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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 understand how progression impacts many different parts of the body over time.

Cycle of Lung Damage

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.

The cycle of pulmonary exacerbations often leads to a condition called bronchiectasis. This is a permanent reshaping of the airways that causes them to become loose and scarred. It can make it harder to clear mucus from the lungs and for the airways to move air in and out as a person breathes.

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.

A change in lung function can't always be felt

Part of what makes this decline so serious is that some people don’t feel the change. So it often isn’t until someone has lost a lot of lung function that he or she begins to feel like something is wrong.

Disease progression is universal

CF is different for everyone. But all cases of CF progress over time. Taking a proactive approach to managing CF is the first step toward managing disease.

CF Fact or Fiction CF Fiction CF Fact or Fiction CF Fiction

Having high lung function means that CF is not progressing.

Here's the truth:
Lung function tests don't always detect early lung damage, so lung damage may be present even when lung function is high.

FACT or FICTION

Long-term Digestive Impact

Over time, scarring may result in other conditions

As someone with CF grows up, the same mucus that caused digestive problems in childhood may have caused scarring in different digestive organs. This scarring can result in the development of other conditions.

The pancreas: cystic fibrosis–related diabetes (CFRD)

Damage caused by thick digestive juices can lead to scarring that makes it more difficult for the pancreas to do its job. As this scarring builds up, many people with CF develop CFRD. This is when the pancreas does not make enough insulin and the body may not be able to properly use the insulin that is produced.

Although CFRD is serious, the symptoms are often similar to CF or not noticeable. It's also important to note that CFRD is different from Type 1 and Type 2 diabetes and therefore is treated differently.

Beginning at an early age, you should expect your care teams to check for CFRD regularly.

The liver: cirrhosis

Over time, the thick mucus causes scarring and damage to the liver.

Up to 30% of people with CF develop liver disease.

It sometimes leads to cirrhosis, an advanced stage of liver disease that makes it difficult for blood to travel through the liver. This can result in many complications, including infections, malnutrition, and sometimes liver failure.

Liver disease is considered to be one of the most serious health risks associated with CF. There are often no symptoms until the scarring and damage have reached an advanced stage. Care teams regularly monitor liver function in people with CF to check for CF progression in the liver.

CF Fact or Fiction CF Fiction CF Fact or Fiction CF Fiction

CFRD and cirrhosis 

are serious, but not 
life-threatening complications of CF.

Here's the truth:
CFRD and cirrhosis 
can be life-threatening, 
so people with CF are tested regularly for these conditions.

FACT or FICTION

Progression in the Bones

For people with CF, 2 different factors contribute to low bone density:

  • Bones don’t grow strong

    For some people, bone development is affected because they may have trouble absorbing enough vitamin D for healthy bone growth. As a result, bones are weak and can fracture more easily

  • Bones lose density

    Some people may lose bone density more easily. The inflammation caused by frequent lung infections, as well as the steroids used to treat these infections, can cause bone tissue to break down

Both of these factors can contribute to osteoporosis, a disease that can lead to stooped posture, loss of height, and bones that break easily.

Care teams begin screening people with CF for bone health at an early age.

About 70% of adults living with CF will develop osteoporosis, compared to 55% of adults who 
do not have CF.

Disease progression is a big part of everyone's CF. So it's important to be proactive in learning about progression and new developments in CF.

CF Fact or Fiction CF fact CF Fact or Fiction CF Fact

Some effects of CF may have no symptoms.

Many effects of CF are silent at first. For example, lung damage may be occurring before it can be detected by some tests, and many conditions in the digestive system do not have noticeable symptoms early on.

FACT or FICTION

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