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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

Esbriet (pirfenidone) Used to Treat IPF

Esbriet (pirfenidone) Approved for Idiopathic Pulmonary Fibrosis

Esbriet (pirfenidone) is one of two medications approved by the FDA (Food and Drug Administration) in October of 2014 to treat idiopathic pulmonary fibrosis. It’s mechanism of Esbriet approved by FDA for Idiopathic Pulmonary Fibrosis action remains somewhat poorly understood. It is described as an antifibrotic agent that acts on multiple pathways that may be involved in scarring of the lung tissue.  Simply put, it turns down the process of scar formation in the lungs.

FDA Approval of Esbriet (pirfenidone)

The FDA approved Esbriet (perfenidone) after reviewing three large clinical trials, ASCEND, CAPACITY I, and CAPACITY II, which were completed over the past decade. The data shows that patients taking Esbriet (perfenindone) had less decline in lung function over time than those patients taking a placebo. The studies specifically compared the forced vital capacities (the amount of air that can be forcibly exhaled from the lungs after taking the deepest breath possible) of patients on medication versus those on a placebo.  FVC (forced vital capacity) is measured via spiromety. Furthermore, when the three studies are viewed together, the medicine improved overall survival.

Esbriet bottleHow Is Esbriet (pirfenidone) Taken?

Esbriet (pirfenidone) is a capsule that should be taken by mouth with food three times a day. Patients start taking one pill (267mg) three times a day and then a week later they increase to two pills three times a day, and a week later they increase to the final dose of three pills (801mg) three times a day. It is important to follow the prescribed dose escalation.  If multiple doses of medication are missed it may be necessary to restart at a lower dose.  Make sure to speak with the prescribing physician prior to stopping or restarting Esbriet (pirfenidone).

Possible Side Effects From Esbriet (pirfenidone)

The most common side effects from Esbriet (pirfenidone) are gastrointestinal. Stomach upset was the most common side effect reported during Sun Precautions for Idiopathic Pulmonary Fibrosis patientsclinical trials. Other side effects that were reported include diarrhea, headache, dizziness, tired feeling, weight loss, cold symptoms such as stuffy nose, sneezing, sore throat, insomnia and joint pain.  The drug makes patients very sensitive to sun exposure. Patients must use SPF 50 sun block, hats and long sleeves if they are in direct sunlight. Failure to take these precautions can lead to an impressive rash in sun-exposed areas. Lastly, patients should have monthly blood tests to monitor their liver function for the first six months then quarterly there after as an elevation in liver enzymes has been documented in some patients taking Esbriet (pirfenidone).

How To Get Started on Esbriet (pirfenidone)

Esbriet (pirfendione) is an expensive specialty medication.  A prescription may cost up to $96,000 per year or $8,000 per month.  For this reason the medication is only available through a network of specialty pharmacies. Once you and your doctor have decided that Esbriet (pirfenidone) is the appropriate medication to treat your IPF your physician will complete an enrollment form and you will be asked to sign the patient authorization form.  Once the enrollment form is submitted to Esbriet CareConnect a nurse case manager from this program will contact the patient.  The nurse case manager will review insurance coverage and available financial assistance programs to reduce the cost of this expensive therapy.  They will then send the prescription on to a specialty pharmacy that will mail the prescription to the patients home.  It is important to return phone calls from Esbriet CareConnect and specialty pharmacies to avoid a delay in starting treatment.  If the out of pocket cost of this medication is prohibitive contact the prescribing physician’s office immediately as they will know of assistance programs or foundations to apply to for help.

 

 

 

 

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IPF patient with doctor

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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