Doctor Discussion Guide: Could I Have IPF?

Medically reviewed by Elaine K. Luo, MD on February 23, 2017Written by Colleen Story

idiopathic pulmonary fibrosis

It’s not unusual to have a dry cough now and then. Many people develop one during the cold winter months. Some cough in response to allergens. A cough can also hang on after you’ve nearly beaten the common cold. In rare cases, though, a dry cough can signal something more serious.

If you’ve had a dry cough for a month or longer, make an appointment with your doctor. If the cough is accompanied by chest pain or shortness of breath, your doctor will probably want to check your lungs.

A number of conditions could cause these symptoms. One of them is idiopathic pulmonary fibrosis (IPF). Though there is no cure for IPF, early detection can lead to treatments that slow its progression. Following are 10 questions to ask your doctor if you’re concerned you might have this condition.

1. What is IPF?

Idiopathic pulmonary fibrosis is a disease that causes scarring inside your lungs. “Idiopathic” means that the cause isn’t known.

In some cases, doctors can find out what’s causing the problem. Some medications are linked to IPF. Exposure to environmental pollutants like heavy metal dust can also increase risk of the disease. But in most cases, the cause remains unknown.

The disease attacks the tissue and space between the air sacs in your lungs, called the interstitium. As this area develops more scar tissue, your lungs don’t work as well as they should. A hard, stiff wall develops around your air sacs. This wall makes it difficult for oxygen to pass through easily.

2. Do I have the symptoms of IPF?

When the disease first starts, you may experience no symptoms at all. As it progresses and the scarring spreads, symptoms begin to appear. They may include the following:

  • dry, hacking cough
  • coughing fits
  • shortness of breath, especially during activity or exercise
  • chest pain
  • unexpected weight loss
  • rapid, shallow breathing
  • constant fatigue
  • muscle aches
  • joint pain
  • fingers and toes that get wider and rounder at the tips (called clubbing)

As IPF progresses, you’ll experience shortness of breath.

3. Do my symptoms mean I have IPF?

You can have some or all of these symptoms listed and still not have IPF. This is a rare lung disease. Other conditions such as pneumonia, high blood pressure in your lungs, and heart disease could be to blame.

Your doctor will start with a full medical examination, and then ask you some questions. Those who are eventually diagnosed with IPF usually share some risk factors. These include:

  • a family history of the disease
  • cigarette smoking
  • certain viral infections, including the Epstein-Barr virus (which causes mononucleosis), hepatitis C virus, HIV, and herpes virus 6
  • exposure to asbestos
  • autoimmune diseases, like systemic lupus erythematosus and rheumatoid arthritis
  • radiation treatments for cancer
  • gastroesophageal reflux disease (GERD)
  • being over the age of 50
  • being male

Smoking is an important risk factor. In a study published in the , researchers noted that up to 75 percent of people with IPF are current or former smokers.

GERD can also put you at risk. have found that people who had severe GERD were more likely to have IPF than those who didn’t have it. This was the case even in people who didn’t have symptoms of IPF. The states that 9 out of 10 people who have IPF also have GERD.

Having these risk factors doesn’t mean you have IPF. They simply increase your risk for the disease. Your doctor will conduct a number of tests to make a correct diagnosis.

4. How will you form a diagnosis?

It takes time to diagnose IPF. Since the symptoms can be caused by other conditions, your doctor will have to do some detective work. Your regular family doctor may refer you to a lung specialist called a pulmonologist for additional tests.

After asking questions about your lifestyle and your risk factors, the doctor will listen to your lungs. Other tests may include:

  • chest X-ray
  • high-resolution CT scan to better see inside your lungs
  • exercise test to check the levels of oxygen in your blood
  • spirometry, which involves blowing hard into a device that measures how much air you exhaled

If these tests don’t reveal your condition, your doctor may recommend a biopsy. In a biopsy, your doctor takes a small sample of your lung tissue to be examined by a lab.

5. If I have IPF, what does that mean?

It can be frightening to get a diagnosis of IPF. Try to realize that everyone is different. The disease progresses quickly in some people, but much more slowly in others. Nevertheless, you’ll probably experience some difficult emotions. Give yourself time to work through those.

There is no cure for IPF. Your symptoms are likely to get worse eventually. Treatments are available, though, that can help manage the disease. You can also make lifestyle changes that will help you to stay active.

6. What treatments are available?

Treatments help to relieve your symptoms and slow the progression of IPF. Some of the treatment options include:

  • Medications: The FDA has approved two medications that can help slow the progression of IPF: nintedanib (Ofev) and pirfenidone (Esbriet).
  • Oxygen therapy: If the oxygen in your blood is low, your doctor may prescribe supplemental oxygen to help.
  • Anti-acid therapy: If you have GERD, treating it can help ease shortness of breath.
  • Pulmonary rehabilitation: This is a training program that helps you learn new breathing techniques, and may also provide psychological and social support.
  • Surgery: Your doctor may recommend a lung transplant if you have a severe form of IPF. It’s usually a last resort.

If your doctor prescribes medications, ask about any potential side effects.

Be sure to ask about pulmonary rehabilitation. These classes can teach you more about your disease, and offer you a chance to share with other people who’ve also been diagnosed.

7. What other symptoms might I experience?

It’s important to ask your doctor what to expect. Your symptoms may be mild to begin with, for instance, but get worse with time. Understanding what may come can help you feel more in control.

For example, you may start to notice more shortness of breath, chest pain, or longer periods of fatigue. Nutritional therapy and careful exercise programs can help with these. Be sure to keep in touch with your doctor as your symptoms change.

8. What complications should I watch for?

Remember that you may go for years without getting worse. Eventually, your lungs will have a more difficult time doing their job. Complications your doctor will watch for include:

  • pulmonary hypertension, or high blood pressure in your lungs
  • heart failure
  • respiratory failure
  • blood clots in your lungs
  • lung infections
  • lung cancer

These are only possible complications. With careful care, you and your doctor can reduce your risk of these conditions.

9. What is the outlook for IPF?

The outlook for people with IPF has improved. Whereas the prognosis once meant a possible survival of two to five years, between 2011 and 2014 significant improvements were made that increased that outlook.

New results have increased understanding of the disease. That’s given people with IPF new hope. New medications have also increased the possibility of extending survival rates.

10. What can I do to feel better?

You can make a number of lifestyle changes to ease your symptoms and improve your quality of life. These include the following:

  • If you smoke, try to quit.
  • Stay as active as possible.
  • Eat a healthy diet with lots of fruits and vegetables.
  • Eat smaller meals more often.
  • Try meditation, yoga, tai chi, and other techniques that foster relaxation and deep breathing.
  • Attend individual or group therapy to deal with the emotional challenges of the disease.
  • Stay up to date with your flu and pneumonia shots.
  • Avoid secondhand smoke.
  • Do your best to avoid catching a cold or virus.

Finally, ask your doctor about possibly participating in a clinical trial. If there is one you could take part in, you could gain access to new treatments before they are widely available.

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