Caring for a Child With Cystic Fibrosis

Cystic fibrosis affects about 35,000 people in the U.S. according to the Centers for Disease Control and Prevention (CDC). Children and adults living with cystic fibrosis often experience problems with breathing and digestion.

“Cystic fibrosis is a complex genetic condition that affects the lungs, gastrointestinal (GI) tract, pancreas, and other organs,” explains Lael Yonker, MD, a Mass General Brigham pediatric pulmonologist. Dr. Yonker cares for patients at Mass General for Children and is co-director of the Cystic Fibrosis Center there.

In this article, she addresses common concerns and questions parents and caregivers may have about caring for a child with cystic fibrosis. While there is currently no cure, Mass General Brigham clinicians and researchers are dedicated to finding new treatments to better help children and adults living with cystic fibrosis.

What causes cystic fibrosis? 

Cystic fibrosis is a genetic condition that’s caused by a change in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. This change in the CFTR gene causes a person’s cells to absorb too much water and sodium (a component of salt), making the mucus in their organs too thick and sticky.  

Early detection is crucial in order to begin treatment. If your child has been diagnosed with cystic fibrosis, they may experience breathing problems, inflammation, infections, and organ damage. It may also be hard for them to get enough nutrition if their pancreas is impacted. 

How is cystic fibrosis treated?

A health care team works with you to create a treatment plan for your child. Your child’s care team may include others in addition to a cystic fibrosis specialist, including nutritionists, physical therapists, genetic counselors, and more. Your team will work together to give your child the best quality of life possible.

Your child’s treatment plan may include:

Airway clearance therapy techniques

Your child may need help clearing the mucus from their airway so they can breathe easier, especially if they are very young.

“A cystic fibrosis specialist helps manage your child’s symptoms and develops a plan that includes steps and techniques to clear the airway when it’s clogged with mucus. This is called airway clearance therapy and is essential for most people with cystic fibrosis,” Dr. Yonker says. 

Your child’s care team may recommend some of the following airway clearance techniques:

• Active cycle of breathing and autogenic drainage. These are controlled breathing techniques to help loosen the mucus and make it easier for your child to cough up. 
• Chest physical therapy, where your child lies on their side and you or a caregiver tap the side of their chest with cupped hands. Gravity and the percussion of your hands helps loosen the mucus in your child’s airways.
• High-frequency chest wall oscillating vest. This device, worn as a vest, gently compresses your child’s chest in and out to promote airflow and loosen mucus.
• Positive expiratory pressure (PEP) or airway oscillating devices, which your child can breathe into to help open up their airways and remove mucus.  

In addition, your child may require breathing support such as oxygen therapy, where a small face mask or tube attached to your child’s nose delivers more oxygen to help them breathe better.

Lifestyle changes for children with cystic fibrosis

Keeping your child as healthy as possible is the main focus of cystic fibrosis care. Your child’s specialist shares certain lifestyle changes you can make to help reduce your child’s symptoms and prevent their cystic fibrosis from getting worse.

Here are some healthy lifestyle changes you can make with your child:

• Stay active. Exercise, even just walking, can benefit your child’s lungs and help clear their airways. Your child’s care team helps you find an exercise program that works best for your child.
• Get enough sleep. Cystic fibrosis can make sleep difficult for your child, so you can try different techniques to help them sleep better and wake up more rested.
• Practice good hygiene and stay up to date on vaccines. Infections can be especially dangerous for children with cystic fibrosis. Keep your child healthy by encouraging everyone in your family to prevent the spread of germs by doing things like regularly washing their hands. Talk to your child’s care team to make sure your child is up to date with recommended vaccinations. Vaccines can help prevent health complications in people with cystic fibrosis.
• Choose healthy foods. Many children with cystic fibrosis cannot absorb food normally due to pancreatic insufficiency. Talk with your doctor about pancreatic enzyme replacement therapy. Additionally, nutritious food like fruits and vegetables, whole grains, and foods rich in protein (like fish, lean meat, and eggs) help improve your child’s overall health. Avoid foods high in unhealthy dietary fat. At times, your child’s doctor may also recommend a diet high in sodium (a component of salt) or a sodium supplement.

You can work with your child’s cystic fibrosis specialist to come up with a healthy eating plan. Learning how to read and understand nutrition labels can also help you make healthy decisions for your child’s food. 

Medication for cystic fibrosis

It’s important to work with your child’s care team to come up with a plan that includes medication in addition to airway clearance therapy. Medications for your child can include:

• Mucus thinners 
• Antibiotics for lung infections 
• Steroids and other drugs to reduce inflammation
• Inhaled bronchodilators to open airways 
• CFTR modulators for improving function of organs affected by cystic fibrosis, including the lungs  

Additional testing for other conditions

Additional tests throughout your child’s life will check for other conditions that can occur as a result of cystic fibrosis, such as diabetes, osteoporosis, and more. These proactive tests are to make sure any developing condition is caught early.

Know when to call your child’s cystic fibrosis care team.

Regular visits to your child’s pediatrician and cystic fibrosis specialist are an essential part of their care. Be sure to call your child’s care team if they:

• Have worsening symptoms 
• Cough or spit up blood 
• Have sudden shortness of breath or chest pain 
• Have severe pain in their belly, which may spread to their chest or back

In some cases, cystic fibrosis can cause respiratory failure where your child has difficulty breathing on their own. This is a medical emergency. Call 9-1-1 if your child:

• Has trouble breathing or talking
• Seems confused
• Has a blueish color on their skin or lips

Treating cystic fibrosis at all ages

As your child gets older, they learn more about cystic fibrosis and how to take control of their health. Mass General Brigham supports people of all ages with cystic fibrosis. Our team works to make sure your child receives consistent care. Clinical trials at Mass General Brigham can offer your child access to new treatments before they are widely available. 

Our researchers are dedicated to looking for new ways to help people with cystic fibrosis live better lives. At the Mass General Brigham Gene and Cell Therapy Institute, researchers are investigating how gene and cell therapy may be able to slow, stop, and even prevent cystic fibrosis.

“People with cystic fibrosis are living longer and better than ever before,” concludes Dr. Yonker. “New treatments offer hope and improved quality of life for children and adults.”