Adult Dosing

• This therapy is not applicable in adults

Pediatric Dosing

Adenosine deaminase deficiency associated with severe combined immunodeficiency disease

• Initial dose: Start 10 U/kg; then increase 5 U/kg qwk until 20 U/kg
• Maintenance dose: 20 U/kg/wk; further increases of 5 U/kg/wk may be needed in some patients
• Max single dose: 30 U/kg

• Adenosine deaminase deficiency associated with severe combined immunodeficiency disease

• Hypersensitivity to any of the ingredients of the product
• Severe thrombocytopenia

• N/A

Renal Dose Adjustment

• Renal impairment: Dose adjustments not defined

Hepatic Dose Adjustment

• Hepatic impairment: Dose adjustments not defined

• Do not dilute or mix with any other drug prior to administration
• Do not use as a replacement for HLA identical bone marrow transplant therapy
• Not intended to be used as a preparatory or support therapy for bone marrow transplantation
• Monitor activity by measuring plasma ADA activity and red blood cell dATP levels; measure ADA and dATP levels at baseline, then q1-2 wks during first 8-12 wks of therapy & then 2-4 times/yr thereafter
• Monitor immune function and clinical status during therapy

Cautions: Use cautiously in

• Thrombocytopenia (contraindicated if severe thrombocytopenia)
• Risk of opportunistic infections

Pregnancy Category:C

Breastfeeding: Safety unknown; manufacturer advises caution.

• CNS: Headache
• LOCAL: Injection site pain

• Modified enzyme; it converts adenosine (toxic) to inosine (less toxic) by deamination
• Metabolism: Unknown
• Excretion: Unknown
• Half-life: Highly variable; 3 to >6 days

US Trade Name(s)

• Adagen

US Availability

Adagen

• INJ: 250 units/mL (1.5 mL vials)

Canadian Trade Name(s)

• N/A

Canadian Availability

• N/A

UK Trade Name(s)

• N/A

UK Availability

• N/A

Australian Trade Name(s)

• N/A

Australian Availability

• N/A

Allergy & Immunology

Biologicals; Miscellaneous

Hematology/Oncology

Biologicals; Miscellaneous