Dosing & Uses
Dosage Forms & Strengths
powder for injection
Serious - Use Alternative
Significant - Monitor Closely
Infusion Reactions (50-55%)
- Decrease frequency with continued treatment
- Bradycardia, flushing, hypertension, hypotension, peripheral edema, tachycardia
- Chills, dizziness, fatigue, feeling hot or cold, headache, pain in extremity, paresthesia, pyrexia, somnolence
- Abdominal pain, diarrhea, nausea, vomiting
- Pruritus, Urticaria
- Chest pain, dyspnea, nasal congestion, throat tightness
Upper respiratory tract infections (44%)
Edema peripheral (21%)
Pain in extremity (19%)
Lower respiratory tract infection (18%)
Back pain (16%)
Feeling cold (11%)
Sr creatinine increased (9%)
Respiratory tract congestion (8%)
Burning sensation (6%)
Chest discomfort (5%)
Fungal infection (5%)
Hot flash (5%)
Muscle spasms (5%)
Ventricular wall thickening (5%)
Viral infections (5%)
Dry mouth (4%)
Localized infection (4%)
Thermal burn (4%)
Hypersensitivity to product or components
Moderate-to-severe HTN, renal impairment, febrile patients, patients with prior sensitivity to agalsidase products
Severe reactions occur may occur despite premedication; decrease infusion rate or temporarily discontinue infusion and/or administer additional antihistamines, antipyretics, and/or steroids to manage infusion reactions; in severe reactions may consider immediate discontinuation of infusion
Development of IgG antibodies reported within 3 months from the onset of therapy; some may also develop IgE antibodies
Premedicate with antipyretics 1 hr before infusion
Use caution in patients with cardiovascular diseases
Fabry Disease Registry has been created to monitor therapeutic responses and adverse effects; www.fabryregistry.com or 1-800-745-4447
Pregnancy & Lactation
Pregnancy Category: B
Lactation: Not known if distributed into 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.
Mechanism of Action
Recombinant alpha-galactosidase A, an enzyme (deficient in Fabry disease) involved in the metabolism of glycosphingolipids (eg, GL-3); corrects abnormal glycosphingolipid metabolism resulting in reduced accumulation of glycosphingolipids and improvement of associated clinical manifestations; reduces GL-3 deposition in capillary endothelium
Half-Life: 86-151 min (children); 45-119 min (adults)
Onset: 10-20 wk
Vd: 247-1097 mL/kg; 81-570 mL/kg
Should not be infused in the same intravenous line with other products.
Reconstitute 35 mg vial by injecting 7.2 mL of SWI & 5 mg vial by injecting 1.1 mL SWI to yield a 5 mg/mL clear, colorless solution
Visually inspect the reconstituted vials for particulate matter & discoloration
Do not use the reconstituted solution if there is particulate matter or if it is discolored
The reconstituted solution should be further diluted with NS to a final total volume of 50-500 mL based on dosage
Remove dose amount of NS from infusion bag & replace w/ drug solution
Reconstituted & diluted solns should be used immediately (drug contains no preservatives); however may be stored in fridge for up to 24 hr
Initial infusion rate
- Not to exceed 0.25 mg/min (15 mg/hr); may incr by 0.05-0.08 mg/min (3-5 mg/hr) for on each subsequent infusion
- <30 kg: not to exceed 0.25 mg/min
- >30 kg: infuse over at least 1.5 hr
Do not shake or agitate product
Do not use filter needles during the preparation of the infusion
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.