Dosing and uses of Adagen (pegademase)
Adult dosage forms and strengths
Not recommended for adults
Pediatric dosage forms and strengths
injectable solution
- 250 units/mL
Adenosine Deaminase Deficiency
Dosing should be individualized based on plasma adenosine deaminase (ADA) activity levels and biochemical markers of ADA deficiency
Do not mix or dilute with other drugs prior to administration
Indicated in patients with severe combined immunodeficiency disease (SCID) who cannot undergo bone marrow transplantation.
Administer q7Days Im
- 1st dose: 10 units/kg IM
- 2nd dose: 15 units/kg IM
- 3rd dose: 20 units/kg IM
- Maintenance: 20 units/kg/week IM, may increase by 5 units/kg/week; not to exceed 30 units/kg
Adagen (pegademase) adverse (side) effects
Frequency not defined
Hematologic events: Hemolytic anemia, autoimmune hemolytic anemia, thrombocythemia, thrombocytopenia and autoimmune thrombocytopenia
Dermatological events: Injection site erythema, urticaria
Lymphomas
Headache
Warnings
Contraindications
Use as a preparatory/support therapy for bone marrow transplantation
Severe thrombocytopenia
Cautions
Use caution in patients with thrombocytopenia; contraindicated in patients with severe thrombocytopenia
Monitor plasma ADA activity & red blood cell dATP levels
Development of antibodies reported, which may result in rapid clearance; adjust dose as necessary in patients developing antibodies
Risk for infection increases in patients that are not able to maintain adequate levels of plasma ADA
Not a substitute for bone marrow transplant
Pregnancy and lactation
Pregnancy category: C
Lactation: Not known if distributed into breast milk, use caution
Pregnancy categories
A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.
B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.
C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.
D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.
X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.
NA: Information not available.
Pharmacology of Adagen (pegademase)
Mechanism of action
Replaces adenosing deaminase deficiency; the enzyme that catalyzes the deamination of deoxyadenosine and adenosine; this results in combined immunodeficiency disease with profound cellular and humoral immunity
Pharmacokinetics
Half-life: 3-6 days
Peak plasma time: 2-3 days
Absorption: Rapid
