Idiopathic Pulmonary Fibrosis (IPF)

What Is Idiopathic Pulmonary Fibrosis (IPF)?

Idiopathic pulmonary fibrosis (IPF) is a serious lung disease. When you breathe in, oxygen moves through tiny air sacs in your lungs and into your bloodstream. From there, it travels to your organs. IPF causes scar tissue to grow inside your lungs and makes it hard to breathe. It gets worse over time.

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.

There’s no cure for IPF. For most people, symptoms don’t get better, but treatments can slow the damage to your lungs. Everyone’s outlook is different. Some people will get worse quickly, while others can live 10 years or more after diagnosis. There are treatments to help you breathe easier and manage your symptoms. You may be able to have a lung transplant.

Idiopathic Pulmonary Fibrosis Symptoms

You can have idiopathic pulmonary fibrosis 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
• Chest pain or tightness
• Leg swelling
• Loss of appetite
• Shortness of breath, especially when you walk or do other activities

Other symptoms of IPF include:

• Feeling more tired than usual
• Joint and muscle aches
• Weight loss without trying
• Clubbing, when the tips of your fingers and toes get wider

Idiopathic Pulmonary Fibrosis Causes and Risk Factors

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

Some risk factors might make you more likely to get IPF:

• Age. Almost everyone diagnosed with IPF is over 50.
• Breathing in wood or metal dust at work or home
• Gender. About 75% of people diagnosed with IPF are men.
• Genetics. Up to 20% of people who have IPF have a family member with a similar lung disease.
• Having acid reflux disease
• Smoking cigarettes

Idiopathic Pulmonary Fibrosis 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 doctor visits to get the right diagnosis. If you have trouble breathing that doesn’t get better, you’ll probably need to see a pulmonologist, a doctor who treats lung problems.

The doctor will use a stethoscope to listen to your lungs. They 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? Which ones?
• 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?

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

• Chest X-ray. It uses radiation in low doses to make images of your organs.
• 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 forehead.
• High-resolution chest CT. This powerful X-ray makes detailed pictures of your lungs. It can help find out how severe your IPF is and possibly what’s causing it.
• Biopsy. The doctor removes small pieces of your lung tissue and looks at them under a microscope. This may be done with surgery or with a flexible tube filled with a small camera that looks down your throat and into your lungs. The flexible tube approach is called a bronchoscopy. During the bronchoscopy, a doctor will 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.

Questions for Your Doctor

You might want to ask:

• How do you know I have IPF?
• Do I need 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?

Idiopathic Pulmonary Fibrosis Treatment

Treatments for idiopathic pulmonary fibrosis 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 suggest:

• Medicine. Two drugs, nintedanib (Ofev) and pirfenidone (Esbriet), are approved to treat IPF. Scientists are still finding out exactly how they work in the lungs, but they know that these treatments can slow lung scarring and damage. Corticosteroids can ease lung inflammation in some people during a flare-up of the disease.
• 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.

Lung Transplants for IPF

Some people with IPF can get a lung transplant. Doctors usually recommend it if your IPF is severe or gets worse quickly. Getting a new lung or lungs can help you live longer, but it is major surgery.

If you fit the criteria for a lung transplant, 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 lung. You’ll also have routine tests to see how well your lungs are working and regular physical therapy.

If you're thinking about a lung transplant, you'll need emotional support from family and friends. Support groups can help by putting you in touch with people who are also getting or have had transplants. 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’re safe and if they work. They often are a way for people to try new medicine not everyone can get. Your doctor can tell you if one of these trials might be good for you.

Living With Idiopathic Pulmonary Fibrosis

Idiopathic pulmonary fibrosis is a serious disease. 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.

Other things you can do to feel better include:

• 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. Eating smaller meals more often will also give your lungs more room to breathe.
• 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 suggest programs to help you quit.
• Get a flu shot. Vaccines can protect you from infections like the flu or pneumonia, which can harm your lungs. You need a flu shot every year. You’ll also need two vaccines to help protect against a serious type of pneumonia. Ask your doctor about when you should get these vaccines. Try to stay away from people who have colds.
• Find ways to relax. Low-key things that you enjoy are great ways to fight stress. Try reading, drawing, or meditating.

It can be hard to manage 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.

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 odds of getting other conditions, including:

• High blood pressure in the lungs, called pulmonary hypertension
• Heart attack
• Stroke
• Blood clots in your lungs
• Lung cancer
• Lung infections

Ask your doctor what you can do to lower your chances of having these problems. There may be treatments that can help with these conditions, too.

Everyone with IPF is different.

For some, the disease gets worse quickly. For others, it can be a slow process in which their lungs stay the same for a long time. Talk to your doctor about your condition and what you can do to manage it.

Average survival is 3 to 5 years, but some people live much longer. A lung transplant can also change your life expectancy.

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

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