Cystic Fibrosis

Healthcare providers often find cystic fibrosis (a genetic disorder) in early infancy, but it can be discovered anytime throughout childhood. CF can have many different symptoms — from mild to severe.

CF causes sticky, thick mucus to build up in your child’s lungs and pancreas. This mucus can clog their breathing tubes (airways), causing your child to wheeze, cough and have frequent lung infections. Mucus from CF can also block ducts in your child’s pancreas and cause digestion problems so they aren’t getting enough nutrients. They might not gain weight like they should and grow slowly. Your child’s expert team will use their vast experience with CF, combined with the most advanced tests available, to confirm your child’s diagnosis.

Cystic fibrosis screening in infants

All newborns in the United States are screened for CF before they leave the hospital. A healthcare provider will take a few drops of blood from your baby’s heel and send it to the lab for testing.

If your baby has certain markers related to cystic fibrosis, we’ll refer you to our cystic fibrosis team, and one of its care coordinators will answer your questions and walk your family through the CF testing process.

Physical exam and medical history

When you and your child come to Cleveland Clinic Children’s for your first visit, your providers will talk with you about any symptoms you’ve noticed. Common ones can include wheezing, coughing with mucus, frequent infections, greasy stools (poop), very salty sweat and trouble gaining weight or growing. We’ll also go over your family medical history and give your child a physical exam.

Sweat test

Sweat test for cystic fibrosis measures the amount of chloride (salt) in your child’s sweat. We’ll put a substance called pilocarpine on an area of your child’s skin. Then, we’ll use a tiny amount of painless electrical stimulation to make the sweat glands produce sweat. We collect a sample of that sweat for testing. People with CF have higher levels of chloride (salt) in their sweat.

Genetic testing

For a child to have cystic fibrosis, they would have inherited two faulty genes, one from each parent. Parents don’t have to have CF for their child to get it. We’ll do a simple blood test to find one of the thousand genetic changes related to CF.

Other tests for CF

We may order more tests to understand how cystic fibrosis is affecting your child’s body, like:

• Fecal test: We may take a sample of your child’s feces (poop) and test it. This can help us find out if your child’s body is using nutrients from food in the right way.
• Pulmonary function testing: We’ll do pulmonary function testing to see how well your child’s lungs work. For example, a pediatric pulmonologist may ask your child to breathe into a mouthpiece and do a test called spirometry to measure how strong their lungs are and how much air they contain.
• Sputum culture: We take a sample of your child’s saliva (spit) and test it for certain bacteria often found in someone who has CF.
• X-rays: Our expert radiologists take pictures inside your child’s chest or sinuses. Chest X-rays and sinus X-rays can show blockages or other changes related to cystic fibrosis.

Meet Our Cystic Fibrosis Team

When your child comes to Cleveland Clinic Children’s for cystic fibrosis diagnosis and treatment, they’ll work with a team of healthcare providers from different specialties so they can get the best, most complete care possible. This team could include:

• Pulmonologists.
• Gastroenterologists.
• Child life specialists.
• Radiologists.
• Respiratory therapists (breathing therapists).
• Social workers.
• Physical therapists.
• Pediatricians or other primary care providers.

Once we know for sure that your child has CF, we’ll schedule regular appointments (at least a few times a year) so we can keep an eye on their health. These visits let us learn how things are going for your child and your family. We also can check if your child is getting enough calories and nutrition from their food. And we’ll make sure their airways and lungs are clear and as healthy as possible to prevent infections.

Getting enough calories and nutrition

Children with cystic fibrosis often have trouble digesting food. Our dietitians can put together an eating plan that makes sure your child eats food with enough calories, protein, vitamins and other nutrients.  

This plan can help them gain weight or maintain a healthy weight. It also helps prevent future health problems, like CF-related diabetes and bone disease, such as osteoporosis. We may also recommend enzymes and supplements to help your child digest food better. And we can talk with you about tube feeding, if needed.

Keeping the airways and lungs clear

Your child’s pulmonologist will likely recommend medications like inhalers or a home nebulizer to help keep their airways clear.

Your child will also see our respiratory therapists, who’ll teach you and your child airway clearance techniques (ACTs). They’ll show you how to do ACTs at home and prescribe tools/devices that can help with at-home ACTs, if needed. Your therapists will also show you and your child how to use inhalers the right way and take care of your equipment.

Preventing and treating infections

Kids with cystic fibrosis tend to get infections pretty easily. Our team makes sure your child is up to date on all of their vaccines (shots). We’ll also watch your child carefully for signs of infection and may recommend antibiotics if they get one. The antibiotics can be given as a pill or through an IV (in a vein in their arm) in the hospital or at home.

Adjusting to life with CF

Our team will help you and your child understand exactly what it means to have CF and what the treatment will be. We’ll also help you both cope with the emotional and social challenges that can come with a chronic illness — things like managing stress and behavioral issues and educating your child’s school, babysitters and others about their condition. And we can help you keep on track with your child’s treatment plan — even when it seems overwhelming.

Taking the Next Step

As you and your child adjust to their cystic fibrosis diagnosis, it’s important to remember that you won’t have to go through treatment alone. At Cleveland Clinic Children’s, we’ll help you get expert care and support so your child can live a longer, fulfilling life.