ELELYSO Powder for solution for injection Ref.[10209] Active ingredients: Taliglucerase alfa

2.1 Recommended Dosage in Patients 4 Years and Older

Treatment-naïve patients

The recommended dosage of ELELYSO for long-term treatment is 60 units/kg of body weight administered every other week as a 60 to 120 minute intravenous infusion.

Patients switching from imiglucerase

Patients currently being treated with imiglucerase for Type 1 Gaucher disease can be switched to ELELYSO. Patients previously treated on a stable dosage of imiglucerase are recommended to begin treatment with ELELYSO at that same units/kg dosage when they switch from imiglucerase to ELELYSO. Administer ELELYSO for long-term treatment every other week as a 60 to 120 minute intravenous infusion. Dosage adjustments can be made based on achievement and maintenance of each patient’s therapeutic goals [see Clinical Studies (14.2)].

2.2 Preparation Instructions

ELELYSO should be reconstituted, diluted, and administered under the supervision of a healthcare professional.

Each vial of ELELYSO provides 200 units of taliglucerase alfa and is intended for one-time use and for only one patient. The reconstitution and dilution steps must be completed using aseptic technique.

ELELYSO should be reconstituted with Sterile Water for Injection, USP and diluted with 0.9% Sodium Chloride Injection, USP, to a final volume of 100 mL to 200 mL, and administered by intravenous infusion.

Prepare ELELYSO according to the following steps. Use aseptic technique.

• Determine the number of vials to be reconstituted based on the patient’s weight and the recommended dose of 60 units/kg, using the following calculations (1–3):
  • Total dose in units = Patient’s weight (kg) × dose (units/kg)
  • Total number of vials = Total dose in units divided by 200 units/vial
  • Round up to the next whole vial.
• Remove the required number of vials from the refrigerator. Do not leave these vials at room temperature longer than 24 hours prior to reconstitution. Do not heat or microwave these vials.
• Reconstitute each vial of ELELYSO with 5.1 mL of Sterile Water for Injection, USP to yield a reconstituted product with a concentration of 40 units/mL and an extractable volume of 5 mL. Upon reconstitution, mix vials gently. DO NOT SHAKE. Prior to further dilution, visually inspect the solution in the vials; the solution should be clear and colorless. Do not use if the solution is discolored or if foreign particulate matter is present.
• Withdraw the calculated dose of drug from the appropriate number of vials and dilute with 0.9% Sodium Chloride Injection, USP, to a final volume of 100 to 200 mL.
  • For pediatric patients, a final volume of 100 to 120 mL should be used.
  • For adult patients, a final volume of 130 to 150 mL may be used. However, if the volume of reconstituted product alone is equal to or greater than 130 to 150 mL, then the final volume should not exceed 200 mL.
• Mix gently. DO NOT SHAKE. Since this is a protein solution, slight flocculation (described as translucent fibers) occurs occasionally after dilution.

2.3 Administration Instructions

After reconstitution and dilution, the preparation should be administered via intravenous infusion and filtered through an in-line low protein-binding 0.2 μm filter.

• For pediatric patients: An initial infusion rate of 1 mL/minute should be used. After tolerability to ELELYSO is established, the infusion rate may be increased, but should not exceed the maximum recommended infusion rate of 2 mL/minute. The total volume of the infusion should be delivered over a minimum of 60 minutes.
• For adult patients: An initial infusion rate of 1.2 mL/minute should be used. After tolerability to ELELYSO is established, the infusion rate may be increased, but should not exceed the maximum recommended infusion rate of 2.2 mL/minute. The total volume of the infusion should be delivered over a minimum of 60 minutes.

As ELELYSO contains no preservative, the product should be used immediately once reconstituted. If immediate use is not possible, the reconstituted product may be stored for up to 24 hours refrigerated at 2°C to 8°C (36°F to 46°F) under protection from light or up to 4 hours at room temperature (20°C to 25°C (68°F to 77°F) without protection from light. The diluted product may be stored for up to 24 hours at 2°C to 8°C (36°F to 46°F) under protection from light. Storage of the reconstituted product and the diluted product should not exceed a total of 24 hours. Do not freeze. Discard any unused product.

Store ELELYSO refrigerated at 2°C to 8°C (36°F to 46°F) in the original carton to protect from light. Do not freeze.