Learn about cystic fibrosis, a genetic disorder that affects the lungs, pancreas, and other organs, and how to treat and live with this chronic disease.
CF is a rare genetic disease found in about 30,000 people in the U.S. If you have CF or are considering testing for it, knowing about the role of genetics in CF can help you make informed decisions about your health care.
If you or your child has just been diagnosed with cystic fibrosis, or your doctor has recommended testing for CF, you may have many questions.
Diagnosing CF is a multistep process. A complete diagnostic evaluation should include a newborn screening, a sweat chloride test, a genetic or carrier test, and a clinical evaluation at a CF Foundation-accredited care center.
Raising a child with cystic fibrosis can bring up many questions because CF affects many aspects of your child’s life. Here you’ll find resources to help you manage your child’s daily needs and find the best possible CF care.
Living with cystic fibrosis comes with many challenges, including medical, social, and financial. By learning more about how you can manage your disease every day, you can ultimately help find a balance between your busy lifestyle and your CF care.
People with CF are living longer, healthier lives than ever before. As an adult with CF, you may reach key milestones you might not have considered. Planning for these life events requires careful thought as you make decisions that may impact your life.
People with cystic fibrosis are living longer and more fulfilling lives, thanks in part to specialized CF care and a range of treatment options.
Cystic Fibrosis Foundation-accredited care centers provide expert care and specialized disease management to people living with cystic fibrosis.
We provide funding for and accredit more than 120 care centers and 53 affiliate programs nationwide. The high quality of specialized care available throughout the care center network has led to the improved length and quality of life for people with CF.
The Cystic Fibrosis Foundation provides standard care guidelines based on the latest research, medical evidence, and consultation with experts on best practices.
As a clinician, you’re critical in helping people with CF maintain their quality of life. We’re committed to helping you partner with patients and their families by providing resources you can use to improve and continue to provide high-quality care.
As part of the Cystic Fibrosis Foundation's mission to help improve the lives of people living with cystic fibrosis, the PSDC initiative taps the CF community to inform key efforts to support the management of daily care.
Your cystic fibrosis care team includes a group of CF health care professionals who partner with you to provide specialized, comprehensive CF care.
Many cystic fibrosis patients and families face complicated issues related to getting the care they need. But CF Foundation Compass makes sure that no one has to do it alone.
For many people with cystic fibrosis, dealing with insurance is as much a part of living with the disease as nebulizers and vests. Many people with CF and their families face issues related to getting the care they need, but no one has to do it alone.
The Cystic Fibrosis Foundation is the world’s leader in the search for a cure for CF and supports a broad range of research initiatives to tackle the disease from all angles.
The CF Foundation offers a number of resources for learning about clinical trials and treatments that are being developed to improve the treatment of cystic fibrosis.
Our understanding of CF continues to evolve as scientists study what causes the disease and how it affects the body. These insights drive the development of new and better treatments and bring us one step closer to a cure.
Researchers, supported by the CF Foundation, have made tremendous advances to improve the health and quality of life of people with CF. We are committed to providing the tools and resources you need to continuously build upon this work.
Although cystic fibrosis affects many parts of the body, the focus is often on the lungs because of the disease's effect on breathing.
Breathing problems (also called respiratory or pulmonary problems) affect the lungs and the airways and are the most serious problems associated with CF. The severity of respiratory problems differs for each person. For most people with CF, lung disease affects how well they do and how long they live. Many treatments are available to keep the lungs as healthy as possible.
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 mucus from these segments. Each segment has airways (bronchi), air sacs (alveoli), and blood vessels (arteries, veins, and capillaries). Oxygen and carbon dioxide are exchanged between the blood and air in the air sacs.
The respiratory system has two main parts:
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.
Cystic fibrosis is caused by abnormalities in a protein called CFTR (cystic fibrosis transmembrane conductance regulator). When working properly, the CFTR protein moves to the surface of cells in the lungs -- and in the pancreas and other organs -- where it helps move chloride, water, and other small molecules out of the cells and onto the surface.
But in people with CF, this protein doesn't get to the cell surface, or it doesn't function properly if it does reach the cell surface. The right amounts of chloride, water, and other molecules do not move into the airways. This results in the buildup of thick, sticky mucus. The mucus becomes so thick that the cilia cannot easily move the mucus to the throat. So, instead of the cilia cleaning the airways, the mucus clogs the airways. When mucus blocks an airway, it is called a mucus plug.
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:
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:
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.
Monitoring and minimizing the buildup of germs, like bacteria in your lungs, is one of the main ways your CF care team can help you protect your lungs.
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 sputum (mucus or phlegm from the lungs) you are producing.
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 “sputum culture.”
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 forced expiratory volume (FEV1), which is the amount of air you can blow out in the first second of a forceful exhalation. Your care team will work with you on ways to keep your FEV1 as high as possible.
Clearing the airways to reduce lung infections and improve lung function with airway clearance techniques is a big part of keeping your FEV1 numbers high.
Watch the webcast below to learn more about CF lung care as Peter Mogayzel, M.D., Ph.D., discusses:
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