Adult Dosing

Fabry disease

• Recommended dose is 1 mg/kg IV infusion q2 wks
• Initial infusion rate should not be >0.25 mg/minute; after the infusion is well tolerated, may increase the rate by 0.05-0.08 mg/minute with each subsequent infusion

• Prior to infusion, administer antipyretic and antihistamine in patients who have experienced infusion reactions
• For patients <30 kg, maximum infusion rate should be maintained at 0.25 mg/min; for patients 30 kg, the duration of administration should not be <1.5 hrs
• Avoid shaking or agitating the product

Pediatric Dosing

Fabry disease

Children >8 yrs

• Recommended dose is 1 mg/kg IV infusion q2 wks
• Initial infusion rate should not be >0.25 mg/minute; after the infusion is well tolerated, may increase the rate by 0.05-0.08 mg/minute with each subsequent infusion

• Prior to infusion, administer antipyretic and antihistamine in patients who have experienced infusion reactions
• For patients <30 kg, maximum infusion rate should be maintained at 0.25 mg/min; for patients 30 kg, the duration of administration should not be <1.5 hrs
• Avoid shaking or agitating the product

[Outline]

• Adult Dosing
• Pediatric Dosing

• Treatment of Fabry disease

• Hypersensitivity to any of the ingredients of the product

• N/A

Renal Dose Adjustment

• Renal impairment: Dose adjustments not defined

Hepatic Dose Adjustment

• Hepatic impairment: Dose adjustments not defined

• Life-threatening and fatal allergic reactions including localized angioedema (including swelling of the face, mouth, and throat), bronchospasm, rash, generalized urticaria, pruritus, dysphagia, dyspnea, hypotension, flushing, chest discomfort, and nasal congestion have been reported during infusion. Consider discontinuation of therapy, if anaphylaxis or severe allergic reactions occur during therapy
• Risks and benefits of re-administration after anaphylaxis or severe allergic reactions should be considered
• If re-administration of the product is indicated, adequate medical support and facilities should be available because of the potential for serious adverse reactions
• Incidences of severe infusion reactions such as chills, vomiting, hypotension, paresthesia, feeling hot or cold, pyrexia, nausea, dyspnea, throat tightness, flushing, headache, pruritus, fatigue, hypertension, tachycardia, chest pain, abdominal pain, diarrhea, dizziness, nasal congestion, urticaria, bradycardia, somnolence, pain in extremity, myalgia, and edema peripheral have occurred during infusion
• Consider pretreatment with antipyretics and antihistamines in patients who have experienced infusion reactions
• If an infusion reaction occurs, consider reducing the infusion rate, temporarily stopping the infusion, or administrating additional antipyretics, antihistamines, or steroids. For severe reactions, discontinue therapy and initiate appropriate medical treatment
• Patients with advanced Fabry disease may have compromised cardiac function, which may predispose them to a higher risk of severe complications from infusion reactions; monitor such patients carefully during therapy
• Incidences of patients developing IgE antibodies and skin test reactivity specific to agalsidase beta have been observed. Patients with a positive skin test to agalsidase beta or those with specific IgE antibodies to agalsidase beta should be re-challenged under the supervision of qualified personnel, with appropriate medical support readily available

Pregnancy Category:B

Breastfeeding: Safety unknown; manufacturer advises caution.

• CNS: Headache, dizziness, somnolence, vertigo, ataxia
• NEURO: Paresthesia
• PSYCHIATRIC: Anxiety, depression
• GI: Nausea, vomiting, abdominal pain, diarrhea, toothache, dry mouth
• CV: Hypertension, chest pain, tachycardia, bradycardia, stroke, cardiac arrhythmia, cardiac arrest, decreased cardiac output, hypotension, ventricular wall thickening, chest discomfort, hot flush, peripheral edema
• RESP: Dyspnea, URTI, lower respiratory tract infection, cough, respiratory tract congestion, wheezing
• EENT: Throat tightness, nasal congestion, hypoacousia, tinnitus, sinusitis, pharyngitis, nasal congestion
• MUSC: Myalgia, back pain, muscle spasm
• GU: Nephrotic syndrome
• METABOLIC: Increased blood creatinine
• DERM: Flushing, pruritus, pallor, urticaria, rash, excoriation, contusion
• MISC: Anaphylaxis, hypersensitivity reactions, chills, pyrexia, feeling hot or cold, fatigue, pain in extremity, back pain, face edema, pain, fungal infection, viral infection, localized infection, procedural pain, burning sensation

• Recombinant human alpha-galactosidase A enzyme; provides an exogenous source of alpha-galactosidase A that catalyzes the hydrolysis of glycosphingolipids, including GL-3
• Metabolism: Unknown
• Excretion: Unknown
• Half-life: 45-102 minutes

US Trade Name(s)

• Fabrazyme

US Availability

Fabrazyme

• PWDR for INJ: 5 mg/vial
• PWDR for INJ: 35 mg/vial

Canadian Trade Name(s)

• Fabrazyme

Canadian Availability

Fabrazyme

• PWDR for INJ: 5 mg/vial
• PWDR for INJ: 35 mg/vial

UK Trade Name(s)

• Fabrazyme

UK Availability

Fabrazyme

• PWDR for INJ: 5 mg/vial
• PWDR for INJ: 35 mg/vial

Australian Trade Name(s)

• Fabrazyme

Australian Availability

Fabrazyme

• PWDR for INJ: 5 mg/vial
• PWDR for INJ: 35 mg/vial

[Outline]

Endocrine/Metabolic

Metabolic Enzymes