Cystic Fibrosis

Cystic Fibrosis Care at UVM Health

At University of Vermont Health, our specialists diagnose, manage and treat all aspects of cystic fibrosis (CF) in adults and pediatric CF in children. As an accredited Cystic Fibrosis Care Center since 1968, we are the only program in the area that meets the rigorous standards of the Cystic Fibrosis Foundation.

Our focus on preventive, high-quality care will help you manage your symptoms and disease progression.

A Porter Hospital physician listens to a patients lungs with a stethoscope.

Why Choose UVM Health?

We offer:

Experienced team: Our committed, compassionate and board-certified providers work closely with a multi-disciplinary care team including nursing, social work, respiratory therapy, psychology, pharmacy, nutrition, otolaryngology (ENT) and endocrinology.
Evidence-based care: Our providers are national leaders and active researchers, providing you with research-driven treatment plans grounded in the most recent discoveries in CF. Participating in the Cystic Fibrosis Therapeutics Development Network (CF TDN), the largest CF clinical trials network in the world, we conduct groundbreaking research to provide you with access to the latest medications and therapies available for treating cystic fibrosis.

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 UVM Health - UVM Children’s Hospital and the Adult Specialty Clinics at UVM Health - UVM Medical Center are committed to helping our pediatric CF patients make a smooth transition from pediatric to adult specialty care.

Transitioning 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.

Ongoing Support

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.

Treatments We Offer

Significant advances have been made in cystic fibrosis therapies in recent years. More patients are able to stay out of the hospital and live healthier lives.

As a Cystic Fibrosis Foundation-accredited center, UVM Health gives you access to the newest and most effective therapies for cystic fibrosis.

Medications for Cystic Fibrosis

A variety of medications can be used to treat cystic fibrosis. Our pharmacist will review your medications, provide education and identify any issues that could interfere with your recommended treatment. You may be prescribed:

Cystic fibrosis transmembrane conductance regulator (CFTR) modulators: Treat the underlying dysfunction of cystic fibrosis
Antibiotics: Treat and prevent lung infections
Mucolytics: Thin sputum and make it easier to expectorate/cough up
Bronchodilators: Keep lung airways open
Oral pancreatic enzymes: Help the digestive tract absorb fat

Respiratory Therapy

Our respiratory therapist will evaluate your airway clearance, nebulized medications and equipment. We may ask that you bring your equipment to a clinic visit. They can help you with ordering or replacing any equipment that you need, such as a nebulizer or an airway clearance VEST.

Pulmonary Rehabilitation for Cystic Fibrosis

Our nationally recognized pulmonary rehabilitation program combines exercise, education and psychosocial support to strengthen your physical and emotional well-being.

Surgical Procedures for Cystic Fibrosis

In some cases, surgery may be part of your care plan. Common procedures include:

Polypectomy: Removes polyps or tissue growths inside nasal passages and sinuses
Bronchoscopy: Suctions mucus from obstructed airways
Feeding tubes: To better meet nutritional needs and improve weight gain
Colonoscopy: Screens for colon cancer

Nutrition

Our dietitian will assess your overall nutritional status, evaluate your diet and make recommendations. They will also follow up on nutrition-related labs and, if needed, provide comprehensive diabetes care.

Exercise

Routine exercise can be beneficial for airway clearance, or addition to current airway clearance regimen. We encourage routine exercise and can offer support in developing safe and sustainable individualized exercise recommendations.

Annual and Routine Care

You will receive annual evaluations by each member of your care team. Routine testing and treatments include:

Chest imaging: Looks for abnormal mucus or dilated airways in the lungs
DEXA scans: Measures bone density and check for CF-related osteoporosis and osteopenia
Oral glucose tolerance tests: For diagnosing CF-related diabetes and/or impaired glucose intolerance
Blood work: To evaluate cell counts, various blood chemistries and markers of inflammation
Sputum cultures: To keep tabs on bacteria common in cystic fibrosis and inform antibiotic treatments
Immunization: For flu, pneumococcal pneumonia and COVID-19

Awards & Certifications

Cystic Fibrosis Foundation

Recognized by the Cystic Fibrosis Foundation, our comprehensive program meets 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.