Active Ingredient: Ivacaftor
Ivacaftor is indicated:
For this indication, competent medicine agencies globally authorize below treatments:
For:
Oral, 150 milligrams ivacaftor, 2 times daily to meals.
Adults and adolescents should be dosed according to the following table.
Dosing recommendations:
| Morning | Evening | |
|---|---|---|
| Ivacaftor as monotherapy | ||
| 6 years and older, ≥25 kg | 150 mg | 150 mg |
| Ivacaftor in combination with tezacaftor/ivacaftor | ||
| 12 years and older | tezacaftor 100 mg/ivacaftor 150 mg | ivacaftor 150 mg |
| Ivacaftor in combination with ivacaftor/tezacaftor/elexacaftor | ||
| 12 years and older | ivacaftor 150 mg/tezacaftor 100 mg/elexacaftor 200 mg | ivacaftor 150 mg |
The morning and evening dose should be taken approximately 12 hours apart with fat-containing food.
If 6 hours or less have passed since the missed morning or evening dose, the patient should be advised to take it as soon as possible and then take the next dose at the regularly scheduled time. If more than 6 hours have passed since the time the dose is usually taken, the patient should be advised to wait until the next scheduled dose.
Patients receiving ivacaftor in a combination regimen should be advised not to take more than one dose of either medicinal product at the same time.
When co-administered with moderate or strong inhibitors of CYP3A, either as monotherapy or in a combination regimen with tezacaftor/ivacaftor or ivacaftor/tezacaftor/elexacaftor, the dose should bIe reduced (see table for the recommended dose). Dosing intervals should be modified according to clinical response and tolerability.
Dosing recommendations for concomitant use with moderate or strong CYP3A inhibitors:
| Moderate CYP3A inhibitors | Strong CYP3A inhibitors | |
|---|---|---|
| Ivacaftor as monotherapy | ||
| 12 years and older, ≥25 kg | One morning tablet of ivacaftor 150 mg once daily. No evening dose. | One morning tablet of ivacaftor 150 mg twice a week, approximately 3 to 4 days apart. No evening dose. |
| Ivacaftor in a combination regimen with tezacaftor/ivacaftor | ||
| 12 years and older | Alternate each morning: - one tablet of tezacaftor 100 mg/ivacaftor 150 mg on the first day - one tablet of ivacaftor 150 mg on the next day Continue alternating tablets each day. No evening dose. | One morning tablet of tezacaftor 100 mg/ivacaftor 150 mg twice a week, approximately 3 to 4 days apart. No evening dose. |
| Ivacaftor in a combination regimen with ivacaftor/tezacaftor/elexacaftor | ||
| 12 years and older | Alternate each morning: - two tablets of ivacaftor 75 mg/tezacaftor 50 mg/ elexacaftor 100 mg on the first day - one tablet of ivacaftor 150 mg on the next day Continue alternating tablets each day. No evening dose. | Two morning tablets of ivacaftor 75 mg/tezacaftor 50 mg/ elexacaftor 100 mg twice a week, approximately 3 to 4 days apart. No evening dose. |
Very limited data are available for elderly patients treated with ivacaftor (administered as monotherapy or in a combination regimen). No dose adjustment specific to this patient population is required.
Ivacaftor tablets should be taken with fat-containing food.
Food or drink containing grapefruit or Seville oranges should be avoided during treatment.
For:
Regimen A: In case that patient age in months is ≥ 4 and patient age in months is ≤ 6 and patient weight is ≥ 5 kg, oral, 25 milligrams ivacaftor, 2 times daily to meals.
Regimen B: In case that patient age in months is ≥ 6 and patient weight is ≥ 5 kg and patient weight is ≤ 7 kg, oral, 25 milligrams ivacaftor, 2 times daily to meals.
Regimen C: In case that patient age in months is ≥ 6 and patient weight is ≥ 7 kg and patient weight is ≤ 14 kg, oral, 50 milligrams ivacaftor, 2 times daily to meals.
Regimen D: In case that patient age in months is ≥ 6 and patient weight is ≥ 14 kg and patient weight is ≤ 25 kg, oral, 75 milligrams ivacaftor, 2 times daily to meals.
Regimen E: In case that patient age in months is ≥ 6 and patient weight is ≥ 25 kg, oral, 150 milligrams ivacaftor, 2 times daily to meals.
Infants aged at least 4 months, toddlers and children should be dosed according to the table.
Dosing recommendations for patients aged 4 months and older:
| Age | Weight | Dose | Total daily dose |
|---|---|---|---|
| 4 months to less than 6 months | ≥5 kg | 25 mg taken orally every 12 hours with fat-containing food | 50 mg |
| 6 months and older | ≥5 kg to <7 kg | 25 mg taken orally every 12 hours with fat-containing food | 50 mg |
| ≥7 kg to <14 kg | 50 mg taken orally every 12 hours with fat-containing food | 100 mg | |
| ≥14 kg to <25 kg | 75 mg taken orally every 12 hours with fat-containing food | 150 mg | |
| ≥25 kg | 150 mg taken orally every 12 hours with fat-containing food | 300 mg | |
Dosing recommendations for a combination regimen with tezacaftor/ivacaftor or ivacaftor/tezacaftor/elexacaftor for children aged 6 years and older:
| Morning | Evening | |
|---|---|---|
| Ivacaftor in combination with tezacaftor/ivacaftor | ||
| 6 years to <12 years, <30 kg | One tezacaftor 50 mg/ivacaftor 75 mg tablet | One ivacaftor 75 mg tablet |
| 6 years to <12 years, ≥30 kg | One tezacaftor 100 mg/ivacaftor 150 mg tablet | One ivacaftor 150 mg tablet |
| Ivacaftor in combination with ivacaftor/tezacaftor/elexacaftor | ||
| 6 years to <12 years, <30 kg | Two ivacaftor 37.5 mg/tezacaftor 25 mg/ elexacaftor 50 mg tablets | One ivacaftor 75 mg tablet |
| 6 years to <12 years, ≥30 kg | Two ivacaftor 75 mg/tezacaftor 50 mg/ elexacaftor 100 mg tablets | One ivacaftor 150 mg tablet |
The morning and evening dose should be taken approximately 12 hours apart with fat-containing food.
If 6 hours or less have passed since the missed morning or evening dose, the patient should be advised to take it as soon as possible and then take the next dose at the regularly scheduled time. If more than 6 hours have passed since the time the dose is usually taken, the patient should be advised to wait until the next scheduled dose.
When co-administered with strong inhibitors of CYP3A in patients aged 6 months and older, the ivacaftor dose should be reduced to one sachet (ivacaftor 25 mg for patients 5 kg to <7 kg; ivacaftor 50 mg for patients 7 kg to <14 kg; ivacaftor 75 mg for patients 14 kg to <25 kg) twice a week.
When co-administered with moderate inhibitors of CYP3A in patients aged 6 months and older, the ivacaftor dose is as above recommended but administered once daily.
Due to the variability in maturation of the cytochrome (CYP) enzymes involved in ivacaftor metabolism, treatment with ivacaftor is not recommended when co-administered with moderate or strong inhibitors of CYP3A in patients aged 4 months to less than 6 months, unless the benefits outweigh the risks. In such cases, the recommended dose is one packet of 25 mg granules twice weekly or less frequently. Dosing intervals should be modified according to clinical response and tolerability.
When co-administered with moderate or strong inhibitors of CYP3A, either as monotherapy or in a combination regimen with tezacaftor/ivacaftor or ivacaftor/tezacaftor/elexacaftor, the dose should be reduced. Dosing intervals should be modified according to clinical response and tolerability.
Dosing recommendations for concomitant use with moderate or strong CYP3A inhibitors in patients aged 6 years and older:
| Moderate CYP3A inhibitors | Strong CYP3A inhibitors | |
|---|---|---|
| Ivacaftor as monotherapy | ||
| 6 years and older, ≥25 kg | One morning tablet of ivacaftor 150 mg once daily. No evening dose. | One morning tablet of ivacaftor 150 mg twice a week, approximately 3 to 4 days apart. No evening dose. |
| Ivacaftor in a combination regimen with tezacaftor/ivacaftor | ||
| 6 years to <12 years, <30 kg | Alternate each morning: - one tablet of tezacaftor 50 mg/ivacaftor 75 mg on the first day - one tablet of ivacaftor 75 mg on the next day. Continue alternating tablets each day. No evening dose. | One morning tablet of tezacaftor 50 mg/ivacaftor 75 mg twice a week, approximately 3 to 4 days apart. No evening dose. |
| 6 years to <12 years, ≥30 kg | Alternate each morning: - one tablet of tezacaftor 100 mg/ivacaftor 150 mg once daily on the first day - one tablet of ivacaftor 150 mg on the next day Continue alternating each day. No evening dose. | One morning tablet of tezacaftor 100 mg/ivacaftor 150 mg twice a week, approximately 3 to 4 days apart. No evening dose. |
| Ivacaftor in a combination regimen with ivacaftor/tezacaftor/elexacaftor | ||
| 6 years to <12 years, <30 kg | Alternate each morning: - two tablets of ivacaftor 37.5 mg/tezacaftor 25 mg/ elexacaftor 50 mg on the first day - one tablet of ivacaftor 75 mg on the next day Continue alternating tablets each day. No evening dose. | Two morning tablets of ivacaftor 37.5 mg/tezacaftor 25 mg/ elexacaftor 50 mg twice a week, approximately 3 to 4 days apart. No evening dose. |
| 6 years to <12 years, ≥30 kg | Alternate each morning: - two tablets of ivacaftor 75 mg/tezacaftor 50 mg/ elexacaftor 100 mg on the first day - one tablet of ivacaftor 150 mg on the next day Continue alternating tablets each day. No evening dose. | Two morning tablets of ivacaftor 75 mg/tezacaftor 50 mg/ elexacaftor 100 mg twice a week, approximately 3 to 4 days apart. No evening dose. |
Each sachet of granules should be mixed with 5 mL of age-appropriate soft food or liquid and completely and immediately consumed. Food or liquid should be at room temperature or below. If not immediately consumed, the mixture has been shown to be stable for one hour and therefore should be ingested during this period. A fat-containing meal or snack should be consumed just before or just after dosing.
Ivacaftor tablets should be taken with fat-containing food.
Food or drink containing grapefruit should be avoided during treatment.
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