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Cystic fibrosis is a genetic condition, which means that it is something you are born with. Cystic fibrosis is known to cause your lungs to produce extra-thick, sticky mucus. This mucus builds up and clogs your airways.
Even before symptoms present, people with cystic fibrosis develop mucus that is thicker and stickier than normal.
Healthy lungs: The lungs naturally produce a thin layer of mucus that helps protect the airways from dangerous bacteria that can cause infections.
Lungs affected by CF: For people living with CF, the mucus in the lungs is thicker than normal. This thick mucus can build up in the airways, allowing germs to thrive and become a source of infection and inflammation. Inflammation is caused by your immune system’s response to something harmful, and inflammation can lead to decreased lung function.
Sticky mucus contributes to the progression of cystic fibrosis over time
Controlling mucus early in the course of cystic fibrosis may be an important part of managing the disease.
When white blood cells fight an infection caught in the mucus, they leave behind remains of the infection. This is called extracellular DNA. The buildup of these remains makes the mucus in lungs affected by CF even thicker than before. This leaves your lungs even more vulnerable to decreased lung function. Because the mucus is even more thick and sticky, it is more difficult for the body to remove.
This may begin a cycle of blockage, infection, and inflammation that may lead to poorer lung function.
Signs and symptoms of pulmonary exacerbations, or flare-ups, from CF include:
- Increased cough
- Changes in mucus (volume or consistency)
- Loss of appetite or weight
- Worsening lung function
Living with Cystic Fibrosis
Get the most out of your treatment
- Understanding treatments: Each treatment works differently to support you
- Establish a routine: Building a routine can help you stay on track and stay healthy
- Maintain a positive attitude: Managing CF can be a challenge, but keeping a positive attitude goes a long way to help you stay on track
Helpful tips on how to stay ahead of CF
Eating well, being active, and establishing treatment routines may help you manage your CF
- Eating a high-calorie, high-fat diet is important to help make sure you are getting the nutrients you need from food
- Exercise as often as you can to help manage lung function. The effect of Pulmozyme on exercise tolerance has not been established in adults or children
- Partner with your CF Care Team to come up with a treatment routine that works best for you
- Make time every day for your Pulmozyme and other cystic fibrosis treatments
It is important to discuss these plans with your doctor and your CF Care Team to find the right balance to fit your unique needs.
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