Dosing and uses of Replagal (agalsidase alfa)
Adult dosage forms and strengths
injectable solution
- 1mg/mL
Fabry Disease (Orphan)
0.2 mg/kg IV infusion q2Weeks over 40 minutes
Antihistamines and corticosteroids may alleviate infusion-related reactions
Pediatric dosage forms and strengths
injectable solution
- 1mg/mL
Fabry Disease (Orphan)
0.2 mg/kg IV infusion q2Weeks over 40 minutes
Antihistamines and corticosteroids may alleviate infusion-related reactions
Replagal (agalsidase alfa) adverse (side) effects
>10%
Flushing (23.6%)
Headache (10.9%)
Infusion reactions (12.7%)
Pyrexia (20%)
Rigors (20%)
1-10%
Abdominal pain
Acne
Asthenia
Chest pain/tightness
Diarrhea
Dizziness
Dyspepsia
Eczema
Erythema, & other skin problems
Edema
Fatigue
GI problems
HTn
Myalgia
Nausea/vomiting
Neuropathic pain
Other pain
Pharyngitis
Throat irritation
Warnings
Contraindications
Hypersensitivity
Concurrent use of chloroquine, amiodarone, benoquin or gentamicin (drugs that inhibit intracellular alpha-galactosidase activity)
Pregnancy and lactation
Pregnancy category: no clinical data, animal studies indicate safe
Lactation: not known whether distributed in 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 Replagal (agalsidase alfa)
Half-Life, Elimination: 1.5-1.8 hr
Half-Life, tissue: 24 hr
Metabolism: likely as other proteins
Excretion: not significantly by renal clearance
Mechanism of action
Recombinant alpha-galactosidase A, an enzyme (deficient in Fabry dz) involved in the metabolism of glycosphingolipids (e.g., GL-3); correcting abnormal glycosphingolipid metabolism resulting in reduced accumulation of glycosphingolipids and improvement of associated clinical manifestations
Administration
IV Preparation
Aseptically dilute required volume of concentrate in 100 mL Ns
Administer within 3 hr of dilution
IV Administration
Infuse over 40 min
Do not administer with other drugs
