AGALSIDASE BETA

AGALSIDASE BETA
(a-gal'si-dase)
Fabrazyme
Classifications: enzyme replacement;
Therapeutic: enzyme replacement

Prototype: Pancrelipase
Pregnancy Category: B

Availability

35 mg/vial injection

Action

Fabry disease is caused by a deficiency of alpha-galactosidase A resulting in accumulation of glycosphingolipids in body tissues causing cardiomyopathy, renal failure, and CVA. Agalsidase beta provides an exogenous source of K-galactosidase A that catalyzes the breakdown of glycosphingolipids including GL-3.

Therapeutic Effect

Reduces globotriaosylceramide (GL-3) deposition in capillary endothelium of the kidney and certain other cell types.

Uses

Treatment of Fabry disease.

Contraindications

Safety and efficacy in children <16 y have not been established. Lactation.

Cautious Use

Hypersensitivity reaction to agalsidase beta or mannitol; compromised cardiac function, mild to severe hypertension; renal impairment; pregnancy (category B).

Route & Dosage

Fabry Disease
Adult: IV 1 mg/kg q2wk

Administration

Intravenous
  • Give antipyretics prior to infusion.

PREPARE: Infusion: Bring Fabrazyme vials and supplied sterile water for injection to room temperature prior to reconstitution. Reconstitute each 35 mg vial slowly injecting 7.2 mL of sterile water for injection down inside wall of vial. Roll and tilt vial gently to mix but do not shake. Reconstituted vial contains 5.0 mg/mL of clear, colorless solution. Do not use if there is particulate matter or if discolored. Must be further diluted in NS to a final total volume of 500 mL; prior to adding the volume of reconstituted agalisidase required for the dose, remove an equal volume of NS from the 500 mL infusion bag.  

ADMINISTER: Infusion: Initial rate should not exceed 0.25 mg/min (15 mg/h; give more slowly if infusion-associated reactions occur). After tolerance to infusion is established, may increase rate in increments of 0.05–0.08 mg/min (increments of 3 to 5 mg/h) for each subsequent infusion.  

INCOMPATIBILITIES Solution/additive: Do not infuse with other products.

  • Store refrigerated until needed. Vials are for single use. Discard any unused portion. Do NOT use after expiration date.

Adverse Effects (≥1%)

Body as a Whole: Fever, skeletal pain, pallor, rigors, temperature change sensation, ataxia, stroke. CNS: Dizziness, headache, paresthesia, anxiety, depression, vertigo. CV: Chest pain, cardiomegaly, hypertension, hypotension, dependent edema, bradycardia, heart failure, exacerbation of preexisting arrhythmias. GI: Dyspepsia, nausea, abdominal pain. Metabolic: Antibody development. Musculoskeletal: Arthrosis, skeletal pain. Respiratory: Bronchitis, bronchospasm, laryngitis, pharyngitis, rhinitis, sinusitis, dyspnea. Skin: Pruritus, urticaria. Special Senses: Hearing loss. Urogenital: Testicular pain, nephrotic syndrome.

Interactions

Drug: Coadministration with amiodarone, chloroquine, hydroxychloroquine, gentamicin is not recommended due to potential of decreased response to agalsidase beta therapy.

Pharmacokinetics

Metabolism: Degraded through peptide hydrolysis. Elimination: Renal elimination expected to be a minor pathway. Half-Life: 45–102 min.

Nursing Implications

Assessment & Drug Effects

  • During infusion, monitor for infusion-related reactions such as hypertension or hypotension, chest pain or chest tightness, dyspnea, fever and chills, headache, abdominal pain, pruritus and urticaria.
  • Slow infusion and notify physician immediately if infusion reaction occurs. Note that additional antipyretic and/or an antihistamine and oral steroid may reduce the symptoms.
  • Monitor cardiac status closely, especially with preexisting heart disease.

Patient & Family Education

  • Notify physician if you have experienced an unusual reaction to agalsidase beta, agalsidase alfa, mannitol, other drugs, foods, or preservatives.
  • Report any of the following to physician immediately: chest pain or chest tightness, rapid heartbeat, shortness of breath or difficulty breathing; depression; dizziness; skin rash, hives or itching; throat tightness; swelling of the face, lips, neck, ears, or extremities.
  • Do not drive or engage in other hazardous activities until reaction to drug is known.

Common adverse effects in italic, life-threatening effects underlined; generic names in bold; classifications in SMALL CAPS; Canadian drug name; Prototype drug

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