Imiglucerase
Imiglucerase (Cerezyme)
Imiglucerase
(ihm-eh- GLEW-sir-ace)
Pregnancy Category: C Cerezyme (Rx)

Classification: Drug for Gaucher's disease

Action/Kinetics: Produced by recombinant DNA technology. Is an analogue of the human enzyme ß-glucocerebrosidase which is a lysosomal glycoprotein enzyme that catalyzes the hydrolysis of glucocerebroside to glucose and ceramide. Clients with Gaucher's disease have a deficiency of ß-glucocerebrosidase resulting in an accumulation of glucocerebrosidase in tissue macrophages; leads to severe anemia, thrombocytopenia, hepatosplenomegaly, and skeletal complications. Imiglucerase replaces the enzyme normally found in the body. t 1/2: 3.6-10.4 min following infusion.

Uses: Long-term replacement therapy for clients with confirmed Type 1 Gaucher's disease.

Contraindications: Hypersensitivity to the product.

Special Concerns: Possible development of IgG antibodies reactive with imiglucerase. Use with caution during lactation.

Side Effects: CNS: Headache, dizziness. GI: Nausea, abdominal discomfort. Hypersensitivity: Pruritus, rash, allergic hypersensitivity reactions. Miscellaneous: Mild decrease in BP, decreased urinary frequency.

How Supplied: Lyophilized for Injection: 200 U/vial

Dosage
?IV Gaucher's disease.
Initial: Dose ranges from 2.5 U/kg 3 times/week to 60 U/kg once a week or q 4 weeks. The usual initial dose is 60 U/kg q 2 weeks. Maintenance: After client response has been established, a decrease in dosage may be attempted. Progressive decreases in dose can be made at intervals of 3-6 months with appropriate monitoring.