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Orkambi Dosage

Generic name: lumacaftor 200mg, ivacaftor 125mg
Dosage form: tablet, film coated
Drug class: CFTR combinations

Medically reviewed by Drugs.com. Last updated on Aug 10, 2023.

Recommended Dosage in Adults and Pediatric Patients Aged 1 Year and Older

The recommended dosage of ORKAMBI in adults and pediatric patients aged one year and older is based on patient's age and weight as described in Table 1.

Table 1: Recommended Oral Dosage of ORKAMBI in Patients Aged 1 Year and Older
​Age Group Weight ORKAMBI Daily Dose (every 12 hours)
Morning Dose Evening Dose
​1 through 2 years 7 kg to <9 kg 1 packet of lumacaftor 75 mg/ivacaftor 94 mg oral granules 1 packet of lumacaftor 75 mg/ivacaftor 94 mg oral granules
9 kg to <14 kg 1 packet of lumacaftor 100 mg/ivacaftor 125 mg oral granules 1 packet of lumacaftor 100 mg/ivacaftor 125 mg oral granules
≥14 kg 1 packet of lumacaftor 150 mg/ivacaftor 188 mg oral granules 1 packet of lumacaftor 150 mg/ivacaftor 188 mg oral granules
​2 through 5 years <14 kg 1 packet of lumacaftor 100 mg/ivacaftor 125 mg oral granules 1 packet of lumacaftor 100 mg/ivacaftor 125 mg oral granules
≥14 kg 1 packet of lumacaftor 150 mg/ivacaftor 188 mg oral granules 1 packet of lumacaftor 150 mg/ivacaftor 188 mg oral granules
​6 through 11 years - 2 tablets of lumacaftor 100 mg/ivacaftor 125 mg
(lumacaftor 200 mg/ivacaftor 250 mg per dose)		 2 tablets of lumacaftor 100 mg/ivacaftor 125 mg
(lumacaftor 200 mg/ivacaftor 250 mg per dose)
​12 years and older - 2 tablets of lumacaftor 200 mg/ivacaftor 125 mg
(lumacaftor 400 mg/ivacaftor 250 mg per dose)		 2 tablets of lumacaftor 200 mg/ivacaftor 125 mg
(lumacaftor 400 mg/ivacaftor 250 mg per dose)

Administration Instructions for ORKAMBI Oral Granules

​The entire content of each packet of oral granules should be mixed with one teaspoon (5 mL) of age-appropriate soft food or liquid and the mixture completely consumed. Some examples of soft foods or liquids include puréed fruits or vegetables, flavored yogurt or pudding, applesauce, water, milk, breast milk, infant formula or juice. Food should be at room temperature or below. Each packet is for single use only. Once mixed, the product has been shown to be stable for one hour, and therefore should be ingested during this period.

​Administration with Fat-Containing Food for ORKAMBI Tablets and Oral Granules

​A fat-containing meal or snack should be consumed just before or just after dosing for all formulations. Examples of appropriate fat-containing foods include eggs, avocados, nuts, butter, peanut butter, cheese pizza, breast milk, infant formula, whole-milk dairy products (such as whole milk, cheese, and yogurt), etc.

​Missed Dose

​If a patient misses a dose and remembers the missed dose within 6 hours, the patient should take the dose with fat-containing food. If more than 6 hours elapsed after the usual dosing time, the patient should skip that dose and resume the normal schedule for the following dose. A double dose should not be taken to make up for the forgotten dose [see Clinical Pharmacology (12.3) and Patient Counseling Information (17)].

Dosage Adjustment for Patients with Hepatic Impairment

For dose adjustment for patients with hepatic impairment, refer to Table 2.

Studies have not been conducted in patients with severe hepatic impairment (Child-Pugh Class C), but exposure is expected to be higher than in patients with moderate hepatic impairment. Therefore, use with caution at a maximum dose of 1 tablet in the morning and 1 tablet in the evening or less frequently, or 1 packet of oral granules once daily or less frequently in patients with severe hepatic impairment after weighing the risks and benefits of treatment [see Dosage and Administration (2.1), Use in Specific Populations (8.6), Clinical Pharmacology (12.3), and Patient Counseling Information (17)].

Table 2: Recommended Dosage for Patients with Hepatic Impairment
​Age Group Weight Morning Dose Evening Dose
*
or less frequently.
​Mild (Child-Pugh Class A) 1 through 2 years 7 kg to <9 kg 1 packet of lumacaftor 75 mg/ivacaftor 94 mg oral granules 1 packet of lumacaftor 75 mg/ivacaftor 94 mg oral granules
9 kg to <14 kg 1 packet of lumacaftor 100 mg/ivacaftor 125 mg oral granules 1 packet of lumacaftor 100 mg/ivacaftor 125 mg oral granules
≥14 kg 1 packet of lumacaftor 150 mg/ivacaftor 188 mg oral granules 1 packet of lumacaftor 150 mg/ivacaftor 188 mg oral granules
2 through 5 years <14 kg 1 packet of lumacaftor 100 mg/ivacaftor 125 mg oral granules 1 packet of lumacaftor 100 mg/ivacaftor 125 mg oral granules
≥14 kg 1 packet of lumacaftor 150 mg/ivacaftor 188 mg oral granules 1 packet of lumacaftor 150 mg/ivacaftor 188 mg oral granules
6 through 11 years - 2 tablets of lumacaftor 100 mg/ivacaftor 125 mg
(lumacaftor 200 mg/ivacaftor 250 mg per dose)		 2 tablets of lumacaftor 100 mg/ivacaftor 125 mg
(lumacaftor 200 mg/ivacaftor 250 mg per dose)
12 years and older - 2 tablets of lumacaftor 200 mg/ivacaftor 125 mg
(lumacaftor 400 mg/ivacaftor 250 mg per dose)		 2 tablets of lumacaftor 200 mg/ivacaftor 125 mg
(lumacaftor 400 mg/ivacaftor 250 mg per dose)
​Moderate (Child-Pugh Class B) 1 through 2 years 7 kg to <9 kg 1 packet of lumacaftor 75 mg/ivacaftor 94 mg oral granules 1 packet of lumacaftor 75 mg/ivacaftor 94 mg oral granules every other day
9 kg to <14 kg 1 packet of lumacaftor 100 mg/ivacaftor 125 mg oral granules 1 packet of lumacaftor 100 mg/ivacaftor 125 mg oral granules every other day
≥14 kg 1 packet of lumacaftor 150 mg/ivacaftor 188 mg oral granules 1 packet of lumacaftor 150 mg/ivacaftor 188 mg oral granules every other day
2 through 5 years <14 kg 1 packet of lumacaftor 100 mg/ivacaftor 125 mg oral granules 1 packet of lumacaftor 100 mg/ivacaftor 125 mg oral granules every other day
≥14 kg 1 packet of lumacaftor 150 mg/ivacaftor 188 mg oral granules 1 packet of lumacaftor 150 mg/ivacaftor 188 mg oral granules every other day
6 through 11 years - 2 tablets of lumacaftor 100 mg/ivacaftor 125 mg
(lumacaftor 200 mg/ivacaftor 250 mg per dose)		 1 tablet of lumacaftor 100 mg/ivacaftor 125 mg
12 years and older - 2 tablets of lumacaftor 200 mg/ivacaftor 125 mg
(lumacaftor 400 mg/ivacaftor 250 mg per dose)		 1 tablet of lumacaftor 200 mg/ivacaftor 125 mg
​Severe (Child-Pugh Class C) 1 through 2 years 7 kg to <9 kg 1 packet of lumacaftor 75 mg/ivacaftor 94 mg oral granules* N/A
9 kg to <14 kg 1 packet of lumacaftor 100 mg/ivacaftor 125 mg oral granules*
≥14 kg 1 packet of lumacaftor 150 mg/ivacaftor 188 mg oral granules*
2 through 5 years <14 kg 1 packet of lumacaftor 100 mg/ivacaftor 125 mg oral granules*
≥14 kg 1 packet of lumacaftor 150 mg/ivacaftor 188 mg oral granules*
6 through 11 years - 1 tablet of lumacaftor 100 mg/ivacaftor 125 mg* 1 tablet of lumacaftor 100 mg/ivacaftor 125 mg*
12 years and older - 1 tablet of lumacaftor 200 mg/ivacaftor 125 mg* 1 tablet of lumacaftor 200 mg/ivacaftor 125 mg*

Dosage Adjustment for Patients Taking CYP3A Inhibitors

No dose adjustment is necessary when CYP3A inhibitors are initiated in patients already taking ORKAMBI. However, when initiating ORKAMBI in patients currently taking strong CYP3A inhibitors, reduce the ORKAMBI dosage for the first week of treatment based on age as follows [see Dosage and Administration (2.1) and Drug Interactions (7.1)]:

  • 1 through 5 years of age: 1 packet of granules every other day
  • 6 years of age and older: 1 tablet daily

Following this one-week period, resume the recommended daily dosage.

If ORKAMBI is interrupted for more than one-week and then re-initiated while taking strong CYP3A inhibitors, reduce the ORKAMBI dosage for the first week of treatment re-initiation based on age as follows:

  • 1 through 5 years of age: 1 packet of granules every other day
  • 6 years of age and older: 1 tablet daily

Following this one-week period, resume the recommended daily dosage.

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Further information

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