Dosage & Administration
Recommendations Prior to ELELYSO Treatment (
To reduce the risk of hypersensitivity reactions, consider pretreatment with antihistamines and/or corticosteroids
Recommended Dosage in Patients 4 Years and Older (
The recommended dosage of ELELYSO is 60 units/kg (based on actual body weight) administered every other week as a 60- to 120-minute intravenous infusion.
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.
Preparation and Administration (
ELELYSO should be reconstituted, diluted, and administered under the supervision of a healthcare professional.
Prepare ELELYSO according to the following steps using aseptic technique:
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.
Elelyso Prescribing Information
• 2 of 72 (3%) ELELYSO-treated patients experienced signs and symptoms consistent with anaphylaxis including urticaria, hypotension, flushing, wheezing, chest tightness, nausea, vomiting, and dizziness. These reactions occurred during ELELYSO infusion.• 21 of 72 (29%) ELELYSO-treated patients experienced hypersensitivity reactions, including the 2 ELELYSO‑treated patients who experienced signs and symptoms consistent with anaphylaxis. Signs and symptoms of these hypersensitivity reactions included pruritus, angioedema, flushing, erythema, rash, nausea, vomiting, cough, chest tightness, and throat irritation. These reactions occurred during ELELYSO infusion and up to 3 hours after the start of infusion[see Adverse Reactions (6.1)].
ELELYSO-treated patients who developed anti-taliglucerase alfa antibodies (referred to as anti-drug antibodies (ADA)) generally had a greater frequency of hypersensitivity reactions compared to those who did not develop ADA
Management of hypersensitivity reactions should be based on the severity of the reaction and includes slowing or temporary interruption of the infusion and/or administration of antihistamines, antipyretics, and/or corticosteroids for mild reactions. To reduce the risk of hypersensitivity reactions, consider pretreatment with antihistamines and/or corticosteroids. If
Consider the risks and benefits of re-administering ELELYSO in patients who have experienced a severe hypersensitivity reaction associated with ELELYSO. Caution should be exercised upon rechallenge
Boxed Warning | 7/2024 |
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)] . | 7/2024 |
Warnings and Precautions ( Life‑threatening h ypersensitivity reactions, including anaphylaxis,have occurred inpatients treated with enzyme replacement therapies, including ELELYSO . In clinical trials, (patients were not routinely pretreated with antihistamines and/or corticosteroids prior to ELELYSO infusions during the clinical trials):
Anaphylaxis has occurred during the early course of enzyme replacement therapy and after extended duration of therapy. Administration of ELELYSO should be supervised by a healthcare provider knowledgeable in the management of hypersensitivity reactions including anaphylaxis. Initiate ELELYSO in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment. Observe patients closely during and after the infusion. ELELYSO-treated patients who developed anti-taliglucerase alfa antibodies (referred to as anti-drug antibodies (ADA)) generally had a greater frequency of hypersensitivity reactions compared to those who did not develop ADA [see Adverse Reactions (6.1)] . Closely monitor for hypersensitivity reactions in patients who develop ADA.Management of hypersensitivity reactions should be based on the severity of the reaction and includes slowing or temporary interruption of the infusion and/or administration of antihistamines, antipyretics, and/or corticosteroids for mild reactions. To reduce the risk of hypersensitivity reactions, consider pretreatment with antihistamines and/or corticosteroids. If a severe hypersensitivity reaction(e.g., anaphylaxis) occurs, discontinue ELELYSO and immediately initiate appropriate medical treatment, including use of epinephrine. Inform patients of the symptoms of life‑threatening hypersensitivity reactions, including anaphylaxis and to seek immediate medical care should symptoms occur. Consider the risks and benefits of re-administering ELELYSO in patients who have experienced a severe hypersensitivity reaction associated with ELELYSO. Caution should be exercised upon rechallenge [see Adverse Reactions (6.2) ]. | 1/2025 |
ELELYSO is indicated for the treatment of patients 4 years of age and older with a confirmed diagnosis of Type 1 Gaucher disease.
Recommendations Prior to ELELYSO Treatment (
To reduce the risk of hypersensitivity reactions, consider pretreatment with antihistamines and/or corticosteroids
• Administration of ELELYSO should be supervised by a healthcare provider knowledgeable in the management of hypersensitivity reactions including anaphylaxis.
Recommended Dosage in Patients 4 Years and Older (
The recommended dosage of ELELYSO is 60 units/kg (based on actual body weight) administered every other week as a 60- to 120-minute intravenous infusion.
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.
• Treatment-naïve: 60 units/kg administered every other week as a 60- to 120-minute intravenous infusion.• Patients switching from imiglucerase: 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 based on achievement and maintenance of each patient’s therapeutic goals.
Preparation and Administration (
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, USP 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 4 years of age and older, 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.
• If the reconstituted ELELYSO vial is not used immediately, refrigerate at 2 °C to 8 °C (36 °F to 46 °F) for up to 24 hours (under protection from light) or store at controlled room temperature at 20 °C to 25 °C (68 °F to 77 °F) for up to 4 hours (without protection from light).• If the diluted solution is not administered immediately, refrigerate at 2 °C to 8 °C (36 °F to 46 °F) for up to 24 hours (under protection from light).• The total storage time for the reconstituted and diluted solution should not exceed 24 hours. Discard the unused reconstituted or diluted solution after 24 hours from the start of preparation.• Do not freeze.
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.
• Reconstitute, dilute and administer under the supervision of a healthcare professional.• See Full Prescribing Information for complete instructions.
For injection: 200 units white to off-white lyophilized powder in a single-dose vial for reconstitution.
The limited available data on ELELYSO use in pregnant women are not sufficient to inform a drug-associated risk. However, there are clinical considerations (
Reproduction studies have been performed with taliglucerase alfa administered during the period of organogenesis in rats and rabbits. In rats, intravenous doses up to 55 mg/kg/day (about 5 times the RHD of 60 units/kg based on the body surface area) did not cause any adverse effects on embryo-fetal development. In rabbits, intravenous doses up to 27.8 mg/kg/day (about 5 times the RHD of 60 units/kg based on the body surface area) did not show any embryo-fetal toxicity.