A Parents’ Guide to Managing Cystic Fibrosis in Children

Cystic fibrosis (CF) is an inherited disease that affects the mucus and sweat glands. CF causes mucus in the body to become thick and sticky, leading to several health problems. CF can affect the lungs, pancreas, liver, intestines, sinuses, and sex organs. People living with CF are also at an increased risk of lung infections. 

CF symptoms vary from person to person, so you may not know how severe your child’s disease will be. In the United States, there are about 30,000 individuals living with CF, and there are 70,000 worldwide. It’s estimated that about 1 in every 30 Americans is considered a CF carrier. 

This article provides an overview of managing cystic fibrosis in children, treatment options, and tips for day-to-day care activities. 

What Parents Should Know About CF

CF is a progressive disease, which means that it worsens over time. It likely feels impossible to learn everything there is to know about this complicated disease. A good goal is to learn a little new information each day.  

It’s helpful to have a basic understanding of how CF affects the body. CF causes long-term damage to the lungs, digestive tract, and other organs because of a cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation. This mutation causes the CFTR protein, which helps to produce sweat and mucus, to malfunction.

When this protein doesn’t function properly, it cannot help move chloride (a component of salt) to the surface of the cells that produce mucus. Without chloride to attract water to the cell, the mucus becomes thick and sticky. A buildup of thick, sticky mucus leads to several problems in the body, including:

• Lungs: Thick mucus in the lungs raises the risk of lung infections and damage.
• Pancreas: A buildup of mucus in the pancreas prevents the release of digestive enzymes that help the body absorb key nutrients. This results in malnutrition and poor growth. 
• Digestive tract: When intestinal secretions are too thick, blockages that may require surgery can occur.
• Liver: Mucus buildup in the liver blocks the bile duct and causes liver diseases like cirrhosis. 
• Reproductive organs: Increased mucus in the reproductive organs can decrease fertility or infertility in men and women. 

CF Is an Inherited Disease

CF is a genetic condition and is not contagious. To have a child with CF, both parents must be carriers of the abnormal CFTR gene and pass it on to their baby. This results in a faulty CFTR protein in the child. 

The CFTR protein is present in every organ that produces mucus. When this protein isn’t functioning normally, the mucus becomes thick and sticky, leading to blockages. Thick mucus is also more likely to trap germs, leading to a serious infection. 

More than 10 million Americans are carriers of the abnormal CFTR gene. There are often no signs of being a carrier, and many people are unaware of their carrier status. 

The risk of CF increases when you have a sibling, half-sibling, or first cousin with CF. CF symptoms tend to worsen over time. Individuals with CF experience a slight decline in lung function each year. 

Healthcare Providers Typically Diagnose Newborns

Because of highly effective health screenings at birth, most people with CF are diagnosed as newborns. It’s estimated that more than 75% of people with CF are diagnosed by age 2, and more than half of people with CF are age 18 or older. 

The following tools are used to diagnose CF:

• Newborn screening: Newborn babies are usually screened for high levels of an enzyme called immunoreactive trypsin. When this enzyme is elevated, it may indicate damage to the pancreas. 
• Genetic testing: More than 2,000 different mutations of the CF gene have been identified. The most common mutation is called deltaF508 and is found in at least 70% of individuals with CF. Parents can undergo genetic testing to find out if they are carriers. 
• Sweat test: Sweat is collected from a small area on the child’s forearm, and the sample is tested for chloride levels because children with CF have higher levels than others. 
• Nasal lining measurement: A healthcare provider runs a small electrical current across the nasal lining (epithelium), which can help determine how well the CFTR protein functions. 

Symptoms 

Symptoms of CF can start in infancy, especially frequent respiratory infections. Common respiratory symptoms include: 

• Chronic cough
• Frequent colds
• Shortness of breath
• Sinus infections 

Digestive symptoms may include:

• Greasy, foul-smelling bowel movements 
• Severe constipation
• Intestinal blockage 
• Inability to gain weight 
• Rectal prolapse 

Other signs to be aware of include: 

• Salty-tasting skin
• Clubbing or enlargement of fingertips and toes 
• Infertility 

Treatment at a Glance 

Because CF can affect so many organs and bodily functions, the treatment plan usually includes several different types of therapies. People with CF require a large number of calories to grow and develop. They need regular physical activity to keep the lungs as healthy as possible. It’s important for children and adults with CF never to be exposed to cigarette smoke or alcohol. 

A typical CF treatment plan may include:

• Airway clearance techniques to loosen and get rid of mucus
• Inhaled medicines to open the airway
• Antibiotics to treat any bacteria that becomes trapped in the thickened mucus
• Pancreatic enzyme supplement capsules to improve nutrient absorption
• Fitness plan to improve energy and lung function
• CFTR modulators to target the defect in the CFTR protein
• Anti-inflammatory medications 

Mucus and Respiratory Management

When your child has CF, your healthcare team will likely teach you about airway clearance techniques. Clearing the airway of excess mucus is an important way to preserve lung function and reduce the risk of breathing problems and infections. 

The goal of airway management is to loosen the mucus in the lungs so that it can be coughed up and cleared out of the body. The mucus in the lungs can be thinned and loosened with the following methods:

• Vibrating therapy vest
• Chest physical therapy
• Medications like bronchodilators and mucus thinners 

The Importance of Nutrition for Healthy Digestion

Staying healthy is an essential part of the treatment plan for anyone with CF. Work with your child’s nutritionist to develop an eating plan with a high number of calories to ensure their nutritional needs are met. Focus on high-energy, high-sodium foods to replace calories and promote growth. 

Your child’s healthcare team will also recommend pancreatic enzyme replacement therapies to help the body absorb more nutrients from their diet. These supplements are usually given before every meal or snack. 

Parent Tips for CF Care 

Finding a way to organize your child’s daily, weekly, and monthly therapies may be helpful and reduce some of the stress that parents feel. Consider writing out a treatment schedule and including your child in the planning as much as possible. 

Children with CF require frequent, regular appointments with their healthcare team. Common tests that need to be conducted regularly include abdominal ultrasound or CT scan, chest X-ray, blood tests, and more.

When caring for a chronically ill child, it is essential to seek out support. Ask your healthcare team for referrals to support groups, therapy services, and financial assistance. Reach out to family and friends for help with meal preparation, babysitting, or housework.

Teaching Your Child CF Self-Care 

Perhaps one of the most challenging aspects of caring for a child with CF is allowing them to start to take over the responsibility for their care. This will be a slow process, so start including your child in their care activities at a young age. 

Talk with your child about the importance of their treatment therapies, including their regular medications, supplements, airway clearance exercises, and medical appointments. Teach them good hand hygiene to prevent infections.

As your child starts to become more independent, it will be important for them to understand when their condition is worsening and what to do. Talk with them about calling their healthcare provider whenever they experience worsening lung symptoms, sudden shortness of breath, or signs of infection.

It may be helpful for your adolescent to connect with others who understand what they are going through. CF Peer Connect will connect your older child with a peer who also has CF. 

Summary

Cystic fibrosis is a progressive genetic condition that is often diagnosed in infancy. Children with CF have thick, sticky mucus that leads to several health problems. Organs affected by thick mucus include the lungs, pancreas, liver, intestines, sinuses, and sex organs. CF's possible symptoms include a chronic cough, frequent colds, shortness of breath, sinus infections, the inability to gain weight, intestinal blockages, salty-tasting skin, and infertility. Treatment options may include airway clearance techniques, medications, enzyme supplements, and a fitness plan. 

A Word From Verywell 

Learning that your child has CF is devastating for many parents. It takes time to adjust to this new diagnosis and begin to understand the disease. Try to be patient with yourself as you learn more each day. Reach out for support whenever you can from family and friends. Work with your healthcare team to find local support groups and financial assistance. Include your child in their care as often as you can. This will help them feel more independent and capable as they grow up.