Pulmonary Fibrosis: How Doctors Treat You When Cause Is Unknown

5 possible treatments + risk factors

Pulmonary Fibrosis: How Doctors Treat You When Cause Is Unknown

Every day, the air you breathe contains smoke, dust and chemicals that can irritate and damage your lungs. For most people, the lungs do a good job of filtering out toxins and repairing themselves. But if you have idiopathic pulmonary fibrosis (IPF), your lungs don’t heal.

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Instead, they form scar tissue. Over time, the scar tissue builds up and makes breathing very difficult. IPF is a chronic, progressing lung disease. But you should know that you and your doctor can slow the disease down.

Pulmonologist Daniel Culver, DO, discusses the basics of IPF, steps you can take to protect your lungs and your best treatment options. 

What are risk factors of IPF?

Shortness of breath and a dry cough that lingers are the main symptoms of idiopathic pulmonary fibrosis, Dr. Culver says.

The cause of the disease is unknown (idiopathic), but certain risk factors make the disease more likely in some people.

You are at greater risk for this disease if you:

  • Are (or were) a smoker
  • Have worked in a dusty environment
  • Are Caucasian
  • Are male
  • Are over age 40

“Most commonly we see idiopathic pulmonary fibrosis in the seventh and eighth decades of life,” Dr. Culver says.

Viruses and acid reflux can also trigger the disease once it is present.

How can you slow disease progression?

IPF gets worse over time in at least 90 percent of patients, and there is no cure. However, there are steps you can take to slow things down.

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The best approaches to treating the disease focus on protecting your lungs against further damage and lightening their load, Dr. Culver says.

Try these tips to help protect your lungs and slow disease progression:

What are possible treatments?

These four treatment options can help lighten the load on your lungs. Your doctor can help decide which are right for you.

1. Breathe easier with oxygen therapy. A mask or similar device delivers oxygen to your lungs.

2. Treat your sleep apnea. If you have sleep apnea it hampers your breathing while you sleep. It can make your IPF worse.

3. Take anti-scarring medications. These drugs can help slow down the rate at which your lungs form scar tissue by about half.

4. Try pulmonary rehabilitation. The goal of a rehabilitation program is to help you learn to breathe and function as well as possible with IPF and other chronic lung diseases. The program likely will focus on diet, exercise and education.

If your doctor recommends pulmonary rehabilitation, make sure those who run the program have a lot of experience in treating lung diseases. Look for a program that treats a high volume of pulmonary patients, Dr. Culver says.

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For best results, choose a rehabilitation program that has educational and strength training components, too, he says. 

Is a lung transplant a good option?

While it’s true that IPF is one of the main reasons for a lung transplant, it’s not an easy fix, Dr. Culver says. It’s difficult to go through a lung transplant. And you often end up trading one set of problems for another.

“We only consider lung transplantation in cases of extremely advanced disease that is not responding well to other treatments,” he says.

A good candidate for a transplant must:

  • Have good heart and kidney function
  • Not be obese
  • Not have underlying connective tissue disease
  • Have a good social support system

If you have an IPF diagnosis, it’s important to take steps from now on to protect your lungs against further damage. Avoid smoke and other lung irritants as much as possible.

Focus on protecting your lungs and work with your doctor to find the treatment options that are best for you. If you do that, you will be well on your way to breathing easier and enjoying a better quality of life.