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.
Bronchiectasis causes loss of lung function
Chronic inflammation and infection often lead to an extensive and progressive condition called bronchiectasis, which eventually affects virtually all people with CF. Bronchiectasis 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. As it worsens, the lungs become more damaged, leading to a loss of lung function.
It’s important to promptly treat pulmonary exacerbations to prevent and slow down the progression of bronchiectasis. If left untreated, the cycle of infection and inflammation can result in a permanent loss of lung function.
A change in lung function is not always felt
Part of what makes this 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.
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.
Long-term Digestive Impact
Over time, scarring may result in other conditions
As someone with CF grows up, the same mucus issues that caused digestive problems in childhood may have caused scarring in different digestive organs. This scarring can result in the development of other conditions.
Pancreatic insufficiency (PI) is when the body has a difficult time digesting because enzymes aren’t able to do their job. People with CF who are pancreatic sufficient (PS) are able to digest food normally because they still have pancreatic function. But even those who are PS can show evidence of pancreatic damage and may be at risk for pancreatitis.
Pancreatitis is a serious condition and occurs when enzymes are not able to pass through blocked pancreatic ducts. This causes the pancreas to become inflamed and can result in severe symptoms such as abdomen and back pain, nausea, vomiting, fever, rapid pulse, and weight loss.
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.
CFRD is a serious condition, but 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. If CFRD is left untreated, CF symptoms can worsen, with reduced ability to fight infection, muscle loss, weight loss, liver disease, nerve problems, complications in the kidneys, and a decline in vision.
The liver: cirrhosis
Over time, the thick mucus causes scarring and damage to the liver.
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 low blood counts, fluid buildup in the abdomen, bleeding in the esophagus, and difficulty breathing.
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.
Progression in Other Parts of the Body
CF affects other parts of the body as well. This can be the result of the disease itself or of the different types of medicines used to treat CF.
For people with CF, different factors contribute to low bone density:
Bones lose density and don't grow as strong as they should
Some people may lose bone density more easily. The inflammation caused by frequent lung infections can cause bone tissue to break down.
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.
These factors can contribute to osteoporosis, a disease that can lead to stooped posture, loss of height, and bones that can break easily.
About 2% of people with CF between the ages of 25 and 35 have Chronic Kidney Disease (CKD). There are two factors that make adults with CF at risk for CKD. These include progressive cystic fibrosis-related diabetes (CFRD) and treating with certain medications for an extended period of time. Some common symptoms of CKD are itching, muscle cramps, nausea and vomiting, loss of appetite, swelling in feet and ankles, and trouble sleeping.
Medications and treatments are extremely important for people with CF, but over time, those medications may cause side effects. Hearing loss is common in people with CF who have been exposed to certain medications such as aminoglycosides for an extended period of time.
CF May Cause Depression and Anxiety
As someone with CF gets older, there’s a possibility that other conditions will develop. Both patients and caregivers dedicate so much time and energy to managing and treating CF that sometimes they don’t focus enough on their emotional and mental health.
People living with or caring for someone with CF are at a higher risk of experiencing depression and anxiety for many reasons. Living with CF or caring for someone with CF takes a lot of patience, discipline, and organization. Depression and anxiety can affect both a person living with CF and their caregiver, which may make it hard for them to stay on track with treatment.