Inborn errors of metabolism:
Indications for VPRIV:
Long-term enzyme replacement therapy (ERT) in patients with Type 1 Gaucher disease.
Adults and Children:
<4yrs: not established. Give by IV infusion over 60mins. Individualize. ≥4yrs: 60 Units/kg every other week; adjust dose based on therapeutic goals. Begin treatment with Vpriv at the same dose when switching from imiglucerase.
Have medical support and resuscitative measures readily available. Discontinue immediately and treat if anaphylactic or other acute reactions occur. Temporarily discontinue and resume with increased infusion time, slowing infusion rate, or administer antihistamines, antipyretics, and/or corticosteroids if hypersensitivity reactions occur. Elderly. Pregnancy. Nursing mothers.
Lysosomal glucocerebroside-specific enzyme.
Hypersensitivity reactions, headache, dizziness, abdominal pain, nausea, back pain, joint pain, activated PTT prolonged, fatigue/asthenia, pyrexia.