Trikafta is a combination of 3 different medications (elexacaftor, tezacaftor, and ivacaftor), all known as cystic fibrosis transmembrane conductance regulator (CFTR) modulators. Trikafta is used to treat cystic fibrosis (CF) in people ages 2 years and older. But it can only help if the CF is caused by certain genetic mutations. This medication is taken by mouth, typically twice daily. Some common side effects of Trikafta include headache, stomach pain, higher risk for respiratory infections (e.g., the common cold). Sometimes, it can also cause acne.
Cystic fibrosis caused by specific mutations
Cystic fibrosis (CF) is caused by genetic changes (mutations) in the CFTR proteins. The mutations result in abnormal proteins that cause problems in different parts of the body, including mucus buildup in the lungs.
Trikafta is a combination of three medications that are all known as CFTR modulators. They act directly on the abnormal CFTR proteins so that they can work properly. As a result, this relieves some of the symptoms of CF.
Keep in mind that there are many different genetic changes that can cause CF. Trikafta can only treat CF that's caused by specific mutations.
Source: DailyMed
Side effects that you should report to your care team as soon as possible:
Side effects that usually do not require medical attention (report these to your care team if they continue or are bothersome):
Combination medication, so more convenient to take
Packaged as weekly "wallets" that make it clear which tablet you're supposed to take and when you're supposed to take them
Can be taken by children as young as 2 years old
Only available as a brand medication
Taken twice daily
Need to take with high-fat meals, such as whole milk, yogurt, or cheese
If you're taking Trikafta tablets, you must swallow the tablets whole. Don't chew, cut, or crush the tablets.
If your child is taking Trikafta granule packets, you can mix the granules with 1 teaspoon of soft foods or liquids before giving the medication to your child. Examples of soft foods or liquids include yogurt, applesauce, water, or juice.
You should take your morning and evening Trikafta doses about 12 hours apart. Set alarms, sticky notes, or other reminders for yourself to help remember to take it on time every 12 hours.
Always take Trikafta with foods high in fat, such as hardboiled eggs or yogurt made with whole milk, to help your body absorb the medication better.
Avoid foods or drinks with grapefruit, because these might raise the amount of Trikafta in your body.
Talk to your provider and pharmacist before starting, changing, or stopping any medications while taking Trikafta. This includes non-prescription medications and supplements as well. Trikafta can interact with certain medications, so it's important that your healthcare team knows about all the medications you take to make sure they're safe for you.
Trikafta can cause some serious health issues. This risk may be even higher for certain groups. If this worries you, talk to your doctor or pharmacist about other options.
Risk factors: History of liver problems
Trikafta can sometimes cause abnormally high liver test results. Your provider will check your liver before you start Trikafta, then every 3 months for the next year, and then every year after that. If you have a history of liver problems, your provider might check your liver labs more often.
If you have severe liver disease, discuss with your provider about the risks and benefits of taking Trikafta, as this medication might cause worsening liver health. This can lead to liver failure that could require transplantation.
While taking Trikafta, if you experience stomach pain, dark-colored urine, pale-colored stools, or yellowing of the skin or eyes, get medical help right away.
Taking Trikafta along with certain medications can affect the amount of Trikafta in your body, how well it works, and the risk of side effects.
Some medications, like the antibiotic, rifampin, and a common supplement, St. John's wort, can lower the amount of Trikafta in your body. This can make Trikafta not work as well.
Other medications can raise the levels of Trikafta in your body. You'll also want to avoid grapefruit and grapefruit juice while taking this medication, because grapefruit might cause you to have too much medication in your body as well. This can lead to a higher risk of side effects.
Trikafta can also affect how other medications work, so tell your provider and pharmacist about all the medications and supplements you're taking before starting Trikafta. They will make sure all your medications are safe for you to take.
Risk factors: Taking steroid medications | Exposure to radiation
The ivacaftor in Trikafta can cause cataracts in children. Your child's provider will likely give your child an eye exam before starting the medication and throughout treatment.
Your provider will determine your dose of Trikafta based on your body weight and age. Trikafta comes as prepackaged blister cards with the exact doses that you need. The morning and evening doses are taken about 12 hours apart, with a high-fat meal or snack.
Morning dose: Each tablet contains elexacaftor 100 mg, tezacaftor 50 mg, and ivacaftor 75 mg. The typical dose is to take 2 tablets by mouth every morning.
Evening dose: Each tablet contains ivacaftor 150 mg. The typical dose is to take 1 tablet by mouth every evening.
Children
Ages 6 to less than 12 years old
Weighing 30 kg or more: See adult dose.
Weighing less than 30 kg:
Morning dose: Each tablet contains elexacaftor 50 mg, tezacaftor 25 mg, and ivacaftor 37.5 mg. The typical dose is to take 2 tablets by mouth every morning.
Evening dose: Each tablet contains ivacaftor 75 mg. The typical dose is to take 1 tablet by mouth every evening.
Ages 2 to less than 6 years old
Weighing 14 kg or more:
Morning dose: Each packet contains elexacaftor 100 mg, tezacaftor 50 mg, and ivacaftor 75 mg. The typical dose is to dissolve 1 packet of granules with 1 teaspoon of soft foods and take by mouth every morning.
Evening dose: Each packet contains ivacaftor 75 mg. The typical dose is to dissolve 1 packet of granules with 1 teaspoon of soft foods and take by mouth every evening.
Weighing less than 14 kg:
Morning dose: Each packet contains elexacaftor 80 mg, tezacaftor 40 mg, and ivacaftor 60 mg. The typical dose is to dissolve 1 packet of granules with 1 teaspoon of soft foods and take by mouth every morning.
Evening dose: Each packet contains ivacaftor 59.5 mg. The typical dose is to dissolve 1 packet of granules with 1 teaspoon of soft foods and take by mouth every evening.
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Gur, M., et al. (2023). Effect of Trikafta on bone density, body composition and exercise capacity in CF: A pilot study. Pediatric Pulmonology.
Hudson, B. N., et al. (2022). Drug-induced acne with elexacaftor/tezacaftor/ivacaftor in people with cystic fibrosis. Journal of Cystic Fibrosis.
Piehler, L., et al. (2023). Effects of elexacaftor/tezacaftor/ivacaftor therapy on mental health of patients with cystic fibrosis. Frontiers in Pharmacology.
Soltman, S., et al. (2021). Body composition in individuals with cystic fibrosis. Journal of Clinical & Translational Endocrinology.
Spoletini, G., et al. (2022). Dose adjustments of elexacaftor/tezacaftor/ivacaftor in response to mental health side effects in adults with cystic fibrosis. Journal of Cystic Fibrosis.
Vertex Pharmaceuticals Incorporated. (n.d.). Am I eligible for Trikafta®?
Vertex Pharmaceuticals Incorporated. (n.d.). How does Trikafta® work?
Vertex Pharmaceuticals Incorporated. (n.d.). How to take Trikafta®?
Vertex Pharmaceuticals Incorporated. (n.d.). Looking for fat-containing food ideas?
Vertex Pharmaceuticals Incorporated. (2023). Trikafta- elexacaftor, tezacaftor, and ivacaftor kit [package insert]. DailyMed.
Zaher, A., et al. (2021). A review of Trikafta: Triple cystic fibrosis transmembrane conductance regulator (CFTR) modulator therapy. Cureus.
Zhang, L., et al. (2022). Impact of elexacaftor/tezacaftor/ivacaftor on depression and anxiety in cystic fibrosis. Therapeutic Advances in Respiratory Disease.
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