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Heart & Lungs

Cystic Fibrosis

Every day when she wakes up, Lisa begins to deal with a condition she’s known all her life — cystic fibrosis (say: SIS-tik fi-BRO-sus). Her parents clap her back and chest for at least 20 minutes to help clear her lungs of the thick mucus that sometimes makes it difficult for her to breathe.

At school, Lisa coughs a lot, so she keeps a box of tissues on her desk just in case she coughs up mucus and needs to spit it out. In gym class, she participates in sports but often gets tired easily. At lunchtime, Lisa takes pills to help her digest her food and get all the vitamins she needs, which helps her keep up her strength to deal with cystic fibrosis.

What Is CF?

Cystic fibrosis (CF) is a disease that causes the body to make thick, sticky mucus (say: MYOO-kus). This causes problems in two major areas: the lungs and the digestive system.

Healthy lungs produce mucus, which protects the airways and makes it easier to breathe. But for a person with CF, the mucus is thick and sticky and can clog up the lungs. This creates a place where bacteria can easily grow — and bacteria cause infections.

And it’s not only the airways and lungs that are affected in a person who has cystic fibrosis. Mucus-producing cells line the digestive tract, including the stomach, intestines, liver, pancreas, and reproductive organs. The pancreas produces enzymes that help digest food and hormones that help absorb sugar. When thick mucus in the pancreas clogs up the narrow passageways, it can make it difficult for people to digest food and get all the vitamins and nutrients they need.

Cystic fibrosis affects more than 30,000 children and young adults in the United States. It can be mild or severe, depending on the person. To make normal mucus, the body needs a special protein. This protein is defective in cystic fibrosis, producing the thick, sticky mucus that causes problems for people with CF.

What Causes CF?

CF is an inherited disease, which means that it’s passed down from parent to child. Someone who has CF was born with it. Maybe you’ve heard someone say, “It’s in your genes.” They don’t mean your blue jeans. Genes make up the blueprint each of your cells follows to make you a unique individual.

Genes determine your eye and hair color and also are responsible for certain health problems. People with CF got the disease because their moms and dads each had a gene for CF. You need two CF genes (one from mom and one from dad) to have cystic fibrosis.

Most people don’t know that they carry the gene for CF until they have a child who has the disease because carriers of the CF gene do not have the disease themselves.

How Is CF Diagnosed?

Although someone with cystic fibrosis is born with it, it isn’t always obvious at birth. It may take a while for symptoms to develop. Doctors may suspect that a baby has CF if he or she coughs a lot and gets a lot of lung infections. The baby also might have unusual large, bulky bowel movements or may not gain weight as expected.

To know for sure, the doctors can do a simple test that doesn’t hurt. Kids with CF have more salt in their sweat than other kids, so by simply collecting a sample of sweat and testing to see how much salt is in it the doctors can determine if a person has CF. Other tests may be done, including one that looks for the gene that causes CF.

How Is CF Treated?

The aim of CF treatment is to keep the lungs clear of mucus and free of infection. It’s also important for someone with CF to eat well.

Let’s start with the lungs. Lisa seems to always have a cough. She’s coughing to clear mucus out of her lungs. When Lisa’s parents clap on her back in the morning, they’re helping to break up the mucus that collected in her lungs overnight, which makes it easier for Lisa to clear it out of her lungs. If the mucus stayed in there, it would make it harder to breathe and would increase the chance of getting a serious lung infection.

A kid with CF will work with a medical team, including doctors, nurses, nutritionists, physical therapists, social workers, and respiratory therapists. A respiratory therapist knows a lot about breathing and how the lungs work. He or she can teach a kid with CF to do special breathing exercises that help get rid of extra mucus. Breathing treatments also help by adding moisture and delivering medicine into the lungs.

Even if everyone does everything right, kids with CF will still get respiratory infections and need to take antibiotics, which kill bacteria. Kids with CF also may need to stay at the hospital for a while. The good news is that newer medicines are more effective and help kids with CF recover more quickly.

Kids with cystic fibrosis have to work a little harder to breathe, and this burns more calories so it’s important that they get enough to eat. Even a kid with CF who eats healthy foods and gets plenty of calories may still have trouble growing and gaining weight.

The problem is that the pancreas is not working and can’t deliver the enzymes needed to break down protein and fat in food. Kids with CF may have more frequent bowel movements (poop) because they are not absorbing the protein and fat that they are eating. Most kids with CF take medicine that helps digest food but may also need to take a vitamin and mineral supplement.

A dietitian can help kids figure out which foods to eat and provide special recipes packed with nutrients and calories. Along with eating right, it’s important for kids with CF to get regular exercise to make their lungs as strong as they can be.

Living With CF

Kids who have CF have to take care of themselves, but thanks to a better understanding of what causes CF and improved treatment, they can go to school and do regular stuff, just like other kids. Meanwhile, researchers are working on even better treatments and hoping that one day there will be a cure.

Reviewed by: Floyd R. Livingston Jr., MD
Date reviewed: April 2014