Generic Name and Formulations:
Pegademase bovine 250 Units/mL; soln for IM inj; preservative-free.
Company:
Sigma-Tau Pharmaceuticals, Inc.
Indications for ADAGEN:
Enzyme replacement therapy for adenosine deaminase (ADA) deficiency in patients with severe combined immunodeficiency disease (SCID) who are not suitable candidates for, or who have failed, bone marrow transplantation.
Adult:
Not applicable.
Children:
Individualize. Give by IM inj every 7 days. 1st dose: 10 Units/kg. 2nd dose: 15 Units/kg. 3rd dose: 20 Units/kg. Maintenance dose: 20 Units/kg per week. May increase by 5 Units/kg/week if necessary; max single dose 30 Units/kg. Biochemical goals: maintain plasma ADA activity (trough levels before maintenance inj) in the range of 15–35μmol/hr/mL; decline in erythrocyte dATP to ≤0.005–0.015μmol/mL packed erythrocytes, or ≤1% of the total erythrocyte adenine nucleotide (ATP + dATP) content, with a normal ATP level, as measured in a pre-injection sample.
Contraindications:
As preparatory or support therapy for bone marrow transplantation. Severe thrombocytopenia.
Warnings/Precautions:
Determine plasma ADA activity and red cell dATP prior to treatment. Measure plasma ADA activity (pre-injection) every 1–2 weeks during the first 8–12 weeks to establish dose. Once dATP level has fallen adequately, measure 2–4 times a year during the remainder of the first year and 2–3 times a year thereafter. Between 3 and 9 months, determine plasma ADA twice a month, then monthly until after 18–24 months of treatment. After 2 years of maintenance therapy, measure plasma ADA every 2–4 months and red cell dATP twice yearly. Monitor immune function and clinical status; improvement may be gradual but should be apparent by end of first year of therapy. Pregnancy (Cat.C). Nursing mothers.
Interactions:
May potentiate vidarabine. May be antagonized by 2'-deoxycoformycin.
Pharmacological Class:
Enzyme.
Adverse Reactions:
Headache, inj site pain; possible antibody formation (perform assay if suspected).
How Supplied:
Single-use vial (1.5mL)—4
