Pronunciation:

el-ex-a-KAF-tor, tez-a-KAF-tor, eye-va-KAF-tor, eye-va-KAF-tor

Brand Names:

• Trikafta

Dosage Forms:

• Tablet
• Granule

Uses of This Medicine:

Elexacaftor/tezacaftor/ivacaftor and ivacaftor combination is used to treat cystic fibrosis (CF) in patients who have at least one copy of F508del mutation in their CF transmembrane conductance regulator (CFTR) gene or another mutation that is responsive to this medicine. Your doctor will use a test to check for the mutation before you receive the medicine.

This medicine is available only with your doctor's prescription.

Before Using This Medicine:

In deciding to use a medicine, the risks of taking the medicine must be weighed against the good it will do. This is a decision you and your doctor will make. For this medicine, the following should be considered:

Allergies—

Tell your doctor if you have ever had any unusual or allergic reaction to this medicine or any other medicines. Also tell your health care professional if you have any other types of allergies, such as to foods, dyes, preservatives, or animals. For non-prescription products, read the label or package ingredients carefully.

Children—

Appropriate studies have not been performed on the relationship of age to the effects of Trikafta® in children younger than 2 years of age. Safety and efficacy have not been established.

Older adults—

Appropriate studies on the relationship of age to the effects of Trikafta® have not been performed in the geriatric population. However, no geriatric-specific problems have been documented to date.

Breast-feeding—

There are no adequate studies in women for determining infant risk when using this medication during breastfeeding. Weigh the potential benefits against the potential risks before taking this medication while breastfeeding.

Other medicines—

Although certain medicines should not be used together at all, in other cases two different medicines may be used together even if an interaction might occur. In these cases, your doctor may want to change the dose, or other precautions may be necessary. When you are taking this medicine, it is especially important that your healthcare professional know if you are taking any of the medicines listed below. The following interactions have been selected on the basis of their potential significance and are not necessarily all-inclusive.

Using this medicine with any of the following medicines is not recommended. Your doctor may decide not to treat you with this medication or change some of the other medicines you take.

• Levoketoconazole
• Nirmatrelvir
• Ritonavir

Using this medicine with any of the following medicines is usually not recommended, but may be required in some cases. If both medicines are prescribed together, your doctor may change the dose or how often you use one or both of the medicines.

• Abametapir
• Adagrasib
• Alfentanil
• Alprazolam
• Amiodarone
• Amprenavir
• Apalutamide
• Aprepitant
• Atazanavir
• Atorvastatin
• Belzutifan
• Bentazepam
• Benzhydrocodone
• Boceprevir
• Bosentan
• Bromazepam
• Buprenorphine
• Carbamazepine
• Ceritinib
• Chlordiazepoxide
• Cimetidine
• Ciprofloxacin
• Clarithromycin
• Clobazam
• Clofazimine
• Clonazepam
• Clorazepate
• Cloxazolam
• Clozapine
• Cobicistat
• Codeine
• Conivaptan
• Crizotinib
• Cyclosporine
• Dabrafenib
• Darunavir
• Delorazepam
• Diazepam
• Digoxin
• Dihydrocodeine
• Dihydroergotamine
• Diltiazem
• Dofetilide
• Dronedarone
• Duvelisib
• Elagolix
• Eliglustat
• Enzalutamide
• Ergotamine
• Erythromycin
• Estazolam
• Everolimus
• Fedratinib
• Fentanyl
• Fexinidazole
• Finerenone
• Flibanserin
• Fluconazole
• Flunitrazepam
• Flurazepam
• Fluvoxamine
• Fosamprenavir
• Fosnetupitant
• Fosphenytoin
• Glimepiride
• Glipizide
• Glyburide
• Grazoprevir
• Halazepam
• Hydrocodone
• Idelalisib
• Imatinib
• Indinavir
• Itraconazole
• Ivosidenib
• Josamycin
• Ketazolam
• Ketoconazole
• Larotrectinib
• Lefamulin
• Lemborexant
• Lenacapavir
• Letermovir
• Lonafarnib
• Lopinavir
• Loprazolam
• Lorazepam
• Lorlatinib
• Lormetazepam
• Lumacaftor
• Mavacamten
• Medazepam
• Meperidine
• Metaclazepam
• Methadone
• Methotrexate
• Mibefradil
• Midazolam
• Mifepristone
• Mitotane
• Morphine
• Morphine Sulfate Liposome
• Nefazodone
• Nelfinavir
• Netupitant
• Nilotinib
• Nitrazepam
• Nordazepam
• Olutasidenib
• Omaveloxolone
• Oxazepam
• Oxycodone
• Pacritinib
• Pentazocine
• Phenobarbital
• Phenytoin
• Pimavanserin
• Pimozide
• Pinazepam
• Pirtobrutinib
• Pitavastatin
• Posaconazole
• Pravastatin
• Prazepam
• Primidone
• Quazepam
• Quinidine
• Remimazolam
• Repaglinide
• Revefenacin
• Ribociclib
• Rifabutin
• Rifampin
• Rifapentine
• Ritlecitinib
• Ritonavir
• Rosuvastatin
• Saquinavir
• Schisandra sphenanthera
• Simvastatin
• Sirolimus
• Sirolimus Protein-Bound
• St John's Wort
• Sufentanil
• Tacrolimus
• Telaprevir
• Telithromycin
• Temazepam
• Temsirolimus
• Tetrazepam
• Tocilizumab
• Tramadol
• Treosulfan
• Triazolam
• Trofinetide
• Troleandomycin
• Tucatinib
• Ubrogepant
• Verapamil
• Voriconazole
• Voxelotor
• Warfarin
• Zolpidem

Other interactions—

Certain medicines should not be used at or around the time of eating food or eating certain types of food since interactions may occur. Using alcohol or tobacco with certain medicines may also cause interactions to occur. The following interactions have been selected on the basis of their potential significance and are not necessarily all-inclusive.

Using this medicine with any of the following is usually not recommended, but may be unavoidable in some cases. If used together, your doctor may change the dose or how often you use this medicine, or give you special instructions about the use of food, alcohol, or tobacco.

• Grapefruit Juice

Other medical problems—

The presence of other medical problems may affect the use of this medicine. Make sure you tell your doctor if you have any other medical problems, especially:

• Exposure to radiation—Use with caution. May increase risk of developing cataracts.

• Kidney disease, severe or
• Liver disease, moderate—Use with caution. The effects may be increased because of slower removal of the medicine from the body.

• Liver disease (eg, ascites, cirrhosis, hepatic encephalopathy, portal hypertension), or history of or—Use with caution. May make this condition worse.

• Liver disease, severe—Should not be used in patients with this condition.

Proper Use of This Medicine:

Take this medicine exactly as directed by your doctor. Do not take more of it, do not take it more often, and do not take it for a longer time than your doctor ordered. To do so may increase the chance of side effects.

This medicine comes with a patient information insert. Read and follow the instructions carefully. Ask your doctor if you have any questions.

Swallow the tablets whole. Do not crush, break, or chew them.

If you are using the oral granules, mix the entire contents of each packet with 1 teaspoon (5 milliliters [mL]) of cool to room temperature soft food (eg, pureed fruits or vegetables, yogurt, applesauce) or liquid (eg, water, milk, juice). Take this mixture within 1 hour after mixing.

Take this medicine with fat-containing foods, including food prepared with butter or oils, cheese, eggs, peanut butter, meat, nuts, or whole milk.

Do not eat grapefruit or drink grapefruit juice while you are using this medicine.

Dosing—

The dose of this medicine will be different for different patients. Follow your doctor's orders or the directions on the label. The following information includes only the average doses of this medicine. If your dose is different, do not change it unless your doctor tells you to do so.

The amount of medicine that you take depends on the strength of the medicine. Also, the number of doses you take each day, the time allowed between doses, and the length of time you take the medicine depend on the medical problem for which you are using the medicine.

• For oral dosage form (granules and tablets):
  • For cystic fibrosis:
    • Adults and children 12 years of age and older—2 orange tablets (containing elexacaftor 100 milligrams (mg), tezacaftor 50 mg, and ivacaftor 75 mg) taken in the morning and 1 light blue tablet (containing ivacaftor 150 mg) taken in the evening, about 12 hours apart.
    • Children 6 to younger than 12 years of age—Dose is based on your body weight and must be determined by your doctor.
      • Weighing 30 kilograms (kg) or more—2 orange tablets (containing elexacaftor 100 milligrams (mg), tezacaftor 50 mg, and ivacaftor 75 mg) taken in the morning and 1 light blue tablet (containing ivacaftor 150 mg) taken in the evening, about 12 hours apart.
      • Weighing less than 30 kg—2 light orange tablets (containing elexacaftor 50 mg, tezacaftor 25 mg, and ivacaftor 37.5 mg) taken in the morning and 1 light blue tablet (containing ivacaftor 75 mg) taken in the evening, about 12 hours apart.
    • Children 2 to younger than 6 years of age—Dose is based on your body weight and must be determined by your doctor.
      • Weighing 14 kilograms (kg) or more—1 white and orange packet of granules (each packet contains 100 mg tezacaftor, 50 mg tezacaftor, and 75 mg ivacaftor) taken in the morning and 1 white and pink packet of granules (containing ivacaftor 75 mg) taken in the evening, about 12 hours apart.
      • Weighing less than 14 kg—1 white and blue packet of granules (each packet contains 80 mg tezacaftor, 40 mg tezacaftor, and 60 mg ivacaftor) taken in the morning and 1 white and green packet of granules (containing ivacaftor 59.5 mg) taken in the evening, about 12 hours apart.
    • Children younger than 2 years of age—Use and dose must be determined by your doctor.

Missed dose—

If you miss a dose of this medicine, take it as soon as possible. However, if it is almost time for your next dose, skip the missed dose and go back to your regular dosing schedule. Do not double doses.

If you miss a dose and it is 6 hours or less from the time you normally take your medicine, take a dose as soon as you can. If it is more than 6 hours since the missed morning dose, take the missed dose as soon as you can, but don't take the evening dose. Take the next morning dose at its usual time. If it is more than 6 hours since the missed evening dose, skip the missed dose and take the next morning dose at the regular time. Do not take extra medicine to make up for a missed dose.

Storage—

Store the medicine in a closed container at room temperature, away from heat, moisture, and direct light. Keep from freezing.

Keep out of the reach of children.

Do not keep outdated medicine or medicine no longer needed.

Ask your healthcare professional how you should dispose of any medicine you do not use.

Precautions While Using This Medicine:

It is very important that your doctor check your or your child's progress at regular visits to make sure that this medicine is working properly. Blood tests will be needed to check for unwanted effects.

This medicine may cause serious allergic reactions, including anaphylaxis and angioedema, which can be life-threatening and require immediate medical attention. Check with your doctor right away if you have chest tightness, cough, difficulty swallowing, dizziness, fast heartbeat, hives, itching, skin rash, large, hive-like swelling on the face, eyelids, lips, tongue, throat, hands, legs, feet, or sex organs, puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue, trouble breathing, or unusual tiredness or weakness.

Check with your doctor right away if you have dark urine, clay-colored stools, stomach pain, or yellow eyes or skin. These may be symptoms of a serious liver problem.

Check with your doctor right away if blurred vision, difficulty with reading, or any other change in vision occurs during or after treatment. Your doctor may want your child's eyes be checked by an ophthalmologist (eye doctor).

Do not take other medicines unless they have been discussed with your doctor. This includes prescription or nonprescription (over-the-counter [OTC]) medicines and herbal (eg, St. John's wort) or vitamin supplements.

Side Effects of This Medicine:

Along with its needed effects, a medicine may cause some unwanted effects. Although not all of these side effects may occur, if they do occur they may need medical attention.

Check with your doctor immediately if any of the following side effects occur:

Incidence not known

Chest tightness

cough

dark urine

difficulty swallowing

dizziness

fast heartbeat

headache

hives, itching, skin rash

large, hive-like swelling on the face, eyelids, lips, tongue, throat, hands, legs, feet, or sex organs

light-colored stools

nausea and vomiting

puffiness or swelling of the eyelids or around the eyes, face, lips, or tongue

stomach pain or bloating

trouble breathing

yellow eyes or skin

unusual tiredness or weakness

Some side effects may occur that usually do not need medical attention. These side effects may go away during treatment as your body adjusts to the medicine. Also, your health care professional may be able to tell you about ways to prevent or reduce some of these side effects. Check with your health care professional if any of the following side effects continue or are bothersome or if you have any questions about them:

More common

Body aches or pain

chills

diarrhea

ear congestion

fever

loss of voice

sneezing

sore throat

stuffy or runny nose

Less common

Anxiety

bladder pain

blemishes on the skin

bloated

bloody or cloudy urine

blurred vision

burning, dry, or itching eyes

cold sweats

coma

confusion

cool, pale skin

cramps

depression

difficult, burning, or painful urination

discharge, excessive tearing

excess air or gas in the stomach or intestines

fast heartbeat

frequent urge to urinate

full feeling

heavy bleeding

increased hunger

lower back or side pain

nervousness

nightmares

pain

passing gas

pimples

redness, pain, swelling of the eye, eyelid, or inner lining of the eyelid

seizures

shakiness

skin rash, encrusted, scaly, and oozing

slurred speech

Other side effects not listed may also occur in some patients. If you notice any other effects, check with your healthcare professional.

Call your doctor for medical advice about side effects. You may report side effects to the FDA at 1-800-FDA-1088.