Idiopathic Pulmonary Fibrosis

What Is Idiopathic Pulmonary Fibrosis (IPF)?

Idiopathic pulmonary fibrosis (IPF) causes scar tissue to grow inside your lungs. Usually, when you breathe in, oxygen moves through tiny air sacs into your bloodstream. This oxygen-rich blood then travels back to your heart. From there, it travels to all the other organs in your body.

IPF scar tissue is thick, like the scars you get on your skin after a cut. It slows oxygen flow from your lungs to your blood, which can keep your body from working as it should. Low oxygen levels and the stiff scar tissue make it hard to breathe.

There’s no cure for IPF. The illness will have an impact on your life and your family. For most people, symptoms don’t get better, but there are new treatments that can slow the damage to your lungs. Everyone’s outlook is different. Some people will get worse quickly, while for others, the symptoms may stay the same for years. There are therapies to help you breathe easier and manage your symptoms. In some cases you may be able to have a lung transplant.

Causes

Some people get pulmonary fibrosis when they're exposed to something in their environment, like pollution, certain medicines, or an infection. But most of the time, doctors don't know what causes IPF. That's what "idiopathic" means.

You might be more likely to get IPF if you:

  • Breathe in wood or metal dust at work or home
  • Have acid reflux disease
  • Smoke cigarettes

Sometimes IPF runs in families. Doctors think broken genes could cause the disease in some people. No one knows yet which genes may be involved.

Symptoms

You can have IPF for a long time without noticing any symptoms. After many years, the scarring in your lungs gets worse, and you might have:

  • A dry, hacking cough that doesn't go away
  • Shortness of breath, especially when you walk or do other activities

You might also notice that:

  • You feel more tired than usual
  • Your joints and muscles ache
  • You've lost weight without trying
  • The tips of your fingers and toes have gotten wider, called clubbing

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Getting a Diagnosis

IPF is hard to tell apart from other lung diseases because it shares many of the same signs. It may take time and a lot of visits to the doctor to get the right diagnosis. If you have trouble breathing that doesn’t get better, you’ll need to see a pulmonologist, a doctor who treats lung problems.

The doctor will use a stethoscope to listen to your lungs. She might ask questions like:

  • How long have you been feeling this way?
  • Have you ever smoked?
  • Do you work with chemicals at your job or home? What kinds?
  • Has anyone in your family been diagnosed with IPF?
  • Do you have any other medical conditions?
  • Have you ever been told you had the Epstein-Barr virus, influenza A, hepatitis C, or HIV?

Your doctor also will give you one or more of these tests:

  • Chest X-ray. It uses radiation in low doses to make images of organs inside your body.
  • Exercise test. You walk on a treadmill or ride a stationary bike while someone checks the levels of oxygen in your blood through a probe on your fingertip or attached to your forehead.
  • High resolution CT, or computed tomography. This is a powerful X-ray that makes detailed pictures of your organs. It can help find out how severe your IPF is and possibly the cause.
  • Biopsy . The doctor removes small pieces of your lung tissue and examines them under a microscope. This may be done with surgery or with a flexible tube and small camera that looks down your throat and into your lungs. This is called bronchoscopy. Sometimes doctors use fluid to wash out your lungs and remove cells to study them. This usually happens in a hospital, and you'll be asleep for it.
  • Pulse oximetry and arterial blood gas tests. They measure how much oxygen is in your blood.
  • Spirometry. You blow as hard as you can into a mouthpiece attached to a device called a spirometer. It measures how well your lungs are working by showing how much air you can blow out.

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Questions for Your Doctor

  • How do you know I have IPF?
  • Do I need any more tests?
  • Do I need to see any other doctors?
  • What treatments might work best for me?
  • How will they make me feel?
  • Will anything help me breathe better right away?
  • Are there any clinical trials that would be good for me?
  • How often should I see you?
  • Will I need a lung transplant?
  • Will my children get IPF?

Treatment

Treatments for IPF won't cure the disease, but they can make it easier for you to breathe. Some may keep your lungs from getting worse quickly. Your doctor may recommend a few options:

  • Medicine. Two drugs, pirfenidone (Esbriet) and nintedanib (Ofev), are approved to treat IPF. Scientists are still finding out exactly how they work, but they do know these treatments slow down scarring and damage in your lungs.
  • Oxygen therapy. You breathe oxygen through a mask or prongs that go in your nose. It boosts the oxygen in your blood so you have less shortness of breath and can be more active. Whether you need to wear oxygen depends on how serious your condition is. Some people with IPF need it only when they sleep or exercise. Others need it 24 hours a day.
  • Pulmonary rehabilitation. You work with a team of doctors, nurses, and therapists on ways to manage your symptoms. You might focus on exercise, healthy eating, relaxation, stress relief, and ways to save your energy. You may visit the hospital for a rehab program or do one at home.

Some people with IPF can get a lung transplant. Doctors usually recommend it for someone whose illness is very severe or gets worse very fast. Getting a new lung or lungs can help you live longer, but it is major surgery.

Your doctor will put you on a waiting list for a lung from a donor. After your transplant, you could be in the hospital for 3 weeks or longer. You'll need to take drugs for the rest of your life that keep your body from rejecting your new organ. You’ll also have lots of tests to see how well your lungs are working and regular physical therapy.

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If you're considering a lung transplant, you'll need emotional support from family and friends. Support groups can help by putting you in touch with people who have already received transplants or are waiting to receive one. . Ask your doctor about programs that can help explain what to expect before and after the surgery.

Scientists are also studying new treatments for IPF in clinical trials. These trials test new drugs to see if they are safe and if they work. They often are a way for people to try new medicine that isn't available to everyone. Your doctor can tell you if one of these trials might be good for you.

Taking Care of Yourself

IPF is a serious disease, and it will have a big effect on your life and your loved ones. To stay as healthy as possible, follow your treatment plan and see your doctor regularly to make sure your treatment is working.

There are other things you can do to feel better:

  • Eat healthy. A well-rounded diet of fruits, vegetables, whole grains, low-fat or fat-free dairy, and lean protein is good for your body in general.
  • Exercise. Take a daily walk or bike ride. It can strengthen your lungs and reduce stress. If it’s hard to breathe when you’re active, ask your doctor about using oxygen during your workouts.
  • Quit smoking. Cigarettes and other tobacco products damage your lungs and make breathing problems worse. Your doctor can recommend programs to help you quit.
  • Get a  flu shot and pneumonia vaccine.  Vaccines can protect you from infections like the flu or pneumonia, which can harm your lungs. You need a flu shot every year. Your doctor will give you one or two doses of the pneumonia vaccine, depending on your age. Try to stay away from people who have colds.
  • Find ways to relax. Low-key activities that you enjoy are great ways to fight stress. Try reading, drawing, or meditating.

It’s hard to live with an illness like IPF. Remember that it’s OK to ask a doctor, counselor, friend, or family member for help with any stress, sadness, or anger you may feel. Support groups are good places to talk to other people who are living with IPF or a similar condition. They can give you and your family advice and understanding.

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What to Expect

The scar tissue in your lungs makes it hard for your body to get oxygen, which puts a strain on your other organs. IPF can raise your risk of getting other conditions, including:

Ask your doctor what you can do to help lower your risk for these problems. There may be treatments that can help with these conditions, too. Everyone with IPF is different. For some people, the disease gets worse quickly. For others, it can be a slow process where their lungs stay the same for a long time. Talk to your doctor about your condition and what you can do to manage it.

Getting Support

To learn more about IPF or find a support group in your area, visit the web site of the Coalition for Pulmonary Fibrosis or the Pulmonary Fibrosis Foundation.

WebMD Medical Reference Reviewed by Melinda Ratini, DO, MS on January 15, 2016

Sources

SOURCES:

American Thoracic Society: "Idiopathic Pulmonary Fibrosis (IPF)."

Cleveland Clinic: "Idiopathic Pulmonary Fibrosis."

Coalition for Pulmonary Fibrosis: "Facts About Idiopathic Pulmonary Fibrosis," "What is Pulmonary Fibrosis?"

National Heart, Lung, and Blood Institute: "How is Idiopathic Pulmonary Fibrosis Treated?" "What is Idiopathic Pulmonary Fibrosis?" "Living With Idiopathic Pulmonary Fibrosis."

Pulmonary Fibrosis Foundation: "About PF."

News release, FDA.

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