Treating cystic fibrosis.

There’s no cure for cystic fibrosis (CF) today. But you can manage it. You can watch for symptoms. You can take your medications. Eating a healthy diet and regular exercise can help, too. You and your doctor work together to find the best treatment plan for you. Be sure to follow all your doctor’s and CF care team’s  instructions. This helps you get the most benefit from your CF treatments.

What do CF treatments do? They can help:

• Prevent and control lung infections
• Loosen and remove the thick, sticky mucus from your lungs
• Prevent blockages in your intestines
• Make sure you get nutrients from your food
• Enhance or even restore defective CFTR proteins and salt and chloride channels

Here are more things you can do to help manage CF:

• Do airway clearance techniques (ACTs) as your health care provider shows you
• Stick to a healthy eating plan
• Exercise and stay active
• Take steps to help prevent infections 
• Take your medications exactly as prescribed
• Keep all appointments with your doctor and health care team

Treating CF with medications.

There are many medications used to help treat CF.

These drugs are called cystic fibrosis transmembrane conductance regulators (CFTR). They target the cause of CF.

Alyftrek (vanzacaftor/tezacaftor/deutivacaftor)
Alyftrek helps the CFTR protein get to the cell surface and work the right way.

It’s used by people with at least one copy of a specific gene mutation (F508del), or who have at least one mutation in the CFTR gene that is responsive to vanzacaftor/tezacaftor/duetivacaftor. Alyftrek is approved for patients 6 years and older. It’s a pill taken by mouth once daily with food that contains fat. It helps improve lung function, helps weight gain, reduces breathing flares and improves sweat chloride levels (salt levels in sweat).

Kalydeco (ivacaftor)
Kalydeco helps the CFTR protein work the right way. It may be prescribed for patients 1 month and older with certain CFTR gene mutations. It’s taken by mouth (as a pill for patients 6 years and older, or a granule pouch for patients 1 month to less than 6 years old) every 12 hours with food that contains fat. Kalydeco helps improve lung function, lower sweat chloride levels and helps with weight gain.

Orkambi (lumacaftor/Ivacaftor)
Orkambi is for anyone with two copies of a specific gene mutation (F508del) that caused their CF. It helps the CFTR protein get to the cell surface and work the right way. It’s taken by mouth (as a pill for patients 6 years and older, or a granule pouch for patients 12 months to less than 6 years old) every 12 hours with food that contains fat. It helps improve lung function, lower sweat chloride levels, helps weight gain and helps reduce breathing flares.

Symdeko (tezacaftor/ivacaftor and ivacaftor)
Symdeko helps the CFTR protein get to the cell surface and work the right way. It’s used by people with two copies of a specific gene mutation (F508del), or who have at least one mutation in the CFTR gene that is responsive to tezacaftor/ivacaftor. Symdeko is approved for patients 6 years and older and is a pill taken by mouth every 12 hours with food that contains fat. It helps improve lung function, helps weight gain and reduces breathing flares.

Trikafta (elexacaftor/tezacaftor/ivacaftor and ivacaftor)
Trikafta helps the CFTR protein get to the cell surface and work the right way. It’s used by people with at least one copy of a specific gene mutation (F508del), or who have at least one mutation in the CFTR gene that is responsive to elexacaftor/tezacaftor/ivacaftor. Trikafta is approved for patients 2 years and older. It’s taken by mouth (as a pill for patients 6 years and older, or a granule pouch for patients 2 years to less than 6 years old) every 12 hours with food that contains fat. It helps improve lung function, helps weight gain, reduces breathing flares and helps improve breathing symptoms.

Other medications can help control the CF symptoms in different parts of your body.

Respiratory system.

• Bronchodilators: Inhaled medications (such as albuterol) that open up the airways to lessen wheezing and shortness of breath
• Mucus thinners: Medications to help thin your mucus so it’s easier to cough up
• Pulmozyme: A nebulized form of medication to help make mucus less sticky
• Bronchitol (mannitol): A dry powder used with other medications to improve lung function
• Anti-inflammatories: Medications (such as ibuprofen, Flovent, and Advair) that reduce swelling in the lungs to help you breathe easier
• Antibiotics: Medications to treat infections of the lungs or prevent your CF symptoms from getting worse, like Cayston, Tobi, Tobi Podhaler, Tobramycin, Bethkis, Kitabis, Zithromax or Cipro
• Hydrators: Medications that help moisten thick mucus, like nebulized hypertonic saline

Digestive system.

• Pancreatic enzymes help you digest your food, like Pancreaze, Creon, Pertzye, Zenpep, Viokace and Ultresa
• Fat-soluble vitamin supplements like ADEKs replace vitamins that don’t get absorbed well enough from the foods you eat
• Diabetes medications, usually insulin, help manage cystic fibrosis-related diabetes (CFRD)  
• Drugs like Zantac, Prilosec and Pepcid help lower acid in your stomach

We’re here to help.

Managing your CF can be a challenge. But you don’t have to do it alone. If you have questions about your CF treatment, call your CVS Specialty® CF CareTeam at 1-866-845-6790 or your CF treatment center.