Healthcare Providers

Get Started on VPRIV

In order to help your patients begin treatment with VPRIV, you should work with your patient to complete, sign and submit a Start Form, available for download.

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If you have questions about the form, please contact us

Data of VPRIV

Learn more about the efficacy and safety of VPRIV in treating type 1 Gaucher disease.

VPRIV data

See how improvement of multiple disease measures is brought together in one treatment.

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Chemical Structure and Cellular Uptake

Details of the Chemical Structure of VPRIV

The Glycan Structure of VPRIV1

glycan graphic

Used with permission from Brumshtein B et al, Oxford University Press, copyright 2010.

  • VPRIV is composed of 497 amino acids with a sequence identical to natural human protein
  • The glycan structure of VPRIV contains high-mannose type glycans consisting of 6 to 9 mannose units with a predominant 9-mannose structure
  • Data are from an in vitro study evaluating the molecular structure and cellular internalization of the human cell line-produced VPRIV
  • In vitro test results do not necessarily correlate with clinical efficacy

Cellular Uptake of VPRIV

VPRIV internalization into differentiated macrophages in vitro1

internalization graph

An in vitro study of crystal structure, kinetics, glycan composition, and internalization into macrophages.

RFU=relative fluorescence units.

  • Internalization of proteins is highly dependent upon their carbohydrate composition
  • The glycan structure of VPRIV contains long-chain, high–mannose-type glycans
  • Data are from an in vitro study evaluating the molecular structure and cellular internalization of the human cell line-produced VPRIV
  • In vitro test results do not necessarily correlate with clinical efficacy
learn the details about dosing and infusion with VPRIV

Reference: 1.
Brumshtein B, Salinas P, Peterson B, et al. Characterization of gene-activated human acid-beta-glucosidase: crystal structure, glycan composition, and internalization into macrophages. Glycobiology. 2010;20(1):24-32.

Indication

  • VPRIV® (velaglucerase alfa for injection) is a hydrolytic lysosomal glucocerebroside-specific enzyme indicated for long-term enzyme replacement therapy (ERT) for pediatric and adult patients with type 1 Gaucher disease.

Important Safety Information

  • The most serious adverse reactions in patients treated with VPRIV were hypersensitivity reactions. Appropriate medical support should be available when VPRIV is administered. If a severe reaction occurs, medical standards for emergency treatment are to be followed.
  • Treatment with VPRIV should be used with caution in patients who have exhibited symptoms of hypersensitivity to the active ingredient, drug product excipients, or to other enzyme replacement therapies.
  • Infusion-related reactions were the most commonly observed adverse reactions in patients treated with VPRIV in clinical studies. The most commonly observed symptoms of infusion-related reactions were: headache, dizziness, hypotension, hypertension, nausea, asthenia/fatigue, and pyrexia. Generally the infusion-related reactions were mild and, in treatment-naïve patients, onset occurred mostly during the first 6 months of treatment and tended to occur less frequently with time.
  • Management of infusion-related reactions should be based on severity of the reaction, such as slowing the infusion rate, treatment with medications such as antihistamines, antipyretics and/or corticosteroids, and/or stopping and resuming treatment with increased infusion time.
  • Other commonly observed adverse reactions in ≥10% of ERT-naïve or imiglucerase-switched patients were: headache, dizziness, abdominal pain, nausea, back pain, joint pain, upper respiratory tract infection, activated PTT prolonged, infusion-related reactions, pyrexia, and asthenia/fatigue.
  • All adult adverse reactions to VPRIV are considered relevant to pediatric patients (ages 4 to 17 years). Adverse reactions more common in pediatric patients (>10% difference) included upper respiratory tract infection, rash, aPTT prolonged, and pyrexia. The safety of VPRIV has not been established in pediatric patients younger than 4 years of age.
  • As with all therapeutic proteins, there is a potential for immunogenicity. In clinical studies, 1 of 54 treatment-naïve patients treated with VPRIV (who received a 45 Units/kg dose) developed IgG class antibodies (neutralizing in an in vitro assay). It is unknown if the presence of IgG antibodies to VPRIV is associated with a higher risk of infusion reactions. Patients with an immune response to other enzyme replacement therapies who are switching to VPRIV should continue to be monitored for antibodies.
  • For more information, please see the full prescribing information or call Shire at 1-866-888-0660
  • To report suspected adverse events, please contact Shire Human Genetic Therapies at 1-866-888-0660 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.
  • For assistance with medical inquiries about VPRIV, please contact Medical Information at 1-866-888-0660 option 2 or US_ShireHGT_MedicalInformation@shire.com.