Breathing problems (also called respiratory or
The right lung has three lobes -- upper, middle, and lower. The left lung has two lobes -- upper and lower. The lobes branch off into smaller segments. Airway clearance techniques (ACTs) help clear the
The Respiratory System
The respiratory system has two main parts:
- Upper respiratory tract: the nose and sinuses
The upper respiratory tract filters out particles such as dust and germs in the air we breathe. It also warms and moistens that air.
- Lower respiratory tract: the airways and lungs
The lower respiratory tract starts at the trachea (the main windpipe), just below the vocal cords, and keeps branching until it gets to the air sacs.
The air sacs are where oxygen (O2) enters into the blood (“in with the good air”) and the carbon dioxide (CO2) comes out (“out with the bad air”). The tubes that go from the windpipe to the air sacs are called the “airways.”
The airways have cells with “house cleaning” tools called cilia (small hair-like structures). They move together to push the dust, germs, and mucus out of the lungs where it can be coughed out. The cilia are normally bathed in a thin layer of fluid that is kept at just the right volume and has a mixture of chemicals (like sodium and chloride) and special chemicals that help fight infections.
CF and the
Cystic fibrosis is caused by abnormalities in a
But in people with CF, this protein doesn't get to the
The following video shows what it looks like when the CFTR protein functions normally in the lungs -- hair-like cilia can move mucus and germs out of the airways -- and what happens when the protein doesn't work.
Mucus plugs keep air from getting into or out of some alveoli (air sacs). Oxygen cannot get into the blood and carbon dioxide cannot get out. The thick, sticky mucus in the small airways:
- Gets in the way of air getting into and out of alveoli
- Makes it hard to remove particles and germs from the airways
If not cleared from the lungs, mucus plugs can lead to lung infections and lung damage. Lung infections cause serious problems for people with CF because:
Infections often cause more mucus to be made. This can lead to more mucus plugs and worse infections.
This infection-inflammation cycle causes lung damage. Mucus plugs airways, allowing germs to grow and cause infection. When the body's defense system -- white blood cells -- attack the germs, the lungs become inflamed. This inflammation spurs the creation of more mucus, which then blocks the airways and allows more germs to grow. As you can see, it's a vicious cycle.
See how the lungs work normally and how cystic fibrosis can affect the lungs over time.
How Your CF Care Team Can Help You Protect Your Lungs
Monitoring and minimizing the buildup of germs, like
Starting at age 5 or older, your CF care team will ask you to take a type of breathing test, known as a pulmonary function test (PFT), during one of your quarterly visits. You will also be asked about your breathing, coughing, and the amount of
Your care team will ask for a sample of your sputum which is tested to see what kinds of bacteria are growing in your lungs and determine which antibiotics might fight the bacteria. This is called a “
Your PFT results will probably look like a sea of numbers and maybe a line graph. Your care center team will review your PFT results with you to help you understand what they mean. One component of your PFT report is the
Clearing the airways to reduce lung infections and improve