Cystic fibrosis (CF) is an inherited disease in which the body makes very thick,
sticky mucus. The mucus causes problems in the lungs,
pancreas, and other organs.
People with cystic fibrosis (pronounced: SIS-tik fye-BROH-sis) get lung infections
often. Over time, they have more trouble breathing. They also have digestive
problems that make it hard to gain weight.
What Are the Signs & Symptoms of Cystic Fibrosis?
CF can cause symptoms soon after a baby is born. Other kids don't have symptoms
until later on.
Some people also might have nasal polyps (small growths of tissue inside the nose),
frequent sinus infections,
How Is Cystic Fibrosis Diagnosed?
Doctors do tests on newborns that check for many health conditions, including cystic
fibrosis. These find most cases of CF. To confirm the diagnosis, doctors do a painless
sweat test. They collect sweat from an area of skin (usually the forearm) to see how
much chloride (a chemical in salt) is in it. People with CF have higher levels of
Most children with CF are diagnosed by the time they're 2 years old. But someone
with a mild form may not be diagnosed until they're a teen.
How Is Cystic Fibrosis Treated?
People with CF will have it all their lives. Doctors use different medicines depending
on a person's needs. But all people with CF need to:
Loosen and clear mucus. There are different ways to do this.
The doctor might recommend that the person:
have chest physical therapy (banging gently on the chest or back)
Prevent or fight lung infections.Washing
hands well and often, avoiding people who are sick, and staying at least 6 feet
away from others with CF can help prevent infections. Taking preventive antibiotics
also can help.
Take enzymes. Most teens with CF need enzymes to help them digest
food and get nutrients from it.
Cystic fibrosis is caused by a change (mutation) in the gene
that makes cystic fibrosis transmembrane regulator (CFTR) protein. To have CF, a baby
must get two copies of the CF gene, one from each parent.
What Happens in Cystic Fibrosis?
In CF, the body makes abnormal CFTR protein or none at all. Without normal CFTR
protein, the cells lining the pathways (tubes) inside some organs make thick, sticky
mucus rather than the normal thin, watery kind.
Thick mucus can trap bacteria
in the lungs, leading to infection, inflammation, and breathing problems. Mucus also
can block the path where digestive enzymes flow between the pancreas and the intestines.
This makes it hard for a teen to digest food and get the vitamins
and nutrients they need from it.
Thick mucus can also affect the liver, the sweat glands, and the reproductive organs.
How Can I Deal With Cystic Fibrosis?
If you have CF, focus on staying as healthy as possible:
Follow the treatment plan. Take medicines as directed, eat high-calorie
meals and snacks, and follow instructions for clearing chest mucus.
Focus on other things. Find pastimes to enjoy, like art, music,
reading, or learning to cook. It's important for teens with CF to get exercise, so
also look for ways to stay physically
Turn to the care team. Your CF care team can offer practical
tips on living with CF, and information about clinical trials, support groups, and
Learn all you can about CF. Experts are always working on new
treatments to help people with CF have a better quality of life and live longer. Online,
turn to resources like the Cystic Fibrosis Foundation
Take charge of your medical care. Start early to help manage
your CF. You can disinfect equipment, refill
prescriptions, and schedule doctor visits. Ask the care team about ways you can
get ready for things like going to college or getting a job. Learning about cystic
fibrosis and its care can help you become a confident adult managing a chronic