Velaglucerase alfa

Pronunciation: VEL-a-GLOO-ser-ase AL-fa
Class: Enzyme replacement

Trade Names

VPRIV
- Injection, lyophilized powder for solution 200 units
- Injection, lyophilized powder for solution 400 units

Pharmacology

Velaglucerase alfa catalyzes the hydrolysis of glucocerebroside to glucose and ceramide.

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Pharmacokinetics

Distribution

Vd is 82 to 108 mL/kg.

Elimination

The half-life is 11 to 12 min. Plasma Cl ranges from 6.72 to 7.56 mL/min/kg.

Indications and Usage

Long-term enzyme replacement therapy for children and adults with type I Gaucher disease.

Contraindications

None well documented.

Dosage and Administration

Adults and Children older than 4 yr of age

IV 60 units/kg every other week. Dosage adjustments are based on achievement and maintenance of each patient's therapeutic goals. Clinical studies have evaluated doses ranging from 15 units/kg to 60 units/kg every other week.

General Advice

  • For IV infusion only. Not for intradermal, subcutaneous, IM, IV bolus, or intra-arterial administration.
  • Reconstitute powder with sterile water for injection: 2.2 mL for 200 unit vial; 4.3 mL for 400 unit vial.
  • Upon reconstitution, mix vial gently. Do not shake.
  • Do not use if reconstituted solution is cloudy or discolored, or contains opaque particles.
  • Withdraw prescribed dose from vial(s) and dilute in 100 mL of sodium chloride 0.9% injection.
  • Administer diluted solution by 60-min IV infusion.
  • Do not infuse with other products in the same infusion tubing because the compatibility in solution with other products has not been evaluated.
  • Diluted solution may be filtered through an in-line, low protein-binding 0.2 mcm filter during administration.
  • Consider pretreatment with antihistamines and/or corticosteroids if hypersensitivity symptoms developed during previous infusion.

Storage/Stability

Store vials at 36° to 46°F. Reconstituted or diluted solution is stable for up to 24 h when stored at 36° to 46°F. The infusion should be completed within 24 h of reconstitution. Do not freeze. Protect from light. Discard any unused reconstituted solution. Do not save for future use.

Drug Interactions

None well documented.

Adverse Reactions

Cardiovascular

Hypertension, hypotension, tachycardia (more than 3%).

CNS

Headache (36%); dizziness (22%); asthenia/fatigue (13%).

Dermatologic

Flushing, rash, urticaria (more than 3%).

GI

Abdominal pain (19%); nausea (10%).

Musculoskeletal

Back pain (18%); joint (knee) pain (15%); bone pain (more than 3%).

Miscellaneous

Infusion-related reaction (52%); upper respiratory tract infection (32%); pyrexia (22%); APTT prolonged (11%).

Precautions

Monitor

Periodically monitor for immunoglobulin G (IgG) antibody formation during first year of treatment.


Pregnancy

Category B .

Lactation

Undetermined.

Children

Safety and efficacy in patients younger than 4 yr of age are not established.

Elderly

Approach dose selection for an elderly patient cautiously; consider potential comorbid conditions.

Hypersensitivity

Has been reported in clinical studies. Use with caution in patients who have exhibited symptoms of hypersensitivity to velaglucerase alfa, excipients in the drug product, or to other enzyme replacement therapies.

Immunogenicity

IgG antibodies to velaglucerase alfa may occur.

Infusion-related reactions

Infusion-related reactions (eg, dizziness, fatigue/asthenia, headache, hypertension, hypotension, nausea, pyrexia) may occur.

Overdosage

Symptoms

No data available.

Patient Information

  • Advise patient or caregiver that medication will be prepared and administered by a health care provider in a health care setting.
  • Advise patient or caregiver that reactions during or shortly after infusion occur commonly and that medications may be given before the infusion in an effort to prevent or reduce the severity of these reactions.
  • Advise patient or caregiver to notify health care provider if any of the following occur: pain, redness, or burning at infusion site; persistent or intolerable nausea, vomiting, stomach pain, diarrhea, or dizziness; any other unexplained symptom or sign.

Copyright © 2009 Wolters Kluwer Health.

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