Cystic fibrosis (CF) is a lifelong condition that children are born with. It can cause severe damage to the lungs, pancreas and other organs. While cystic fibrosis is serious, early diagnosis and treatment can help children grow, thrive and stay as healthy as possible.
At Banner Health, we care for children with cystic fibrosis. Our pulmonary specialists work closely with families to provide advanced treatments, manage symptoms and support your child’s overall health. Together, we help your child live their best life.
Cystic fibrosis is a lifelong genetic condition that affects how the body works. It is inherited, meaning it is passed down through families. CF starts at birth and gets worse over time.
In a healthy body, mucus is thin and watery. It protects and keeps the lungs, digestive system and other organs working well. In CF, a faulty gene or genetic mutation causes mucus to become thick and sticky. This thick mucus builds up in the lungs and other organs, causing breathing problems, trouble digesting food and a higher risk for lung infections.
While cystic fibrosis is considered a lung disease, it affects many other areas. The name comes from the cysts and scarring (fibrosis) it causes in the pancreas, an organ that helps with digestion.
Cystic fibrosis can affect many parts of the body. Here’s how it works:
Lungs: The thick mucus in the lungs makes it difficult to breathe. It traps germs, which can cause lung infections like pneumonia. Over time, these infections can lead to lung damage.
Pancreas: The thick mucus from CF blocks enzymes from leaving the pancreas, preventing the body from breaking down and properly absorbing food. This may cause people with cystic fibrosis to have trouble gaining weight or getting the nutrition they need. Because other parts of the pancreas control the level of sugar in the blood, some people with CF may develop type 1 diabetes.
Sinuses: The thick mucus in CF can also build up in the nasal cavities (sinuses), causing chronic sinus infections. This can cause headaches, congestion and problems breathing through the nose.
Liver and bile ducts: The mucus can block the bile ducts, which carry bile from the liver and gallbladder to the small intestine. This can cause liver damage over time. In some rare cases, people with CF may develop liver disease.
Sweat glands: People with CF sweat more salt than usual. This can lead to dehydration and imbalances in salt levels, which are important for the body to work well.
Reproductive system: CF can also affect fertility, especially in males, because it can block the tubes that carry sperm.
Cystic fibrosis is caused by mutations in the CFTR gene (cystic fibrosis transmembrane conductance regulator). This gene controls the movement of salt and water in and out of cells. In people with CF, the mutation makes a CFTR protein that does not work properly, causing mucus to become thick and sticky.
There are more than 2,000 known mutations of the CFTR gene. Some mutations cause milder symptoms (atypical cystic fibrosis), while others cause more severe problems (classic cystic fibrosis).
CF is a genetic condition, which means it is inherited. To develop the disease, a child must inherit the faulty CFTR gene from both parents.
Parents do not need to have CF themselves to pass the gene to their child. This is because cystic fibrosis is a recessive gene, meaning a parent can carry one faulty copy of the gene without having symptoms. They are called carriers. Many people are carriers of the CF gene without knowing it. It’s estimated that about 1 in 30 people in the U.S. are carriers.
If both parents are carriers, there is a:
You can find out if you are a carrier through a carrier screening or genetic testing. This test looks at your DNA from a blood, saliva or cheek swab sample. A genetic counselor can help explain the results and what they mean for you and your family.
Cystic fibrosis affects many parts of the body. Symptoms range from mild to severe. They may include wheezing, coughing that produces mucus, muscle and joint pain and clubbing or bulging of the fingers or toes.
Early diagnosis is important for starting treatment as soon as possible and managing complications. Learn more about the signs and symptoms of CF, possible complications and how CF is diagnosed.
While there is no cure for cystic fibrosis, many people now live longer, healthier lives. Early diagnosis and treatment can make a big difference. Treatment focuses on managing symptoms, preventing infections and improving quality of life.
Learn more about the treatments available and how to manage daily life with CF.
If you or your child has recently been diagnosed with cystic fibrosis, our experienced team is here to provide comprehensive, personalized care. With an interdisciplinary family-centered approach, we’re here to support your family every step of the way. Schedule an appointment today.
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