Posology : מינונים

2 DOSAGE AND ADMINISTRATION

2.1 Recommendations Prior to ELELYSO Treatment
Administration of ELELYSO should be supervised by a healthcare provider knowledgeable in the management of hypersensitivity reactions including anaphylaxis [see Warnings and Precautions (5.1)].

Initiate ELELYSO in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment [see Warnings and Precautions (5.1)].

To reduce the risk of hypersensitivity reactions, consider pretreatment with antihistamines and/or corticosteroids [see Warnings and Precautions (5.1)].


2.2 Recommended Dosage
Treatment-naïve patients: The recommended dosage for treatment-naïve adult and pediatric patients above 2 years of age and older is 60 units per kg of body weight administered every other week as a 60 to 120 minute intravenous infusion.

Patients switching from imiglucerase: If it is acceptable to switch from a stable imiglucerase dosage to ELELYSO, initiate ELELYSO intravenous treatment (60- to 120-minute infusion) with the same units/kg imiglucerase dosage and subsequently administer ELELYSO every other week. Dosage adjustments can be made based on achievement and maintenance of each patient’s therapeutic goals 
2.3 Preparation Instructions
ELELYSO should be reconstituted, diluted, and administered under the supervision of a healthcare professional.

Prepare ELELYSO according to the following steps using aseptic technique:  a. Determine the number of vials to be reconstituted based on the patient’s weight in kg and the recommended dose [see Dosage and Administration (2.2)]. Round the number of vials up to the next whole number.
 b. 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.
 c. Reconstitute each vial of ELELYSO with 5.1 mL of Sterile Water for Injection to yield a reconstituted product with a concentration of 40 units/mL and an extractable volume of 5 mL.

(1) Upon reconstitution, mix vials gently. DO NOT SHAKE.
(2) Prior to further dilution, visually inspect the reconstituted solution in the vials for particulate matter and discoloration. The solution should be clear and colorless. Discard if particulate matter is present or the solution is discolored.
 d. 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. Discard any unused reconstituted solution.
1. For pediatric patients, use a final volume of 100 to 120 mL.
2. For adult patients, may use a final volume of 130 to 150 mL . 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.
 e. Mix the diluted solution gently. DO NOT SHAKE. Since this is a protein solution, slight flocculation (described as translucent fibers) occurs occasionally after dilution.
 f.   Discard any unused diluted solution.


2.4 Administration Instructions

After reconstitution and dilution, administer via intravenous infusion over a minimum of 60 minutes and with an in-line low protein-binding 0.2 micron filter.
•   For pediatric patients who weigh (based on actual body weight): o Less than 30 kg use an infusion rate of 1 mL/minute.
o Greater than or equal to 30 kg, use an initial infusion rate of 1 mL/minute. After tolerability to ELELYSO is established, may increase the infusion rate to a maximum of 2 mL/minute.
For adult patients, use an initial infusion rate of 1.2 mL/minute. After tolerability to ELELYSO is established, may increase the infusion rate to a maximum of 2.2 mL/minute.
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 at 2 to 8 °C (36 to 46 °F) under protection from light. The diluted product may be stored for up to 24 hours at 2 to 8 °C (36 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 the unused reconstituted or diluted solution after 24 hours from the start of preparation