Adult Dosing

Type 1 Gaucher disease

• Initial: 2.5 U/kg IV over 1-2 hrs 3 times/wk; alternate dosing: 60 U/kg q2 wks

• Individualize dosage and increase or decrease based on therapeutic response
• Pre-medication with antihistamines and corticosteroids would reduce infusion reactions

Pediatric Dosing

Type 1 Gaucher disease

Child 2-16 yrs

• Initial: 2.5 U/kg IV over 1-2 hrs 3 times/wk; alternate dosing: 60 U/kg q2 wks

• Individualize dosage and increase or decrease based on therapeutic response
• Premedication with antihistamines and corticosteroids would reduce infusion reactions

[Outline]

• Adult Dosing
• Pediatric Dosing

• Long-term enzyme replacement therapy in with Type 1 Gaucher disease that results in one or more of the non-neuropathic conditions below
• Anemia
• Thrombocytopenia
• Bone disease
• Hepatomegaly
• Splenomegaly

• Hypersensitivity to any of the ingredients of the product

• N/A

Renal Dose Adjustment

• Renal impairment: Dose adjustments not defined

Hepatic Dose Adjustment

• Hepatic impairment: Dose adjustments not defined

• Therapy with imiglucerase may lead to development of IgG antibodies, which may lead to symptoms of hypersensitivity
• Periodically monitor IgG antibody formation during the first year of treatment
• Safety and effectiveness in patients younger than 2 have not been established

Cautions: Use cautiously in

• Hypersensitivity
• Pulmonary hypertension
• Pneumonia
• Patients previously treated with alglucerase
• Lactation

Pregnancy Category:C

Breastfeeding: Imiglucerase is likely to be destroyed in the infant's GI tract and not absorbed systemically. Limited amount of data supports safety .This information is based upon LactMed database (available at http://toxnet.nlm.nih.gov/cgi-bin/sis/htmlgen?LACT last accessed 12 July 2011). Manufacture advises caution.

• LOCAL: Infusion reactions, injection site discomfort, pruritis, burning, swelling, sterile abscess
• CV: Pulmonary hypertension, tachycardia
• HYPERSENSITIVITY: Pruritis, flushing, urticaria, angioedema, chest discomfort, dyspnea, cough, cyanosis, hypotension, anaphylactoid reactions
• GI: Nausea, abdominal pain, vomiting, diarrhea
• CNS: Fatigue, dizziness, headache
• DERM: Rash, flushing, pruritus, urticaria
• MUSC: Backache
• MISC: Fever, chills, peripheral edema

• Replacement enzyme; catalyzes the hydrolysis of glucocerebroside to glucose and ceramide
• Metabolism: Unknown
• Excretion: Unknown
• Half life: 3.6-10.4 minutes

US Trade Name(s)

• Cerezyme

US Availability

Cerezyme

• PWDR for INJ: 200 units/vial
• PWDR for INJ: 400 units/vial

Canadian Trade Name(s)

• Cerezyme

Canadian Availability

Cerezyme

• PWDR for INJ: 200 units/vial
• PWDR for INJ: 400 units/vial

UK Trade Name(s)

• Cerezyme

UK Availability

Cerezyme

• PWDR for INJ: 200 U
• PWDR for INJ: 400 U

Australian Trade Name(s)

• N/A

Australian Availability

• N/A

[Outline]

Endocrine/Metabolic

Replacement Enzymes