Indication:
Proprietary Name- Vpriv
Established Name - Velaglucerase Alfa
Applicant- Shire Human Genetic Therapies Inc
Indication-
Indicated for long term enzyme replacement therapy (ERT) for
pediatric and adult patients with Type 1 Glaucher Disease
Approved by FDA on 26-2-2010 (Ref- FDA approved List- 2010)
Adverse Reaction:
Most common adverse reactions during clinical studies include-
headache, dizziness, abdominal pain, nausea, back pain, joint pain,
prolonged activated PTT, fatigue/asthenia, and pyrexia
Contra-Indications:
CONTRAINDICATIONS
None
WARNINGS AND PRECAUTIONS
Hypersensitivity
Hypersensitivity reactions including anaphylaxis have occurred.
Ensure that personnel administering product are adequately
trained in cardiopulmonary resuscitative measures, and have ready
access to emergency medical services
.
Consider slowing infusion rate, treatment with antihistamines,
antipyretics and/or corticosteroids, and/or stopping treatment if a
hypersensitivity reaction occurs during an infusion.
Consider pre-treatment with antihistamines and/or corticosteroids
in patients with prior reactions
Dosages/ Overdosage Etc:
Indication-
Indicated for long term enzyme replacement therapy (ERT) for
pediatric and adult patients with Type 1 Glaucher Disease
INDICATIONS AND USAGE
VPRIV is a hydrolytic lysosomal glucocerebroside-specific enzyme
indicated for long-term enzyme replacement therapy (ERT) for patients
with type 1 Gaucher disease
DOSAGE AND ADMINISTRATION
Recommended Starting Dose in Adults and Pediatric Patients 4 Years of
Age or Older:or Patients Naïve to Enzyme Replacement Therapy: 60 Units/kg
Patients being treated with stable imiglucerase dosages for Gaucher
disease: Can switch to VPRIV at previous imiglucerase dose two weeks
after last imiglucerase dose
Determine number of vials to be reconstituted based on patient’s actual
weight and prescribed dose
DOSAGE FORMS AND STRENGTHS
Lyophilized powder to be reconstituted and diluted for infusion
.
Available in 400 Units single-use vials
Patient Information:
1.Advise patients that VPRIV is a treatment that is given intravenously every
other week. The infusion typically takes up to 60 minutes.
2.Advise patients that VPRIV may cause hypersensitivity reactions
Pharmacology/ Pharmacokinetics:
1. Mechanism of Action
Gaucher disease is an autosomal recessive disorder caused by mutations
in the GBA gene, which results in a deficiency of the lysosomal enzyme
beta-glucocerebrosidase. Glucocerebrosidase catalyzes the conversion
of the sphingolipid glucocerebroside into glucose and ceramide.
The enzymatic deficiency causes an accumulation of glucocerebroside
primarily in the lysosomal compartment of macrophages, giving rise to
foam cells or -Gaucher cells-. Velaglucerase alfa catalyzes the hydrolysis
of glucocerebroside, reducing the amount of accumulated glucocerebroside.
2. P h a rm a co k i n et i cs
In. a multicenter study conducted in pediatric ( 4 to 17 years old)
and adult ( 19 to 62 years old) patients with type 1 Gaucher disease,
pharmacokinetic evaluations were performed at Weeks 1 and 37 following
60-minute intravenous infusions of VPRIV 60 Units/kg every other week.
Serum velaglucerase alfa concentrations declined rapidly with a mean
half life of 11 to 12 minutes.
.
Pregnancy and lactation:
1 Pregnancy
Pregnancy Category B
Risk Summary-There are no adequate and well controlled studies with VPRIV
in pregnant women and there is limited experience in pregnant women.
Because animal reproduction studies are not always predictive of human response,
VPRIV should be used during pregnancy only if clearly needed.
2. Nursing Mothers
It is not known whether VPRIV is present in human milk. The developmental and
health benefits of breastfeeding should be considered along with any potential
adverse effects on the breastfed child from the drug or from the underlying
maternal condition.
Exercise caution when VPRIV is administered to a nursing woman.
3. Pediatric Use
The safety and effectiveness of VPRIV have been established for enzyme
replacement therapy (ERT) in patients between 4 and 17 years of age with
type 1 Gaucher disease.
The safety and efficacy profiles were similar between pediatric and adult
patients.
4.Geriatric Use
In clinical studies of VPRIV in Gaucher’s disease,,the adverse reaction profile
in elderly patients was consistent with that previously observed across
pediatric and adult patients.
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