agalsidase beta

agalsidase beta,

a miscellaneous agent used to treat Fabry disease.

contraindications Known hypersensitivity to this drug prohibits its use.

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agalsidase beta

Fabrazyme, Fibrazyme

Pharmacologic class: Homodimeric glycoprotein

Therapeutic class: Recombinant human alpha-galactosidase enzyme

Pregnancy risk category B

Action

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

Availability

Powder for reconstitution: 37 mg (5 mg/ml)

⊘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.

Contraindications

None

Precautions

Use cautiously in:
• cardiac dysfunction
• pregnant or breastfeeding patients
• children.

Administration

• 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.

Route	Onset	Peak	Duration
I.V.	End of infusion	90 min	Up to 5 hr

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)

Interactions

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.