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Drug overview for FABRAZYME (agalsidase beta):
Generic name: AGALSIDASE BETA (a-GAL-si-dase BAY-ta)
Drug class: Metabolic Disease Enzyme Replacement, Fabry's Disease
Therapeutic class: Metabolic Disease Enzyme Replacement Agents
Agalsidase beta is a biosynthetic (recombinant DNA origin) form of human alpha-galactosidase, an enzyme that metabolizes glycosphingolipids, including globotriaosylceramide (GL-3).
No enhanced Uses information available for this drug.
Generic name: AGALSIDASE BETA (a-GAL-si-dase BAY-ta)
Drug class: Metabolic Disease Enzyme Replacement, Fabry's Disease
Therapeutic class: Metabolic Disease Enzyme Replacement Agents
Agalsidase beta is a biosynthetic (recombinant DNA origin) form of human alpha-galactosidase, an enzyme that metabolizes glycosphingolipids, including globotriaosylceramide (GL-3).
No enhanced Uses information available for this drug.
DRUG IMAGES
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The following indications for FABRAZYME (agalsidase beta) have been approved by the FDA:
Indications:
Fabry disease
Professional Synonyms:
Alpha-galactosidase A deficiency
Angiokeratoma corporis diffusum universale
Angiokeratoma corporis diffusum
Ceramide trihexosidase deficiency
Diffuse angiokeratoma
Fabry's disease
Glycolipid lipidosis
Indications:
Fabry disease
Professional Synonyms:
Alpha-galactosidase A deficiency
Angiokeratoma corporis diffusum universale
Angiokeratoma corporis diffusum
Ceramide trihexosidase deficiency
Diffuse angiokeratoma
Fabry's disease
Glycolipid lipidosis
The following dosing information is available for FABRAZYME (agalsidase beta):
Dosage of agalsidase beta is expressed in mg. The specific activity of agalsidase beta is approximately 70 units/mg, with 1 unit defined as the amount of activity that results in hydrolysis of 1 mcmol of a synthetic substrate, p-nitrophenyl-alpha-d-galactopyranoside, per minute under the assay conditions.
For the management of Fabry's disease in adults, the recommended dosage of agalsidase beta is 1 mg/kg given as an IV infusion once every 2 weeks.
For the management of Fabry's disease in adults, the recommended dosage of agalsidase beta is 1 mg/kg given as an IV infusion once every 2 weeks.
No enhanced Administration information available for this drug.
| DRUG LABEL | DOSING TYPE | DOSING INSTRUCTIONS |
|---|---|---|
| FABRAZYME 35 MG VIAL | Maintenance | Adults infuse 1 mg/kg by intravenous route every 2 weeks |
| FABRAZYME 5 MG VIAL | Maintenance | Adults infuse 1 mg/kg by intravenous route every 2 weeks |
No generic dosing information available.
The following drug interaction information is available for FABRAZYME (agalsidase beta):
There are 0 contraindications.
There are 4 severe interactions.
These drug interactions can produce serious consequences in most patients. Actions required for severe interactions include, but are not limited to, discontinuing one or both agents, adjusting dosage, altering administration scheduling, and providing additional patient monitoring. Review the full interaction monograph for more information.
| Drug Interaction | Drug Names |
|---|---|
| Agalsidase/Chloroquine SEVERITY LEVEL: 2-Severe Interaction: Action is required to reduce the risk of severe adverse interaction. MECHANISM OF ACTION: Chloroquine may inhibit the intracellular activity of alpha-galactosidase.(1,2) CLINICAL EFFECTS: Concurrent use of agalsidase with chloroquine may result in decreased effectiveness of agalsidase.(1,2) PREDISPOSING FACTORS: None determined. PATIENT MANAGEMENT: The manufacturer of agalsidase alpha in Canada(1) and the manufacturer of agalsidase beta in the United Kingdom(2) state that agalsidase should not be administered with chloroquine. DISCUSSION: Because of a theoretical risk of inhibited intracellular activity of alpha-galactosidase activity, the manufacturer of agalsidase alpha in Canada(1) and the manufacturer of agalsidase beta in the United Kingdom(2) state that agalsidase should not be administered with chloroquine. |
CHLOROQUINE PHOSPHATE |
| Agalsidase/Amiodarone SEVERITY LEVEL: 2-Severe Interaction: Action is required to reduce the risk of severe adverse interaction. MECHANISM OF ACTION: Amiodarone may inhibit the intracellular activity of alpha-galactosidase.(1,2) CLINICAL EFFECTS: Concurrent use of agalsidase with amiodarone may result in decreased effectiveness of agalsidase.(1,2) PREDISPOSING FACTORS: None determined. PATIENT MANAGEMENT: The manufacturer of agalsidase alpha in Canada(1) and the manufacturer of agalsidase beta in the United Kingdom(2) state that agalsidase should not be administered with amiodarone. DISCUSSION: Because of a theoretical risk of inhibited intracellular activity of alpha-galactosidase activity, the manufacturer of agalsidase alpha in Canada(1) and the manufacturer of agalsidase beta in the United Kingdom(2) state that agalsidase should not be administered with amiodarone. |
AMIODARONE HCL, AMIODARONE HCL-D5W, NEXTERONE, PACERONE |
| Agalsidase/Monobenzone (Benoquin) SEVERITY LEVEL: 2-Severe Interaction: Action is required to reduce the risk of severe adverse interaction. MECHANISM OF ACTION: Monobenzone (benoquin) may inhibit the intracellular activity of alpha-galactosidase.(1,2) CLINICAL EFFECTS: Concurrent use of agalsidase with monobenzone (benoquin) may result in decreased effectiveness of agalsidase.(1,2) PREDISPOSING FACTORS: None determined. PATIENT MANAGEMENT: The manufacturer of agalsidase alpha in Canada(1) and the manufacturer of agalsidase beta in the United Kingdom(2) state that agalsidase should not be administered with monobenzone (benoquin). DISCUSSION: Because of a theoretical risk of inhibited intracellular activity of alpha-galactosidase activity, the manufacturer of agalsidase alpha in Canada(1) and the manufacturer of agalsidase beta in the United Kingdom(2) state that agalsidase should not be administered with monobenzone (benoquin). |
MONOBENZONE |
| Agalsidase/Gentamicin SEVERITY LEVEL: 2-Severe Interaction: Action is required to reduce the risk of severe adverse interaction. MECHANISM OF ACTION: Gentamicin may inhibit the intracellular activity of alpha-galactosidase.(1,2) CLINICAL EFFECTS: Concurrent use of agalsidase with gentamicin may result in decreased effectiveness of agalsidase.(1,2) PREDISPOSING FACTORS: None determined. PATIENT MANAGEMENT: The manufacturer of agalsidase alpha in Canada(1) and the manufacturer of agalsidase beta in the United Kingdom(2) state that agalsidase should not be administered with gentamicin. DISCUSSION: Because of a theoretical risk of inhibited intracellular activity of alpha-galactosidase activity, the manufacturer of agalsidase alpha in Canada(1) and the manufacturer of agalsidase beta in the United Kingdom(2) state that agalsidase should not be administered with gentamicin. |
GENTAMICIN SULFATE, GENTAMICIN SULFATE IN NS |
There are 0 moderate interactions.
The following contraindication information is available for FABRAZYME (agalsidase beta):
Drug contraindication overview.
No known contraindications.
No known contraindications.
There are 0 contraindications.
There are 0 severe contraindications.
There are 1 moderate contraindications.
Clinically significant contraindication, where the condition can be managed or treated before the drug may be given safely.
| Moderate List |
|---|
| Chronic heart failure |
The following adverse reaction information is available for FABRAZYME (agalsidase beta):
Adverse reaction overview.
The most common and severe adverse effects occurring in patients receiving agalsidase beta are infusion reactions, including tachycardia, hypertension, throat tightness, chest pain/tightness, dyspnea, fever, chills/rigors, abdominal pain, pruritus, urticaria, nausea, vomiting, lip or ear edema, and rash. Transient mild to moderate infusion reactions were reported in approximately 60% of patients requiring the drug during the controlled and/or open-label studies.
The most common and severe adverse effects occurring in patients receiving agalsidase beta are infusion reactions, including tachycardia, hypertension, throat tightness, chest pain/tightness, dyspnea, fever, chills/rigors, abdominal pain, pruritus, urticaria, nausea, vomiting, lip or ear edema, and rash. Transient mild to moderate infusion reactions were reported in approximately 60% of patients requiring the drug during the controlled and/or open-label studies.
There are 25 severe adverse reactions.
| More Frequent | Less Frequent |
|---|---|
|
Chest pain Dyspnea Hypertension Throat constriction |
Cardiac arrest Cardiac arrhythmia Cardiomegaly Fungal infection Hearing loss Low cardiac output Lower respiratory infection Nephrotic syndrome Viral infection |
| Rare/Very Rare |
|---|
|
Acute myocardial infarction Anaphylaxis Angioedema Bronchospastic pulmonary disease Cerebrovascular accident Heart failure Hypersensitivity angiitis Hypersensitivity drug reaction Lymphadenopathy Pneumonia Renal failure Sepsis |
There are 46 less severe adverse reactions.
| More Frequent | Less Frequent |
|---|---|
|
Chest tightness Chills Dizziness Fatigue Fever Headache disorder Myalgia Nausea Paresthesia Peripheral edema Pharyngitis Pruritus of skin Rhinitis Skin rash Tachycardia Upper respiratory infection Vomiting |
Acute abdominal pain Ataxia Bradycardia Bronchitis Chest discomfort Depression Drowsy Dyspepsia Facial edema Flushing Hypotension Muscle spasm Pain Pallor Sensation of cold Stinging of skin Tinnitus Toothache Urticaria Vertigo |
| Rare/Very Rare |
|---|
|
Arthralgia Erythema General weakness Hyperhidrosis Laryngitis Palpitations Rhinorrhea Sinusitis Symptoms of anxiety |
The following precautions are available for FABRAZYME (agalsidase beta):
Safety and efficacy of agalsidase beta have not been established in children younger than 16 years of age; however, many experts recommend initiation of enzyme replacement therapy in children with clinical manifestations of Fabry's disease based on experience with other enzyme replacement infusions in children with similar genetic diseases.
Contraindicated
Severe Precaution
Management or Monitoring Precaution
Contraindicated
| None |
Severe Precaution
| None |
Management or Monitoring Precaution
| None |
Category B. (See Users Guide.) Also see Fabry's Disease Registry under Uses: Fabry's Disease.
It is not known whether agalsidase beta is distributed into milk. Because of the potential for adverse reactions to agalsidase beta in nursing infants, the drug should be used with caution in nursing women. (See Fabry's Disease Registry under Uses: Fabry's Disease.)
Clinical trials of agalsidase beta did not include sufficient numbers of patients 65 years of age and older to determine whether they respond differently than younger adults.
The following prioritized warning is available for FABRAZYME (agalsidase beta):
WARNING: Agalsidase beta may cause serious (sometimes fatal) allergic reactions. Get medical help right away if you have any symptoms of a serious allergic reaction such as rash, itching/swelling (especially of the face/tongue/throat), severe dizziness, or trouble breathing.
WARNING: Agalsidase beta may cause serious (sometimes fatal) allergic reactions. Get medical help right away if you have any symptoms of a serious allergic reaction such as rash, itching/swelling (especially of the face/tongue/throat), severe dizziness, or trouble breathing.
The following icd codes are available for FABRAZYME (agalsidase beta)'s list of indications:
| Fabry disease | |
| E75.21 | Fabry (-anderson) disease |
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