Version May 2024
Note: Pretreatment with antihistamines and/or corticosteroids can be considered for prevention of subsequent infusion reactions in patients with hypersensitivity reactions requiring symptomatic treatment; during clinical studies, patients were not routinely premedicated prior to infusion.
Gaucher disease (type 1): IV: 60 units/kg administered every 2 weeks; adjust dose based upon disease activity (range: 15 to 60 units/kg evaluated in clinical trials).
Note: When switching from imiglucerase to velaglucerase alfa in stable patients, initiate treatment 2 weeks after the last imiglucerase dose and at the same dose.
No dosage adjustment provided in manufacturer's labeling.
No dosage adjustment provided in manufacturer's labeling.
Refer to adult dosing.
(For additional information see "Velaglucerase alfa (glucocerebrosidase): Pediatric drug information")
Note: Pretreatment with antihistamines and/or corticosteroids can be considered for prevention of subsequent infusion reactions in patients with hypersensitivity reactions 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 every other week; adjust based upon disease activity; dosing range: 15 to 60 units/kg every other week (Ref). Note: When switching from imiglucerase to velaglucerase alfa, initiate velaglucerase alfa treatment 2 weeks after the last imiglucerase dose and at the same dose as stable imiglucerase therapy.
There are no dosage adjustments provided in manufacturer's labeling.
There are no dosage adjustments provided in manufacturer's labeling.
The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified.
>10%:
Gastrointestinal: Abdominal pain (15% to 19%)
Hematologic & oncologic: Prolonged partial thromboplastin time (5% to 11%; more common in children and adolescents)
Hypersensitivity: Hypersensitivity reaction (23% to 52%; including anaphylaxis: <1%)
Nervous system: Dizziness (8% to 22%), fatigue (≤15%), headache (30% to 35%)
Neuromuscular & skeletal: Arthralgia (8% to 15%; knee), asthenia (≤15%), back pain (17% to 18%)
Miscellaneous: Fever (13% to 22%; more common in children and adolescents)
1% to 10%:
Cardiovascular: Flushing (>2%), hypertension (>2%), hypotension (>2%), tachycardia (>2%)
Dermatologic: Skin rash (>2%; more common in children and adolescents), urticaria (>2%)
Gastrointestinal: Nausea (6% to 10%)
Immunologic: Antibody development (neutralizing: 2%)
Neuromuscular & skeletal: Ostealgia (>2%)
Postmarketing: Gastrointestinal: Vomiting
There are no contraindications listed in the US manufacturer's labeling.
Canadian labeling: Hypersensitivity to velaglucerase alfa or any component of the formulation.
Concerns related to adverse effects:
• Antibody formation: The development of IgG antibodies has been reported; the clinical significance is unknown. Patients with an immune response to other enzyme replacement therapies who are switching to velaglucerase alfa should be monitored for antibody development.
• Hypersensitivity reactions: Use with caution in patients who have exhibited hypersensitivity reactions to velaglucerase alfa or other enzyme replacement therapies. Anaphylaxis has occurred; appropriate medical support should be readily available in the event of a serious reaction. The most common hypersensitivity reactions reported in clinical trials include asthenia, dizziness, fatigue, fever, headache, hyper-/hypotension, nausea, and pyrexia. Most reactions were mild and occurred during the first 6 months of treatment. Management strategies of more severe reactions include symptomatic treatment, pretreatment with antihistamines, antipyretics, and/or corticosteroids, and slowing of the infusion rate. Treatment should be discontinued if anaphylaxis or other acute reactions occur.
Pediatric patients (4 to 17 years) may experience a higher frequency of some adverse effects than adults (>10% difference in incidence), including fever, rash, and prolonged aPTT.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution Reconstituted, Intravenous [preservative free]:
Vpriv: 400 units (1 ea)
No
Solution (reconstituted) (Vpriv Intravenous)
400 unit (per each): $1,753.03
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Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution Reconstituted, Intravenous:
Vpriv: 400 units (1 ea)
IV: Infuse over 1 hour; use an inline, low protein-binding 0.2- or 0.22-micron filter during infusion. Do not infuse other products in the same infusion tubing.
Parenteral: For IV administration. Infuse over 60 minutes through an in-line low protein-binding 0.2 or 0.22 micron filter. Do not infuse other products in the same infusion tubing.
Gaucher disease: For long-term enzyme replacement therapy for pediatric and adult patients with type 1 Gaucher disease.
None known.
There are no known significant interactions.
Based on available information, an increased risk of adverse pregnancy outcomes has not been observed following maternal use of velaglucerase alfa (Elstein 2014; Lau 2018). 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, may occur.
It is not known if velaglucerase alfa is present in breast milk.
According to the manufacturer, the decision to breastfeed during therapy should consider the risk of infant exposure, the benefits of breastfeeding to the infant, and benefits of treatment to the mother.
Disease monitoring: CBC, liver enzymes, IgG antibodies; MRI, CT, or US of liver and spleen; bone density studies; monitor antibodies in those patients who developed antibodies to other enzyme replacement therapies
Velaglucerase alfa, which contains the same amino acid sequence as endogenous glucocerebrosidase, catalyzes the hydrolysis of glucocerebroside to glucose and ceramide in the lysosome. In patients with type 1 Gaucher’s disease, glucocerebrosidase deficiency results in accumulation of glucocerebroside in macrophages, thereby causing the associated signs and symptoms. Velaglucerase alfa is used to diminish hepatosplenomegaly and improve anemia, thrombocytopenia, and bone disease.
Note: Values reported below based on combined pediatric patient (4-17 years) and adult data.
Distribution: Vd: Steady-state: 0.08-.0.11 L/kg
Half-life elimination: 11-12 minutes
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