Cystic Fibrosis Medications: What You Need to Know
Cystic fibrosis is a life-threatening, genetic disease that affects patients’ ability to breathe and is marked by persistent lung infections. While it currently has no cure, a number of treatments, therapeutics, and supplements exist to help cystic fibrosis patients maintain their health and well-being. These include medications approved for the disease, but treatment plans are unique and tailored to each patient’s specific health characteristics and needs. Cystic fibrosis medications range from CFTR modulators and enzyme supplements, to mucolytics, antibiotics, and vitamins.
NOTE: The following is not necessarily an exhaustive list of cystic fibrosis medications and treatments. Cystic Fibrosis News Today continues to add to its resource pages for cystic fibrosis and relevant medications, treatments, and supplements. Have a suggestion? Be sure to contact us.
CFTR Modulators for Cystic Fibrosis
The cystic fibrosis transmembrane conductance regulator (CFTR) is a protein that functions as a channel across the membrane of cells that produce mucus, sweat, saliva, tears, and digestive enzymes. The channel transports negatively charged particles called chloride ions in and out of cells, to help control the movement of water in tissues, which is necessary for the production of thin and freely flowing mucus. Cystic fibrosis patients have mutations in the CFTR gene — more than 1,000 have been identified, according to the National Institutes of Health — that cause a malfunction in the CFTR protein, resulting in a buildup of mucus, persistent lung infections, pancreatic damage, and other complications. CFTR modulators are the main course of treatment for cystic fibrosis and include:
Ivacaftor (Kalydeco)
Enzymes to Treat Cystic Fibrosis
According to the Cystic Fibrosis Foundation (CFF), 87 percent of people with cystic fibrosis need to take enzymes. These supplements come in the form of oral capsules that work in the intestines to help patients absorb nutrients from their food. Each capsule has small beads with digestive enzymes, and is covered with a special enteric coating to allow the beads to dissolve in the small intestine. It is important that patients take the right amount of enzymes, just before a meal, and should check with the dietitian on their CF care team for the exact amount of enzymes to take. Enzymes help patients digest carbohydrates, proteins and fats; gain and maintain a healthy weight; and absorb essential nutrients, such as vitamins and minerals. Enzymes for patients with cystic fibrosis include:
Pancrelipase (Cotazym, Cotazym-S, Creon, Dygase, Ku-Zyme, Ku-Zyme HP, Kutrase, Lapase, Lipram, Lipram UL, Palcaps 10, Pancrease MT, Pancreaze, Pancrecarb MS, Pangestyme CN 10, Pangestyme EC, Panocaps, Panocaps MT 16, Panokase, Pertzye, Ultrase, Viokace, Viokase, Zenpep)
Pancreatin (Hi-Vegi-Lip, Pan-2400, Pancreatin 4X)
Mucolytics for Patients with Cystic Fibrosis
Mucolytics are in a category of cystic fibrosis medications designed to help thin mucus in the lungs so patients can cough and expel mucus more easily. These inhaled medications “cut up” the DNA strands outside the cell that are responsible for making CF mucus thick and sticky. The DNA strands are formed in the white blood cells, and are able to fight lung infections. By moving the mucus out of the lungs, the damage caused by chronic lung infections is delayed or reduced. The class of mucolytic medications include:
Dornase alfa (Pulmozyme)
Hypertonic saline
Antibiotics to Help Cystic Fibrosis Patients
Lung infections are particularly common in cystic fibrosis, and antibiotics are used to fight or control infection-causing bacteria. There are oral antibiotics (liquids, tablets or capsules to be swallowed), intravenous (IV) antibiotics (liquid medicine administered directly into the blood through a catheter), and inhaled antibiotics (an aerosol or mist inhaled directly to the airways). The choice of antibiotic drug, dosage, and duration of treatment depend on the patient and the infection, but the options include:
Tobramycin inhalation solution (TOBI, Bethkis)
Aztreonam for inhalation solution (Cayston, Azactam)
Tobramycin inhalation powder (TOBI Podhaler)
Azithromycin (Zithromax, Zmax)
Amikin (Amikacin)
Gentamicin (Garamycin)
A fuller list is also available on the CFF website.
Vitamins to Ease Cystic Fibrosis Symptoms
Vitamins can help the bodies of patients with cystic fibrosis to grow, function better, and fight infections. While vitamin A is used to improve vision, bone and tooth formation, cell function, and immunity, vitamin D helps to build and maintain strong bones and teeth. Vitamin E is particularly important as it is an antioxidant that protects compounds in the body from combining with oxygen, keeping red blood cells healthy and helping to fight infections. In addition, vitamin K helps the blood to clot and helps maintain the health of the bones. Some vitamins of note include:
Alpha E
Amino-Opti-E
Aqua Gem-EAqua-E
Aquasol E
Aquavite-E
Centrum Singles-Vitamin E
E Pherol
E-400 Clear
E-600
E-Gems
Nutr-E-Sol
Vita-Plus E Natural
Note: Cystic Fibrosis News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.
