Pediatric Cystic Fibrosis

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Children and young adults with cystic fibrosis (CF) need coordinated care from a team of experts in a wide range of specialties. Specialists with the Duke Children's Cystic Fibrosis Center provide the latest treatments and personalized care to help manage your child's condition and improve their quality of life.

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About Cystic Fibrosis

Cystic fibrosis (CF) is a genetic disease that affects many systems in the body. It causes thick, sticky mucus to block the airways and build up in the lungs and other organs. The sticky mucus obstructs airways, which can result in troubled breathing. It also tends to cause repeated lung infections that may eventually damage the lung. CF can also interfere with the body’s ability to absorb nutrients from foods and can cause sinus and reproductive problems.

While there’s no cure for CF, there have been significant advances in treatment in recent decades. We use these advances to create individualized treatment programs, which have proven successful in helping people with cystic fibrosis live longer, fuller, and more active lives than ever before.

People born with cystic fibrosis -- also called CF – face a range of serious health challenges including difficulty breathing, lung damage, poor growth, and a shorter lifespan. Currently there is no cure, but promising new medications called CFTR modulators are helping people with CF achieve improved lung function, growth, and overall health. Here Shatha Yousef, MD, a pediatric pulmonologist at Duke Health, discusses CF symptoms, long-term effects, and new treatments that are changing the lives of people with the disease.

People with cystic fibrosis (CF) can live longer, better-quality lives thanks to new treatments called CFTR modulators. These medications target gene mutations present in CF and can significantly minimize symptoms and reduce complications. When started early, they can even eliminate some of the worst effects of CF. Here, Mai ElMallah, MD, a pediatric pulmonologist at Duke Health, discusses CFTR modulators, how they work, and who can benefit.

Cystic Fibrosis Patient Thriving on Trikafta | Duke Health

Treatment Options

Our team of specialists provides a complete range of traditional and new treatments based on CF care guidelines. Many treatments are given at home on a daily basis. However, your child may need to be hospitalized for treatment if their lung function worsens. Our pediatric CF specialists will make sure you understand each treatment and support you and your child every step of the way.

Respiratory Therapy

A respiratory therapist may administer breathing treatments and work with a physical therapist to do chest physiotherapy to remove mucus from the airways. Treatments can be performed with various devices, including a vest that vibrates to loosen lung secretions. Therapists typically stay in the room during the course of each treatment. Your child may receive more frequent therapies during a stay in the hospital.

Medication

Your child's pulmonologist may prescribe antibiotics for lung infections, anti-inflammatories to reduce airway swelling, and bronchodilators to help keep airways open. Other medicines can loosen mucus and reduce its stickiness. If the genetic mutation causing your child’s CF is one targeted by medications called CFTR modulator drugs, one of these medications may be prescribed.

Physical Therapy

A physical therapist (PT) will evaluate the impact of cystic fibrosis on your child's heart and lungs and develop a treatment plan of activities to encourage improved lung functioning. The PT will also encourage regular physical activity in the hospital and at home.

Diet and Enzyme Supplements

Children with CF can become malnourished, which can contribute to worsening lung function. A registered dietitian (RD) and nutritionist may recommend a high-calorie diet -- at least 3,000 calories each day -- with extra fat and protein to maintain your child’s weight. They will also recommend a daily multivitamin, as cystic fibrosis can interfere with absorption of certain vitamins. Some children with cystic fibrosis lack pancreatic enzymes they need to break down food. Your child’s doctor may recommend supplements with extra enzymes to be taken before meals and snacks. In the new era of CFTR modulators, our RD will ensure that your child has a healthy weight and avoid excessive weight gain.

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Tests

Most children with cystic fibrosis are diagnosed through newborn screening. Once diagnosed, your child will see our specialists about every month for the first year, and every three months thereafter. We'll perform tests to check your child's health, answer your questions, and make sure you are managing well with airway clearance techniques. For your child's protection, we follow infection control guidelines, and we ask that they wear a surgical mask when entering the hospital and outside the exam room.

Diagnostic Testing

Newborns are screened for cystic fibrosis via a blood test within the first few days after they are born. When newborn screening suggests CF, a sweat test confirms the diagnosis by measuring the level of salt in your child's perspiration. It is also used to diagnose cystic fibrosis in older children and teens who were not diagnosed as infants but have symptoms of the disease.

Genetic Testing

Cystic fibrosis is caused by a mutation in what’s called the CFTR gene. Some newer CF medications, called CFTR modulators, are targeted to specific mutations of this gene. Genetic testing can determine the type of mutation your child has and whether these medications may help.

Routine Visit Tests

Your child's doctors may order imaging tests such as X-rays and CT scans; lung function tests; sputum (respiratory mucus) culture to check for certain bacteria; annual blood work to check your child's nutritional status, liver function, vitamin levels, and other health issues; and screenings for depression and anxiety, as well as diabetes and osteoporosis, which can occur with cystic fibrosis.

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Accredited CF Center

Our CF Center is accredited by the Cystic Fibrosis Foundation (CFF), which ensures that your child receives expert, high-quality care in line with CFF guidelines. As an accredited center, we offer:

Access to a Comprehensive Team of Experts
Your child’s team will include doctors who specialize in pulmonology, endocrinology, gastroenterology, and infectious diseases. The team will also include a CF nurse coordinator, nutritionists, respiratory therapists, and physical therapists as well as licensed clinical social workers and psychologists.

Access to Clinical Trials
People in both our pediatric and adult centers who meet certain qualifications can participate in trials of new cystic fibrosis treatments or observational studies monitoring the impact of some of these treatments.

Seamless Transition to Adult Care
Our comprehensive program includes helping young adults begin taking responsibility for their own care. We start preparing for the transition to our Adult Cystic Fibrosis Center when your child reaches age 16, providing support and guidance as they begin to manage home treatments, monitor their symptoms, and learn when to seek help from their doctor.

Ongoing Care and Support
Our licensed clinical social workers guide you and your family through the medical system and coordinate the health services your child needs. They can help you communicate with other members of the care team to ensure that the needs of your child and family are being met. They can provide support, help you work with insurance providers, connect you with resources, and help you manage details related to your child’s care. Your case manager coordinates your child’s transition from hospital to home.

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This page was medically reviewed on 08/19/2024 by

Shatha W. Yousef, MD | Pediatric Pulmonologist