Version May 2024
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: Dosing based on actual body weight: 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
There are no dosage adjustments provided in the manufacturer's labeling.
There are no dosage adjustments provided in the manufacturer's labeling.
Refer to adult dosing.
(For additional information see "Taliglucerase alfa (glucocerebrosidase): Pediatric drug information")
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: IV: 60 units/kg/dose every 2 weeks; use actual body weight to calculate dose.
Conversion from imiglucerase: Initiate taliglucerase alfa using the patient's same previous imiglucerase dose and administer every 2 weeks; doses may be adjusted if needed to achieve and maintain therapeutic goals. Note: Conversion to taliglucerase alfa is based on a single study of patients stabilized on a biweekly imiglucerase dose for ≥6 months.
There are no dosage adjustments provided in the manufacturer’s labeling.
There are no dosage adjustments provided in the manufacturer’s labeling.
The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified.
>10%:
Central nervous system: Headache (13% to 19%)
Hypersensitivity: Hypersensitivity reaction (≤29%; increased risk in antibody-positive patients; patients switching from imiglucerase: 6%)
Immunologic: Antibody formation (≤53%; neutralizing 63%)
Neuromuscular & skeletal: Arthralgia (13%), limb pain (10%)
1% to <10%:
Cardiovascular: Flushing (6%)
Central nervous system: Dizziness (9%), fatigue (9%)
Dermatologic: Pruritus (6%), urticaria (6%)
Gastrointestinal: Nausea (9%), vomiting (≥6%), abdominal pain (6%)
Hypersensitivity: Anaphylaxis (3%)
Frequency not defined:
Hypersensitivity: Angioedema
Postmarketing: Back pain, diarrhea, fixed drug eruption (type III immune-mediated)
There are no contraindications listed in the US labeling.
Canadian labeling: Severe hypersensitivity to taliglucerase alfa or any component of the formulation.
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 ADA; the presence of antibodies may be related to a higher risk of hypersensitivity 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. If severe reactions occur, immediately discontinue infusion and initiate appropriate treatment; rechallenge with caution.
In a clinical trial evaluating the safety of taliglucerase alfa, treatment-naïve pediatric patients experienced a higher incidence of vomiting compared to treatment-naïve adults (44% vs 6%). Vomiting may be a symptom of hypersensitivity; monitor patients carefully.
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]
No
Solution (reconstituted) (Elelyso Intravenous)
200 unit (per each): $1,047.20
Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution Reconstituted, Intravenous:
Elelyso: 200 units (1 ea) [contains polysorbate 80]
Product access is restricted to the Gaucher Personal Support (GPS) program. Healthcare providers and patients may obtain additional information by contacting the GPS program at 855-353-5976.
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.
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.
IV infusion: Administer IV using a low protein-binding infusion set with a 0.2 micron in-line filter; infusion rate based on patient actual body weight.
Pediatric patients:
Weight <30 kg: Infuse at a rate of 1 mL/minute.
Weight ≥30 kg: Initiate infusion at a rate of 1 mL/minute; the infusion rate may be increased if tolerated to a maximum of 2 mL/minute (minimum infusion time: 60 minutes).
Adult patients: Initiate infusion at a rate of 1.2 mL/minute; the infusion rate may be increased if tolerated to a maximum of 2.2 mL/minute (minimum infusion time: 60 minutes).
Gaucher disease: Treatment of adult and pediatric patients ≥4 years of age with a confirmed diagnosis of type 1 Gaucher disease.
None known.
There are no known significant interactions.
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.
It is not known if taliglucerase alfa is excreted in breast milk. Enzyme ingested by a nursing infant would likely degrade in their digestive system. The benefits of nursing to the infant should be weighed against the potential for additional bone loss in the mother (Zimran 2009). According to the manufacturer, the decision to continue or discontinue breast-feeding during therapy should take into account the risk of exposure to the infant and the benefits of treatment to the mother.
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; signs and symptoms of hypersensitivity and infusion reactions for 3 hours after the start of the infusion.
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
Half-life elimination: Pediatric patients: 33 to 37 minutes; Adults: 19 to 29 minutes (dose-dependent; increased with higher doses)
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