Kalydeco (ivacaftor) is a medication used to treat cystic fibrosis (CF) in people aged 4 months and older. It's only for people who have a special mutation (change in DNA) in the CFTR (cystic fibrosis transmembrane conductance regulator) gene that responds to Kalydeco (ivacaftor). This medication is typically only available at certain pharmacies.
Cystic fibrosis
Kalydeco (ivacaftor) is a CFTR potentiator. It helps the CFTR protein work better by keeping chloride "gates" open so that salt and water can move more freely in the lungs. This helps prevent mucus buildup in the lungs, which helps improve cystic fibrosis and its related symptoms.
Source: DailyMed
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:
More common
Body aches or pain
chills
difficulty with breathing or trouble breathing
ear congestion
headache
loss of voice
runny or stuffy nose
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:
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.
Available in an oral granule form, which can be mixed in soft foods or liquids for children who cannot swallow tablets
Works well to treat cystic fibrosis in people with a CFTR mutation
Dose can be adjusted for people with mild liver disease
Keep in mind that taking Kalydeco (ivacaftor) every 12 hours is not the same as taking it twice a day. Set alarms or reminders for yourself to help remember to take it on time every 12 hours.
Always take Kalydeco (ivacaftor) with foods that are high in fat to help your body absorb the medication better.
Avoid foods or drinks with grapefruit, because these might raise the amount of Kalydeco (ivacaftor) in your body.
If you're taking the Kalydeco (ivacaftor) oral granules, pour and mix all of the granules into 1 teaspoon (about 5 mL) of soft food or liquid that's at or below room temperature. Some examples of soft foods include puréed fruits or vegetables, applesauce, yogurt, infant formula, or juice. Make sure your child takes the mixture within an hour of mixing it, right before or right after eating a high-fat meal.
Talk to your provider before starting, changing, or stopping any medications while taking Kalydeco (ivacaftor), including prescription and non-prescription medications, vitamins, and supplements. Kalydeco (ivacaftor) can interact with a lot of medications, so it's important that your provider knows about all the medications you take or plan to take to make sure they're safe for you.
Kalydeco (ivacaftor) 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
Kalydeco (ivacaftor) can sometimes cause abnormally high liver test results. Your provider will check your liver before you start Kalydeco (ivacaftor), 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. While taking this medication, if you experience stomach pain, dark-colored urine, pale-colored stools, or yellowing of the skin or eyes, get medical help right away, as these can be signs of a liver problem.
Taking Kalydeco (ivacaftor) along with certain medications can affect the amount of Kalydeco (ivacaftor) 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 Kalydeco (ivacaftor) in your body. This can make Kalydeco (ivacaftor) not work as well.
Other medications, like the antifungal, fluconazole (Diflucan), can raise the levels of Kalydeco (ivacaftor) in your body. You'll also want to avoid grapefruit and grapefruit juice while taking this medication, because grapefruit can cause you to have too much medication in your body as well. This can lead to a higher risk of side effects.
In addition, Kalydeco (ivacaftor) can affect how other medications work, so make sure to tell your provider and pharmacist about all the medications and supplements you're taking before starting Kalydeco (ivacaftor).
Risk factors: Taking corticosteroid medications | Exposure to radiation
Kalydeco (ivacaftor) can cause cataracts (clouding of the lens of the eye) in children. Your child's provider will likely give your child an eye exam before starting the medication and throughout treatment.
Tablets for adults and children age 6 years and older: The typical dose is 150 mg by mouth every 12 hours with a high-fat meal.
Oral granules for children younger than 6 years of age: The dose will depend on the child’s age and weight. Dosage starts from 25 mg to 75 mg by mouth every 12 hours with a high-fat meal.
The dose might differ if you or your child have liver problems or if you're taking certain medications that can interact with Kalydeco (ivacaftor).
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.
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