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WHAT IS IDIOPATHIC PULMONARY FIBROSIS (IPF)?

IPF is a specific type of interstitial lung disease (ILD), or diseases that cause inflammation and scarring of the lungs. 

  • The I in IPF stands for "idiopathic," which means the cause of IPF is unknown
  • "Pulmonary" is another word for lungs
  • "Fibrosis" refers to scarring

Know the characteristics of idiopathic pulmonary fibrosis (IPF)

IPF is progressive. This means that the scarring caused by IPF will continue to happen, and lung function will continue to get worse

IPF is unpredictable. It is difficult to know how slowly or quickly IPF will progress. Each person's experience is different

IPF is irreversible. Once there is scarring in the lungs, it cannot be reversed. Today, there is no cure for IPF, but there are treatment options that may slow its progression

Ask your doctor about the progression of IPF over time

Progression of Idiopathic Pulmonary Fibrosis over time

IPF is progressive, which means that over time the scarring becomes more widespread and lung function declines. IPF progresses rapidly for some people and slowly for others


How IPF affects the lungs

IPF is scarring of tissue in your lungs

Impact of IPF on lungs

This scarring, or fibrosis, may be the result of tiny injuries deep in your lungs that do not heal properly.

Impact of IPF on alveoli

These injuries lead to progressive scarring of tissue in the small air sacs in your lungs (called alveoli). This scarring, or fibrosis, causes the lungs to stiffen.

Impact of IPF on oxygen exchange

Fibrosis may damage the  lungs' ability to exchange gases such as oxygen with the blood.

Did you know that the average lung function loss in adults without IPF is 30 mL/year? But in idiopathic pulmonary fibrosis (IPF), the progressive scarring in the lung can lead to much greater annual loss.


IPF permanently damages lung function

Currently, there is no cure for IPF, but there are treatment options that may help delay disease progression.

Patient Ambassador Janet

I want people to realize that IPF is just a disease. The diagnosis is not the end; it is the beginning-the beginning of personal development, potential, and change."

Janet
Esbriet Ambassador

Learn about lung function

IPF MAKES LUNG FUNCTION WORSE

Learn more about how IPF damages the lungs.

Esbriet® (pirfenidone) capsules 267 mg

A TREATMENT OPTION FOR IPF

Preserving more of your lung function by slowing disease progression is essential, and Esbriet may help.

About Esbriet

Esbriet® (pirfenidone) is a prescription medicine used to treat people with a lung disease called idiopathic pulmonary fibrosis (IPF).

It is not known if Esbriet is safe and effective in children.

Before you take Esbriet, tell your doctor if you:
  • have other medical conditions (particularly liver or kidney problems).
  • are a smoker.
  • are or plan to become pregnant or breastfeed (Esbriet has not been studied in these patients).
  • are taking any prescription or over-the-counter medicines, vitamins, or herbal supplements.
What are the possible side effects of Esbriet?

Esbriet may cause serious side effects, including:

  • liver problems. Call your doctor if you have symptoms such as yellowing of your skin or eyes, dark or brown urine, pain on the upper right side of your stomach area, bleeding or bruising more easily than normal, or increased fatigue. Your doctor will also do regular blood tests to check your liver.
  • sun sensitivity and rash. When you are outside, use sunscreen (SPF 50) and wear a hat and clothes that cover your skin to avoid getting a sunburn.
  • stomach problems. Esbriet may cause stomach problems such as nausea, vomiting, diarrhea, indigestion, heartburn, and stomach pain.

Your doctor may change your dose or discontinue Esbriet if side effects do not go away.

The most common side effects of Esbriet include upper respiratory tract infections, feeling tired, headache, dizziness, loss of appetite, sinusitis, insomnia, or weight loss.

These are not all the possible side effects of Esbriet.

What should you avoid while taking Esbriet?
  • Direct exposure to sunlight, or light from sunlamps and tanning beds.
  • Other medicines that can make your skin sensitive to sunlight.
  • Smoking, which may affect how well Esbriet works.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088 or www.fda.gov/medwatch or to Genentech at 1-888-835-2555.

Please see full Prescribing Information, including Patient Information, for additional important safety information.

    • Centers for Medicare & Medicaid Services. Advance notice of methodological changes for calendar year (CY) 2018 for Medicare Advantage (MA) capitation rates, Part C and Part D payment policies and 2018 call letter. https://www.cms.gov/Medicare/Health-Plans/MedicareAdvtgSpecRateStats/Downloads/Advance2018.pdf. Posted February 1, 2017. Accessed October 17, 2017.

      Centers for Medicare & Medicaid Services. Advance notice of methodological changes for calendar year (CY) 2018 for Medicare Advantage (MA) capitation rates, Part C and Part D payment policies and 2018 call letter. https://www.cms.gov/Medicare/Health-Plans/MedicareAdvtgSpecRateStats/Downloads/Advance2018.pdf. Posted February 1, 2017. Accessed October 17, 2017.