Medically reviewed on June 7, 2018
Commonly used brand name(s)
In the U.S.
Available Dosage Forms:
Therapeutic Class: Respiratory Agent
Uses For This Medicine
Ivacaftor is used to treat cystic fibrosis (CF) in patients 2 years of age and older. It is only used for patients who have the following mutations in the CF transmembrane conductance regulator (CFTR) gene: G551D, G1244E, G1349D, G178R, G551S, R117H, S1251N, S1255P, S549N, or S549R mutations. Your doctor will use a test to check for the mutation before you receive the medicine. Ivacaftor is a CFTR potentiator, and works by moving more chloride into the cells of your body.
Ivacaftor 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 ivacaftor, the following should be considered:
Tell your doctor if you have ever had any unusual or allergic reaction to ivacaftor 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.
Appropriate studies performed to date have not demonstrated pediatric-specific problems that would limit the usefulness of ivacaftor in children. However, safety and efficacy have not been established in children younger than 2 years of age.
Although appropriate studies on the relationship of age to the effects of ivacaftor have not been performed in the geriatric population, no geriatric-specific problems have been documented to date.
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.
Interactions with 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 ivacaftor, 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 ivacaftor 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.
- Morphine Sulfate Liposome
- St John's Wort
Interactions with Food/Tobacco/Alcohol
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.
Other Medical Problems
The presence of other medical problems may affect the use of ivacaftor. Make sure you tell your doctor if you have any other medical problems, especially:
- Cataracts—Use with caution. May make this condition worse.
- Kidney disease, severe or
- Liver disease, moderate or severe—Use with caution. The effects may be increased because of slower removal of the medicine from the body.
Proper Use of This Medicine
Take ivacaftor 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.
Ivacaftor comes with a patient information insert. Read and follow the instructions in the insert carefully. Ask your doctor if you have any questions.
Take ivacaftor with fat-containing foods such as butter, cheese pizza, eggs, peanut butter, or whole milk dairy products (eg, whole milk, cheese, yogurt).
To use the oral granules:
- Shake the packet gently before opening it.
- Open one packet and pour the contents into 1 teaspoon of cold soft food or liquid (eg, applesauce, pureed fruits or vegetables, yogurt, water, milk, or juice).
- Mix the granules with the soft food or liquid.
- After mixing, give ivacaftor to your child within 1 hour. Make sure all medicine is taken.
Do not eat grapefruit or drink grapefruit juice while you are using ivacaftor. Grapefruit and grapefruit juice may cause the amount of medicine in the blood to be too high.
Do not eat Seville oranges (bitter or sour oranges) or drink Seville orange juice while taking ivacaftor. Seville oranges or juice may cause the amount of medicine in the blood to be too high.
The dose of ivacaftor 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 ivacaftor. 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 forms (granules or tablets):
- For cystic fibrosis:
- Adults and children 6 years of age and older—150 milligrams (mg) every 12 hours, taken with fat-containing food.
- Children 2 to 5 years of age and weighing 14 kilograms (kg) or more—Dose is based on body weight and must be determined by your doctor. The dose is usually one 75 mg packet every 12 hours, taken with fat-containing food.
- Children 2 to 5 years of age and weighing less than 14 kg—Dose is based on body weight and must be determined by your doctor. The dose is usually one 50 mg packet every 12 hours, taken with fat-containing food.
- Children younger than 2 years of age—Use is not recommended.
- For cystic fibrosis:
If you miss a dose of ivacaftor, 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, take it as soon as you can. If your next regular dose is less than 6 hours away, wait until then to use the medicine and skip the missed dose.
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 the progress of you or your child at regular visits to make sure that ivacaftor is working properly. Blood tests will be needed to check for unwanted effects.
Check with your doctor right away if you or your child have pain or tenderness in the upper stomach, pale stools, dark urine, loss of appetite, nausea, unusual tiredness or weakness, or yellow eyes or skin. These could be symptoms of a serious liver problem.
Ivacaftor may make you dizzy. Avoid driving, using machines, or doing anything else that could be dangerous if you are not alert.
Check with your doctor immediately if blurred vision, difficulty in reading, or any other change in vision occurs during or after treatment. Your doctor may want you to have your eyes 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.
This Medicine Side Effects
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:
- Body aches or pain
- chest pain
- difficulty with breathing or troubled breathing
- ear congestion
- loss of voice
- nasal congestion
- runny nose
- sore throat
- unusual tiredness or weakness
- Dark urine
- fever with or without chills
- light-colored stools
- loss of appetite
- nausea and vomiting
- stomach pain
- yellow eyes or skin
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:
- Blemishes on the skin
- difficulty with moving
- muscle aches
- muscle pain or stiffness
- pain in the joints
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.
See also: Side effects (in more detail)
Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.
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More about ivacaftor
- Ivacaftor Side Effects
- During Pregnancy
- Dosage Information
- Drug Interactions
- Support Group
- En Español
- 1 Review – Add your own review/rating
- Drug class: CFTR potentiators
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