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When Shortness of Breath Is a Sign of IPF

Medically reviewed by Judith Marcin, MD on June 14, 2017Written by Ashley Marcin on December 19, 2016

Overview

Shortness of breath can be an early sign of idiopathic pulmonary fibrosis (IPF), a rare and serious lung disease that generally affects middle-aged to older adults between the ages of 50 and 70. Unfortunately, shortness of breath can also be a symptom of other chronic conditions like heart disease, asthma, and COPD.

With IPF, the small air sacs in your lungs called alveoli become thick and stiff or scarred. This also means that your lungs have difficulty moving oxygen into the bloodstream and to your organs. The scarring in the lungs often gets worse as time goes on. Breathing and oxygen delivery, as a result, become more and more impaired.

There’s no cure for IPF. The course of the disease varies greatly among individuals. Some people have a rapid worsening, some have episodes of on and off worsening, some have a slow progression, and others remain stable for years. While experts agree that the median length of survival in individuals with IPF is typically around three to five years from diagnosis, those with stable disease can live much longer. Respiratory failure is the most common cause of death in IPF, but other causes can include:

Shortness of breath

One of the earliest signs of IPF is shortness of breath. You may notice you are winded while walking down the street or going upstairs. You may have difficulty breathing while performing other physical tasks and need to take breaks to complete them. This happens because IPF causes stiffening or thickening and scarring within your lungs. As your lungs become more rigid, it’s harder for them to inflate and they can’t hold as much air.

Shortness of breath is also called dyspnea. In the later stages of the disease, shortness of breath may make it difficult to talk on the phone, eat, or even take full breaths while at rest.

Other symptoms

Coughing is another early symptom of IPF. This cough is typically dry and does not bring up any phlegm or mucus.

Other symptoms of the disease, can include:

  • abnormal breathing sounds (crackles)
  • clubbing of fingers or toes
  • fatigue
  • muscle and joint aches
  • unexplained weight loss

Experts agree that the course of IPF can be unpredictable.

When to see your doctor

If you’re experiencing shortness of breath or have any of the other signs of IPF, make an appointment with your doctor for a physical exam. They may refer you to a pulmonologist — a lung specialist who can evaluate X-rays, breathing tests, heart tests, biopsies, and blood oxygen level tests.

You may want to answer the following questions before your appointment so you can give your doctor a better picture of your medical history:

  • What are your symptoms? When did they start?
  • What is your current or previous occupation?
  • Do you have other medical conditions?
  • What medications and/or supplements are you currently taking?
  • Do you smoke? If yes, how often and for how many years?
  • Do you know of family members who have had chronic lung disease or IPF specifically?
  • Is there anything else that you think your doctor should know about your health?

Outlook and self-management

Shortness of breath may be an early sign that you have IPF. If you’re experiencing this symptom, head to your doctor for a physical exam and evaluation. Getting an accurate diagnosis as soon as possible may help you get treatments that can slow the progress of the disease and improve your quality of life.

If you’re diagnosed, there are a few things you can do to help yourself feel better:

  • If you smoke, quit. Smoking is harmful to your lungs. Your doctor can give you support to help you stop smoking or you can start your quit plan today on CDC.gov.
  • Eat a healthy diet. When breathing is difficult, you may not feel like eating, which can cause you to lose weight. Add fruits, vegetables, whole grains, low-fat or fat-free dairy, and lean meats to your daily routine. Eat smaller and more frequent meals.
  • Exercise. Though you may worry about having shortness of breath, moving your body can actually help maintain your lung function and lower stress levels.
  • Get good rest. Sleeping and taking time to rest is just as important as exercising. It can help with your energy levels and with managing stress.
  • Consider vaccinations. The pneumonia vaccine, whooping cough vaccine, and flu shots can safeguard you against respiratory infections that might make your IPF worse.
  • Take medications as directed by your doctor. While you’re at it, make sure you’re keeping up with your appointments, reporting any new or unusual symptoms, and following any other instructions your doctor has given you.

In the past, medications focused on treating inflammation. More recent medications focus on treating scarring. Pirfenidone and nintedanib, two medications approved in 2014 for the treatment of pulmonary fibrosis, are often now considered the standard of care. These medications have been shown to slow the progression of the disease as well as slow the worsening of lung function.

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