Why is this medication prescribed?

Tezacaftor and Ivacaftor are medications used in combination to treat cystic fibrosis (CF) in patients who have specific genetic mutations. CF is a genetic disorder that affects the lungs and digestive system, causing thick mucus to build up and leading to various complications. Tezacaftor and Ivacaftor work together to improve the function of the defective CFTR protein, which is responsible for regulating the flow of salt and water in and out of cells. By improving CFTR function, these medications help to reduce mucus buildup and improve lung function in patients with CF.

Tezacaftor is a corrector medication that helps to improve the processing and trafficking of the defective CFTR protein to the cell surface, while Ivacaftor is a potentiator medication that helps to enhance the function of the CFTR protein once it reaches the cell surface.

The combination of Tezacaftor and Ivacaftor is usually prescribed for patients with CF who have specific mutations in the CFTR gene. It is important to note that these medications are not effective for all mutations associated with CF, and their use should be determined by genetic testing and in consultation with a healthcare provider familiar with CF management.

How should this medicine be used?

Dosage and Administration:

• Tezacaftor/Ivacaftor Combination: The combination therapy of Tezacaftor and Ivacaftor is usually prescribed as a fixed-dose combination tablet. The dosage strength and frequency are typically determined by a healthcare provider based on factors such as the patient’s age, weight, other medications being taken, and the specific mutations present.
• Administration: The tablets are usually taken orally with food to improve absorption. It’s important for patients to follow the prescribed dosing schedule provided by their healthcare provider.
• Monitoring: Patients prescribed Tezacaftor and Ivacaftor may require regular monitoring, including lung function tests, to assess the effectiveness of the treatment and monitor for any potential side effects.
• Missed Dose: If a dose is missed, patients should take it as soon as they remember. However, if it’s almost time for the next dose, they should skip the missed dose and continue with their regular dosing schedule. It’s important not to double the dose to make up for a missed one.
• Adherence: Patients should be encouraged to adhere to the prescribed treatment regimen consistently to optimize the benefits of Tezacaftor and Ivacaftor therapy.

As with any medication, it’s essential for patients to communicate regularly with their healthcare providers regarding their CF management, including any concerns or questions about Tezacaftor and Ivacaftor therapy.

Other uses for this medicine

Some off-label uses may include:

• Tezacaftor and ivacaftor are specifically approved for the treatment of CF in patients with certain genetic mutations, such as those with two copies of the F508del mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene.
• They are not typically used for other conditions besides CF.

What special precautions should I follow?

As for special precautions to follow when taking Tezacaftor and Ivacaftor:

• Pregnancy and Breastfeeding: It’s essential to inform your healthcare provider if you are pregnant, planning to become pregnant, or breastfeeding before starting Tezacaftor and Ivacaftor therapy. The safety of these medications during pregnancy and breastfeeding has not been established, so the potential risks and benefits should be discussed with a healthcare provider.
• Liver Function: Tezacaftor and Ivacaftor can affect liver function, so regular monitoring of liver enzymes may be necessary during treatment. Patients should promptly report any symptoms of liver problems, such as jaundice, dark urine, or abdominal pain, to their healthcare provider.
• Drug Interactions: Tezacaftor and Ivacaftor may interact with other medications, including certain antibiotics, antifungals, and seizure medications. It’s crucial to inform your healthcare provider about all the medications, supplements, and herbal products you are taking before starting Tezacaftor and Ivacaftor therapy.
• Side Effects: Common side effects of Tezacaftor and Ivacaftor may include headache, upper respiratory tract infection, abdominal pain, and diarrhea. Patients should report any unusual or severe side effects to their healthcare provider.
• Allergic Reactions: Individuals who have had a hypersensitivity reaction to Tezacaftor or Ivacaftor or any of their components should not take these medications. Symptoms of an allergic reaction may include rash, itching, swelling, severe dizziness, or difficulty breathing.

It’s important to follow the prescribed dosage and instructions provided by your healthcare provider and to attend regular follow-up appointments to monitor your response to treatment and any potential side effects.

What special dietary instructions should I follow?

Regarding special dietary instructions for Tezacaftor and Ivacaftor, there are no specific dietary restrictions associated with these medications. However, taking Tezacaftor and Ivacaftor with food, particularly a meal containing fat, can help improve the absorption of the medications. Therefore, it’s generally recommended to take the medications with food to optimize their effectiveness.

What should I do if I forget a dose?

If you forget to take a dose of Tezacaftor and Ivacaftor, you should take it as soon as you remember, unless it is almost time for your next scheduled dose. If it is close to the time for your next dose, skip the missed dose and continue with your regular dosing schedule. Do not double the dose to make up for a missed one. If you have any concerns or questions about missed doses, consult your healthcare provider or pharmacist for guidance.

What side effects can this medication cause?

Tezacaftor and ivacaftor, when used individually or in combination, may cause side effects in some individuals. Common side effects of tezacaftor and ivacaftor combination therapy may include:

• Respiratory Effects:
  • Chest tightness
  • Cough
  • Increased sputum production
  • Nasal congestion
  • Sinus congestion or headache
• Gastrointestinal Effects:
  • Nausea
  • Diarrhea
  • Abdominal pain or discomfort
  • Increased levels of liver enzymes (as seen in blood tests)
• Other Common Side Effects:
  • Fatigue
  • Headache
  • Rash or itching
  • Increased blood creatinine phosphokinase (an enzyme found in muscles)
• Less Common Side Effects:
  • Respiratory tract infections
  • Joint pain
  • Elevated blood glucose levels
  • Eye symptoms such as blurred vision or eye pain
  • Irregular heartbeat (palpitations)
• Serious Side Effects:
  • Liver problems: Symptoms may include yellowing of the skin or eyes (jaundice), dark urine, or persistent nausea or vomiting.
  • Respiratory issues: Some individuals may experience worsening respiratory symptoms, which could indicate a serious reaction.
  • Allergic reactions: Rarely, severe allergic reactions such as swelling of the face, lips, tongue, or throat may occur, along with difficulty breathing or swallowing.

It’s essential to report any side effects to your healthcare provider promptly, especially if they are severe, persistent, or concerning. Your healthcare provider may adjust your dosage or recommend alternative treatments if side effects are problematic. Additionally, always follow your healthcare provider’s instructions for medication use and attend regular check-ups to monitor your response to treatment and any potential side effects.

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

Storage and Disposal:

• Storage:
  • Store tezacaftor and ivacaftor at room temperature away from moisture and heat.
  • Keep both medications in their original packaging to protect them from light.
  • Do not transfer them to other containers unless specifically instructed to do so by your healthcare provider or pharmacist.
  • Keep them out of reach of children and pets.
• Disposal:
  • Follow any specific disposal instructions provided by your pharmacist or healthcare provider.
  • If no specific instructions are given, you can dispose of tezacaftor and ivacaftor by mixing them with an unpalatable substance (like dirt or cat litter) in a sealed plastic bag and throwing it away in the trash. This prevents accidental ingestion by pets or children.
  • Do not flush them down the toilet or pour them down the drain unless instructed to do so by specific guidelines or local regulations.

In case of emergency/overdose

• In case of an overdose or emergency, call your local poison control center at 1-800-222-1222 immediately or seek emergency medical attention.
• Symptoms of overdose may include severe dizziness, fainting, chest pain, irregular heartbeat, or seizures.
• Be prepared to provide information about the type and amount of medication taken and any symptoms experienced.

What other information should I know?

• Always take tezacaftor and ivacaftor exactly as prescribed by your healthcare provider.
• Inform your doctor of any other medications, vitamins, or supplements you are taking, as they may interact with tezacaftor and ivacaftor.
• Attend all scheduled appointments with your healthcare provider for monitoring and adjustments to your treatment regimen.
• Report any unexpected or severe side effects to your healthcare provider promptly.
• Keep a list of emergency contacts, including your healthcare provider’s phone number, readily available.

As with any medication, it’s essential to follow the prescribed dosage and instructions carefully and seek medical attention if you have any concerns or experience adverse effects.