Elexacaftor, Tezacaftor, and Ivacaftor

The combination of elexacaftor, tezacaftor, and ivacaftor may cause life-threatening liver damage. Tell your doctor if you have or have ever had liver disease. If you experience any of the following symptoms, stop taking the combination of elexacaftor, tezacaftor, and ivacaftor and call your doctor immediately: pain, swelling, or discomfort in the upper right abdominal area, yellowing of your skin or eyes, confusion, dark urine, nausea, vomiting, or loss of appetite.

Keep all appointments with your doctor and the laboratory. Your doctor will order certain tests to check your body's response to the combination of elexacaftor, tezacaftor, and ivacaftor before and during your treatment.

You will be given the Medication Guide. Read the information carefully and ask your doctor or pharmacist if you have any questions.

The combination of elexacaftor, tezacaftor, and ivacaftor is used to treat certain types of cystic fibrosis (CF; an inherited condition that causes affects the lungs and other parts of the body). Elexacaftor and tezacaftor are in a class of medications called cystic fibrosis transmembrane conductance regulator (CFTR) correctors. Ivacaftor is in a class of medications called cystic fibrosis transmembrane conductance regulator (CFTR) potentiators. These medications work by improving the function of a protein in the body to decrease the build-up of thick mucus in the lungs and improve other CF symptoms.

Elexacaftor, tezacaftor, and ivacaftor come as tablets and as granules to take by mouth. Take with fatty foods twice a day, 12 hours apart. Take elexacaftor, tezacaftor, and ivacaftor at around the same times every day.

Take elexacaftor, tezacaftor, and ivacaftor exactly as directed. Do not take more or less of it or take it more often than prescribed by your doctor.

If you are taking the tablets, each daily dose is usually given as 2 different tablets that have different ingredients. One tablet (combination of elexacaftor, tezacaftor, and ivacaftor) is orange and the other tablet (ivacaftor) is blue. Your doctor will probably tell you to take elexacaftor, tezacaftor, and ivacaftor (2 orange tablets) every morning and ivacaftor (1 blue tablet) every evening.

Swallow the tablets whole; do not split, chew, or crush them.

If you are taking the granules, each daily dose is usually given as 2 different packets of granules: one packet contains granules of elexacaftor, tezacaftor, and ivacaftor and the other packet contains granules of ivacaftor. Your doctor will probably tell you to take 1 packet of elexacaftor, tezacaftor, and ivacaftor granules every morning and 1 packet of ivacaftor granules every evening.

To prepare a dose of elexacaftor, tezacaftor, and ivacaftor granules, mix the entire packet of granules in 1 teaspoon (5 mL) of cold or room temperature soft food or liquid, such as pureed fruits or vegetables, yogurt, applesauce, water, milk, or juice. Take the mixture within 1 hour of mixing the granules with food or a liquid.

Take elexacaftor, tezacaftor, and ivacaftor tablets and granules with fatty foods such as eggs, butter, nuts, peanut butter, cheese pizza, and whole-milk dairy products (such as whole milk, cheese, and yogurt). Talk to your doctor about other fatty foods to eat with these medications.

The combination of elexacaftor, tezacaftor, and ivacaftor controls cystic fibrosis, but does not cure it. Continue to take these medications even if you feel well. Do not stop taking these medications without talking to your doctor.

This medication may be prescribed for other uses; ask your doctor or pharmacist for more information.

Before taking the combination of elexacaftor, tezacaftor, and ivacaftor,

• tell your doctor or pharmacist if you are allergic to this medication, any part of this medication, or any other medications, foods or substances. Tell your doctor or pharmacist about the allergy and what symptoms you had.
• tell your doctor and pharmacist what prescription and nonprescription medications, vitamins, nutritional supplements, and herbal products you are taking or plan to take while taking the combination of elexacaftor, tezacaftor, and ivacaftor. Your doctor may need to change the doses of your medications and monitor you carefully for side effects.
• you should know that the following nonprescription or herbal products may interact with elexacaftor, tezacaftor, and ivacaftor: St. John's wort. Be sure to let your healthcare provider know that you are taking this medication before you start taking elexacaftor, tezacaftor, and ivacaftor. Do not start this medication while taking elexacaftor, tezacaftor, and ivacaftor without talking to your healthcare provider.
• tell your doctor if you have or have ever had kidney disease.
• tell your doctor if you are pregnant, plan to become pregnant, or are breast-feeding. If you become pregnant while taking the combination of elexacaftor, tezacaftor, and ivacaftor, call your doctor.
• you should know that depression, anxiety, thinking about or committing self harm or suicide, and sleep difficulties have been reported with elexacaftor, tezacaftor, and ivacaftor. Before beginning elexacaftor, tezacaftor, and ivacaftor, tell your doctor if you have or have ever had depression, anxiety, thoughts of harming yourself or attempting suicide in the past, or sleep difficulties. During treatment, call your doctor right away if you start experiencing any of the following: changes in mood, mood swings, difficulties regulating emotion, trouble sleeping, anxious thoughts, or thoughts of harming yourself or others.

Do not eat grapefruit or drink grapefruit juice while taking these medications.

Take the missed dose as soon as you remember if it is within 6 hours of the missed dose. However, if it is more than 6 hours, skip the missed dose and continue your regular dosing schedule.  Do not take a double dose to make up for a missed one.

The combination of elexacaftor, tezacaftor, and ivacaftor may cause side effects. Tell your doctor if any of these symptoms are severe or do not go away:

• headache
• runny nose, sneezing, and stuffiness; fever; cough; or other signs of infection
• diarrhea
• gas
• pink eye
• stomach pain

Some side effects can be serious. If you experience any of these symptoms or those listed in the IMPORTANT WARNING section, call your doctor immediately or get emergency medical treatment:

• rash; itching; hives; difficulty breathing or swallowing; swelling of the face eyes, lips, tongue, mouth, or throat; or lightheadedness or dizziness
• unusual headache, blurred vision, double vision, or vision loss

The combination of elexacaftor, tezacaftor, and ivacaftor may cause cataracts (clouding of the lens of the eye that may cause vision problems) in children and teenagers. Children and teenagers taking elexacaftor, tezacaftor, and ivacaftor should see an eye doctor before and during their treatment. Talk to your child's doctor about the risks of giving elexacaftor, tezacaftor, and ivacaftor to your child.

The combination of elexacaftor, tezacaftor, and ivacaftor may cause other side effects. Call your doctor if you have any unusual problems while taking this medication.

If you experience a serious side effect, you or your doctor may send a report to the Food and Drug Administration's (FDA) MedWatch Adverse Event Reporting program online (https://www.fda.gov/Safety/MedWatch) or by phone (1-800-332-1088).

Keep this medication in the container it came in, tightly closed, and out of reach of children. Store it at room temperature and away from excess heat and moisture (not in the bathroom).

Keep all medication out of sight and reach of children as many containers are not child-resistant. Always lock safety caps. Place the medication in a safe location – one that is up and away and out of their sight and reach. https://www.upandaway.org

Dispose of unneeded medications in a way so that pets, children, and other people cannot take them. Do not flush this medication down the toilet. Use a medicine take-back program. Talk to your pharmacist about take-back programs in your community. Visit the FDA's Safe Disposal of Medicines website https://goo.gl/c4Rm4p for more information.

In case of overdose, call the poison control helpline at 1-800-222-1222. Information is also available online at https://www.poisonhelp.org/help. If the victim has collapsed, had a seizure, has trouble breathing, or can't be awakened, immediately call emergency services at 911.

Keep all appointments with your doctor and the laboratory. Your doctor may order a blood test before you start treatment to see if this medication is right for you.

Do not let anyone else take your medication. Ask your pharmacist any questions you have about refilling your prescription.

Keep a written list of all of the prescription and nonprescription (over-the-counter) medicines, vitamins, minerals, and dietary supplements you are taking. Bring this list with you each time you visit a doctor or if you are admitted to the hospital. You should carry the list with you in case of emergencies.

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