Kalydeco (Ivacaftor)
Complete patient guide for uses, dosage, side effects, and safety information
Prescription RequiredQuick Facts
What Is Kalydeco Used For?
Kalydeco is a specialized medication designed to treat cystic fibrosis in patients who have specific genetic mutations that respond to this treatment.
Primary Uses
Kalydeco is used for the chronic treatment of cystic fibrosis (CF) in patients aged 1 month and older who have at least one mutation in the CFTR gene that is responsive to ivacaftor potentiation based on clinical and/or in vitro assay data.
How It Works
Kalydeco belongs to a group of medicines called CFTR modulators. In people with G551D or other gating mutations and in people with an R117H mutation, the CFTR protein is present in normal amounts but its ability to allow chloride to pass through is reduced. Kalydeco is a CFTR potentiator, which increases the opening of the CFTR protein, allowing more chloride to pass through and improving the flow of chloride and water in patients with cystic fibrosis.
Good to know: Kalydeco is not addictive.
How to Take Kalydeco
Your doctor will determine the exact amount of Kalydeco you need based on your age, weight, and condition. The medication comes in both tablet and granule forms.
Dosage Instructions
For patients 1 month to less than 2 months old (>3 kg): One sachet of 13.4 mg granules every 24 hours. For patients 2 months to less than 4 months old (≥3 kg): One sachet of 13.4 mg granules every 12 hours. For patients 4 months to less than 6 months old (≥5 kg): One sachet of 25 mg granules every 12 hours. For patients 6 months and older: dosing varies by weight from 25 mg to 150 mg granules every 12 hours, or 150 mg tablets every 12 hours for patients ≥25 kg. Always take with fat-containing food and avoid grapefruit-containing foods.
What If You Miss a Dose?
If you miss your dose, take it only if less than 6 hours have passed since the missed dose time. Otherwise, wait until your next scheduled dose. Take Kalydeco regularly at the same time each day for best results.
Important: Do not take a double dose to make up for a missed dose. This may increase the chance of unwanted side effects.
Available Tablet Strengths
Side Effects
Like all medications, this medicine can cause side effects. Most are mild and temporary, but some require medical attention.
Common Side Effects
These side effects occur relatively frequently and usually don’t require emergency care:
- Upper respiratory tract infection (common cold), including sore throat and nasal congestion
- Headache
- Abdominal pain
- Diarrhea
- Rash
- Sinus congestion
- Red throat
- Runny nose
- Dizziness
- Changes in the type of bacteria in mucus
- Ear pain or discomfort
- Ringing in the ears
- Redness inside the ear
Seek immediate medical attention if you experience:
- Significant pain or discomfort in the upper right abdominal area
- Yellowing of your skin or the white parts of your eyes
- Rash or hives
- Tightness of the chest or throat or difficulty breathing
- Light-headedness or dizziness
- Loss of appetite
- Nausea or vomiting
- Dark urine
Important Warnings
Before taking Kalydeco, it’s important to discuss your medical history and current medications with your doctor to ensure safe use.
Who Should Not Take Kalydeco
Do not use Kalydeco if you are allergic to ivacaftor or any of the ingredients listed in the medication. Do not give to children weighing less than 3 kg or under 1 month of age.
Medical Conditions to Discuss
Tell your doctor if you have liver or kidney disease, as your dose may need adjustment. Your doctor will perform blood tests to check your liver function before and during treatment, particularly during the first year. Eye examinations may be performed in children as cataracts without vision effects have been noted in some children receiving Kalydeco.
Pregnancy and Breastfeeding
Check with your doctor if you are pregnant or intend to become pregnant. It is unknown whether Kalydeco is excreted in human milk, so discuss with your doctor if you plan to breastfeed.
Drug Interactions
Several medications can interfere with Kalydeco and affect how it works. You may need different amounts of your medicines or different medicines entirely.
Grapefruit interaction: Avoid food containing grapefruit during treatment with Kalydeco as they may increase the amount of Kalydeco in your body.
- Antifungal medicines: ketoconazole, itraconazole, posaconazole, voriconazole, fluconazole
- Antibiotic medicines: telithromycin, clarithromycin, erythromycin, rifampicin, rifabutin
- Anticonvulsant medicines: phenobarbital, carbamazepine, phenytoin
- Anti-inflammatory medicines: dexamethasone, high-dose prednisone
- Herbal medicine: St. John’s wort (Hypericum perforatum)
- Benzodiazepines: midazolam, alprazolam, diazepam, triazolam
- Immunosuppressants: ciclosporin, tacrolimus
- Cardiac glycosides: digoxin
- Anticoagulants: warfarin
Alcohol & Driving
Kalydeco may affect your ability to drive or operate machinery safely.
Alcohol
No specific alcohol interactions are mentioned in the prescribing information.
Driving and Operating Machinery
Be careful before you drive or use any machines or tools until you know how Kalydeco affects you. Kalydeco may cause dizziness in some people. Do not drive or use machines unless you are sure that you are not affected. If your child is taking Kalydeco, ensure they do not ride bikes or do activities requiring full attention unless you are sure they are not affected.
Storage
Store Kalydeco below 30°C, in a dry place away from moisture, heat or sunlight. Keep this medicine where young children cannot reach it. Do not store in the bathroom, near a sink, in the car, or on window sills. Once mixed with food, Kalydeco granules are stable for one hour.
Overdose Information
If you think you have used too much Kalydeco, you may need urgent medical attention. Immediately phone the Poisons Information Centre (by calling 13 11 26), contact your doctor, or go to the Emergency Department at your nearest hospital. Do this even if there are no signs of discomfort or poisoning.
Frequently Asked Questions
The source does not specify how long Kalydeco takes to work. Your doctor will monitor your progress through regular appointments to assess how well the medication is working for you.
Yes, you must take Kalydeco with fat-containing food. Examples include meals prepared with butter or oils, eggs, cheese, whole milk, meats, oily fish, avocados, nuts, and fat-containing nutritional bars or drinks.
No, Kalydeco is not addictive.
Do not stop using Kalydeco or change the dose without first checking with your doctor. Always speak to your doctor or pharmacist before deciding to stop taking any of your medicines.
The prescribing information does not mention specific alcohol interactions with Kalydeco. However, you should discuss alcohol consumption with your doctor.
If you miss your dose, take it only if less than 6 hours have passed since the missed dose time. Otherwise, wait until your next scheduled dose. Never take a double dose to make up for a missed dose.
No, you should avoid food containing grapefruit during treatment with Kalydeco as they may increase the amount of Kalydeco in your body.
Important Disclaimer
This information is intended for general educational purposes only and should not be considered medical advice. Always consult your healthcare provider or pharmacist before starting, stopping, or changing any medication.
If you think you may have a medical emergency, call your doctor or emergency services immediately.