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Elexacaftor, Tezacaftor, and Ivacaftor

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Why is this medication 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.

How should this medicine be used?

Elexacaftor, Tezacaftor, and Ivacaftor are available as oral tablets and granules. Usually, it is taken twice a day, separated by 12 hours, with fatty meals. Every day, take ivacaftor, tezacaftor, and elexacaftor at the same times. Pay close attention to the instructions on the label of your prescription and ask your pharmacist or doctor to explain anything you do not understand. Follow the directions on these drugs precisely. Never take more or less of it, or take it more frequently than your doctor has instructed.

Each daily dosage, if you’re taking the tablets, is often administered as two separate tablets with different components. One tablet is orange (containing elexacaftor, tezacaftor, and ivacaftor), whereas the other tablet is blue (containing ivacaftor). It is likely that your doctor will advise you to take two orange tablets of elexacaftor, tezacaftor, and ivacaftor every morning and one blue tablet of ivacaftor every evening.

Do not split, chew, or crush the tablets; instead, swallow them whole.

When using granules, each daily dosage is often administered as two separate packets: one package contains ivacaftor granules, and the second packet contains tezacaftor, elexacaftor, and ivacaftor granules. You will likely be prescribed one package of elexacaftor, tezacaftor, and ivacaftor granules in the morning and one packet of ivacaftor granules in the evening by your doctor.

The full packet of granules should be mixed with 1 teaspoon (5 mL) of cold or room temperature soft food or drink, such as pureed fruits or vegetables, yogurt, applesauce, water, milk, or juice, in order to produce a dose of elexacaftor, tezacaftor, and ivacaftor. After combining the granules with food or fluids, take the concoction within an hour.

Consume fatty meals like eggs, butter, nuts, peanut butter, cheese pizza, and whole-milk dairy products (such whole milk, cheese, and yogurt) along with elexacaftor, tezacaftor, and ivacaftor pills and granules. Consult your doctor about consuming additional fatty meals while taking these prescriptions.

Elexacaftor, Tezacaftor, and Ivacaftor together manage cystic fibrosis, but they do not treat it. Even if you feel better, keep taking these pills. Don’t stop taking these drugs without first consulting your physician.

Request a copy of the patient’s manufacturer’s information from your pharmacist or physician.

Other uses for this medicine

Elexacaftor, tezacaftor, and ivacaftor are primarily used in combination therapy to treat cystic fibrosis (CF), as mentioned earlier. However, they are not indicated for other medical conditions besides CF. These drugs target specific defects in the cystic fibrosis transmembrane conductance regulator (CFTR) protein, and their efficacy and safety profiles have been established primarily in CF patients.

What special precautions should I follow?

Regarding special precautions for these medications, it’s crucial to follow the advice and instructions provided by your healthcare provider. Here are some general precautions:

  • Pregnancy and breastfeeding: It’s essential to inform your healthcare provider if you are pregnant, planning to become pregnant, or breastfeeding. The safety of these medications during pregnancy and breastfeeding is not well-established, so your doctor will weigh the potential risks and benefits.
  • Drug interactions: Inform your healthcare provider about all medications, supplements, and herbal products you are currently taking, as they may interact with elexacaftor, tezacaftor, or ivacaftor. Certain medications may need to be adjusted or avoided when taking CF medications to prevent adverse effects or reduced efficacy.
  • Liver function: These medications may affect liver function, so your healthcare provider may monitor your liver enzymes regularly, especially when starting treatment or if you have pre-existing liver conditions.
  • Lung function: Your healthcare provider will likely monitor your lung function regularly to assess the effectiveness of the treatment and make any necessary adjustments.
  • Adverse reactions: Be aware of potential side effects associated with these medications, such as respiratory symptoms, gastrointestinal issues, elevated liver enzymes, and changes in blood glucose levels. Contact your healthcare provider if you experience any concerning symptoms.
  • Genetic testing: Before initiating treatment with these medications, genetic testing is usually performed to identify specific mutations in the CFTR gene. This helps determine whether the patient is a suitable candidate for elexacaftor, tezacaftor, and ivacaftor therapy.

Always follow your healthcare provider’s recommendations and instructions for taking these medications to ensure safe and effective treatment. If you have any concerns or questions about elexacaftor, tezacaftor, or ivacaftor, discuss them with your doctor.

What special dietary instructions should I follow?

  • Fat-containing Food: Take elexacaftor, tezacaftor, and ivacaftor with fat-containing food. This helps optimize absorption and effectiveness of the medications.

What should I do if I forget a dose?

If you miss a dose of elexacaftor, tezacaftor, or ivacaftor, take it as soon as you remember. However, if it is almost time for your next scheduled dose, skip the missed dose and continue with your regular dosing schedule. Do not take a double dose to make up for a missed one. If you are unsure about what to do, contact your healthcare provider or pharmacist for guidance.

What side effects can this medication cause?

Elexacaftor, tezacaftor, and ivacaftor, like all medications, can cause side effects. These side effects can vary in severity and frequency among individuals. It’s important to note that not everyone will experience side effects, and some individuals may experience side effects that are not listed here. Common side effects associated with elexacaftor, tezacaftor, and ivacaftor may include:

  • Respiratory Symptoms: These may include cough, nasal congestion, sinus congestion, and throat irritation.
  • Gastrointestinal Issues: Common gastrointestinal side effects may include nausea, diarrhea, abdominal pain, and increased liver enzymes.
  • Rash or Skin Reactions: Some individuals may experience rash, itching, or other skin reactions.
  • Fatigue: Feeling tired or fatigued may occur in some individuals.
  • Headache: Headaches are a potential side effect of these medications.
  • Increased Blood Glucose Levels: Some individuals may experience increased blood glucose levels while taking these medications.
  • Dizziness: Feeling dizzy or lightheaded may occur in some individuals.
  • Changes in Taste: Alterations in taste perception have been reported as a side effect of these medications.
  • Menstrual Irregularities: Changes in menstrual cycles or menstrual irregularities may occur in female individuals taking these medications.
  • Joint Pain: Some individuals may experience joint pain or discomfort.

It’s important to contact your healthcare provider if you experience any side effects while taking elexacaftor, tezacaftor, or ivacaftor, especially if the side effects are severe, persistent, or concerning. Your healthcare provider can provide guidance on managing side effects and may adjust your treatment regimen if necessary.

Additionally, this list is not exhaustive, and other side effects may occur. Always read the medication package insert or consult your healthcare provider or pharmacist for a comprehensive list of potential side effects and further guidance.

What should I know about storage and disposal of this medication?

Storage and disposal of elexacaftor, tezacaftor, and ivacaftor:


  • Room Temperature: Store elexacaftor, tezacaftor, and ivacaftor at room temperature away from light and moisture.
  • Specific Requirements: Follow specific storage instructions provided on the medication packaging or by your healthcare provider or pharmacist.
  • Keep Out of Reach of Children: Store these medications in a location where they are inaccessible to children and pets.


  • Follow Local Regulations: Dispose of unused or expired medications according to local regulations or guidelines. Do not flush medications down the toilet or pour them into a drain unless instructed to do so.
  • Take-back Programs: Many communities offer drug take-back programs to safely dispose of unused medications. Check with your local pharmacy or government authorities for information on available programs in your area.
  • Ask Your Pharmacist: If you are unsure how to properly dispose of elexacaftor, tezacaftor, or ivacaftor, ask your pharmacist for guidance.

In case of emergency/overdose

  • Seek Medical Attention: In case of an overdose or emergency situation, such as accidentally taking too much medication, experiencing severe side effects, or exhibiting signs of an allergic reaction, seek immediate medical attention by calling emergency services or going to the nearest emergency room.
  • Poison Control: You can also contact your local poison control center for guidance on managing an overdose or emergency situation.

What other information should I know?

  • Regular Monitoring: Your healthcare provider may recommend regular monitoring, including blood tests and lung function tests, to assess the effectiveness of treatment and monitor for any potential side effects or complications.
  • Genetic Testing: Before starting treatment with elexacaftor, tezacaftor, and ivacaftor, genetic testing is usually performed to identify specific mutations in the CFTR gene. This helps determine whether you are a suitable candidate for these medications.
  • Follow Prescribing Instructions: Always follow the prescribing instructions provided by your healthcare provider and take elexacaftor, tezacaftor, and ivacaftor exactly as directed.
  • Inform Healthcare Providers: Inform all healthcare providers involved in your care, including specialists and dentists, that you are taking elexacaftor, tezacaftor, and ivacaftor, as they may interact with other medications or treatments.
  • Keep Appointments: Attend all scheduled appointments with your healthcare provider for regular check-ups and monitoring of your condition.
  • Patient Support Programs: Some pharmaceutical companies offer patient support programs that provide resources, assistance with medication costs, and other support services for individuals taking elexacaftor, tezacaftor, and ivacaftor. You may inquire about these programs with your healthcare provider or pharmacist.

Always consult your healthcare provider or pharmacist if you have any questions or concerns about the storage, disposal, emergency management, or other aspects of taking elexacaftor, tezacaftor, and ivacaftor.

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