Treating cystic fibrosis.

While there is no cure for cystic fibrosis (CF), you can manage it with medications and other treatments. Your doctor will work with you to find the best treatment for you. Be sure to follow your doctor’s instructions exactly to get the most benefit from your CF treatments.

Treatments for CF are designed to help:

• Prevent and control infections in your lungs
• Loosen and remove the thick, sticky mucus from your lungs
• Prevent blockages in your intestines
• Make sure you absorb the nutrients you need

In addition to the treatment your doctor prescribes, there are things you can do to help manage your condition. Things under your control include:

• Using airway clearance techniques (ACTs) as your doctor told you Sticking to a healthy eating plan that’s right for you
• Exercising on a regular basis
• Taking precautions to reduce infection risk
• Staying on track with your medications and taking them exactly as prescribed
• Keeping all appointments with your doctor and other members of your health care team

Treating CF with medications.

There are a variety of medications used to help treat CF. Kalydeco®, Orkambi® and Symdeko® help treat the disease itself.

• Kalydeco (ivacaftor)
  Kalydeco is a cystic fibrosis transmembrane conductance regulator (CFTR) modulator. It helps the CFTR protein work the right way in patients 12 months and older with certain CF gene mutations. It’s taken by mouth (as a pill for patients six years and older or a granule pouch for patients 12 months to less than six years old) twice a day with fat-containing food. Kalydeco helps improve lung function, lower sweat chloride levels and increase weight gain.
• Orkambi (lumacaftor/Ivacaftor)
  Orkambi is a combination CFTR potentiator and corrector. For patients two years old and older with two copies of a specific gene mutation (F508del), it helps the CFTR protein get to the cell surface and work the right way. It’s a pill taken by mouth twice a day with fat-containing food. It helps improve lung function, lower sweat chloride levels, increase weight gain and lower pulmonary exacerbations.
• Symdeko (tezacaftor/ivacaftor and ivacaftor)
  Symdeko is a combination CFTR potentiator that helps the CFTR protein get to the cell surface and work the right way in 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 12 years and older and is a pill taken by mouth twice daily with fat-containing food. It helps improve lung function, increase weight gain and reduce pulmonary exacerbations.

Other medications are used to control the CF symptoms that may affect different parts of your body.

Respiratory system.

• Bronchodilators: Inhaled medications that open up the airways to ease wheezing and shortness of breath (such as albuterol)
• Mucus thinners: Medications to help make mucus less thick and easier to cough up
• Pulmozyme®: A nebulized form of human recombinant deoxyribonuclease (DNase) that reduces mucus stickiness
• Anti-inflammatories: Medications that reduce swelling in the lungs and ease breathing (such as ibuprofen, Flovent® and Advair®)
• Antibiotics: Medications to treat infections of the lungs or prevent your CF symptoms from getting worse (such as Cayston®, TOBI®, TOBI® Podhaler®, Bethkis®, Kitabis®, Zithromax®, Cipro®)
• Hydrators: Medications that help increase moisture in thick mucus (such as nebulized hypertonic saline)

Digestive system.

Pancreatic enzymes: Medications that aid in digestion (such as Pancreaze®, Creon®, Pertzye®, Zenpep®, Viokace®, Ultresa®)

Fat-soluble vitamin supplements: These replace vitamins not absorbed from the foods you eat (such as ADEKs)

Diabetes medications: Used to treat patients who develop cystic fibrosis related diabetes (CFRD), typically insulin is the treatment for CFRD rather than oral medications

Acid reducers: To reduce the production of acid in the stomach (such as Zantac®, Prilosec®, Pepcid®)

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.