Dosing and uses of Cerezyme (imiglucerase)
Adult dosage forms and strengths
powder for injection
- 200 units
- 400 units
Gaucher Disease
Indicated for type 1 Gaucher's disease; associated with one or more of the following: anemia, thrombocytopenia, bone disease, hepatomegaly, splenomegaly
2.5 units/kg IV infused over 1-2 hr 3 times per week OR 30-60 unit/kg IV q2Weeks initially
May initiate at a higher dose/frequency based on disease severity
Up to 240 unit/kg q2Weeks has been safely used
Pediatric dosage forms and strengths
powder for injection
- 200 units
- 400 units
Gaucher Disease
<2 years: Safety and efficacy not estabilshed
>2 years: 2.5 units/kg IV (1-2 hours infusion) 3 times per week OR 30-60 unit/kg IV q2Weeks initially
May initiate at a higher dose/frequency based on disease severity
Up to 240 unit/kg q2Weeks has been safely used
Cerezyme (imiglucerase) adverse (side) effects
1-10%
Hypersensitivity with infusion (6.6%)
Nausea, abdominal pain, vomigin, diarrhea (1.5%)
Rash (1.5%)
Fatigue (1.5%)
Headache (1.5%)
Fever (1.5%)
Dizziness (1.5%)
Chills (1.5%)
Backache (1.5%)
Tachycardia (1.5%)
<1%
Discomfort
Pruritus
Burning
Swelling
Sterile abscess at site of venipuncture
Pulmonary hypertension
Pneumonia
Warnings
Contraindications
None listed in the manufacturer's labeL
Cautions
Antibodies to imiglucerase that may lead to infusion reactions reported in up to 15% of patients within 6 months from the onset of therapy; antibody formation rare after 12 months of therapy
Most patients with infusion reactions are able to continue treatment if pre-treated with antihistamines and/or corticosteroids and infused at a lower rate
Pregnancy and lactation
Pregnancy category: C
Lactation: Not known if distributed into breast milk, use caution
Pregnancy categories
A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.
B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.
C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.
D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.
X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.
NA: Information not available.
Pharmacology of Cerezyme (imiglucerase)
Mechanism of action
Analog of beta-glucocerebrosidase, lysosomal enzyme replacement that plays a role in the hydrolysis of glucocerebroside to glucose and ceramide
Pharmacokinetics
Half-Life: 3.6-10.4 min
Onset: 30 min
Vd: 0.09-0.15 L/kg
Renal Clearance: 9.8-20.3 mL/min/kg
Administration
IV Preparation
Reconstitute with 5.1 mL SWI for 200 u vial & 10.2 mL for 400 U vial (resulting conc 40 U/mL)
Stable for 12 hr at room temp & fridge
IV Administration
Dilute to a final volume 100-200 mL w/ Ns
Diluted soln stable for 24 hr in fridge
Infuse over 1-2 hr
Storage
Store vials at 2-8°C (36-46°F)
