Pediatric Cystic Fibrosis
Pediatric Cystic Fibrosis at Golisano Children's Hospital
Typically discovered during the first few weeks of life, cystic fibrosis (CF) is a rare genetic condition that causes mucus, which is normally thin, to become thick and sticky and clog organs like the lungs, pancreas and digestive tract (gut). This lifelong disease can lead to problems with breathing and digestion and other health issues in infants and children.
Today, advances in care, early screening and innovative therapies mean that children with cystic fibrosis are able to better manage their condition and achieve a higher quality of life. At Golisano Children's Hospital at University of Vermont Health, we are here for you and your child and are dedicated to helping you manage your child’s condition now and into the future.
Why Choose Golisano Children's Hospital?
Our nationally accredited Cystic Fibrosis (CF) Pediatric Program is the only one of its kind serving Vermont and northeastern New York. Recognized by the Cystic Fibrosis Foundation (CFF) with the Quality Care Award, our program provides a comprehensive team approach to treat and manage pediatric cystic fibrosis from the first days of life.
We offer:
- Exceptional pediatric expertise: Children with CF need regular monitoring of their lungs and nutritional needs. We provide comprehensive care for young patients of all ages in a family-friendly supportive environment. Our multidisciplinary pediatric CF clinic is made up of fellowship-trained pediatric pulmonary medicine and gastroenterology specialists who work closely with pediatric specialists in otolaryngology (ENT), endocrinology, infectious diseases and other specialties.
- Family-centered services: Our child-friendly approach revolves around the unique needs of each family to help and support your child’s CF journey. We help you and your family make informed decisions about your child’s care so they can reach their highest potential throughout their lives.
- Advanced CF testing: All infants in the United States are now screened for CF shortly after birth using a tiny sample of your baby’s blood. When you come to a Cystic Fibrosis Foundation-accredited center, you can be assured your child has access to a wide array of CF diagnostic exams using the latest technology.
- Research-driven care: Active researchers, our physicians make all diagnostic and treatment recommendations based on the latest thinking in the field. Participating in the Cystic Fibrosis Therapeutics Development Network (CF TDN), the largest CF clinical trial network in the world, we provide your child with access to the most effective medications and therapies available for treating cystic fibrosis.
What Causes Pediatric Cystic Fibrosis?
Cystic fibrosis is an inherited condition that affects the lungs and digestive system. A mutation of the CFTR gene, which normally helps balance salt and water on many surfaces in the body such as the lungs, often leads to the thick and sticky mucus that causes many of the symptoms of CF.
Children who inherit the CFTR mutation from both parents will have cystic fibrosis. CF carriers are people who have only inherited the genetic abnormality from one parent and typically have no symptoms.
As cystic fibrosis is a recessive condition, many people who have CF have no known family history. CF is found in all races and ethnicities.
Symptoms of Pediatric Cystic Fibrosis
Cystic fibrosis affects every child differently and can lead to a broad range of symptoms, including:
- Daily cough
- Shortness of breath
- Repeated lung infections
- Persistent nasal congestion or repeated sinus infections
- Poor growth or malnutrition
- Foul-smelling stools or trouble with bowel movements
Our Cystic Fibrosis Pediatric Program follows the proven diagnostic guidelines of the national CF Foundation to accurately identify CF so that your child can begin treatment as soon as possible. Our trained technicians safely and carefully perform the testing, sending samples for processing in our state-of-the-art laboratories.
We have had the best experiences with this team. The attention to detail and connections made with our family make it very family centered.
Child Life Program
Medical care can be overwhelming for children. Our Child Life Specialists help your child cope with stress, prepare for procedures and understand their care. These highly trained specialists offer your family support as you navigate our health system.
Diagnosing Pediatric Cystic Fibrosis
Diagnosing cystic fibrosis as early as possible can make a significant difference in delaying or preventing a child from developing the serious lifelong health issues associated with CF. Studies show that early CF treatment and care leads to healthier children than those who are diagnosed later in life. Prenatal genetic testing may allow expecting parents to learn if their child has the disease or is a CF carrier. For individuals and couples considering pregnancy, carrier screening provides them with the opportunity to discover if they are at risk of conceiving a child with CF.
Most diagnoses of cystic fibrosis take place shortly after birth, during routine newborn screenings that hospitals perform to identify babies born with certain health conditions like CF.
A positive newborn screening result should always be confirmed with more sophisticated testing such as a sweat chloride exam to ensure your child receives the most accurate diagnosis.
Some of the common diagnostic tests our specialists may order for assessing CF in your child include:
- Sweat chloride testing, a non-invasive test to see if your child’s sweat contains more salt than normal. This test is the 'gold standard' for diagnosising CF.
- Genetic testing to analyze blood or saliva for the specific defective gene (CFTR mutation) causing your child’s cystic fibrosis
Treatments for Pediatric Cystic Fibrosis
When you bring your child to Golisano Children's Hospital for CF care, you can trust us to provide your child with the most comprehensive care available in a safe and comfortable setting. Our Cystic Fibrosis Pediatric Program offers clinical services in the cozy, brightly decorated child-friendly environment of Golisano Children’s Hospital. Our certified Child Life specialists are always on hand to help your child feel at ease during appointments and support your family in every way possible.
Major advances in CF therapeutics are keeping more children out of the hospital and helping them to grow and thrive with their disease into adulthood. Because we also offer a comprehensive CF program for patients 18 years and older, we can easily transition young adults from our pediatrics program to the adult clinical services when they are ready.
As members of a network of more than 130 Cystic Fibrosis Foundation-accredited care centers found in every state, we ensure continuity of CF care across the country, which is especially important for college students moving in or out of our service area.
Our top performing CFF-sanctioned center status ensures we can provide your child with the newest and most effective therapies, including:
A variety of drug therapies can be used to treat your child’s cystic fibrosis and reduce symptoms impacting their ability to function and thrive. Your child’s CF provider may prescribe:
- CFTR modulators to treat the underlying cause of CF
- Antibiotics to treat and prevent lung infections
- Mucus-thinning drugs to improve lung function
- Anti-inflammatory medications
- Oral pancreatic enzymes to help the digestive tract absorb nutrients
Chest physical therapy techniques are often used to make breathing easier for children with cystic fibrosis. Your child’s respiratory therapist may use several different therapeutic approaches, including:
- Manual chest percussion, a hands-on technique to loosen mucus
- Mechanical vest, a device worn around the chest that vibrates at high frequency to loosen and thin mucus
- Breathing tools, such as handheld devices (acapella, flutter) and mechanical percussors or IPV
- Exercise is an important part of airway clearance
Therapy may include:
- Nutritional counseling
- Mental health screening and counseling (individual or group support)
- Case management assistance to help with insurance coverage for the proper medications and treatments
In some cases, surgery may be necessary to treat your child’s cystic fibrosis. Common procedures for improving common cystic fibrosis symptoms include:
- Endoscopic sinus surgery, such as polypectomy, to remove blockages from the sinus
- Flexible bronchoscopy and lavage for suctioning mucus from obstructed airways and to obtain samples to identify causes of lung infections
- GI Endoscopy to evaluate digestive disorders
- Feeding tubes to better meet nutritional needs and improve weight gain
- Bowel surgery to treat gastrointestinal blockages, such as meconium ileus, caused by CF-related digestive problems
Transitioning from Pediatric to Adult Specialty Care
Getting ready for adult CF health care, known as a health care transition, is a journey that happens over time. The Children’s Specialty Center at the Golisano Children’s Hospital and the Adult Specialty Clinics at University of Vermont Medical Center are committed to helping our CF patients make a smooth transition from pediatric to adult specialty care.
At around age 13, we work with you and your family to prepare for the transition to the “adult” model of care. Some visits will take place without your family present to assist you in learning how to set health priorities and supporting you to become more independent with your health care. Health care transition typically occurs sometime between 18 to 21 years of age. We will provide resources to help guide you through this transition.
Your health care provider will act as a partner in supporting your health goals along the way. Your health is important to us and our team is here to help you throughout this journey.
Awards & Certifications
Cystic Fibrosis Foundation
Recognized by the Cystic Fibrosis Foundation, our comprehensive program received the Quality Care Award by meeting the rigorous standards of this world leader in the fight against cystic fibrosis by providing expert care and specialized disease management. We have been a foundation-accredited Cystic Fibrosis Care Center since 1968.
Cystic Fibrosis Therapeutics Development Network
As a site of the Cystic Fibrosis Therapeutics Development Network (CF TDN), we contribute to innovations in cystic fibrosis care by conducting breakthrough clinical studies in a wide range of therapeutic areas. Our participation provides patients with many opportunities to enroll in and benefit from clinical trials evaluating the latest cystic fibrosis therapies and procedures.
Locations Near You
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111 Colchester Avenue
Burlington, VT 05401-1473
