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
People with cystic fibrosis (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. The first sign a baby might have
cystic fibrosis is an intestinal blockage called meconium ileus. Other kids don't
have symptoms until later on. Cystic fibrosis can be mild or severe, depending
on the person.
Some kids also might have nasal polyps (small growths of tissue inside the nose),
frequent sinus infections,
How Is Cystic Fibrosis Diagnosed?
screening tests catch most cases of CF. If the screening test is positive, or
if a child has cystic fibrosis symptoms, 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 are a teen.
How Is Cystic Fibrosis Treated?
Kids with CF will have it all their lives. Doctors use different medicines depending
on a child's needs. But all people with CF need to:
Loosen and clear mucus. There are different ways to do this.
The doctor might recommend a child:
have chest physical therapy (when a parent or trained person bangs 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 kids with CF need enzymes to help them digest
food and get nutrients from it.
Eat a high-calorie diet and take vitamin supplements, when needed.
What Causes Cystic Fibrosis?
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 child 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 Parents Help?
To help your child:
Follow the treatment plan. Help your child stay as healthy as
possible. Give medicines as directed, serve high-calorie
meals and snacks, and follow instructions for clearing chest mucus.
Offer encouragement. Help your child find pastimes to enjoy,
like art, music, reading, or learning to cook. It's important for kids with CF to
get exercise, so also look for ways your child can stay physically active. Maybe you
can do some of them together.
Turn to the care team. Your child's care team can offer practical
tips on living with CF, and information about clinical
trials, support groups, and new therapies.
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
Teach self-care as your child gets older. Start early to help
your child understand and manage CF. Encourage an older child or teen to handle some
parts of their health
care, like disinfecting equipment or asking questions at doctor visits. Ask the
care team about ways you can help your child get ready for things like going to college
or getting a job. Learning about cystic fibrosis and its care helps kids and teens
become confident adults managing a chronic health condition.