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Ivacaftor

Ivacaftor is a CFTR potentiator used to treat cystic fibrosis in patients aged 1 month and older who have at least one responsive mutation in the CFTR gene.

Brand name Kalydeco
Drug class Cystic Fibrosis Transmembrane Conductance Regulator Potentiator
Rx Forms 2
Dr. Brian Staiger, PharmD, BCPS
Reviewed by Last reviewed Jul 10, 2026 Content updated Oct 2, 2026
🧬 Where this information comes from Click to expand

The clinical label records used on this page are FDA-filed Structured Product Labeling (SPL) retrieved through openFDA. We identified 1 clinical label set within the page’s selected clinical scope after exact ingredient or ingredient-combination matching and applicable route/form matching. Forms, routes, brands, labelers, strengths, and NDC product-record counts are built separately from matching FDA National Drug Code Directory records. The linked DailyMed document is a representative official source for verification, not the page’s only clinical source. View the linked label on DailyMed →

✓No current FDA shortage listed · checked Oct 5, 2026
Ivacaftor at a glance The quick version — the full detail is in the Patient Information Guide below.
Dr. Brian Staiger, PharmD, BCPS
The Pharmacist’s Take By · Clinical pharmacist · Written

Ivacaftor (Kalydeco), on the market since about 2012 and the first targeted drug of its kind, treats cystic fibrosis by helping a faulty CFTR channel (a cell gateway for chloride, part of salt) open more often so chloride flows more normally, but only in people whose genetic test shows a responsive mutation. Most people tolerate it well, and the side effects they notice are usually headache, cold-like symptoms such as a stuffy nose or sore throat, and some stomach upset. The one thing to watch is the liver, so call promptly about yellowing skin or eyes or dark urine, and report any new anxiety, depression or sleeplessness right away.

Clinical pearls

  • Every dose needs fat-containing food, such as eggs, cheese, peanut butter, whole milk or breast milk, or much less is absorbed.
  • Avoid grapefruit and grapefruit juice, and skip St. John’s wort, which can sharply weaken how well ivacaftor works.
  • Antifungals and antibiotics such as fluconazole or clarithromycin raise ivacaftor levels, so the dose usually needs adjusting while taking them.
  • Expect liver blood tests before starting and regularly through treatment, and children also need eye exams because cataracts have been reported.
How we write & review this content — our editorial policy →
How you get itPrescription only
Comes asOral powder, Tablet
Earliest FDA record2012
Companies listing it with the FDA1
FDA label last updatedMar 23, 2026
FDA’s strongest warning (boxed)None on the label

Patient information guide A plain-language walkthrough of Ivacaftor, written from its FDA label.

What Ivacaftor is used for

▾

Ivacaftor (Kalydeco) treats cystic fibrosis in patients with specific CFTR gene mutations. It is not effective for all CF patients — only those whose mutation allows the CFTR protein to be potentiated by ivacaftor.

  • Kalydeco is indicated for cystic fibrosis in patients aged 1 month and older who have at least one CFTR gene mutation responsive to ivacaftor, based on clinical data or lab testing
  • Responsive mutations include (but are not limited to) G551D, R117H, S549N, S549R, G178R, G551S, G1244E, G1349D, S1251N, S1255P, F508C, and many others listed in the full prescribing information
  • If the patient's genetic mutation is unknown, an FDA-cleared CF mutation test must be used before starting treatment
  • Kalydeco tablets are approved for patients aged 6 years and older; Kalydeco oral granules are approved for patients aged 1 month to less than 6 years
  • Kalydeco is not recommended for infants under 1 month of age

How Ivacaftor works

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Cystic fibrosis is caused by mutations in the CFTR gene, which make the CFTR protein — a chloride channel on the surface of cells lining the lungs and other organs — work poorly. Ivacaftor is a CFTR potentiator: it binds to the CFTR protein and helps the channel open more often (increasing what's called "channel gating"), allowing chloride to flow more normally across cell surfaces. This improvement helps correct the underlying problem in certain types of CF mutations, particularly those where the CFTR protein reaches the cell surface but doesn't function properly.

The degree of benefit depends on which mutation a patient has and how much functional CFTR protein is present at the cell surface. Ivacaftor is not effective for mutations where little or no CFTR protein reaches the surface at all.

Ivacaftor dosage

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Kalydeco is taken by mouth twice daily, roughly every 12 hours. It must always be taken with fat-containing food — the drug absorbs up to 4 times better with fat. For children under 6 years, Kalydeco oral granules are mixed into a small amount of soft food or liquid and consumed within one hour. For patients 6 years and older, Kalydeco tablets are swallowed whole.

  • Dosing for children under 6 is based on age and body weight — your prescriber will determine the right packet strength
  • Patients with moderate or severe liver impairment, or those taking certain interacting medications, may need a reduced dosing frequency
  • Follow your prescriber's exact instructions — do not adjust the dose on your own

Dosing details from the FDA-approved label

From the Ivacaftor prescribing information (“Dosage and Administration”). Doses are set by your prescriber for your product, condition, kidney function and age — never change a dose on your own.

  • Adults and pediatric patients aged 6 years and older
    • 150 mg orally every 12 hours (300 mg total daily dose) with fat-containing food
    • Swallow tablets whole; take with fat-containing food
  • 1 month to less than 2 months, 3 kg or greater
    • One packet (containing 5.8 mg ivacaftor) every 12 hours
    • Oral granules mixed with one teaspoon (5 mL) of soft food or liquid, given with fat-containing food; not recommended under 1 month of age
  • 2 months to less than 4 months, 3 kg or greater
    • One packet (containing 13.4 mg ivacaftor) every 12 hours
    • Oral granules mixed with one teaspoon (5 mL) of soft food or liquid, given with fat-containing food
  • 4 months to less than 6 months, 5 kg or greater
    • One packet (containing 25 mg ivacaftor) every 12 hours
    • Oral granules mixed with one teaspoon (5 mL) of soft food or liquid, given with fat-containing food
  • 6 months to less than 6 years, 5 kg to less than 7 kg
    • One packet (containing 25 mg ivacaftor) every 12 hours
    • Oral granules with fat-containing food
  • 6 months to less than 6 years, 7 kg to less than 14 kg
    • One packet (containing 50 mg ivacaftor) every 12 hours
    • Oral granules with fat-containing food
  • 6 months to less than 6 years, 14 kg or greater
    • One packet (containing 75 mg ivacaftor) every 12 hours
    • Oral granules with fat-containing food
  • Moderate hepatic impairment (Child-Pugh Class B), 6 months and older
    • 6 months to less than 6 years: one packet (25 mg, 50 mg, or 75 mg) of oral granules once daily based on age and weight in Table 1
    • 6 years and older: 150 mg orally once daily
    • Not recommended under 6 months of age
  • Severe hepatic impairment (Child-Pugh Class C), 6 months and older
    • 6 months to less than 6 years: one packet (25 mg, 50 mg, or 75 mg) of oral granules once daily or less frequently
    • 6 years and older: 150 mg orally once daily or less frequently
    • Use with caution; not recommended under 6 months of age
  • Patients 6 months and older taking CYP3A inhibitors
    • Moderate inhibitors: once daily (packet 25, 50 or 75 mg for 6 months to less than 6 years
    • 150 mg for 6 years and older)
    • Strong inhibitors: packet twice a week (6 months to less than 6 years)
    • 150 mg twice weekly (6 years and older)
    • Not recommended under 6 months of age; avoid grapefruit
Read the label’s full dosing text

2 DOSAGE AND ADMINISTRATION Age Weight Dosage Administration 1 month to less than 2 months 3 kg or greater One 5.8 mg packet every 12 hours Mixed with one teaspoon (5 mL) of soft food or liquid and administered orally with fat-containing food 2 months to less than 4 months 3 kg or greater One 13.4 mg packet every 12 hours 4 months to less than 6 months 5 kg or greater One 25 mg packet every 12 hours 6 months to less than 6 years 5 kg to less than 7 kg One 25 mg packet every 12 hours 7 kg to less than 14 kg One 50 mg packet every 12 hours 14 kg or greater One 75 mg packet every 12 hours 6 years and older - One 150 mg tablet every 12 hours Taken orally with fat-containing food See full prescribing information for the recommended dosage in patients aged 6 months and older with moderate or severe hepatic impairment. ( 2.3 , 8.6 ) See full prescribing information for dosage modifications due to drug interactions with KALYDECO. ( 2.4 , 7.1 ) Not recommended in pediatric patients less than 1 month of age. ( 2.2 , 8.4 ) Not recommended in patients 1 month to less than 6 months of age with any level of hepatic impairment and/or taking concomitant moderate or strong CYP3A inhibitors. ( 2.3 , 2.4 , 8.6 ) 2.1 Recommended Dosage in Adults and Pediatric Patients Aged 6 Years and Older The recommended dosage of KALYDECO for adults and pediatric patients aged 6 years and older is 150 mg orally every 12 hours (300 mg total daily dose) with fat-containing food [ see Dosage and Administration (2.5) ]. 2.2 Recommended Dosage in Pediatric Patients Aged 1 Month to Less than 6 Years The recommended dosage of KALYDECO (oral granules) for pediatric patients aged 1 month to less than 6 years is weight-based provided in Table 1. Take KALYDECO orally with fat-containing food [see Dosage and Administration (2.5) ] . Table 1: Recommended Dosage of KALYDECO Oral Granules by Body Weight in Pediatric Patients Aged 1 Month to Less than 6 Years Age Body Weight (kg) KALYDECO Dosage 1 month to less than 2 months KALYDECO is not recommended for use in pediatric patients under 1 month of age. Use of KALYDECO in pediatric patients aged 1 to less than 6 months born at a gestational age less than 37 weeks has not been evaluated.

Excerpted — the section continues on the label. Read the full Dosage and Administration section on DailyMed →

Ivacaftor side effects

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The most commonly reported side effects with Kalydeco include:

Common side effects

  • Headache
  • Upper respiratory tract infection (common cold symptoms)
  • Nasal congestion
  • Sore throat or throat pain
  • Stomach pain
  • Diarrhea or nausea
  • Rash
  • Dizziness

When to get medical help

  • Call your doctor right away if you notice yellowing of the skin or eyes, dark urine, or severe stomach pain — these may be signs of liver problems
  • Get emergency help for signs of a serious allergic reaction: hives, swelling of the face or throat, difficulty breathing (anaphylaxis has been reported)
  • Seek prompt medical care for unusual or severe headaches, blurred or double vision, or any changes in eyesight — these may signal increased pressure in the skull (intracranial hypertension)
  • Contact your doctor immediately if you notice new or worsening anxiety, depression, trouble sleeping, or any thoughts of self-harm
  • Tell your doctor if your child develops any vision changes — cataracts have been reported in pediatric patients

More serious reactions have also been reported: liver enzyme elevations (watch for yellowing skin or eyes, unusual fatigue), severe allergic reactions including anaphylaxis, increased pressure in the skull, and neuropsychiatric symptoms like depression or suicidal thoughts. Contact your doctor promptly if any of these occur.

Ivacaftor warnings and precautions

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  • Liver toxicity: Elevated liver enzymes have been reported, more commonly in younger children and those with prior liver enzyme abnormalities. Liver function must be tested before starting treatment and monitored regularly throughout. Dosing should be paused if levels rise significantly.
  • Neuropsychiatric events: Anxiety, depression, insomnia, and suicidal thoughts or behavior have been reported — in adults and children alike, sometimes within the first three months. Monitor carefully and report any changes in mood or behavior to a doctor right away.
  • Intracranial hypertension (increased pressure in the skull): Has been reported with this drug class. Seek prompt evaluation for unusual headaches, blurred or double vision, or any vision changes.
  • Severe allergic reactions (anaphylaxis): Stop taking Kalydeco and get emergency help immediately if you have signs like throat swelling, hives, or difficulty breathing.
  • Cataracts: Non-congenital cataracts (lens clouding) have been found in pediatric patients. Baseline and follow-up eye exams are recommended for all children starting Kalydeco.
  • Strong CYP3A inducers: Drugs like rifampin and St. John's wort sharply reduce ivacaftor levels, making it less effective; avoid combining them.

Ivacaftor interactions

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Several drugs and foods can significantly change how much ivacaftor is in your body, affecting either its safety or effectiveness:

  • Strong CYP3A inhibitors (ketoconazole, itraconazole, voriconazole, posaconazole, clarithromycin, telithromycin) — raise ivacaftor levels dramatically (up to ~8.5-fold); dose reduction required for patients 6 months and older; not recommended at all for those under 6 months
  • Moderate CYP3A inhibitors (fluconazole, erythromycin) — raise ivacaftor levels about 3-fold; dose reduction required for patients 6 months and older; not recommended under 6 months
  • Strong CYP3A inducers (rifampin, rifabutin, carbamazepine, phenytoin, phenobarbital, St. John's wort) — drastically reduce ivacaftor levels; avoid co-administration
  • Grapefruit and grapefruit juice — contain natural CYP3A inhibitors that can raise ivacaftor levels; avoid entirely during treatment
  • Warfarin — ivacaftor may affect warfarin's blood-thinning effect; INR monitoring is recommended
  • Digoxin, cyclosporine, tacrolimus — ivacaftor may raise levels of these drugs; use with caution and monitor closely
  • Glimepiride and glipizide (diabetes medications) — ivacaftor may increase their exposure; use with caution

Interactions listed in the FDA-approved label

From the Ivacaftor prescribing information (“Drug Interactions”): what the manufacturer studied or reported to the FDA.

  • Strong CYP3A inhibitors (e.g., ketoconazole, itraconazole, posaconazole, voriconazole, telithromycin, clarithromycin): Ketoconazole increased ivacaftor exposure (AUC) by 8.5-fold. Reduce KALYDECO dosage in patients aged 6 months and older; not recommended in patients less than 6 months of age.
  • Moderate CYP3A inhibitors (e.g., fluconazole, erythromycin): Fluconazole increased ivacaftor exposure by 3-fold. Reduce KALYDECO dosage in patients aged 6 months and older; not recommended in patients less than 6 months of age.
  • Grapefruit juice / food or drink containing grapefruit: Contains components that moderately inhibit CYP3A and may increase ivacaftor exposure. Avoid food or drink containing grapefruit during treatment.
  • Strong CYP3A inducers (rifampin, rifabutin, phenobarbital, carbamazepine, phenytoin, St. John's wort): Rifampin decreased ivacaftor exposure (AUC) by approximately 9-fold. Co-administration is not recommended.
  • Ciprofloxacin: Had no effect on the exposure of ivacaftor. No dosage adjustment is necessary.
  • CYP2C9 substrates (warfarin, glimepiride, glipizide): Ivacaftor may inhibit CYP2C9; exposure to glimepiride and glipizide may be increased. Monitor INR with warfarin; use glimepiride and glipizide with caution.
  • CYP3A and/or P-gp substrates (digoxin, cyclosporine, tacrolimus): Ivacaftor weakly inhibits CYP3A and P-gp; midazolam exposure rose 1.5-fold and digoxin exposure 1.3-fold, which may increase or prolong therapeutic effect and adverse events. Use caution and appropriate monitoring when co-administering with sensitive CYP3A and/or P-gp substrates.
Read the label’s full interactions text

7 DRUG INTERACTIONS Potential for other drugs to affect ivacaftor CYP3A inhibitors: Reduce KALYDECO dosage in patients aged 6 months and older when co-administered with strong CYP3A inhibitors (e.g., ketoconazole) or moderate CYP3A inhibitors (e.g., fluconazole). KALYDECO is not recommended in patients aged 1 month to less than 6 months when co-administered with strong or moderate CYP3A inhibitors. Avoid food or drink containing grapefruit. ( 2.4 , 7.1 ) 7.1 Inhibitors of CYP3A Ivacaftor is a sensitive CYP3A substrate. Co-administration with ketoconazole, a strong CYP3A inhibitor, significantly increased ivacaftor exposure [measured as area under the curve (AUC)] by 8.5-fold. Based on simulations of these results, a reduction of the KALYDECO dosage is recommended for patients aged 6 months and older taking concomitant strong CYP3A inhibitors, such as ketoconazole, itraconazole, posaconazole, voriconazole, telithromycin, and clarithromycin. KALYDECO is not recommended for patients less than 6 months of age taking strong CYP3A inhibitors [see Dosage and Administration (2.4) and Clinical Pharmacology (12.3) ] . Co-administration with fluconazole, a moderate inhibitor of CYP3A, increased ivacaftor exposure by 3-fold. Therefore, a reduction of the KALYDECO dosage is recommended for patients aged 6 months and older taking concomitant moderate CYP3A inhibitors, such as fluconazole and erythromycin. KALYDECO is not recommended for patients less than 6 months of age taking moderate CYP3A inhibitors [see Dosage and Administration (2.4) and Clinical Pharmacology (12.3) ] . Co-administration of KALYDECO with grapefruit juice, which contains one or more components that moderately inhibit CYP3A, may increase exposure of ivacaftor. Therefore, avoid food or drink containing grapefruit during treatment with KALYDECO [ see Clinical Pharmacology (12.3) ].

Excerpted — the section continues on the label. Read the full Drug Interactions section on DailyMed →

Not a complete list. The label covers the interactions the manufacturer studied or reported, and our own interaction database does not cover every medicine either. Bring your full medication list, including vitamins and herbal products, to your pharmacist.

🌿138 supplements and herbs with documented Ivacaftor interactions, ranked by severity Check Ivacaftor against a supplement or herb →

Before taking Ivacaftor

▾
  • Patients with liver impairment: dose adjustments are needed for moderate or severe liver disease in patients 6 months and older; Kalydeco is not recommended at any dose for patients under 6 months with any level of liver impairment
  • Patients with severe kidney disease or end-stage renal disease: use with caution; Kalydeco has not been studied in these populations
  • Pregnancy: human data are limited; animal studies did not show birth defects at typical doses, but higher doses caused fetal weight changes; discuss risks and benefits with your doctor
  • Breastfeeding: it is not known whether ivacaftor passes into human breast milk; discuss with your healthcare provider before breastfeeding while on this medication
  • Infants under 1 month of age: safety and effectiveness have not been established; Kalydeco is not recommended
  • Infants 1 to less than 6 months born before 37 weeks of pregnancy (premature): use in this group has not been evaluated
  • Pediatric patients: baseline eye exams and regular follow-up are recommended due to cataract risk
  • All patients: liver function tests are required before starting and throughout treatment
  • Older adults (65+): CF primarily affects younger patients; there is limited data in those 65 and over

Ivacaftor overdose

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There have been no reported cases of overdose with Kalydeco. There is no specific antidote available. If an overdose is suspected, treatment is supportive — monitoring vital signs and the patient's overall condition. Contact Poison Control or seek emergency care right away if you think too much has been taken.

What Ivacaftor does in the body

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One measurable effect of ivacaftor is a reduction in sweat chloride levels — a standard biomarker used to assess CFTR function in CF. Across clinical trials, Kalydeco produced meaningful reductions in sweat chloride in patients with responsive mutations, including in very young infants. However, the size of the sweat chloride drop does not directly predict how much lung function improves for a given patient. Studies also showed that ivacaftor at standard and higher doses did not cause meaningful changes in heart rhythm (QTc interval), which is reassuring from a cardiac safety standpoint.

How your body processes Ivacaftor

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Ivacaftor is taken by mouth and reaches peak blood levels in about 4 hours when taken with a fatty meal. Absorption increases 2.5- to 4-fold when taken with fat, which is why food requirements are strict. The drug is about 99% bound to proteins in the blood and is broken down primarily by liver enzymes (CYP3A) into metabolites, one of which (M1) retains some activity. The elimination half-life is roughly 12 hours, and steady-state levels are reached within 3 to 5 days of twice-daily dosing. Most of the drug (about 88%) leaves the body through the stool after metabolism; very little is excreted unchanged in the urine.

How to store Ivacaftor

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Store at 20°C-25°C (68°F-77°F); excursions permitted to 15°C-30°C (59°F-86°F) .

Written from the FDA-approved label with AI and reviewed by our pharmacy team. How we use AI →

138

Supplements & herbs that interact with Ivacaftor

138 supplements and herbal products have documented interactions with Ivacaftor in the licensed clinical database we use. Most are manageable — but your pharmacist should know about everything you take, including vitamins and herbals.

Severity mix
  • Major · 3
  • Moderate · 94
  • Minor · 41
ACTIVATED CHARCOAL Major GRAPEFRUIT Major ST. JOHN'S WORT Major ACACIA RIGIDULA Moderate ADRAFINIL Moderate AFRICAN WILD POTATO Moderate AMERICAN ELDER Moderate ASTAXANTHIN Moderate +130 more
Every supplement checked against Ivacaftor — ranked by severity The full interaction hub: 3 major · 94 moderate · 41 minor · every one linked to its full report. Check Ivacaftor against your exact supplements

Educational information from a licensed clinical database (Natural Medicines) — see our data sources & update cadence. Not medical advice: an interaction being documented does not mean it will happen to you; do not start or stop medications or supplements without professional guidance.

Ivacaftor: pharmacist answers to common questions

Quick, plain-English answers to the questions patients ask most about Ivacaftor — the kind of thing our pharmacy team would talk through with you at the counter.

What exactly does Kalydeco do for cystic fibrosis?

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Cystic fibrosis is caused by a faulty protein called CFTR, which is supposed to act like a gate letting salt and water move across cell surfaces in your lungs and other organs. In CF, that gate is broken in different ways depending on your specific mutation. Kalydeco helps prop that gate open more often — so chloride can flow more normally. It doesn't cure CF, but in people with the right mutations, it helps the underlying problem work better, which can improve lung function and overall health.

Does Kalydeco work for everyone with cystic fibrosis?

▾
No — and this is really important. Kalydeco only works if you have a CFTR mutation that responds to ivacaftor. There are many different CF mutations, and not all of them make a protein that this drug can help. Your doctor will confirm your genetic mutation before prescribing it. If your specific mutation isn't on the responsive list, Kalydeco won't be effective for you, and a different CF treatment would be more appropriate.

Why do I have to eat fatty food every time I take it?

▾
This isn't just a suggestion — it's essential for the medicine to work properly. Studies show that ivacaftor absorbs up to four times better into your bloodstream when taken with fat-containing food compared to taking it on an empty stomach. Good options include eggs, peanut butter, cheese, whole milk, yogurt, or cheese pizza. If you consistently take it without fat, you may not be getting enough of the drug in your system to get the full benefit.

What side effects should I watch out for and which ones need a doctor's call?

▾
The most common side effects are things like headache, nasal congestion, sore throat, stomach pain, nausea, rash, and dizziness — mostly manageable and often mild. But there are some things that need a call to your doctor right away: any yellowing of the skin or eyes (which can signal liver trouble), unusual or severe headaches and vision changes (which can mean increased pressure in the skull), new anxiety, depression, or thoughts of self-harm, and in children, any changes in vision that could relate to cataracts. A serious allergic reaction — throat swelling, hives, difficulty breathing — is a 911 situation.

Why does my doctor keep checking my liver blood tests while I'm on this?

▾
Kalydeco has been linked to elevated liver enzymes in some patients, meaning the liver shows signs of stress. This is more common in younger children and those who already had some liver enzyme changes before starting. The good news is that when caught early through regular blood tests, the problem can usually be managed by pausing the medication. Your doctor will check your levels before you start, every three months in the first year, and once a year after that. Don't skip these — they're a key part of staying safe on this medication.

Are there any medications or foods I need to avoid while taking Kalydeco?

▾
Yes, a few important ones. Grapefruit and grapefruit juice should be avoided completely — they can raise ivacaftor levels in your blood in an unpredictable way. Several common prescription drugs can also interfere: some antifungals (like ketoconazole or fluconazole), certain antibiotics (like clarithromycin or erythromycin), and some seizure medications (like carbamazepine or phenytoin) can either raise ivacaftor levels too high or drop them so low the drug stops working. The herbal supplement St. John's wort has the same problem. Always tell every doctor and pharmacist you see that you're taking Kalydeco before starting any new medication.

Is Ivacaftor available over the counter?

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No. Ivacaftor is a prescription medicine: every Ivacaftor product listed with the FDA is prescription only, so it is not sold over the counter. Your prescriber decides whether it is appropriate for you, and your pharmacist dispenses it.

When was Ivacaftor first available?

▾
The earliest FDA record we hold for Ivacaftor is from 2012: its first approval in Drugs@FDA or its earliest product listing in the NDC Directory, whichever is older. Many older medicines were in use before their first formal FDA approval, so this is the earliest date on record, not necessarily when Ivacaftor was first used.

Answers drafted from the FDA-approved label with AI and reviewed by our pharmacy team. How we use AI →

More about Ivacaftor on HelloPharmacist

📊Ivacaftor supplement interactions138 checked — ranked by severity 🌿Drug & Supplement CheckerIvacaftor vs. vitamins & herbs 💬Ask a PharmacistFree answer, usually < 24h 🔎NDC & pricing lookupFind Ivacaftor NDCs & costs 🅰️Browse Drugs A–ZLook up any medication
Detailed data & references for Ivacaftor Forms & strengths, pricing, billing codes, manufacturers, safety reports and sourcing — the data behind the page. Most people can stop here. Each section below opens on a tap.

Ivacaftor forms and strengths (how it comes)

Ivacaftor is available in 2 forms. Different forms and brands can have different approved uses, doses, schedules, and directions. Follow the instructions for your exact product, and do not switch forms without guidance from your prescriber or pharmacist.

Oral powder

By mouth
Brands Kalydeco
How this form is used
What it may be used for
  • Kalydeco oral granules are used to treat cystic fibrosis in patients aged 1 month to less than 6 years who have at least one responsive CFTR gene mutation
  • Kalydeco oral granules are dosed based on the child's age and body weight, as directed by the prescriber
Important instructions
  • Kalydeco oral granules should be mixed with one teaspoon (5 mL) of age-appropriate soft food or liquid (such as puréed fruit, yogurt, applesauce, water, breast milk, or infant formula) at or below room temperature
  • Administer Kalydeco oral granules immediately before or after fat-containing food
  • Once mixed, the full dose must be consumed within one hour
  • Each dose of Kalydeco oral granules is given every 12 hours (twice daily) unless your prescriber has adjusted the schedule due to liver problems or drug interactions
Available strengths 5 strengths
5.8 mg 13.4 mg 25 mg 50 mg 75 mg
13 FDA-listed product records ⓘ

Tablet

By mouth
Brands Kalydeco
How this form is used
What it may be used for
  • Kalydeco tablets are used to treat cystic fibrosis in patients aged 6 years and older who have at least one responsive CFTR gene mutation
Important instructions
  • Kalydeco tablets are taken by mouth every 12 hours (twice daily)
  • Kalydeco tablets must always be taken with fat-containing food such as eggs, peanut butter, cheese, or whole-milk dairy products
  • Swallow Kalydeco tablets whole — do not crush or chew
Available strengths 1 strength
150 mg
5 FDA-listed product records ⓘ

Ivacaftor on the U.S. market: products, makers and brands

How Ivacaftor appears in the FDA’s National Drug Code Directory — including its dosage forms, strengths, brand names, manufacturers, and FDA-listed product records. These are directory records, not sales or prescription volume.

1 company lists 6 Ivacaftor product records with the FDA, mostly as oral powder, tablet; the earliest marketing or approval year on record is 2012. Brand names on the directory include Kalydeco; the rest are generics.

2
Dose forms ⓘ
1
Labelers (makers, repackagers, distributors)
1
Brand names
6
FDA-listed product records ⓘ
2012
Earliest FDA record ⓘ

FDA-listed product records by dose form (tablet variants merged) — FDA National Drug Code Directory. Record counts are not sales or prescription volume.

Also listed with the FDA, not a patient dose form: Powder (20) · Granule (1) — bulk drug substance sold to compounders/manufacturers and multi-item kits. These aren’t counted in the dose-form total above.

How widely Ivacaftor is used (Medicaid data)

A general measure of how commonly Ivacaftor is prescribed, based on Medicaid — one of the largest public drug programs. The figure below is prescriptions filled in Q1–Q4 2025, summed across every form and brand of the drug.

Think of this as a proxy for how widely Ivacaftor is used, drawn from freely available public data. Medicaid is just one program — these numbers don’t include Medicare, other government coverage, or commercial and cash-pay prescriptions, so real-world use is higher than what’s shown here.

53,353
Medicaid prescriptions filled (Q1–Q4 2025)
$1.43B gross Medicaid reimbursement (before mandatory rebates — net cost is lower)

How commonly is Ivacaftor prescribed? Of the 1,601 medications we measure, Ivacaftor ranks #621 by Medicaid prescriptions — more than 61% of them.

Where Ivacaftor ranks among the drugs we measure · Q1–Q4 2025 · neighbors ±5
Total prescriptions — #621 of 1,601
#616 Ivermectin 55,437 Rx #617 Abatacept Injection 55,268 Rx #618 Fluticasone Topical 55,053 Rx #619 Darunavir, Cobicistat,... 54,928 Rx #620 Entecavir 54,796 Rx #621 Ivacaftor 53,353 Rx #622 Tadalafil 53,237 Rx #623 Cefepime Injection 52,968 Rx #624 Sulindac 52,720 Rx #625 Leucovorin Injection 52,618 Rx #626 Cefprozil 52,006 Rx

Utilization by Ivacaftor product

Pick a product to see its own Medicaid numbers. Each chip is one clinical product — a specific strength & dosage form as defined by RxNorm — with brand-name and generic versions combined.

The 150 mg oral tablet is the most-dispensed Ivacaftor product — about 2% of these Medicaid prescriptions.

ivacaftor 150 MG Oral Tablet Most dispensed

1,012 Medicaid prescriptions (Q1–Q4 2025) 2% of Ivacaftor prescriptions shown $28.3M gross reimbursement (before rebates) ≈ $27,922 per prescription (gross)

Summed across the 1 of 5 listed NDC products with Medicaid activity — every brand and generic of this exact strength & form. RxNorm 1243046

ivacaftor 50 MG Oral Granules

206 Medicaid prescriptions (Q1–Q4 2025) <1% of Ivacaftor prescriptions shown $5.6M gross reimbursement (before rebates) ≈ $27,163 per prescription (gross)

Summed across the 1 of 1 listed NDC products with Medicaid activity — every brand and generic of this exact strength & form. RxNorm 1606862

ivacaftor 75 MG Oral Granules

186 Medicaid prescriptions (Q1–Q4 2025) <1% of Ivacaftor prescriptions shown $6.5M gross reimbursement (before rebates) ≈ $35,117 per prescription (gross)

Summed across the 1 of 1 listed NDC products with Medicaid activity — every brand and generic of this exact strength & form. RxNorm 1606868

Other Ivacaftor products — combination packs and listings not yet matched to a specific clinical product (kept visible so every prescription is accounted for)

51,949 Medicaid prescriptions (Q1–Q4 2025) 97% of Ivacaftor prescriptions shown $1.39B gross reimbursement (before rebates) ≈ $26,845 per prescription (gross)

Summed across the 6 of 22 listed NDC products with Medicaid activity.

Shares are of the Medicaid prescriptions shown here. Product grouping follows RxNorm (the National Library of Medicine’s drug terminology), so “one product” means one strength & dosage form regardless of manufacturer. Dollar figures are gross Medicaid reimbursement before mandatory rebates — rebates (often large, especially for brand-name drugs) are confidential, so actual net cost to Medicaid is lower than shown.

Source: Medicaid State Drug Utilization Data (CMS), aggregated by generic ingredient — summed across every NDC (all brands, strengths, salt forms and dosage forms) of Ivacaftor, and across all states and both fee-for-service and managed-care claims. A general popularity signal; it excludes Medicare, commercial insurance and cash prescriptions, so it is not total U.S. use. Based on the 9 of 29 listed Ivacaftor NDCs with Medicaid activity.

Compare Ivacaftor products

A representative set of FDA-listed product records for Ivacaftor — across manufacturers, strengths, and dosage forms, grouped by form with each product’s manufacturer and how the FDA approved it. The same medicine may be made and packaged by different companies, so a single drug can have many directory entries. (It’s also why a refill can look different — a new shape, color, or box — even though it contains the same medication.)

Strength Route Manufacturer / Labeler Category ⓘ Details
💊Oral powder
5.8 mg Oral Vertex Pharmaceuticals Incorporated NDA View NDC →
13.4 mg Oral Vertex Pharmaceuticals Incorporated NDA View NDC →
25 mg Oral Vertex Pharmaceuticals Incorporated NDA View NDC →
50 mg Oral Vertex Pharmaceuticals Incorporated NDA View NDC →
75 mg Oral Vertex Pharmaceuticals Incorporated NDA View NDC →
💊Tablet
150 mg Oral Vertex Pharmaceuticals Incorporated NDA View NDC →

Showing 6 of 6 products — FDA National Drug Code Directory. See every product, package size and price: browse all 6 Ivacaftor NDCs →

Ivacaftor recall history

A recall is when a specific batch of a medicine is pulled from the market — usually a manufacturing issue, not a problem with the drug itself. Class I is most serious, Class III least.

The FDA’s enforcement reports list no Ivacaftor recalls since July 2021.

✅No Ivacaftor recalls on record since July 2021

Source: FDA enforcement (recall) reports, via openFDA; our copy was last updated Sep 24, 2026.

RxNorm Concept Web — From Ingredient to Every Form and Strength

This page starts with one ingredient concept (IN). The branches below show its dose-form concepts (SCDF), then the available clinical drug and strength concepts (SCD). Combination products remain separate concepts with their own pages.

Ingredient (IN) Ivacaftor RxCUI 1243041 2 dose forms · 8 strengths
  1. Dose form (SCDF) Oral Granules RxCUI 1606861 In current FDA listings
    Clinical drug / strength (SCD) 5.8 MG RxCUI 2636791 13.4 MG RxCUI 2636787 25 MG RxCUI 2166399 50 MG RxCUI 1606862 59.5 MG RxCUI 2635020 75 MG RxCUI 1606868
  2. Dose form (SCDF) Oral Tablet RxCUI 1243045 In current FDA listings
    Clinical drug / strength (SCD) 75 MG RxCUI 2174388 150 MG RxCUI 1243046

Look up RxCUI 1243041 in the RxCUI Atlas →

RxNorm is the U.S. National Library of Medicine’s normalized drug vocabulary. Its concept hierarchy can include forms that are not currently marketed; the status on each branch compares that concept with current FDA listings. RxCUIs identify vocabulary concepts, not individual packages or manufacturers.

Ivacaftor brand names

Ivacaftor is sold under one brand name in the FDA directory — Kalydeco. A brand and its generic contain the same active ingredient; the brand name belongs to one company's product.

Kalydeco

Who makes Ivacaftor: manufacturers and labelers

One company lists Ivacaftor products with the FDA. By number of product records, the largest are Vertex Pharmaceuticals Incorporated. Labelers include manufacturers and repackagers; the product in your bottle depends on which one your pharmacy stocks.

Vertex Pharmaceuticals Incorporated 6

Ivacaftor drug class (RxNorm)

Ivacaftor belongs to the Cystic Fibrosis Transmembrane Conductance Regulator Potentiator class.

Pharmacologic class Cystic Fibrosis Transmembrane Conductance Regulator Potentiator
Drug family (ATC) Other respiratory system products
How it works Chloride Channel Activation Potentiators, P-Glycoprotein Inhibitors, Cytochrome P450 2C9 Inhibitors, Cytochrome P450 3A Inhibitors

Classified by RxNorm RxClass — the U.S. National Library of Medicine's standardized drug-classification service (Established Pharmacologic Class from the FDA, the ATC drug family from the WHO, and mechanism of action). Reference only, not medical advice.

Sources for this Ivacaftor page

Core label, product, RxNorm, safety, and utilization data on this page come from the sources identified below. AI-written, editorial, and licensed sections are labeled separately.

📄
FDA-filed drug labels (SPL) Uses, dosing, warnings & side effects from SPL records retrieved through openFDA; the linked representative set can be verified on DailyMed. ✓ Linked label effective Mar 23, 2026
🏭
Forms, brands & labelersAggregated from ingredient-matched FDA National Drug Code Directory product records for Ivacaftor.
📈
Real-world reportsThe FDA Adverse Event Reporting System (FAERS), served through openFDA.
🚨
Recalls & shortagesopenFDA drug-enforcement reports and the FDA drug-shortage database.
🧾 Clinical label set — FDA-filed SPL labels retained for Ivacaftor after exact ingredient or ingredient-combination matching and applicable route/form matching
Clinical labels in scope
1
Finished products in the current FDA directory
6
Companies listing them (makers, repackagers, distributors)
1
FDA labeler codes behind them ⓘ
1
Linked representative label ⓘ
Vertex Pharmaceuticals Incorporated
Linked label effective
Mar 23, 2026
Composite sections available
22
Linked SPL Set ID
0ab0c9f8-3eee-4e0f-9f3f-c1e16aaffe25
Linked SPL Document ID
13eb3fc2-0dac-4ada-96a8-a2e0cea711b7
Open the linked representative label on DailyMed ↗

How this page is built

HelloPharmacist combines public FDA and NLM records and does not author the underlying clinical facts. The plain-language sections are written with AI from the FDA-approved label text and reviewed by our pharmacy team before publication. Vertex Pharmaceuticals Incorporated is the representative DailyMed label linked for verification and dates. This is general education, not medical advice: always talk to your doctor or pharmacist about your own medicines. How we build and review drug pages →