Pediatric Cystic Fibrosis Diagnosis

Cystic fibrosis management is complex, so getting treatment at a center that specializes in cystic fibrosis and pediatric pulmonology is the best option. The Pediatric Cystic Fibrosis Program at Fletcher Allen is a multidisciplinary clinic focused on the care and treatment of children with cystic fibrosis.  

At Fletcher Allen in Burlington, Vermont, our pediatric cystic fibrosis team works together to provide effective and timely treatment. Meet our:

Cystic Fibrosis Nurse Coordinator - The Cystic Fibrosis (CF) Nurse Coordinator is responsible for coordination of the entire CF Program, and will work with you in the following capacities: 

  • Managing the CF clinic schedule and appointments
  • Assigning your visits with the CF team members
  • Scheduling tests and procedures 
  • Education 
  • Conducting pre- and post-clinic conferences 

In addition to the roles played related directly to you and your families care, the coordinator also provides education to schools, day care, nursing, medical students, and other health care providers and serves as the research coordinator for several research studies.

Respiratory Therapist - A Respiratory Therapist (RT) is trained to look at lung health. RT’s make suggestions to the physician about what is available and teach patients, their families and others such as day care providers and school nurses how to perform proper airway clearance.  When in clinic an RT will collect spit (called sputum) samples, perform Pulmonary Function Tests (PFT’s), and teach and review airway clearance.

Registered Dietician - At each clinic visit, the Clinic Dietitian checks weight and height to be sure your child is growing properly. The dietician is also available to work with patients and families to improve food choices for better growth and overall health when necessary, and to help parents work with picky eaters and other nutrition challenges related to their CF and growth.

Social Worker - Social workers help to improve the patients and their family’s quality of life by offering information, resources and support services. Working with the medical team, patient and family, the social worker also helps plan the care and treatment of patients.

Child Life Specialist - The health care environment brings with it many new and sometimes stressful experiences for children, teenagers, and families. Child Life staff work with patients and their families to help them understand and cope with these experiences. We provide opportunities for children to grow and develop normally.  While in the hospital, we prepare patients and families for procedures, and we help them cope with all aspects of their health care experiences.

Pediatric Cystic Fibrosis Diagnosis

You may have genetic counseling either before or after you conceive.  A genetic counselor helps you understand how cystic fibrosis runs in families. The counselor also arranges genetic testing and interprets these tests for you and your family.

Newborn screening (NBS) for CF is now mandatory across the United States because early diagnosis is tied to better health.  The test involves a small blood sample taken from a heel stick shortly after birth. The sample is already used to test for other congenital metabolic disorders.

Many infants with CF have evidence of injury to the pancreas. The NBS test measures an immunoreactive trypsinogen (IRT) level which, when elevated, may be a sign of this injury. Babies with high IRT levels then have their blood sample tested for a limited number of CF mutations. Depending on the level, they are referred for a sweat test at an accredited CF Center and further evaluation if necessary. Many infants with elevated IRT levels, however, do not have CF. Therefore, it is important that the other testing occur. Even though the IRT level is a good NBS for CF, there are some infants who will be missed with this test.

Learn more about treatments for Pediactric Cystic Fibrosis.

Find a Fletcher Allen physician or call 802-847-8600.