Treatment for Idiopathic Pulmonary Fibrosis

If you have idiopathic pulmonary fibrosis (IPF), you can do a lot of things to help you feel better. Whether you've got a hacking cough or feel short of breath, treatments can make a big difference. There's no cure, but medication and other kinds of therapy can ease your symptoms and make your life easier.

Some new drugs may help slow down the disease. They can prevent more scar tissue from forming in your lungs. Other treatments help you breathe more easily, control your cough, give you more energy, or prevent infections that could make you sicker.

The options you pick depend on your specific symptoms, overall health, and age. Talk to your doctor to figure out the best plan so you can enjoy your life and stay active.

Medicine

Two drugs may help prevent new scarring from forming in your lungs, which is the cause of your breathing troubles.

Nintedanib (Ofev) and pirfenidone (Esbriet) block a process in your body that leads to lung scarring. They may keep your IPF from getting worse and can help you breathe better.

Other medications fight the inflammation that makes you feel sick or causes scarring. Your doctor may suggest:

  • Corticosteroids like prednisone
  • Drugs that slow down your immune system, like azathioprine (Imuran), cyclophosphamide (Cytoxan), and mycophenolate mofetil (CellCept)

Therapies That Can Help

Drugs may treat your IPF scarring or symptoms, but you can do other things to breathe better and get more energy.

Oxygen therapy. It helps you feel less short of breath so you can stay active. You'll carry a small, light oxygen tank with you and breathe in through a small tube in your nose.

Pulmonary rehab. You do exercises to help your breathing and build up your strength.

Your therapist can give you tips for a healthy diet so you keep your weight and energy levels up. He can also show you how to save your energy for when you need it most.

Surgery

If your IPF scarring is severe, your doctor may suggest a lung transplant. Most people only think about doing this if other treatments aren't working.

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Most people who get lung transplants are younger than 65. But if you're older and have no other serious medical problems, it may be an option for you.

It's a major operation. There aren't many lungs available from donors, so you'll go on a list to receive one. While you wait, avoid smoking, eat a healthy diet, do your breathing exercises, and stay as active as possible.

Once you get word that a donor has been found, you may have to go to the hospital quickly for your surgery, so make sure your doctor can reach you at all times.

You won't be awake during your surgery. Doctors put tubes into your mouth and windpipe that help you breathe while it's going on.

During the operation, your surgeon removes your lung and the blood vessels that connect it to your heart. He then attaches your new lung to these blood vessels.

If you're getting two new lungs, you'll need a heart-lung bypass machine to pump oxygen into your blood during the surgery.

A single lung transplant takes 4-8 hours, and a double transplant takes 6-12 hours.

After surgery, you'll recover in the hospital for up to 3 weeks. You'll need to cough to clear fluid from your lungs. You'll also do breathing exercises to learn to take slow, deep breaths.

For 3 months after your surgery, your doctor will test your blood and lungs to make sure your body doesn't reject your new lung or you don't get any infections. You'll take medicines for the rest of your life to help prevent this from happening.

It can take weeks or months to recover. If you're thinking about getting a transplant, you'll need a lot of emotional support. Ask your doctor about support groups that have people facing the same concerns as you. Also find out about educational workshops that can explain what to expect before and after surgery.

WebMD Medical Reference Reviewed by Jennifer Robinson, MD on April 22, 2018

Sources

SOURCES:

National Heart, Lung, and Blood Institute: "How Is Idiopathic Pulmonary Fibrosis Treated," "What Is a Lung Transplant?"

American Lung Association: "Pulmonary Fibrosis: Symptoms, Diagnosis, and Treatment."

Pulmonary Fibrosis Foundation: "Treatment Options."

FDA.

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