Cystic Fibrosis
Medical Center
Pulmonary and Critical Care Medicine
111 Colchester Avenue
Main Campus, East Pavilion, Level 5
Burlington, VT 05401-1473
Cystic Fibrosis is an inherited condition that affects the lungs and digestive system. It is life-threatening and requires daily care. With advances in diagnosis & treatment, people with the condition are living longer, better lives.
Cystic Fibrosis Care at UVM Medical Center
Cystic Fibrosis is a condition where the cells that make mucus, sweat and digestive juices are thick and sticky instead of thin and slippery. These secretions end up blocking ducts, passageways
Due to its complex nature, it is best to get treatment at a center that specializes in Cystic Fibrosis. Each of our patients is scheduled for 4 visits per year. Routine care for our Cystic Fibrosis patients includes:
- Annual chest CT scans
- DEXA bone scans
- Oral glucose tolerance tests
- Yearly blood work
Cystic Fibrosis Diagnosis
At the UVM Medical Center, we are members of the Northern New England Cystic Fibrosis Consortium. We participate in a variety of clinical trials to help with the discovery of new therapies. When you come to UVM Medical Center for diagnosis, our specialists evaluate the following symptoms of cystic fibrosis:
- A cough that doesn't seem to go away and makes thick mucus and spit (called sputum)
- Wheezing
- Breathlessness
- A decreased ability to exercise
- Repeated lung infections
- Inflamed nasal passages or a stuffy nose
- Bad-smelling, greasy bowel movements
- Reduced weight gain and growth
- Intestinal blockage
- Severe constipation
Based on your symptoms, our doctor may prescribe any of the following tests in order to diagnose Cystic Fibrosis:
- Sweat test - your sweat is collected and tested to see if it's saltier than normal.
- Genetic testing - blood or saliva can be checked for the specific defective gene that's responsible for cystic fibrosis.
Treatments for Cystic Fibrosis
The treatment options for cystic fibrosis at The University of Vermont Medical center include:
- Medications, including:
- Antibiotics - to treat and prevent lung infections
- Mucus-thinning drugs - to help you cough up the mucus, which improves lung function
- Bronchodilators - help keep your airways open
- Oral pancreatic enzymes - help your digestive tract absorb nutrients
- Chest physical therapy, including:
- Chest clapper - a hand-held machine that helps loosen mucus
- Inflatable vest - a device worn around the chest that vibrates at high frequency
- Breathing tools - typically a tube or mask that you exhale through while doing breathing exercises
- Oxygen therapy - breathing pure oxygen can help prevent high blood pressure in the lungs (also called pulmonary hypertension)
- Lung rehabilitation, including:
- Exercise training
- Nutritional counseling
- Techniques to save energy
- Breathing exercises
- Counseling or group support or both
- Surgical procedures, including:
- Nasal polyp removal - if your breathing is obstructed
- Endoscopy and lavage - mucus is suctioned from obstructed airways through an endoscope
- Feeding tube - a feeding tube can deliver extra nutrition while you sleep by threading the tube through your nose to your stomach or surgically implanted into the abdomen
- Bowel surgery - to remove a bowel blockage or if a section of bowel has folded in on itself