agalsidase beta

Fabrazyme, Fibrazyme

Pharmacologic class: Homodimeric glycoprotein

Therapeutic class: Recombinant human alpha-galactosidase enzyme

Pregnancy risk category B


Provides exogenous source of alpha-galactosidase A (which is deficient in Fabry disease) and reduces deposits of globotriaosylceramide in kidney and other body tissues


Powder for reconstitution: for injection, lyophilized: 5 mg, 35 mg

Indications and dosages

Fabry disease

Adults: 1 mg/kg I.V. q 2 weeks. Infuse no faster than 0.25 mg/minute; if tolerated, increase rate by 0.05 to 0.08 mg/minute in subsequent infusions.




Use cautiously in:

• advanced Fabry disease, cardiac dysfunction

• pregnant or breastfeeding patients

• children.


• Premedicate with antipyretics, as prescribed.

• To reconstitute, slowly inject 7.2 ml of sterile water for injection into vial; then roll and tilt vial gently to mix drug.

• Don't shake drug, and don't use filter needles.

• Dilute reconstituted solution with normal saline injection to a final volume of 500 ml.

• Infuse through separate I.V. line; don't mix with other drugs.

Adverse reactions

CNS: anxiety, depression, dizziness, paresthesias

CV: dependent edema, chest pain, cardiomegaly

EENT: rhinitis, sinusitis, laryngitis, pharyngitis

GI: nausea, dyspepsia

GU: testicular pain

Musculoskeletal: arthrosis, bone pain

Respiratory: bronchitis, bronchospasm

Skin: pallor

Other: pain, allergic reactions, infusion reactions (hypertension, chest tightness, dyspnea, fever, rigors, hypotension, abdominal pain, pruritus, myalgia, headache, urticaria)


Drug-drug. Amiodarone, chloroquine, gentamicin, monobenzone: inhibition of intracellular agalsidase activity

Patient monitoring

• Watch closely for signs and symptoms of allergic or infusion reaction.

• Monitor vital signs and fluid intake and output. Stay alert for dependent edema, blood pressure changes, and chest pain.

• Measure temperature. Watch for signs and symptoms of infection (particularly EENT and respiratory infections).

• Evaluate patient's mood. Report significant anxiety or depression.

Patient teaching

Teach patient to recognize and immediately report signs and symptoms of allergic or infusion reaction.

• Caution patient to avoid driving and other hazardous activities until he knows how drug affects mood, balance, and blood pressure.

• Advise patient to report signs and symptoms of infection (particularly EENT and respiratory infections).

• Inform patient that drug can cause depression and anxiety. Instruct him to notify prescriber if these effects occur.

• As appropriate, review all other significant and life-threatening adverse reactions and interactions, especially those related to the drugs mentioned above.

McGraw-Hill Nurse's Drug Handbook, 7th Ed. Copyright © 2013 by The McGraw-Hill Companies, Inc. All rights reserved

agalsidase beta

(a-gal-si-dase) ,


(trade name)


Therapeutic: replacement enzyme
Pharmacologic: enzymes
Pregnancy Category: B


Fabry disease.


Replaces a deficient enzyme.

Therapeutic effects

Prevents damage from accumulation of globotriaosylceramide in capillary endothelium.


Absorption: IV administration results in complete bioavailability.
Distribution: Widely distributed.
Metabolism and Excretion: Unknown.
Half-life: 45–102 min.

Time/action profile

IVunknownend of infusion2 wk


Contraindicated in: None noted.
Use Cautiously in: Compromised cardiac function (may have ↑ risk of infusion reactions); Obstetric / Lactation / Pediatric: Safety not established.

Adverse Reactions/Side Effects

Central nervous system

  • anxiety
  • depression
  • dizziness
  • headache


  • bronchospasm


  • edema (most frequent)
  • cardiomegaly
  • hypotension (most frequent)
  • hypertension


  • dyspepsia
  • nausea


  • testicular pain


  • pallor


  • skeletal pain (most frequent)
  • arthrosis


  • infusion reactions
  • rigors (most frequent)
  • fever


Drug-Drug interaction

None noted.


Intravenous (Adults) 1 mg/kg every 2 wk.


Lyophilized powder for injection: 5 mg/mL in 5– and 35–mL vials

Nursing implications

Nursing assessment

  • Assess for infusion reactions (fever, rigors, chest tightness, hypertension, hypotension, pruritus, myalgia, dyspnea, urticaria, abdominal pain, headache). Pre-treat patients with antipyretics. If an infusion reaction occurs, regardless of pre-treatment, decrease infusion rate, temporarily stop the infusion, and/or administer additional antipyretics, antihistamines, and/or oral corticosteroids. Infusion reactions decline in frequency with continued use; serious reactions may still occur with extended of therapy.
  • Monitor cardiac function during treatment in patients with previously compromised cardiac function.

Potential Nursing Diagnoses

Deficient knowledge, related to medication regimen (Patient/Family Teaching)


  • Intravenous Administration
  • Administer antipyretics prior to infusion.
  • Intermittent Infusion: Allow refrigerated agalsidase and diluent to reach room temperature prior to reconstitution (30 min). Determine number of 35 mg and 5 mg vials needed for dose. Reconstitute each vial by slowly injecting 7.2 mL and 1.1 mL of Sterile Water for Injection into each 35 mg vial and 5 mg vial, respectively. Inject down the inside wall of vial. Roll and tilt each vial gently. Solution is clear and colorless; do not administer solutions that are discolored or contain particulate matter. Diluent: Remove an equal volume of 0.9% NaCl from a 500 mL infusion bag, then dilute further with 0.9% NaCl for a final volume of 500 mL. Inject reconstituted agalsidase directly into 0.9% NaCl, do not inject into air space. Discard unused portion of vial. Gently invert bag to mix solution, avoid vigorous shaking or agitation. Diluted solution may be filtered through an in-line low protein-binding 0.2 micrometer filter during administration. Use reconstituted and diluted solutions immediately or within 24 hrs..
  • Rate: Initial infusion rate should not exceed 0.25 mg/min (15 mg/hr). Infusion rate may be slowed if infusion reaction occurs. After tolerance to rate is well established, infusion may be increased in increments of 0.05–0.08 mg/min (increments of 3–5 mg/hr)each subsequent infusion.
  • Y-Site Incompatibility: Do not infuse agalsidase in the same IV line with other products.

Patient/Family Teaching

  • Inform patients that a Registry has been established to better understand Fabry disease and to monitor the effects of agalsidase. Encourage patients to participate; participation is voluntary and may be long term. For information visit www.fabryregistry.com or call 800–745–4447.

Evaluation/Desired Outcomes

  • Reduction of globotriaosylceramide deposition in capillary endothelium of the kidney and certain other cell types.
Drug Guide, © 2015 Farlex and Partners
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References in periodicals archive ?
The BRIGHT study is a 12 month, open-label switchover study to assess the safety, efficacy and pharmacokinetics of pegunigalsidase alfa 2 mg/kg administered every 4 weeks in up to 30 Fabry patients previously treated with an enzyme replacement therapy: Fabrazyme or Replagal.
The Fabry Registry, Genzyme (617-591-5500), is studying the use in pregnancy of agalsidase beta (Fabrazyme) for Fabry disease.
Agalsidase beta (Fabrazyme, Genzyme Corporation, Cambridge, Massachusetts) is used at a dose of 1 mg/kg every 2 weeks.
Galaford is first orally administered drug competing with injectable enzyme replacement therapies (ERT), which includes the likes of Sanofi's (EPA: SAN) Fabrazyme (agalsidase beta) and Shire's (LON: SHP) Replagal (agalsidase alfa).
Of them, 23 were not treated with enzyme replacement therapy (ERT), while 21 had received ERT for at least 12 months with agalsidase-[beta] 1 mg/kg every fortnight (Fabrazyme, Genzyme Corp., Cambridge, MA, USA).
This is in contrast to drugs such as Fabrazyme (a synthetic enzyme that replaces a required enzyme that is deficient in those suffering from Fabry's Disease) or Synagis (which is used as a "natural antibody," dosed to pediatric patients to provide passive immune protection against respiratory syncitial virus (RSV) in cases where infection is progressing too rapidly to safely rely on the child's own antibody production).
* Since the approval of Fabrazyme and Replagal in the EU in 2001, no other drugs have been approved for the treatment of Fabry disease.
alfa (Fabrazyme) ** Genzyme/Sanofi Protein C (Xigris) Lonza for Eli Lilly Factor VIII (Kogenate-FS) ** Bayer Interferon beta (Rebif) ** Merck-Serono I-2 receptor mAb (Simulect) Novartis TNF mAb (Remicade) ** Janssen/J&J FSH (Gonal-F) Merck-Serono Galactosidase.
Two different recombinant a-galactosidase A preparations, algalsidase alfa (Replagal, Shire HGT) and agalsidase [beta] (Fabrazyme, Genzyme Corp), are approved for the treatment of Fabry disease.
As agents for enzyme replacement therapy (ERT), [alpha]-Gal[beta] (Fabrazyme) and [alpha]-Gala (Replagal) were approved in Europe in 2001 and in the USA in 2003.
biotech's manufacturing problems, which led to shortages of the Cerezyme and Fabrazyme medicines.