Dosing & Uses
Dosage Forms & Strengths
Dosage Forms & Strengths
Fabry Disease (Orphan)
0.2 mg/kg IV infusion q2Weeks over 40 minutes
Antihistamines and corticosteroids may alleviate infusion-related reactions
Serious - Use Alternative
Significant - Monitor Closely
Infusion reactions (12.7%)
Erythema, & other skin problems
Concurrent use of chloroquine, amiodarone, benoquin or gentamicin (drugs that inhibit intracellular alpha-galactosidase activity)
Pregnancy & Lactation
Pregnancy Category: no clinical data, animal studies indicate safe
Lactation: not known whether distributed in breast milk, use caution
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.
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
Aseptically dilute required volume of concentrate in 100 mL NS
Administer within 3 hr of dilution
Infuse over 40 min
Do not administer with other drugs
Adding plans allows you to compare formulary status to other drugs in the same class.
To view formulary information first create a list of plans. Your list will be saved and can be edited at any time.
Adding plans allows you to:
- View the formulary and any restrictions for each plan.
- Manage and view all your plans together – even plans in different states.
- Compare formulary status to other drugs in the same class.
- Access your plan list on any device – mobile or desktop.
The above information is provided for general informational and educational purposes only. Individual plans may vary and formulary information changes. Contact the applicable plan provider for the most current information.
|1||This drug is available at the lowest co-pay. Most commonly, these are generic drugs.|
|2||This drug is available at a middle level co-pay. Most commonly, these are "preferred" (on formulary) brand drugs.|
|3||This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs.|
|4||This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.|
|5||This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.|
|6||This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.|
|NC||NOT COVERED – Drugs that are not covered by the plan.|
Drugs that require prior authorization. This restriction requires that specific clinical criteria be met prior to the approval of the prescription.
Drugs that have quantity limits associated with each prescription. This restriction typically limits the quantity of the drug that will be covered.
Drugs that have step therapy associated with each prescription. This restriction typically requires that certain criteria be met prior to approval for the prescription.
Drugs that have restrictions other than prior authorization, quantity limits, and step therapy associated with each prescription.