velaglucerase alfa

Prevents the accumulation of glucocerebrosidases in cells. Replaces glucocerebrosidases that are deficient in type 1 Gaucher disease. Replaces the deficient enzyme in type 1 Gaucher disease.

Therapeutic effects:

Improvement in symptoms of Gaucher disease (anemia, thrombocytopenia, bone disease, splenomegaly, and hepatomegaly).

Pharmacokinetics ⬆ ⬇

Absorption: IV administration results in complete bioavailability.

Distribution: Unknown.

Metabolism/Excretion: Unknown.

Half-Life: 11–12 min.

Time/Action Profile ⬆ ⬇

ROUTE	ONSET	PEAK	DURATION
IV	unknown	unknown	unknown

Contraind./Precautions ⬆ ⬇

Contraindicated in:

None.

Use Cautiously in:

Previous hypersensitivity reactions (pretreatment required);
OB: Use during pregnancy only if clearly needed;
Lactation: Safety not established in breastfeeding;
Pedi: Children <4 yr (safety and effectiveness not established).

Adv. Reactions/Side Effects ⬆ ⬇

CV: hypertension, hypotension

GI: abdominal pain, nausea

Derm: rash (↑ in children), flushing, urticaria

Hemat: ↑aPTT (↑ in children)

MS: back pain, joint pain, bone pain

Neuro: dizziness, fatigue, weakness, headache

Misc: fever (↑ in children), infusion-related reactions, HYPERSENSITIVITY REACTIONS (INCLUDING ANAPHYLAXIS)

Interactions ⬆ ⬇

Drug-drug:

None reported.

Route/Dosage ⬆ ⬇

IV (Adults ): 60 units/kg every other wk.

Availability ⬆ ⬇

Lyophilized powder for injection: 400 units/vial

Assessment ⬆ ⬇

Monitor for an improvement in symptoms including hepatomegaly, splenomegaly, anemia, thrombocytopenia, bone demineralization, and ↑ appetite and energy level periodically during therapy.
Assess and monitor for signs and symptoms of anaphylaxis (urticaria, hypotension, flushing, wheezing, chest tightness, nausea, vomiting, dizziness). If anaphylaxis occurs stop infusion immediately and initiate appropriate treatment.
Monitor for signs and symptoms of infusion-related reactions (headache, dizziness, hypotension, hypertension, nausea, fatigue, pyrexia). Most reactions are mild, occur within the 1st 6 mo of therapy, and diminish with time. Treat by slowing infusion rate, administering antihistamines, antipyretics, corticosteroids, and/or stopping infusion and resuming with ↑ infusion time.

Lab Test Considerations:

Monitor hemoglobin and platelet count monthly to determine effectiveness of therapy.
- May cause prolonged activated partial thromboplastin time.

Implementation ⬆ ⬇

Patients currently treated with imiglucerase may switch to velaglucerase. If on a stable imiglucerase dose, begin treatment with velaglucerase at the same dose.
Administer antihistamine, antipyretics, and/or corticosteroids prior to infusion, if required, to prevent hypersensitivity reactions.
Administer in a health care setting with appropriate medical monitoring and supportive measures (cardiopulmonary resuscitation equipment) available during administration.
If a severe hypersensitivity reaction (e.g., anaphylaxis) occurs, discontinue immediately and initiate appropriate medical treatment (epinephrine). Consider the risks and benefits of readministering following severe hypersensitivity reactions.

IV Administration:

Intermittent Infusion: Reconstitution: Determine correct amount of velaglucerase and appropriate number of vials. Reconstitute each 400-unit vial with 4.3 mL of sterile water for injection. Mix vials gently; do not shake. Solution is clear to slightly opalescent and colorless; do not administer solutions that are discolored or contain particulate matter. Concentration: 100 units/mL. Vials are for single use only; discard unused solution.Diluent: Dilute total volume in 100 mL of 0.9% NaCl. Administer within 24 hr of reconstitution. Do not freeze; protect from light.
Rate: Infuse diluted solution over 1 hr through an in-line low-protein-binding 0.2-micron filter.
Y-Site Incompatibility: Do not administer other products through infusion tubing.

Patient/Family Teaching ⬆ ⬇

Explain the purpose and side effects of velaglucerase alfa to patient and/or caregiver. Emphasize the importance of treatment at least every 4 wk. Velaglucerase helps control the symptoms but does not cure Gaucher disease. Lifelong therapy may be required.
Advise patient and/or caregiver to readPatient Information before starting therapy
Advise patient and/or caregiver to notify health care professional of all Rx or OTC medications, vitamins, or herbal products being taken and to consult health care professional before taking other medications.
Advise patient and/or caregiver if a dose is missed to notify a health care professional.
Teach patient the signs and symptoms of an hypersensitivity reaction; if any occur, seek immediate medical care.
Teach patient and/or caregiver the signs and symptoms of infusion-related reactions.
Educate patient and/or caregiver on the importance of follow-up examinations and lab tests.
Rep: Advise females of reproductive potential to notify health care professional if pregnancy is planned or suspected or if breastfeeding.

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Pronunciation ⬇

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