Maureen
Living with CF

How CF Progresses

Cystic fibrosis (CF) feels a little different for everyone, but everyone experiences progression of CF over time, whether they feel it or not. Progression can occur when the thick mucus building up throughout the body causes inflammation and scarring in many different organs, leading to permanent damage. This damage is often present before it can even be detected by tests, so it's important to stay proactive in managing CF.

How CF Progresses

Cystic fibrosis (CF) feels a little different for everyone, but everyone experiences progression of CF over time, whether they feel it or not. Progression can occur when the thick mucus building up throughout the body causes inflammation and scarring in many different organs, leading to permanent damage. This damage is often present before it can even be detected by tests, so it's important to stay proactive in managing CF.

Long-term Impact on the Lungs

Repeated pulmonary exacerbations cause lung damage

Beginning early, the buildup of thick, sticky mucus in the lungs results in a cycle of infection, inflammation, and more mucus buildup. Pulmonary exacerbations are often a key part of this cycle.

This is especially serious because a pulmonary exacerbation means more than just a hospital stay or an extra round of antibiotics. It may cause permanent lung damage that advances disease progression.

Bronchiectasis causes loss of lung function

It’s important to promptly treat pulmonary exacerbations to prevent and slow down the progression of bronchiectasis, a permanent reshaping of the airways that causes them to become loose and scarred.

Bronchiectasis eventually affects almost all people with CF and can make it harder to clear mucus from the lungs and move air in and out of the airways. As it worsens, the lungs become more damaged, leading to a loss of lung function.

If left untreated, this cycle of infection and inflammation causes bronchiectasis, and can result in a permanent loss of lung function.

In CF, permanent lung damage may occur even before it can be detected by lung function tests.

Starting from a young age, people with CF may experience a lung function decline of 1 to 3 percentage points each year on average.

It’s never too early to talk to a healthcare provider about potential lung damage.

Part of what makes lung function decline so serious is that some people don’t always feel the change. So someone may lose a lot of lung function before they begin to feel like something is wrong.

STAY CF SMART: CF is different for everyone. But all cases of CF progress over time. The first step toward managing CF is to take a proactive approach to care.

CF fact or fiction
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Having high lung function means that CF is not progressing.
Fact
or
Fiction
CF fiction CF fact

Here’s the fact: Lung damage may be present even when lung function is high.

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