Cystic fibrosis treatment can be a lot on little bodies. Here’s what parents should know about managing their child’s symptoms on a long term basis. 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
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.
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:
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.
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:
Symptoms of CF can start in infancy, especially frequent respiratory infections. Common respiratory symptoms include:
Digestive symptoms may include:
Other signs to be aware of include:
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:
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:
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.
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.
Search for a local chapter of the CF Foundation to connect with other parents of children with CF.
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.
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.
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.
Because of effective newborn screenings conducted at birth, most children with cystic fibrosis are diagnosed as newborns.
Cystic fibrosis affects several areas of school and education for most children. Work with your child’s school to develop accommodations for physical activity requirements, online education options, and extended deadlines on exams and projects.
Because of improved treatments, most individuals with CF can expect to live into their 40s, 50s, and beyond.