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
[Outline]
- 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.
