Elexacaftor, Tezacaftor, and Ivacaftor

WHY is this medicine prescribed?

The combination of elexacaftor, tezacaftor, and ivacaftor is used to treat certain types of cystic fibrosis (an inborn disease that causes problems with breathing, digestion, and reproduction) in adults and children 2 years of age and older. It should be used only in people with a certain genetic make-up. Your doctor may order a blood test to help decide if this medication is right for you. 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 cystic fibrosis symptoms.

Are there OTHER USES for this medicine?

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

HOW should this medicine be used?

Elexacaftor, tezacaftor, and ivacaftor come as tablets and as granules to take by mouth. It is usually taken with fatty foods twice a day, 12 hours apart. Take elexacaftor, tezacaftor, and ivacaftor at the same times every day. Follow the directions on your prescription label carefully, and ask your doctor or pharmacist to explain any part you do not understand. Take these medications 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 works to control 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.

Ask your pharmacist or doctor for a copy of the manufacturer's information for the patient.

What SPECIAL PRECAUTIONS should I follow?

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

• tell your doctor and pharmacist if you are allergic to elexacaftor, tezacaftor, and ivacaftor any other medications, or any of the ingredients in elexacaftor, tezacaftor, and ivacaftor tablets or granules. Ask your pharmacist for a list of the ingredients.

• tell your doctor and pharmacist what other prescription and nonprescription medications, vitamins, nutritional supplements, you are taking or plan to take. Your doctor may need to change the doses of your medications or monitor you carefully for side effects.

• 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 liver or 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.

What SPECIAL DIETARY instructions should I follow?

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

What should I do IF I FORGET to take a dose?

If you remember the morning or evening missed dose within 6 hours of the time you were scheduled to take it, take the missed dose right away with a fat-containing food and continue your regular dosing schedule. However, if more than 6 hours have passed since the scheduled time to take your morning dose, take the missed morning dose as soon as possible and skip the evening dose, then continue your regular dosing schedule. If more than 6 hours have passed since the scheduled time to take the evening dose, skip the missed evening dose and continue your regular dosing schedule. Do not take morning and evening doses together to make up for a missed one.

What SIDE EFFECTS can this medicine cause?

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
• rash
• gas
• pink eye
• itching
• dizziness

Some side effects can be serious. If you experience any of these symptoms, call your doctor immediately or get emergency medical treatment:

• yellowing of the skin or eyes
• loss of appetite
• nausea
• vomiting
• pale stools
• pain in the upper right part of the stomach
• dark urine

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 (http://www.fda.gov/Safety/MedWatch) or by phone (1-800-332-1088).

What should I know about STORAGE and DISPOSAL of this medication?

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).

It is important to keep all medication out of sight and reach of children as many containers (such as weekly pill minders and those for eye drops, creams, patches, and inhalers) are not child-resistant and young children can open them easily. To protect young children from poisoning, always lock safety caps and immediately place the medication in a safe location – one that is up and away and out of their sight and reach. http://www.upandaway.org

Unneeded medications should be disposed of in special ways to ensure that pets, children, and other people cannot consume them. However, you should not flush this medication down the toilet. Instead, the best way to dispose of your medication is through a medicine take-back program. Talk to your pharmacist or contact your local garbage/recycling department to learn about take-back programs in your community. See the FDA's Safe Disposal of Medicines website (http://goo.gl/c4Rm4p) for more information if you do not have access to a take-back program.

What should I do in case of OVERDOSE?

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.

What OTHER INFORMATION should I know?

Keep all appointments with your doctor and the laboratory. Your doctor will order an eye exam (for children and teenagers) and certain lab tests such as liver tests before and during your treatment to check your body's response to elexacaftor, tezacaftor, and ivacaftor.

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

It is important for you to keep a written list of all of the prescription and nonprescription (over-the-counter) medicines you are taking, as well as any products such as vitamins, minerals, or other dietary supplements. You should bring this list with you each time you visit a doctor or if you are admitted to a hospital. It is also important information to carry with you in case of emergencies.