Skip to Content

Velaglucerase Alfa

Medically reviewed by Drugs.com. Last updated on Aug 27, 2020.

Pronunciation

(vel a GLOO ser ase AL fa)

Index Terms

  • Gene-Activated Human Acid-Beta-Glucosidase
  • GlcCerase

Dosage Forms

Excipient information presented when available (limited, particularly for generics); consult specific product labeling.

Solution Reconstituted, Intravenous [preservative free]:

Vpriv: 400 units (1 ea)

Brand Names: U.S.

  • Vpriv

Pharmacologic Category

  • Enzyme

Pharmacology

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.

Distribution

Vd: Steady-state: 0.08-.0.11 L/kg

Half-Life Elimination

11-12 minutes

Use: Labeled Indications

Gaucher disease: For long-term enzyme replacement therapy for pediatric and adult patients with type 1 Gaucher disease.

Contraindications

There are no contraindications listed in the US manufacturer's labeling.

Canadian labeling: Hypersensitivity to velaglucerase alfa or any component of the formulation.

Dosing: Adult

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’s disease (type 1): IV: 60 units/kg administered every 2 weeks; adjust dose based upon disease activity (range: 15-60 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.

Dosing: Geriatric

Refer to adult dosing.

Dosing: Pediatric

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 (Elstein 2017). 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.

Reconstitution

Reconstitute the vials with 4.3 mL of SWFI to a final concentration of 100 units/mL. Gently mix vials; do not shake. The solution for infusion should be further diluted in 100 mL of NS. Slight flocculation may occur; this is acceptable for administration.

Administration

IV: Infuse over 1 hour; use an inline, low protein-binding 0.2 micron filter during infusion. Do not infuse other products in the same infusion tubing.

Storage

Store intact vials at 2°C to 8°C (36°F to 46°F). Once reconstituted, the product should be used immediately. If immediate use is not possible, the reconstituted or diluted product may be stored for up to 24 hours at 2°C to 8°C (36°F to 46°F). The infusion should be completed within 24 hours of reconstitution. Do not freeze. Protect from light. Discard any unused solution.

Drug Interactions

There are no known significant interactions.

Adverse Reactions

>10%:

Central nervous system: Headache (30% to 35%), dizziness (8% to 22%), asthenia (≤15%), fatigue (≤15%)

Gastrointestinal: Abdominal pain (15% to 19%)

Hematologic & oncologic: Prolonged partial thromboplastin time (5% to 11%; more common in children)

Hypersensitivity: Hypersensitivity reaction (23% to 52%)

Neuromuscular & skeletal: Back pain (17% to 18%), arthralgia (8% to 15%; knee)

Miscellaneous: Fever (13% to 22%; more common in children)

1% to 10%:

Cardiovascular: Flushing (>2%), hypertension (>2%), hypotension (>2%), tachycardia (>2%)

Dermatologic: Skin rash ( >2%; more common in children), urticaria (>2%)

Gastrointestinal: Nausea (6% to 10%)

Immunologic: Antibody development (neutralizing: 2%)

Neuromuscular & skeletal: Ostealgia (>2%)

<1%, postmarketing, and/or case reports: Anaphylaxis, chest discomfort, dyspnea, pruritus, vomiting

Warnings/Precautions

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.

Monitoring Parameters

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

Pregnancy Considerations

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.

Patient Education

What is this drug used for?

• It is used to treat Gaucher disease.

All drugs may cause side effects. However, many people have no side effects or only have minor side effects. Call your doctor or get medical help if any of these side effects or any other side effects bother you or do not go away:

• Abdominal pain

• Back pain

• Joint pain

WARNING/CAUTION: Even though it may be rare, some people may have very bad and sometimes deadly side effects when taking a drug. Tell your doctor or get medical help right away if you have any of the following signs or symptoms that may be related to a very bad side effect:

• Infusion reaction

• Chest pain

• Severe nausea

• Vomiting

• Loss of strength and energy

• Fatigue

• Fast heartbeat

• Shortness of breath

• Vision changes

• Dizziness

• Passing out

• Headache

• Signs of an allergic reaction, like rash; hives; itching; red, swollen, blistered, or peeling skin with or without fever; wheezing; tightness in the chest or throat; trouble breathing, swallowing, or talking; unusual hoarseness; or swelling of the mouth, face, lips, tongue, or throat.

Note: This is not a comprehensive list of all side effects. Talk to your doctor if you have questions.

Consumer Information Use and Disclaimer: This information should not be used to decide whether or not to take this medicine or any other medicine. Only the healthcare provider has the knowledge and training to decide which medicines are right for a specific patient. This information does not endorse any medicine as safe, effective, or approved for treating any patient or health condition. This is only a limited summary of general information about the medicine's uses from the patient education leaflet and is not intended to be comprehensive. This limited summary does NOT include all information available about the possible uses, directions, warnings, precautions, interactions, adverse effects, or risks that may apply to this medicine. This information is not intended to provide medical advice, diagnosis or treatment and does not replace information you receive from the healthcare provider. For a more detailed summary of information about the risks and benefits of using this medicine, please speak with your healthcare provider and review the entire patient education leaflet.

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.