Fabrazyme (agalsidase beta)
Metabolic Disease Enzyme Replacement, Fabry's Disease Intravenous Vial (Ea)
Last reviewed · Source: First Databank (FDB) drug data
Overview
Agalsidase beta is a biosynthetic (recombinant DNA origin) form of human alpha-galactosidase, an enzyme that metabolizes glycosphingolipids, including globotriaosylceramide (GL-3).
Agalsidase beta is a biosynthetic (recombinant DNA origin) form of human alpha-galactosidase (alpha-galactosidase A, also known as ceramide trihexosidase); this enzyme catalyzes the hydrolysis of GL-3 and other alpha-galactyl-terminated neutral glycosphingolipids, such as galabiosylceramide and blood group B substances to ceramide dihexoside and galactose. Agalsidase beta is prepared from cultures of genetically modified mammalian (Chinese hamster ovary) cells using recombinant DNA technology. In patients with Fabry's disease, agalsidase beta provides an exogenous source of alpha-galactosidase.
Agalsidase beta is internalized and transported into lysosomes where it exerts enzymatic activity and reduces accumulated GL-3.
Agalsidase beta exhibits nonlinear pharmacokinetics following IV infusion of various weight-based doses in adults. The AUC and maximum plasma concentration increased greater than dose proportional with increasing doses.
USES: This medication is used to treat a certain inherited disorder (Fabry disease) that causes buildup of a certain fat substance (GL-3) in some parts of your body. Worsening of this disorder can lead to kidney disease, heart attack, or stroke. Agalsidase beta works by replacing an enzyme that your body lacks, which helps break down the fat substance (GL-3) in your body.
This can help prevent symptoms such as pain (especially in the hands and feet), dark red spots on the skin, a decreased ability to sweat, cloudiness of the front part of the eye, stomach/intestinal problems, ringing in the ears (tinnitus), and hearing loss.
Generic name: agalsidase beta
Pronunciation: (a-GAL-si-dase BAY-ta)
Drug class: Metabolic Disease Enzyme Replacement, Fabry's Disease
Therapeutic class: Metabolic Disease Enzyme Replacement Agents
Reference product & biosimilars
No reference, biosimilar or interchangeable associated with this drug.
Today’s intelligence on Fabrazyme
Indications
The following indications are listed for Fabrazyme:
- Fabry Disease
Also known as: Alpha-Galactosidase A Deficiency, Angiokeratoma Corporis Diffusum, Angiokeratoma Corporis Diffusum Universale, Ceramide Trihexosidase Deficiency, Diffuse Angiokeratoma, Fabry's Disease, Glycolipid Lipidosis.
Dosing
No dosing information is available for this tab.
Agalsidase beta is available as 5 mg and 35 mg single-dose vials. Agalsidase beta is administered as an IV infusion following reconstitution and dilution. Agalsidase beta should be administered using an in-line low protein binding 0.2-(upsilon)m filter and should not be infused in the same IV line as other products.
| Drug label | Dosing type | Instructions |
|---|---|---|
| Fabrazyme 35 Mg Vial | Maintenance | Infuse 1 mg/kg by intravenous route every 2 weeks. |
| Fabrazyme 5 Mg Vial | Maintenance | Infuse 1 mg/kg by intravenous route every 2 weeks. |
No dosing information is available for this tab.
Drug interactions
No formal drug interaction studies have been performed to date. However, some clinicians state that biosynthetic forms of alpha-galactosidase should not be administered concurrently with chloroquine, amiodarone, monobenzone, or gentamicin because of a theoretical risk of inhibited intracellular alpha-galactosidase activity.
Contraindicated Drug Combination: This drug combination is contraindicated and generally should not be dispensed or administered to the same patient.
Agalsidase/Amiodarone
SEVERITY LEVEL: 1-Contraindicated Drug Combination: This drug combination is contraindicated and generally should not be dispensed or administered to the same patient.
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.
Agalsidase/Chloroquine
SEVERITY LEVEL: 1-Contraindicated Drug Combination: This drug combination is contraindicated and generally should not be dispensed or administered to the same patient.
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.
Agalsidase/Gentamicin
SEVERITY LEVEL: 1-Contraindicated Drug Combination: This drug combination is contraindicated and generally should not be dispensed or administered to the same patient.
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.
Agalsidase/Monobenzone (Benoquin)
SEVERITY LEVEL: 1-Contraindicated Drug Combination: This drug combination is contraindicated and generally should not be dispensed or administered to the same patient.
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).
There are no severe interactions for this drug.
There are no moderate interactions for this drug.
Contraindications
*None.
There are no contraindication contraindications for this drug.
There are no severe contraindications for this drug.
- Chronic heart failure
Adverse reactions
The most common adverse reactions (>=20%) with agalsidase beta include upper respiratory tract infection, chills, pyrexia, headache, cough, paresthesia, fatigue, peripheral edema, dizziness, and rash.
More Frequent
- Chest pain
- Dyspnea
- Hypertension
- Throat constriction
Less Frequent
- Cardiac arrest
- Cardiac arrhythmia
- Cardiomegaly
- Fungal infection
- Hearing loss
- Low cardiac output
- Lower respiratory infection
- Nephrotic syndrome
- Viral infection
Rare or Very Rare
- Acute myocardial infarction
- Anaphylaxis
- Angioedema
- Bronchospastic pulmonary disease
- Cerebrovascular accident
- Heart failure
- Hypersensitivity angiitis
- Hypersensitivity drug reaction
- Lymphadenopathy
- Pneumonia
- Renal failure
- Sepsis
More 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
Less Frequent
- 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 or Very Rare
- Arthralgia
- Erythema
- General weakness
- Hyperhidrosis
- Laryngitis
- Palpitations
- Rhinorrhea
- Sinusitis
- Symptoms of anxiety
Precautions
No enhanced messaging available.
Safety and efficacy of agalsidase beta have been established in pediatric patients based on results from adequate and well-controlled studies in adults, a single-arm, open-label study in 16 pediatric patients (8 to 16 years of age) with Fabry disease, and additional data in 24 pediatric patients (2 to 7 years of age) with the condition. The overall safety profile of agalsidase beta was similar between adults and pediatric patients.
Clinical data from a Fabry Disease registry pregnancy sub-study, postmarketing case reports, and case series involving the administration of agalsidase beta during pregnancy did not reveal a drug-associated risk of major birth defects, miscarriage, or other adverse maternal or fetal outcomes. In the Fabry Disease registry substudy, 33 pregnancies exposed to agalsidase beta prior to or during pregnancy had a known outcome; 5 exposures occurred during the first trimester.
No evidence of effects on embryofetal development were seen in rats at doses up to 68 times the human dose.
Agalsidase beta has been detected in human milk in small amounts; however, data are insufficient to determine the effects of agalsidase beta on a breastfed infant or milk production. The developmental and health benefits of breastfeeding should be considered along with the mother's clinical need for the drug and any potential adverse effects of the drug on the breastfed child or from the underlying maternal condition.
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.
ICD codes
| Code | Type | Description |
|---|---|---|
| Fabry disease | ||
| E75.21 | ICD10CM | Fabry (-anderson) disease |
ICD code information from First Databank (FDB).
Formulary status
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Last reviewed · Source: First Databank (FDB) drug data
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