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How Does Idiopathic Pulmonary Fibrosis (IPF) Affect Me?

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How IPF Impacts Lung Function

  • “Lung function” refers to:
    • How well the lungs can breathe in and out and exchange gases such as oxygen
    • Oxygen is vital to the body
  • IPF reduces lung function by causing permanent scarring around parts of the lungs. This scarring keeps the lungs from breathing in and transporting as much oxygen as they used to
  • It’s important to talk to your doctor about your lung function and how it impacts your body

With IPF, once damage occurs, it cannot be undone. That is why it is important to slow your IPF from progressing. The goal of treatment is to help slow progression of the disease.


Signs and Symptoms of IPF

Recognizing common IPF symptoms can help in taking early action

Many people only learn that they have IPF after experiencing symptoms for quite some time, or even after the disease has already progressed. That’s because early symptoms of IPF often resemble those of other conditions. Some of the early signs will be noticeable to you, while others may only be detected during an examination by your doctor.

Understanding the common signs and symptoms of IPF and discussing them with your doctor can often help to detect IPF early and give you more time to work with your doctor on a treatment plan. Esbriet® (pirfenidone) does not treat the symptoms of IPF and does not reverse the scarring in your lungs.

Early symptoms of IPF

  • An ongoing cough that does not produce any phlegm
  • Feeling tired or weak
  • Having shortness of breath, even during normal daily activities
  • A doctor may also hear “Velcro®-like” crackles at the bottom of your lungs

Additional symptoms may appear as IPF progresses

  • Shortness of breath, even at rest
  • A dry cough that becomes more hacking. It may include bouts of uncontrollable coughing
  • Tiring quickly, even during light activities
  • Clubbing (enlarging) of fingertips that develops over time
  • Decreased oxygen levels in the blood

Keep in mind that each person responds to IPF differently and may experience some or all of these signs and symptoms.


How Your Doctor May Test Lung Function

There are a few different types of tests that your doctor might do to check how your IPF is progressing. They include

Pulmonary function tests
Pulmonary function tests

There are tests that measure lung function. Your doctor can give you more information. One test your doctor may do is a forced vital capacity (FVC) test:

  • FVC is a test that measures the amount of air you can exhale with force after you inhale as deeply as possible. It measures how well the lungs work
  • A device called a spirometer measures FVC. You take in a big breath and blow out as hard as you can to generate a reading
  • Your treatment team may check your FVC at every visit. If your FVC measurement goes down over time, it may mean that lung function is decreasing and IPF may be progressing
  • Remember that no matter what your test results say, both you and your healthcare team want what’s best for your health. You’re both working toward the same goal
Exercise tests
Exercise tests

Your doctor may have you do physical activities. For example, one type of test looks at how far you can walk in a set amount of time. Another test checks your oxygen saturation while you walk. A third one measures how quickly you recover after walking.

Lung scans
Lung scans

There are scans your doctor can do to see what is happening inside your lungs. One type of scan is called an HRCT scan.

Since everyone is different, your doctor will decide which tests are best for measuring how your IPF is progressing. Ask your doctor about which tests may be right for you.

Take action now and talk to your doctor.

  • Discuss ways to manage your IPF symptoms and to support a healthy lifestyle
  • Make a plan for how to treat IPF and discuss ways to hopefully slow the progression of IPF

Questions To Ask Your Doctor

Take an active role in managing your condition.

Select Important Safety Information and Indication

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.
  • severe skin reactions. Call your doctor right away if you have a severe skin reaction such as skin blisters, rash, sores in the mouth, hives or any other severe skin symptoms.
  • 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 Legacy Pharma Inc. SEZC at 1-800-727-7151.

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