velaglucerase alfa(vel-a-gloo-ser-ase al-fa) ,
Pregnancy Category: B
ClassificationTherapeutic: replacement enzyme
Enzyme replacement therapy (ERT) for pediatric and adult patients with type 1 Gaucher disease.
Prevents the accumulation of glucocerebrosides in cells. Replaces glucocerebrosidases that are deficient in type 1 Gaucher’s disease. Replaces the deficient enzyme in type 1 Gaucher disease.
Improvement in symptoms of Gaucher’s disease (anemia, thrombocytopenia, bone disease, splenomegaly, and hepatomegaly).
Absorption: IV administration results in complete bioavailability.
Metabolism and Excretion: Unknown.
Half-life: 11–12 min.
Contraindicated in: None noted.
Use Cautiously in: Previous hypersensitivity reactions; pretreament required; Geriatric: consider concurrent disease states and drug therapy; Obstetric: Use during pregnancy only if clearly needed; Lactation: Use catiously if breast-feeding; Pediatric: Safe and effective use in children <4 yr not established.
Adverse Reactions/Side Effects
Central nervous system
- dizziness (most frequent)
- fatigue (most frequent)
- weakness (most frequent)
- abdominal pain (most frequent)
- nausea (most frequent)
- rash (↑ in children) (most frequent)
- ↑ aPTT(↑ in children) (most frequent)
- back pain (most frequent)
- joint pain (most frequent)
- bone pain
- hypersensitivity reactions including anaphylaxis (life-threatening)
- fever (↑ in children) (most frequent)
- infusion-related reactions (most frequent)
Drug-Drug interactionNone noted.
Oral (Adults) 60 Units/kg every other week.
Lyophilized powder for IV injection (requires reconstitution and dilution): 200 Units/vial, 400 Units/vial
- Monitor for an improvement in symptoms including hepatomegaly, splenomegaly, anemia, thrombocytopenia, bone demineralization, and ↑ appetite and energy level periodically during therapy.
- Monitor patient for signs of hypersensitivity reactions (pruritus, flushing, urticaria, angioedema, chest pain, dyspnea, hypotension). Pretreatment with antihistamines and decreasing rate of infusion usually allows patient to continue use.
- 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. May be treated with slowing infusion rate, 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.
Potential Nursing DiagnosesFatigue (Indications)
Risk for injury (Indications)
- Patients currently treated with imiglucerase may switch to velaglucerase. If on a stable imiglucerase dose, begin treatment with velaglucerase at the same dose.
- Pre-treatment with antihistamines, and/or corticosteroids may prevent subsequent infusion-related reactions.
- Determine correct amount of velaglucerase and appropriate number of vials. Reconstitute each 200 unit vial with 2.2 mL and 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. Withdraw 2 mL from each 200 Unit vial and 4 mL from each 400 Unit vial. Diluent: Dilute total volume in 100 mL of 0.9% NaCl. Administer within 24 hrs of reconstitution. Do not freeze; protect from light.
- Rate: Infuse diluted solution over 1 hr through an in-line low protein-binding 0.2 mcg filter.
- Additive Incompatibility: Do not infuse with other solutions or products.
- Inform patient of the purpose of this medication and the importance of treatment at least every 4 wk. Velaglucerase helps control the symptoms but does not cure Gaucher’s disease. Lifelong therapy may be required.
- Advise female patient to notify health care professional if pregnancy is planned or suspected or if breastfeeding.
- Emphasize the importance of follow-up examinations and lab tests.
- Increasing hemoglobin and platelet counts and decreasing acid phosphatase levels, hepatomegaly, and splenomegaly. In pediatric patients, cachexia and wasting should diminish.