Understanding Pulmonary Fibrosis
This video was created to help you better understand your disease. The more you know about your disease, the more control you can take over your health.
Based on the specific diagnosis you received, you may be wondering: What does this mean and how will it affect me?
Interstitial lung diseases, or ILDs, are a group of over 200 diseases, some of which can cause scarring, or fibrosis, in the lungs.
When ILD causes fibrosis of the lungs, it is often referred to as pulmonary fibrosis.
ILDs are considered rare diseases, which is why you may not have heard of them before.
Here are just some of the more common ILDs. Certain types of ILDs are prone to being chronic and progressive. This means that the scarring in the lungs can get worse over time.
Idiopathic pulmonary fibrosis, or IPF, is the most common type of chronic and progressive ILD.
So how does a chronic ILD with fibrosis affect the lungs?
First, it helps to understand how healthy lungs work.
The main role of the lungs is to help supply the body with the oxygen it needs.
When you inhale, oxygen-rich air travels through the nose and mouth...then into the lungs, until it reaches the alveoli, the tiny air sacs of the lungs.
Oxygen molecules pass through the very thin walls of the alveoli and into the bloodstream. The oxygen in the blood is then delivered to tissues throughout your body.
A chronic ILD with fibrosis first develops when scarring occurs in the interstitium–the layer of tissue between the alveoli and blood vessels of the lungs.
At this early stage, there is not much scarring, so a majority of oxygen molecules can pass from the alveoli into the bloodstream. Oxygen levels may be only a little lower than normal.
Over time, the disease will reach an advanced stage in which the walls of the alveoli may become so scarred...that very little oxygen can pass through and get into the bloodstream.
Oxygen levels can become very low and you may need supplemental oxygen to help with your breathing.
There is important information you should know about acute exacerbations.
Acute exacerbations are most commonly recognized in patients with IPF, but can also occur in other types of ILDs.
An acute exacerbation is a serious and sudden worsening of lung function that is associated with disease progression.
Acute exacerbations may require medical attention and, in some cases, hospitalization. So it’s important to know the signs and symptoms to look out for.
Signs and symptoms that point to an acute exacerbation include:
More difficulty breathing on your own…
A worsening cough…
Or fever and flu-like symptoms.
Your oxygen levels may also become dangerously low.
If you ever suddenly start to feel worse than usual, or your symptoms quickly become severe, it is important to seek medical help right away.
If you notice your symptoms of shortness of breath and dry cough are getting worse over time, it’s important to see your healthcare provider...as it could be a sign that your disease is progressing.
In addition to shortness of breath and chronic dry cough, let your healthcare provider know if you experience other worsening symptoms, including more fatigue than usual, chest discomfort, and enlarged and rounded fingertips, called clubbing.
Your healthcare provider will listen to your breath sounds…and may hear dry, inspiratory crackles at the base of your lungs when you breathe in.
Your doctor may order pulmonary function tests, or PFTs–the same tests used to help diagnose ILD–to determine how well your lungs are working and to monitor your disease progression. The test results appear as a pattern of waves, as shown here, that form as you breathe in and out.
Your doctor may also order a high-resolution computed tomography scan, or HRCT, to help monitor disease progression. This may have been the same test used to confirm your ILD diagnosis. HRCT is a main tool for identifying a chronic ILD with fibrosis because it shows detailed, specific features of ILD.
Your healthcare provider may order PFTs, HRCTs, and other tests periodically to monitor your disease progression and use the results to determine how your condition is affecting your health.
It’s important to take an active role in monitoring your symptoms since living with a chronic ILD with fibrosis is not easy. You do not have to do this alone. Your healthcare team members can be your best allies. They understand you and your condition and they can recommend help based on your individual needs. Be sure to ask your healthcare provider if there is more you can do about your chronic ILD with fibrosis.
You can also call Lungs&You at 1-844-473-2638 if you have questions about what you’re going through. You can also visit lungsandyou.com and follow Lungs&You on Facebook. Lungs&You does not provide medical advice. Please consult with your healthcare provider if you have specific questions about your health.
Support is available. Depending on your condition, you can find information about pulmonary fibrosis and ILDs associated with autoimmune diseases, including the following.
Thank you for watching this video. We hope it has provided you with a greater understanding of chronic ILDs with fibrosis and how you can take an active role in monitoring your condition.
Watch this informative video to learn about interstitial lung disease (ILD), particularly idiopathic pulmonary fibrosis (IPF)—a common type of ILD—its symptoms, how it affects the body, how it can be managed, and types of support available.