension in patients with IPF is a very active area of clinical research.  In our practice we have a variety of clinical trials that are ongoing for such patients.  In patients that do not qualify for research trials we selectively offer treatment for their pulmonary hypertension.

Clinical Research

The majority of clinical research studies now and looking forward will be comparing an experimental medicine to placebo but will allow treatment with either OFEV or Esbriet.  It is imperative that patients participate in clinical research studies so that we may make further progress in treating IPF.

Lung Transplantation

In patients that are getting worse, it is important to have an early discussion about lung transplantation as a treatment option.  This is not the right path for every patient and even for many patients that might be interested.  The evaluation process is rigorous and excludes patients with other advanced medical problems, obesity, inadequate supports and very advanced age.  Nonetheless, I still have a frank and open discussion about lung transplantation with all of my patients.

End of Life Planning

In patients that have very advanced disease and are not candidates for lung transplantation, it is appropriate to discuss end of life planning.  Although this topic is scary for patients and hard for doctors, an open discussion about this is important and productive.  My experience is that patients are often afraid to ask about this topic but are receptive to the discussion when offered in a supportive environment.

Filed Under: IPF Symptoms

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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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