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Fabrazyme

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Overview

What is Fabrazyme?

Fabrazyme (agalsidase beta) is a recombinant human α-galactosidase A enzyme with the same amino acid sequence as the native enzyme. Purified agalsidase beta is a homodimeric glycoprotein with a molecular weight of approximately 100 kD. The mature protein is comprised of two subunits of 398 amino acids (approximately 51 kD), each of which contains three N-linked glycosylation sites. α‑galactosidase A catalyzes the hydrolysis of globotriaosylceramide (GL-3) and other α‑galactyl-terminated neutral glycosphingolipids, such as galabiosylceramide and blood group B substances to ceramide dihexoside and galactose. The specific activity of Fabrazyme is approximately 70 U/mg (one unit is defined as the amount of activity that results in the hydrolysis of 1 µmole of a synthetic substrate, p-nitrophenyl-α-D-galactopyranoside, per minute under the assay conditions).

Fabrazyme is produced by recombinant DNA technology in a Chinese Hamster Ovary mammalian cell expression system.

Fabrazyme is intended for intravenous infusion. It is supplied as a sterile, nonpyrogenic, white to off-white, lyophilized cake or powder for reconstitution with Sterile Water for Injection, USP. Each 35 mg vial contains 37 mg of agalsidase beta, as well as 222 mg mannitol, 20.4 mg sodium phosphate monobasic monohydrate, and 59.2 mg sodium phosphate dibasic heptahydrate. Following reconstitution as directed, 35 mg of agalsidase beta (7 mL) may be extracted from each 35 mg vial.

Each 5 mg vial contains 5.5 mg of agalsidase beta, as well as 33.0 mg mannitol, 3.0 mg sodium phosphate monobasic monohydrate, and 8.8 mg sodium phosphate dibasic heptahydrate. Following reconstitution as directed, 5 mg of agalsidase beta (1 mL) may be extracted from each 5 mg vial.



What does Fabrazyme look like?



What are the available doses of Fabrazyme?

Fabrazyme is supplied as a sterile, nonpyrogenic, white to off-white, lyophilized cake or powder for reconstitution with Sterile Water for Injection, USP to yield a concentration of 5 mg/mL; and then further diluted with 0.9% Sodium Chloride Injection, USP for intravenous infusion.

Single-use vials are available in 35 mg and 5 mg dosages.

What should I talk to my health care provider before I take Fabrazyme?

How should I use Fabrazyme?

Fabrazyme(agalsidase beta) is indicated for use in patients with Fabry disease.  Fabrazyme reduces globotriaosylceramide (GL‑3) deposition in capillary endothelium of the kidney and certain other cell types.

The recommended dosage of Fabrazyme is 1 mg/kg body weight infused every two weeks as an intravenous (IV) infusion.  Patients should receive antipyretics prior to infusion

The initial IV infusion rate should be no more than 0.25 mg/min (15 mg/hr).  The infusion rate may be slowed in the event of infusion reactions.  After patient tolerance to the infusion is well established, the infusion rate may be increased in increments of 0.05 to 0.08 mg/min (increments of 3 to 5 mg/hr) with each subsequent infusion.  For patients weighing < 30 kg, the maximum infusion rate should remain at 0.25 mg/min (15 mg/hr).  For patients weighing ≥ 30 kg, the administration duration should not be less than 1.5 hours (based on individual patient tolerability). 

Patients who have had a positive skin test to Fabrazyme or who have tested positive for anti-Fabrazyme IgE may be successfully re-challenged with Fabrazyme.  The initial re-challenge administration should be a low dose at a lower infusion rate, e.g., 1/2 the therapeutic dose (0.5 mg/kg) at 1/25 the initial standard recommended rate (0.01 mg/min).  Once a patient tolerates the infusion, the dose may be increased to reach the approved dose of 1 mg/kg and the infusion rate may be increased by slowly titrating upwards (doubled every 30 minutes up to a maximum rate of 0.25 mg/min), as tolerated.


What interacts with Fabrazyme?

Sorry No Records found


What are the warnings of Fabrazyme?

Sorry No Records found


What are the precautions of Fabrazyme?

Sorry No Records found


What are the side effects of Fabrazyme?

Sorry No records found


What should I look out for while using Fabrazyme?

None.


What might happen if I take too much Fabrazyme?

There have been no reports of overdose with Fabrazyme. In clinical trials, patients received doses up to 3 mg/kg body weight.  The adverse reactions experienced by patients who received treatment with 3 mg/kg were similar to the adverse reactions experienced by patients who received treatment with 1 mg/kg.


How should I store and handle Fabrazyme?

Store at 20°-25°C (68°-77°F) [see USP Controlled Room Temperature].Dispense in a tight, light-resistant container as defined in the USP/NF with a child-resistant closure.A Schedule CIII Narcotic.Store at 20°-25°C (68°-77°F) [see USP Controlled Room Temperature].Dispense in a tight, light-resistant container as defined in the USP/NF with a child-resistant closure.A Schedule CIII Narcotic.Store at 20°-25°C (68°-77°F) [see USP Controlled Room Temperature].Dispense in a tight, light-resistant container as defined in the USP/NF with a child-resistant closure.A Schedule CIII Narcotic. Fabrazyme is supplied as a sterile, nonpyrogenic, white to off-white lyophilized cake or powder. Fabrazyme 35 mg vials are supplied in single-use, clear Type I glass 20 mL (cc) vials. The closure consists of a siliconized butyl stopper and an aluminum seal with a plastic purple flip-off cap. Fabrazyme 5 mg vials are supplied in single-use, clear Type I glass 5 mL (cc) vials. The closure consists of a siliconized butyl stopper and an aluminum seal with a plastic gray flip-off cap. 35 mg vial: NDC 58468-0040-15 mg vial: NDC 58468-0041-1Refrigerate vials of Fabrazyme at 2° to 8°C (36° to 46°F).  DO NOT USE Fabrazyme after the expiration date on the vial.  Reconstituted and diluted solutions of Fabrazyme should be used immediately.  This product contains no preservatives.  If immediate use is not possible, the reconstituted and diluted solution may be stored for up to 24 hours at 2° to 8°C (36° to 46°F). Fabrazyme is supplied as a sterile, nonpyrogenic, white to off-white lyophilized cake or powder. Fabrazyme 35 mg vials are supplied in single-use, clear Type I glass 20 mL (cc) vials. The closure consists of a siliconized butyl stopper and an aluminum seal with a plastic purple flip-off cap. Fabrazyme 5 mg vials are supplied in single-use, clear Type I glass 5 mL (cc) vials. The closure consists of a siliconized butyl stopper and an aluminum seal with a plastic gray flip-off cap. 35 mg vial: NDC 58468-0040-15 mg vial: NDC 58468-0041-1Refrigerate vials of Fabrazyme at 2° to 8°C (36° to 46°F).  DO NOT USE Fabrazyme after the expiration date on the vial.  Reconstituted and diluted solutions of Fabrazyme should be used immediately.  This product contains no preservatives.  If immediate use is not possible, the reconstituted and diluted solution may be stored for up to 24 hours at 2° to 8°C (36° to 46°F). Fabrazyme is supplied as a sterile, nonpyrogenic, white to off-white lyophilized cake or powder. Fabrazyme 35 mg vials are supplied in single-use, clear Type I glass 20 mL (cc) vials. The closure consists of a siliconized butyl stopper and an aluminum seal with a plastic purple flip-off cap. Fabrazyme 5 mg vials are supplied in single-use, clear Type I glass 5 mL (cc) vials. The closure consists of a siliconized butyl stopper and an aluminum seal with a plastic gray flip-off cap. 35 mg vial: NDC 58468-0040-15 mg vial: NDC 58468-0041-1Refrigerate vials of Fabrazyme at 2° to 8°C (36° to 46°F).  DO NOT USE Fabrazyme after the expiration date on the vial.  Reconstituted and diluted solutions of Fabrazyme should be used immediately.  This product contains no preservatives.  If immediate use is not possible, the reconstituted and diluted solution may be stored for up to 24 hours at 2° to 8°C (36° to 46°F). Fabrazyme is supplied as a sterile, nonpyrogenic, white to off-white lyophilized cake or powder. Fabrazyme 35 mg vials are supplied in single-use, clear Type I glass 20 mL (cc) vials. The closure consists of a siliconized butyl stopper and an aluminum seal with a plastic purple flip-off cap. Fabrazyme 5 mg vials are supplied in single-use, clear Type I glass 5 mL (cc) vials. The closure consists of a siliconized butyl stopper and an aluminum seal with a plastic gray flip-off cap. 35 mg vial: NDC 58468-0040-15 mg vial: NDC 58468-0041-1Refrigerate vials of Fabrazyme at 2° to 8°C (36° to 46°F).  DO NOT USE Fabrazyme after the expiration date on the vial.  Reconstituted and diluted solutions of Fabrazyme should be used immediately.  This product contains no preservatives.  If immediate use is not possible, the reconstituted and diluted solution may be stored for up to 24 hours at 2° to 8°C (36° to 46°F).


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Clinical Information

Chemical Structure

No Image found
Clinical Pharmacology

Non-Clinical Toxicology
None.

Use of daunorubicin in a patient who has previously received doxorubicin increases the risk of cardiotoxicity. Daunorubicin hydrochloride should not be used in patients who have previously received the recommended maximum cumulative doses of doxorubicin or daunorubicin hydrochloride. Cyclophosphamide used concurrently with daunorubicin hydrochloride may also result in increased cardiotoxicity.

Dosage reduction of daunorubicin hydrochloride may be required when used concurrently with other myelosuppressive agents.

Hepatotoxic medications, such as high-dose methotrexate, may impair liver function and increase the risk of toxicity.

Life-threatening anaphylactic and severe allergic reactions have been observed in patients during Fabrazyme infusions. Reactions have included localized angioedema (including swelling of the face, mouth, and throat), bronchospasm, hypotension, generalized urticaria, dysphagia, rash, dyspnea, flushing, chest discomfort, pruritus, and nasal congestion. Interventions have included cardiopulmonary resuscitation, oxygen supplementation, IV fluids, hospitalization, and treatment with inhaled beta-adrenergic agonists, epinephrine, and IV corticosteroids.

In clinical trials and postmarketing safety experience with Fabrazyme, approximately 1% of patients developed anaphylactic or severe allergic reactions during Fabrazyme infusion. 

If anaphylactic or severe allergic reactions occur, immediately discontinue the administration of Fabrazyme and initiate necessary emergency treatment. Because of the potential for severe allergic reactions, appropriate medical support measures should be readily available when Fabrazyme is administered.

The risks and benefits of re-administering Fabrazyme following an anaphylactic or severe allergic reaction should be considered. Extreme care should be exercised, with appropriate medical support measures readily available, if the decision is made to re-administer the product .

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Reference

This information is obtained from the National Institute of Health's Standard Packaging Label drug database.
"https://dailymed.nlm.nih.gov/dailymed/"

While we update our database periodically, we cannot guarantee it is always updated to the latest version.

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Clonazepam Description Each single-scored tablet, for oral administration, contains 0.5 mg, 1 mg, or 2 mg Clonazepam, USP, a benzodiazepine. Each tablet also contains corn starch, lactose monohydrate, magnesium stearate, microcrystalline cellulose, and povidone. Clonazepam tablets USP 0.5 mg contain Yellow D&C No. 10 Aluminum Lake. Clonazepam tablets USP 1 mg contain Yellow D&C No. 10 Aluminum Lake, as well as FD&C Blue No. 1 Aluminum Lake. Chemically, Clonazepam, USP is 5-(o-chlorophenyl)-1,3-dihydro-7-nitro-2H-1,4-benzodiazepin-2-one. It is a light yellow crystalline powder. It has the following structural formula: C15H10ClN3O3 M.W. 315.72
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Interactions

Interactions

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