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What is Cystic Fibrosis?

Hear from Dr. Donaldson on Cystic Fibrosis on the Chairs Corner Podcast


Cystic Fibrosis (CF) is the most common lethal genetic disease in the Caucasian population, affecting one in 3300 births. Other ethnic populations are affected less frequently, ranging from one in 10,000 – 15,000 births in Hispanic and African-American populations, to one in 30,000 Asian births. The CF gene is responsible for controlling salt and water transport across the cells lining the lung, pancreas, and other organs. When this gene is abnormal, secretions in these organs become dehydrated and sticky, and eventually clog airways and may block other organs (pancreas, intestines, male reproductive tract, bile ducts) as well. CF has a number of common symptoms, which may include: salty-tasting skin; persistent coughing, wheezing, or recurrent pneumonia; failure to gain weight despite a big appetite; and frequent, bulky stools. The standard test used to diagnose CF is the sweat test. This simple, painless test measures the amount of salt in sweat. Direct testing for abnormalities in the CF gene (from a blood test) is possible most of the time.

The treatment for CF lung disease is based upon the clearance of thick airway secretions and treating infections.  Highly effective, mutation specific therapies that target the genetic defect in CF are now available for 90% of adults with cystic fibrosis. Symptomatic therapies include use of devices that help clear mucus from airways, inhaled mucus modifying treatments and exercise. Antibiotics that fight the particular bacteria found in CF are used when the amount of infection in the chest becomes problematic, and may be administered by mouth, inhaled, or intravenously. The maintenance of proper nutrition is also essential, and requires a high calorie diet, supplementation of certain vitamins, and the use of enzymes that help to digest meals.

Clinical Services

At UNC, adult and pediatric CF patients are cared for in separate, multidisciplinary CF clinics. Staffed by physicians and advanced practice providers, our robust team includes clinical nurse coordinators, dietitians, respiratory therapists, social workers, mental health coordinators, pharmacists, and research personnel with special expertise in CF. We have a strong behavioral health collaborative care model with colleagues in Psychiatry, and we co-manage patients with colleagues in Endocrinology, Gastroenterology, Reproductive Health and Transplant Pulmonology when needed.
If you are interested in learning more about the adult CF clinic, please click here.
For appointments, please call the clinic at: 984-974-5703. Referring physicians can reach out to the CF Nurse Coordinator via email with any questions.
For inquiries into the pediatric CF clinic please visit UNC Pediatric Pulmonology or call 919-966-1055.

Clinical Studies

Click here to learn about clinical and translational studies being carried out by our team.

Additional Resources

  • Cystic Fibrosis Foundation (CFF): A non-profit US organization working to cure CF and help patients suffering from the disease.
  • Clinical Trials Finder is a website maintained by the CFF that lists all active and completed clinical trials in CF. We’re not able to do every study here, but if you find a trial on this website that you are interested in, we will help connect you!

CF Patient Registry

The CF Patient Registry collects information on the health status of people with cystic fibrosis who receive care in CF Foundation-accredited care centers and agree to participate in the Registry. This information is used to create CF care guidelines, assist care teams providing care to individuals with CF and guide quality improvement initiatives at care centers. Researchers also use the Patient Registry to study CF treatments and outcomes and to design CF clinical trials. For further information visit the CF Foundation’s Patient Registry website or contact the Adult CF Nurse Coordinator.

Quality Improvement

The Cystic Fibrosis Quality Improvement team has been involved in many national QI collaboratives sponsored by the CF Foundation, both as participants and as QI coaches and faculty. In 2019, we were recognized for our efforts by the Cystic Fibrosis Foundation Quality Care Award. Our entire multidisciplinary team participates in our QI initiatives, and we continue to seek and receive input from patients and CF caregivers. The team members translate this feedback into new ways of delivering care, with the goal of prolonging and improving the lives of our patients. Recent and upcoming projects have focused on:

  • Improving CF-related diabetes care
  • Home spirometry program
  • Reproductive Health Collaboration
  • Virtual Patient Support Groups/Mental Health
  • Food Insecurity
  • C.F. RISE
  • Elevating Remote CF Care: CF@Home Program

We Need You!

The CF Quality Improvement team at UNC is eager to hear your input and ideas. If you as a patient or family member have any feedback or suggestions- no matter how small- please do not hesitate to contact us.