Ivacaftor oral granules

What is this medicine?

IVACAFTOR (EYE va KAF tor) is used to treat cystic fibrosis (CF) in patients who have certain mutations in their CF gene.

How should I use this medicine?

Take this medicine by mouth just before or just after eating fat-containing food such as eggs, peanut butter, cheese pizza, or whole-milk dairy products. Follow the directions on the prescription label. First, mix the contents (granules) of the packet with 1 teaspoonful (5 mL) of soft food or liquid, such as yogurt, applesauce, water, breast milk, infant formula, milk, or juice. Mix well and eat or drink the mixture immediately. Take your medicine at regular intervals. Do not take it more often than directed. Do not stop taking except on your doctor's advice.

Talk to your pediatrician regarding the use of this medicine in children. While this drug may be prescribed for children as young as 6 months for selected conditions, precautions do apply.

What side effects may I notice from receiving this medicine?

Side effects that you should report to your doctor or health care professional as soon as possible:

• allergic reactions like skin rash, itching or hives, swelling of the face, lips, or tongue

• changes in vision

• dark urine

• general ill feeling of flu-like symptoms

• light-colored stools

• loss of appetite

• right upper belly pain

• unusually weak or tired

• yellowing of the eyes or skin

Side effects that usually do not require medical attention (report to your doctor or health care professional if they continue or are bothersome):

• diarrhea

• dizziness

• headache

• joint pain

• nausea

What may interact with this medicine?

Do not take this medicine with any of the following medications:

• certain medicines for seizures like carbamazepine, phenobarbital, phenytoin

• grapefruit juice

• rifabutin

• rifampin

• Seville oranges

• St. John's Wort

This medicine may also interact with the following medications:

• certain antibiotics like clarithromycin, erythromycin, telithromycin

• certain medicines for anxiety or sleep

• certain medicines for fungal infections like fluconazole, itraconazole, ketoconazole, posaconazole, and voriconazole

• cyclosporine

• digoxin

• tacrolimus

What if I miss a dose?

If you miss a dose, take it as soon as you can. If your next dose is to be taken in less than 6 hours, then do not take

the missed dose. Take the next dose at your regular time. Do not take double or extra doses.

Where should I keep my medicine?

Keep out of the reach of children.

Store at room temperature between 15 to 30 degrees C (59 to 86 degrees F). Throw away any unused medicine after the expiration date.

What should I tell my health care provider before I take this medicine?

They need to know if you have any of these conditions:

• kidney disease

• liver disease

• an unusual or allergic reaction to ivacaftor, other medicines, foods, dyes, or preservatives

• pregnant or breast-feeding

What should I watch for while using this medicine?

Tell your doctor or healthcare professional if your symptoms do not start to get better or if they get worse.

Your vision and blood may be tested before and during use of this medicine.

Tell your doctor or health care professional right away if you have any change in your eyesight.

You may get dizzy. Do not ride a bicycle, drive, or do anything that needs mental alertness until you know how this medicine affects you. Do not stand or sit up quickly. This reduces the risk of dizzy or fainting spells. Avoid cough and cold medicines, alcohol, and activities that may make you drowsy or dizzy.