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About
Progression
Let’s talk about progression and why it’s an important factor to consider as you explore your treatment options. For some people, interstitial lung disease (ILD) with fibrosis may be a progressive disease. This means the lung fibrosis—scarring of the lung tissue—continues to get worse over time.
While it is difficult to know if and/or how quickly lung fibrosis will advance, understanding how it progresses is key. This section takes a closer look at how an ILD with fibrosis may progress—and why slowing it down with treatment may be important.
Quick Fact: Not all types of ILD with fibrosis are progressive. Some forms are more likely to produce progressive scarring than others.
Progression Of Fibrosis
In The Lungs
For people diagnosed with ILD, it is important to understand that progressive lung fibrosis (also known as lung scarring) causes the lungs to stiffen, making it more difficult to fully fill them with air. As the lung scarring progresses, it can continue to get worse over time and can make it harder to breathe. The damage this scarring causes is believed to be irreversible.
Lung Fibrosis Simulator Tool
Watch how lung fibrosis progresses over time.
The Progression of Fibrosis in the Lungs
In chronic ILD with worsening fibrosis, the body’s repair processes overreact to
tiny injuries in the walls of the lungs’ air sacs, also called alveoli, and
surrounding tissues.
Special immune cells called myofibroblasts produce too much scar tissue, which builds up between the walls of the air sacs and the blood vessels.
The scar tissue interferes with the flow of oxygen into the blood.
As the fibrosis worsens, less and less oxygen is able to pass from the lungs into the blood.
Healthy Lungs
In normal, healthy lungs, little or no scarring is present. Oxygen enters the lungs
and flows freely from the air sacs to the bloodstream as shown here by the flow of
the blue oxygen molecules through the outer ring.
Early Disease
Early in the course of chronic ILD with worsening fibrosis, little scarring is
present. The scarring in pulmonary fibrosis usually starts in the lower half of the
lungs and progresses upward. The majority of the oxygen that enters the air sacs
passes easily into the bloodstream.
Intermediate Disease
You can clearly see that the scarring is spreading in the lungs. The scarring around
the air sacs has made it harder for the oxygen to get through to the bloodstream,
and much of it remains in the air sacs. This means that less oxygen is available for
your other organs.
Advanced Disease
For those with advanced pulmonary fibrosis, the scarring around the air sacs allows
very little oxygen to pass into the bloodstream. Much of the lungs have lost their
ability to function.
Fibrosis in the Lungs
Fibrosis causes permanent damage to the lungs, impacting how well your lungs
function. Once scarring occurs, it cannot be reversed. Scarring may spread
throughout the
lungs over time.
Progression
And Your Diagnosis
Every person is unique. For some, lung fibrosis can progress very rapidly. For others, progression may happen more slowly. In some situations, lung function can even remain the same for long periods of time. Since disease progression varies and is often difficult to predict, monitoring progression is a crucial part of managing the disease.
The scarring caused by ILD is believed to be permanent.
Consider the Following Facts
While everyone experiences decline in lung function as they age, people with idiopathic pulmonary fibrosis (IPF) lose 3 to 4 times more lung function (150 to 200 mL measured by ) than those without IPF.
For people with IPF, it is important to remember that lung fibrosis is always progressive.
It’s estimated that 18% to 32% of those diagnosed with an ILD that is not IPF can develop a form that is chronic and progressive.
Quick Fact: Slowing the rate of lung function decline can be an important part of a treatment plan. OFEV may help.
Monitoring
Lung Function Decline
It is important to see your doctor regularly to test your lung function. The sooner lung function decline is detected, the sooner you and your doctor can discuss and consider what treatment options might be right for you.
One of the ways doctors will keep track of how fibrosis is affecting lung function is by testing FVC. Here’s what you need to know about FVC:
This test is often referred to as a pulmonary or lung function test and is measured using a spirometer.
For this test, you take your deepest breath, and then blow as hard and as long as possible into the spirometer.
If your FVC is progressively decreasing over time, it may be a sign that your condition is getting worse. And—if you’re not already on treatment—it may be time to ask your doctor about beginning treatment to help slow the decline in lung function.
It’s important to remember, however, that even if your FVC isn’t decreasing, lung fibrosis may still be progressing, which is why discussing treatment options with your doctor is important.
Quick Fact: Testing FVC is just one method your doctor may use to evaluate how well your lungs are working. Other tests include: walking tests, tests that measure how well oxygen moves from your lungs to your bloodstream, and other pulmonary function tests.
How Treatment
May Help
If lung scarring progresses slowly, it may not noticeably affect lung function for some time—meaning a person with an ILD with fibrosis may not see or feel the effects of progression. Despite this, it’s important to remember the scarring may still progress. Left untreated, it may progress at a faster rate than if on treatment.
Keep fighting to inspire the ones you love. Work with your doctor to discuss treatment options. Starting treatment can be an important step to help slow disease progression.
Ask your doctor if OFEV® (nintedanib) capsules could be the right next step for you.