Taliglucerase alfa
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From MedlinePlus, U.S. National Library of Medicine
Boxed warning
WARNING: HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS Patients treated with enzyme replacement therapies have experienced life‑threatening hypersensitivity reactions, including anaphylaxis. Anaphylaxis has occurred during the early course of enzyme replacement therapy and after extended duration of therapy. Initiate ELELYSO in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment. 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 [see Warnings and Precautions (5.1) ] . WARNING: HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS See full prescribing information for complete boxed warning. • Anaphylaxis has occurred during the early course of enzyme replacement therapy and after extended duration of therapy. ( 5.1 ) • Initiate ELELYSO in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment.
( 5.1 ) • If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue ELELYSO and immediately initiate appropriate medical treatment, including use of epinephrine. ( 5.1 )
Hydrolytic Lysosomal Glucocerebroside-specific Enzyme
Dosing in kidney impairment
This label has no separate kidney-impairment dosing section. Check warnings and clinical pharmacology in the full label.
Preparation and administration
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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 ] . Initiate ELELYSO in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment [see Warnings and Precautions ] .
To reduce the risk of hypersensitivity reactions, consider pretreatment with antihistamines and/or corticosteroids [see Warnings and Precautions ] .
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 ] .
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.
Upon reconstitution, mix vials gently. DO NOT SHAKE. 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. For pediatric patients 4 years of age and older, use a final volume of 100 to 120 mL. 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.
Storage and Handling of the Reconstituted and 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.
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.
Contraindications
None. None ( 4 )
Safety data
Reported adverse events (FAERS)
Liver injury (LiverTox)
LiverTox has no chapter for taliglucerase alfa. Check the label's hepatic warnings.
Pregnancy and lactation
Pregnancy
8.1 Pregnancy Risk Summary Available data, including data from a product registry and pharmacovigilance reports, have not identified a drug-associated risk of major birth defects, miscarriage, or other adverse maternal or fetal outcomes in pregnant women exposed to ELELYSO. Additionally, there are risks associated with untreated symptomatic Type I Gaucher disease in pregnant women ( see Clinical Considerations ). In animal reproduction studies when pregnant rats and rabbits were administered taliglucerase alfa at intravenous doses up to 5 times the recommended human dose (RHD), there was no evidence of embryo-fetal toxicity ( see Data ).
The background risk of major birth defects and miscarriage for the indicated population is unknown. In the U.S. general population, the estimated background risk of major birth defects and miscarriage in clinically recognized pregnancies is 2 to 4% and 15 to 20%, respectively. Clinical Considerations Disease-Associated Maternal and/or Embryo/Fetal Risk Women with Type 1 Gaucher disease have an increased risk of spontaneous abortion if disease symptoms are not treated and controlled pre-conception and during a pregnancy. Pregnancy may exacerbate existing Type 1 Gaucher disease symptoms or result in new disease manifestations.
Type 1 Gaucher disease manifestations may lead to adverse pregnancy outcomes, including hepatosplenomegaly which can interfere with the normal growth of a fetus and thrombocytopenia which can lead to increased bleeding and possible postpartum hemorrhage requiring transfusion. Data Animal Data 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.
Breastfeeding (LactMed)
Taliglucerase alfa is a biosynthetic synthetic enzyme closely related to beta-glucocerebrosidase, which is a normal component of human milk. Because it is a large protein molecule with a molecular weight of about 61,000 Da, the amount in milk is likely to be very low and absorption is unlikely because it is probably destroyed in the infant's gastrointestinal tract. Preliminary evidence indicates that breastfeeding while taking Taliglucerase does not harm the breastfed infant. International panels of clinicians that treat Gaucher's disease recommend breastfeeding with enzyme replacement therapy such as imiglucerase. Consider limiting the duration of breastfeeding to about 6 months to avoid excessive bone loss in the nursing mother.-
- Alglucerase
- Imiglucerase
Supply and cost
Drug shortage status
Recalls (last 24 months)
Therapeutic equivalence (Orange Book)
Price ladder
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