Trikafta contains elexacaftor, tezacaftor, and ivacaftor, formulated as fixed-dose combination tablets or granules for oral use. The tablets are available in varying strengths: either orange tablet with 100 mg elexacaftor, 50 mg tezacaftor, 75 mg ivacaftor or a lighter orange tablet with half those amounts. The ivacaftor-only tablet is available in 150 mg or 75 mg strengths, and both forms come with several inactive ingredients like croscarmellose sodium and hypromellose. The granules are unit-dose packets available in similar strengths as the tablets, designed for convenient oral administration.

How should I use this medication?

Take this medication by mouth with fat-containing foods such as butter, oil, eggs, peanut butter, nuts, meat, or whole-milk dairy products such as whole milk, cheese, or yogurt. Take it as directed on the prescription label. Mix the entire packet with 1 teaspoon of soft food or liquids. These can include pureed fruits or vegetables, yogurt, applesauce, water, milk, or juice. Food or liquid should be at room temperature or below. Take the dose within an hour of mixing. Do not take this medication with grapefruit juice. Keep taking it unless your care team tells you to stop. Talk to your care team about the use of this medication in children. While it may be prescribed for children as young as 2 years for selected conditions, precautions do apply.

Overdosage: If you think you have taken too much of this medicine contact a poison control center or emergency room at once. NOTE: This medicine is only for you. Do not share this medicine with others.

What if I miss a dose?

It is important not to miss any doses. Talk to your care team about what to do if you miss a dose.

Where should I keep my medication?

Keep out of the reach of children and pets. Store at room temperature between 20 and 25 degrees C (68 and 77 degrees F). Get rid of any unused medication after the expiration date. To get rid of medications that are no longer needed or have expired: -Take the medication to a medication take-back program. Check with your pharmacy or law enforcement to find a location. -If you cannot return the medication, check the label or package insert to see if the medication should be thrown out in the garbage or flushed down the toilet. If you are not sure, ask your care team. If it is safe to put it in the trash, empty the medication out of the container. Mix the medication with cat litter, dirt, coffee grounds, or other unwanted substance. Seal the mixture in a bag or container. Put it in the trash.

NOTE: This sheet is a summary. It may not cover all possible information. If you have questions about this medicine, talk to your doctor, pharmacist, or health care provider.

What side effects may I notice from receiving this medication?

Side effects that you should report to your care team as soon as possible: -Allergic reactions—skin rash, itching, hives, swelling of the face, lips, tongue, or throat -Change in vision such as blurry vision, seeing halos around lights, vision loss -Liver injury—right upper belly pain, loss of appetite, nausea, light-colored stool, dark yellow or brown urine, yellowing skin or eyes, unusual weakness or fatigue Side effects that usually do not require medical attention (report these to your care team if they continue or are bothersome): -Diarrhea -Headache -Runny or stuffy nose -Sinus pain or pressure around the face or forehead -Skin rash -Stomach pain

This list may not describe all possible side effects. Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088.

What should I tell my care team before I take this medication?

They need to know if you have any of these conditions: -Kidney disease -Liver disease -An unusual reaction to elexacaftor, tezacaftor, ivacaftor, other medications, foods, dyes, or preservatives -Pregnant or trying to get pregnant -Breast-feeding

What may interact with this medication?

Do not take this medication with any of the following: -Certain medications for seizures, such as carbamazepine, phenobarbital, phenytoin -Grapefruit juice -Rifabutin -Rifampin -St. John's Wort This medication may also interact with the following: -Certain antibiotics, such as clarithromycin, erythromycin, and telithromycin -Certain medications for fungal infections, such as fluconazole, itraconazole, ketoconazole, posaconazole, and voriconazole -Cyclosporine -Digoxin -Everolimus -Glimepiride -Glipizide -Sirolimus -Tacrolimus -Warfarin

This list may not describe all possible interactions. Give your health care provider a list of all the medicines, herbs, non-prescription drugs, or dietary supplements you use. Also tell them if you smoke, drink alcohol, or use illegal drugs. Some items may interact with your medicine.

What should I watch for while using this medication?

Visit your care team for regular checks on your progress. Tell your care team if your symptoms do not start to get better or if they get worse. Your vision and blood may be tested before and during use of this medication. Tell your care team right away if you have any change in your eyesight.

What are the most serious risks of this medication?

This medication can cause serious liver injury. Your care team will check your liver with blood tests before and during treatment. Stop this medication and call your care team right away if you have symptoms of liver injury. These may include feeling weak or tired, loss of appetite, nausea, belly pain, light-colored stool, dark yellow or brown urine, or yellowing skin or eyes.