(tal i GLOO ser ase AL fa)
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution Reconstituted, Intravenous [preservative free]:
Elelyso: 200 units (1 ea) [contains polysorbate 80]
Brand Names: U.S.
Taliglucerase alfa is an analogue of glucocerebrosidase; it is produced by recombinant DNA technology using plant (carrot) cell culture. Glucocerebrosidase is an enzyme deficient in Gaucher's disease. It is needed to catalyze the hydrolysis of glucocerebroside to glucose and ceramide, thereby reducing liver and spleen size and improving anemia and thrombocytopenia.
Distribution: Vss: Pediatric patients: 8.8 to 14.9 L; Adults: 10.7 to 11.7 L
Pediatric patients: 33 to 37 minutes; Adults: 19 to 29 minutes (dose-dependent; increased with higher doses)
Use: Labeled Indications
Gaucher disease: Treatment of adult and pediatric patients with a confirmed diagnosis of type 1 Gaucher disease.
There are no contraindications listed in the US labeling.
Canadian labeling: Severe hypersensitivity to taliglucerase alfa or any component of the formulation.
Note: Pretreatment with antihistamines and/or corticosteroids can be considered for prevention of subsequent infusion reactions in patients with an infusion reaction requiring symptomatic treatment; during clinical studies, patients were not routinely premedicated prior to infusion.
Gaucher disease (type 1): IV: 60 units/kg every 2 weeks; dosing is individualized based on disease severity.
Conversion from imiglucerase: Initiate taliglucerase alfa using the patient’s same previous imiglucerase dose and administer every 2 weeks. Note: Conversion to taliglucerase alfa is based on a single study of patients stabilized on a biweekly imiglucerase dose for ≥6 months
Refer to adult dosing.
Note: Pretreatment with antihistamines, antipyretics, and/or corticosteroids can be considered for prevention of subsequent infusion reactions in patients with an infusion reaction requiring symptomatic treatment; during clinical studies, patients were not routinely premedicated prior to infusion.
Gaucher disease (type 1): Children ≥4 years and Adolescents: Refer to adult dosing.
Dosing: Renal Impairment
There are no dosage adjustments provided in the manufacturer’s labeling.
Dosing: Hepatic Impairment
There are no dosage adjustments provided in the manufacturer’s labeling.
Calculate the necessary dose; round up to the next whole vial when determining the number of vials needed. Reconstitute each vial with 5.1 mL of SWFI; mix gently; do not shake. Solution should be clear and colorless. Withdraw 5 mL of reconstituted solution from each vial (reconstituted vials contain 5.3 mL) and further dilute in NS to a final volume of 100 to 200 mL. For pediatric patients, use a final volume of 100 to 120 mL; for adults, a final volume of 130 to 150 mL may be used. Final volume should not exceed 200 mL for adults using ≥130 to 150 mL or more of reconstituted product. Slight flocculation may occur following dilution; this is acceptable for administration. Discard any unused product.
IV: Administer IV over a minimum infusion time of 60 minutes (usual infusion time: 60 to 120 minutes). Administer using a low protein-binding infusion set with a 0.2 micron in-line filter.
Pediatric patients: Initiate infusion at a rate of 1 mL/minute; rate may be increased, but do not exceed 2 mL/minute based on patient tolerance.
Adult patients: Initiate infusion at a rate of 1.2 mL/minute; rate may be increased, but not exceed 2.2 mL/minute based on patient tolerance.
Store intact vials at 2°C to 8°C (36°F to 46°F); protect from light. Do not freeze. If not used immediately, the reconstituted solution may be stored at 2°C to 8°C (36°F to 46°F) for ≤24 hours (protect from light) or at 20°C to 25°C (68°F to 77°F) for ≤4 hours (without protection from light). The solution diluted in NS for infusion may be stored at 2°C to 8°C (36°F to 46°F) for ≤24 hours; protect from light. In total, the reconstituted and diluted products can be stored for ≤24 hours. Do not freeze.
There are no known significant interactions.
Central nervous system: Headache (13% to 19%)
Gastrointestinal: Vomiting (children and adolescents: 44%; adults: 6%)
Hypersensitivity: Hypersensitivity reaction (22% to 29%; antibody-positive patients: 35%; patients switching from imiglucerase: 6%)
Immunologic: Antibody formation (adults: 13% to 53%; neutralizing but effect on therapeutic response not evaluated: 10%; positive at baseline: 6%; children and adolescents: 22%)
Neuromuscular & skeletal: Arthralgia (13%), limb pain (10%)
Respiratory: Pharyngitis (4% to 19%)
1% to <10%:
Cardiovascular: Flushing (6%)
Central nervous system: Dizziness (9%), fatigue (9%)
Dermatologic: Pruritus (6%), urticaria (6%), skin rash (≥5%)
Gastrointestinal: Nausea (9%), abdominal pain (6%)
Hypersensitivity: Fixed drug eruption (4%; type III immune-mediated; mild and intermittent), anaphylaxis (3%)
Neuromuscular & skeletal: Back pain (≥5%)
<1%, postmarketing, and/or case reports: Diarrhea
Concerns related to adverse effects:
• Antibody formation: The development of IgG anti-drug antibodies (ADA) has been reported; the clinical significance is unknown. Patients who develop immune or infusion reactions to taliglucerase alfa or who have had an immune response to other enzyme replacement therapies and who are switching to taliglucerase alfa should be monitored for antibody development; it is unknown if presence of antibodies is related to a higher risk of infusion reactions.
• CNS effects: Dizziness and fatigue have been observed with therapy; caution patients about performing dangerous tasks (eg, driving, operating machinery).
• Hypersensitivity/anaphylactoid reactions: Serious hypersensitivity reactions, including anaphylaxis, may occur; these reactions have occurred up to 3 hours after start of infusion. Appropriate medical support should be readily available. Base management of reaction on severity; may include slowing or temporary interruption of infusion and/or premedication (eg, antihistamine, antipyretics, corticosteroids) for mild reactions. Pretreatment may prevent subsequent reactions. Observe patient during and after infusion. If severe reactions occur, immediately discontinue infusion and initiate appropriate treatment; rechallenge with caution.
Hemoglobin, platelet count, angiotensin converting enzyme, tartrate-resistant acid phosphatase, chitotriosidase, IgG anti-drug antibody formation (in patients who experience, or previously experienced, immune or infusion reactions to enzyme replacement therapy); liver volume, spleen volume; bone density; ECG, echocardiogram, chest x-ray
Adverse effects were not observed in animal reproduction studies. Pregnancy may exacerbate existing type I Gaucher disease or result in new symptoms. Women with type I Gaucher disease have an increased risk of spontaneous abortion if disease is not well controlled. Adverse pregnancy outcomes, including hepatosplenomegaly and thrombocytopenia (which may result in increased bleeding and postpartum hemorrhage requiring transfusion), may occur.
• Discuss specific use of drug and side effects with patient as it relates to treatment. (HCAHPS: During this hospital stay, were you given any medicine that you had not taken before? Before giving you any new medicine, how often did hospital staff tell you what the medicine was for? How often did hospital staff describe possible side effects in a way you could understand?)
• Patient may experience headache, diarrhea, joint pain, loss of strength and energy, abdominal pain, painful extremities, or back pain. Have patient report immediately to prescriber severe dizziness, passing out, flushing, cough, nausea, vomiting, or pharyngitis (HCAHPS).
• Educate patient about signs of a significant reaction (eg, wheezing; chest tightness; fever; itching; bad cough; blue skin color; seizures; or swelling of face, lips, tongue, or throat). Note: This is not a comprehensive list of all side effects. Patient should consult prescriber for additional questions.
Intended Use and Disclaimer: Should not be printed and given to patients. This information is intended to serve as a concise initial reference for health care professionals to use when discussing medications with a patient. You must ultimately rely on your own discretion, experience, and judgment in diagnosing, treating, and advising patients.
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- Drug class: lysosomal enzymes
Other brands: Elelyso