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Idiopathic Pulmonary Fibrosis Disease: Understanding Your Diagnosis

What is idiopathic pulmonary fibrosis?

Fibrosis is the buildup of scar tissue. Pulmonary fibrosis is the formation of such scar tissue in the lungs, between the air sacs. The term “idiopathic” means that your doctors aren’t sure exactly what is causing it.

To put it all together, idiopathic pulmonary fibrosis (IPF) is when the tissue in the lungs becomes damaged or scarred, and the cause may be unknown. This thickened, sometimes stiff scar tissue can make it difficult for your lungs to work properly, leading to reduced oxygen levels in your bloodstream.

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What are the symptoms?

Because your lungs aren’t able to work as efficiently, you may feel shortness of breath. This tends to worsen as the disease progresses. You may also experience other symptoms, including:

  • fatigue
  • dry, hacking cough
  • muscle or joint pain
  • sudden or unexplained weight loss
  • clubbing, or when the fingertips and nails become enlarged or rounded

How did I get it?

Because it’s idiopathic, your doctors aren’t sure how you developed IPF. Many clinicians believe that something either inside or outside of the lungs attacks the lung tissue. The following are some of the possible causes of these attacks.

Radiation therapy

Sometimes damage can occur in the lungs of people who received radiation treatments for breast or lung cancer.

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Certain medications

A handful of medications have been shown to increase the risk for pulmonary fibrosis. These include:

Environmental toxins

Exposure to certain types of pollutants, especially over a long period of time, may contribute to IPF. Some of these pollutants are:

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  • asbestos
  • silica and metal dust
  • bird droppings and animal dander
  • dust from grains
  • regular household dust and the various bacteria and other matter it contains
  • cigarette smoke
  • fumes of various types

Medical conditions

Medical conditions that can damage the lungs increase the risk for IPF include:

Genetics

IPF may run in the family. For this reason, it’s thought that genetics may play a role, at least for some people who develop pulmonary fibrosis.

What can I expect?

IPF is a serious and progressive disease, with no known cure. You should talk to your doctor so that you can understand your risk and be prepared for complications, such as:

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  • pulmonary hypertension (high blood pressure in the lungs)
  • stroke
  • pulmonary embolism (blood clots in the lungs)
  • heart attack
  • respiratory failure
  • heart failure in the right ventricle, because of how hard the heart is working to pump blood through the damaged arteries in the lungs

What treatments are available?

Unfortunately, few treatment options currently exist. The available treatments vary in effectiveness. Your doctor may recommend a combination of treatments, such as:

  • medications like corticosteroids and antibiotics
  • oxygen therapy
  • pulmonary rehabilitation
  • flu and/or pneumonia vaccines
  • lung transplant, usually as a last resort

Two new treatment options available include pirfenidone and nintedanib. Both medications are believed to decrease the buildup of scar tissue in the lungs and to slow the progression of the disease. One trial found that the disease slowed in progression in people who took 150mg of nintedanib twice a day.

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How can I improve my quality of life?

To make living with IPF a bit easier, it’s important to keep yourself as healthy as possible. Here are some general tips to keep in mind:

  • Stop smoking. Quitting can help you cut down on further damage to your lungs.
  • Eat a nutritious diet. You may find that eating becomes more difficult as your breathing gets harder. Eating smaller meals more frequently can make breathing easier.
  • Stay active. Ask your doctor about moderate exercise that can help you stay healthy. You may need to use an oxygen tank while exercising.
  • Get adequate rest.
  • Stay away from places at high altitudes, if possible, and avoid traveling by airplane.

Look for support groups in your area, to find others you can talk to and share tips with. Check with the Pulmonary Fibrosis Association, and the American Lung Association.

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