# Kanuma Dosage

Generic name: Sebelipase alfa 2mg in 1mL
Dosage form: injection, solution, concentrate

Medically reviewed by Drugs.com. Last updated on Aug 12, 2020.

### Dosage

Patients with Rapidly Progressive LAL Deficiency Presenting within the First 6 Months of Life:

The recommended starting dosage is 1 mg/kg administered once weekly as an intravenous infusion. For patients who do not achieve an optimal clinical response, increase to 3 mg/kg once weekly.

Pediatric and Adult Patients with LAL Deficiency:

The recommended dosage is 1 mg/kg administered once every other week as an intravenous infusion.

### Preparation Instructions

KANUMA is for intravenous infusion only. Prepare KANUMA using the following steps.

1. Determine the number of vials needed based on the patient's weight and the recommended dose of 1 mg/kg or 3 mg/kg, using the following calculations (a-b):
1. Total dose (mg) = Patient's weight (kg) × Recommended dose (mg/kg)
2. Total number of vials = Total dose (mg) divided by 20 mg/vial
2. Round to the next whole vial and remove the required number of vials from the refrigerator to allow them to reach room temperature.
1. Volume (mL) of calculated total dose = Total dose (mg) divided by the 2 mg/mL concentration
2. Volume (mL) of 0.9% Sodium Chloride for dilution = Total infusion volume (mL) for patient's weight (see Table 1) - Volume (mL) of calculated total dose
Table 1: Total Infusion Volumes*
Weight Range (kg) 1 mg/kg dose 3 mg/kg dose
Total Infusion Volume (mL) Total Infusion Volume (mL)
*
The infusion volume should be based on the prescribed dose and should be prepared to a final KANUMA concentration of 0.1 mg/mL to 1.5 mg/mL.
For patients with LAL deficiency presenting within the first 6 months of life who do not achieve an optimal clinical response with a dose of 1 mg/kg.
1 to 10.9 10 25
11 to 24.9 25 50
25 to 49.9 50 100
50 to 99.9 100 250
100 to 120.9 250 500
3. Mix gently by inversion. Do not shake the vials or the prepared infusion.
4. The solution should be inspected visually for particulate matter and discoloration prior to administration. The solution should be a clear to slightly opalescent, colorless to slightly colored solution. Thin, translucent particles or fibers may be present in the vials or diluted solution. Do not use if the solution is cloudy or if other particulate matter is observed.
5. Vials are for single-use only. Discard any unused product. Do not freeze.

Administer the diluted solution as an intravenous infusion using a low-protein binding infusion set with an in-line, low-protein binding 0.2 micron filter.

Infuse over at least 2 hours. Consider further prolonging the infusion time for patients receiving the 3 mg/kg dose or those who have experienced hypersensitivity reactions . A 1-hour infusion may be considered for those patients receiving the 1 mg/kg dose who tolerate the infusion.

### Storage of Diluted Solution

KANUMA contains no preservatives; therefore, product should be used immediately after dilution. If immediate use is not possible, the diluted product may be stored up to 24 hours in the refrigerator at 2°C to 8°C (36°F to 46°F). Do not freeze or shake. Protect from light.