Pediatric Cystic Fibrosis
Children and young adults with cystic fibrosis 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.
About Cystic Fibrosis
Cystic fibrosis (CF) is a chronic, inherited disease that affects many systems in the body. It causes thick, sticky mucus to build up in the lungs and other organs. The sticky mucus obstructs airways, which results in troubled breathing. It also tends to cause repeated lung infections and eventually lung damage. CF can also interfere with the body’s ability to absorb nutrients from foods and can cause reproductive problems in men and women.
While there’s no cure for cystic fibrosis, 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.
Accredited CF Center
Our CF Center is accredited by the Cystic Fibrosis Foundation, which ensures that your child receives expert, high-quality care. 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 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.
- Seamless transition to adult care. Our comprehensive program includes helping young adults begin taking responsibility for their own care. We start teaching about 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.
CYSTIC FIBROSIS IN CHILDREN
Our team of specialists provides a complete range of traditional and new treatments. Many treatments are given at home on a daily basis. However, your child may need to be hospitalized for treatment if his or her lung function worsens. Our pediatric cystic fibrosis specialists will make sure you understand each treatment and support you and your child every step of the way.
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 his or her stay in the hospital.
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 of the ones targeted by CFTR modulator drugs (ivacaftor and lumacaftor), doctors may prescribe one of these medications for your child starting at age 6 or older.
A physical therapist 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.
Children with CF can become malnourished, which can contribute to worsening lung function. A registered dietician 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 may 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.
CYSTIC FIBROSIS IN CHILDREN
Most children with cystic fibrosis are diagnosed during newborn screening; however, children with mild symptoms may not be diagnosed until they are older. Once diagnosed, your child will see our specialists about every three months. 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 ask that they wear a surgical mask when entering the hospital and outside the exam room.
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.
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.
Your child's doctors may order imaging tests such as X-rays and CT scans; lung function tests; sputum 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.