Cystic Fibrosis (CF)
About Cystic Fibrosis (CF)
Cystic Fibrosis is a rare inherited disease that mainly affects the respiratory and digestive systems. The mucus normally secreted by cells in the lung airways (breathing tubes), pancreatic ducts, gastrointestinal tract, and the reproductive system becomes thickened and blocked, causing frequent infections and loss of function in the affected organs.
How Common Is Cystic Fibrosis?
There are about 30,000 people with cystic fibrosis in the United States and approximately 70,000 people worldwide. Approximately 1 in 30 Americans is a carrier.
What Causes Cystic Fibrosis?
Cystic fibrosis affects the production of a protein called the cystic fibrosis transmembrane conductance regulator (CFTR) protein. People with CF produce a faulty CFTR protein that affects the cells of the body that produce mucus and sweat.
Who Can Get Cystic Fibrosis?
Cystic Fibrosis is an inherited genetic condition. Both parents must be carriers of an abnormal gene that gets passed on to their child for the child to have CF. Approximately 1 in 30 Americans is a carrier.
What Are the Symptoms of Cystic Fibrosis?
CF symptoms can vary depending on the severity of the disease, and symptoms may worsen or improve over time. Patients diagnosed with CF in adulthood may experience milder symptoms.
CF causes thick and sticky mucus that can clog the airways of the lungs, leading to symptoms that include:
- Persistent cough
- Thick mucus (sputum)
- Difficulty with exertion/exercise
- Repeated lung infections
- Inflammation of nasal passages
In addition to respiratory symptoms, CF can cause digestive and reproductive issues.
Can Cystic Fibrosis Be Cured?
There is currently no cure for CF, but treatment is available. Because of improved care, the average life expectancy has been steadily increasing. As we develop a better understanding of this condition, we are researching new avenues for treatment. Clinical trials offer CF patients an opportunity to access the latest and most advanced treatments available.
How Is Cystic Fibrosis Treated?
There have been many advances in CF treatment. Patients can take medications to help thin and clear the thick mucus from the airways, enzymes to help absorb fat and nutrients, and antibiotics to treat infections.
There are also new treatments that target fixing the CFTR protein. Recent medications called modulators significantly improve symptoms of CF and may slow the disease process in many individuals.
In severe cases, lung transplantation may be an option.
Cystic Fibrosis Care at Columbia
The Gunnar Esiason Adult Cystic Fibrosis and Lung Program at Columbia University Irving Medical Center is a premier site for adult cystic fibrosis (CF) care and research in the New York region. We offer a comprehensive approach to the diagnosis and treatment of cystic fibrosis and related conditions in adults, and our patients benefit from integration with other leading Columbia programs.