CFTR Proteins

What are CFTR proteins and what do they do?

CFTR proteins are found on the surface of some cells in your body, for example, the cells that line the airways in your lungs. Normal CFTR proteins act like channels on the surface of the cells. They open and close to let chloride ions move into and out of the cell—a process called “gating.” This helps maintain a healthy balance of salt and water in the tissues.

Normal CFTR proteins

Proteins that work as they should allow chloride ions to pass into or out of the cells.

Clear Airway

Little particles called chloride ions move into and out of the cells through CFTR protein channels to help keep a balance of salt and water in the lungs. Mucus stays thin and watery, so the lungs can work well.


What is KALYDECO® (ivacaftor)?

KALYDECO is a prescription medicine used for the treatment of cystic fibrosis (CF) in patients age 2 years and older who have at least one mutation in their CF gene that is responsive to KALYDECO.

Talk to your doctor to learn if you have an indicated CF gene mutation.

It is not known if KALYDECO is safe and effective in children under 2 years of age.

Important Safety Information

Who should not take KALYDECO?

Do not take KALYDECO if you take certain medicines or herbal supplements such as: the antibiotics rifampin (Rifamate®, Rifater®) or rifabutin (Mycobutin®); seizure medications such as phenobarbital, carbamazepine (Tegretol®, Carbatrol®, Equetro®) or phenytoin (Dilantin®, Phenytek®); or St. John’s wort.

Talk to your doctor before taking KALYDECO if you take any of these medicines or supplements.