Vpriv (Velaglucerase alfa) is an enzyme with amino acid sequences that are similar to the endogenous glucocerebrosidase enzyme. It is used in children and adults with type 1 Gaucher disease for the long term enzymatic replacement. It results in an improvement in anemia, thrombocytopenia, skeletal pains, bony disease, and visceromegaly.

Vpriv (Velaglucerase alfa) Dose in Adults

Note:

• Patients with hypersensitivity reactions to the drug must be pretreated with corticosteroids and antihistamines.

Vpriv dosage in the treatment of Type 1 Gaucher’s disease:

• 60 units/kg intravenous every other week.
• The dose should be adjusted based on disease severity.
• The usual dosage range between 15 - 60 units/kg on alternate weeks.

Note:

• Velaglucerase alfa treatment may be initiated after 2 weeks of the last dose of imiglucerase at the same dose if switching the treatment is required.

Vpriv dose in Children:

Note:

Patients with hypersensitivity reactions to the drug must be pretreated with corticosteroids and antihistamines.

Vpriv dose in the treatment of Type 1 Gaucher’s disease:

• Children and Adolescents 4 - 17 years:

  • 60 units/kg intravenous every other week.
  • The dose should be adjusted based on disease severity.
  • The usual dosage range between 15 - 60 units/kg on alternate weeks.

Note:

• Velaglucerase alfa treatment may be initiated after 2 weeks of the last dose of imiglucerase at the same dose if switching the treatment is required.

Vpriv Pregnancy Risk Factor B

• If Gaucher's disease isn't controlled, patients with the disease could have an abortion.
• Pregnancy can also increase the severity of the condition and may cause new symptoms. 
• Pregnancy may also be a time when you might see thrombocytopenia or hepatosplenomegaly.
• Animal reproductive studies have not shown any evidence of teratogenic effects.

Use Velaglucerase alfa during breastfeeding

• It is unknown if the drug will be excreted into breastmilk.
• It is recommended that lactating mothers use it with caution.

Vpriv (Velaglucerase alfa) Dose in Kidney Disease:

• Adjustment in the dose has not been provided by the manufacturer in patients with liver disease.

Vpriv (Velaglucerase alfa) Dose in Liver Disease:

• Adjustment in the dose has not been provided by the manufacturer in patients with liver disease.

Common Side Effects of Vpriv (Velaglucerase alfa) Include:

• Central Nervous System:

  • Headache
  • Dizziness
  • Fatigue
  • Weakness

• Gastrointestinal:

  • Abdominal Pain

• Hematologic & Oncologic:

  • Prolonged Partial Thromboplastin Time

• Hypersensitivity:

  • Hypersensitivity Reaction

• Neuromuscular & Skeletal:

  • Back Pain
  • Arthralgia

• Miscellaneous:

  • Fever

Less Common Side Effects of Vpriv Include:

• Cardiovascular:

  • Flushing
  • Hypertension
  • Hypotension
  • Tachycardia

• Dermatologic:

  • Skin rash
  • Urticaria

• Gastrointestinal:

  • Nausea

• Immunologic:

  • Immunogenicity

• Neuromuscular & skeletal:

  • Ostealgia

Contraindication to Vpriv (Velaglucerase alfa) Include:

• The manufacturer has not listed any contraindications.

Warnings and precautions

• Formation of antibodies:
  • Although Ig G antibodies could develop, their clinical significance remains unknown.
  • Patients receiving other enzymatic therapies might also develop antibodies to the enzyme. This should be monitored regularly.
• Hypersensitivity reactions
  • Patients with a hypersensitivity reaction to Vpriv (velaglucerasealfa) or similar enzyme therapies should be advised to discontinue use of the drug.
  • Patients may develop severe reactions that may require immediate administration of fluids or adrenaline. You should infuse it in a setting that has all of the necessary facilities and lifesaving medicine.
  • Other common allergic reactions include fatigue, dizziness and fever.
  • Most patients develop mild hypersensitivity reactions during the first six months of the treatment that resolve with symptomatic treatments, antihistamines, corticosteroids, and temporary withholding the infusion or slowing the infusion rate.
  • If the condition is severe, it should be stoppe  

Monitor:

• Monitor for visceromegaly (clinically and radiologically using MR, CT, or USG)
• CBC,
• Liver function tests,
• IgG antibodies
• Bone densitometry

How to administer Vpriv (Velaglucerase alfa)?

• It is administered intravenously as an intravenous infusion over one hour.
• A special low protein-binding 0.2-micron filter should be used.
• Other products should not be infused in the same line/ tubing.

Mechanism of action of Velaglucerase alfa (Vpriv):

• Velaglucerase Alfa is an enzyme preparation which contains the same amino acids sequence as endogenous glucosecerebrosidase.
• Type 1 Gaucher's Disease is characterised by a deficiency in glucocerebrosidase, which results in an accumulation of glucocerebroside within the macrophages.
• Vpriv is able to catalyze the hydrolysis in the lysosome of glucocerebroside, glucose, and ceramide. It reduces anemia, thrombocytopenia and skeletal pains.

It has a half-life elimination of 11-12 minutes

International Brands of Velaglucerase alfa:

• Vpriv

Velaglucerase alfa Brands in Pakistan:

No Brands Available in Pakistan.