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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
• 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.
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
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
• 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.
☞ 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.
agalsidase beta(a-gal-si-dase) ,
ClassificationTherapeutic: replacement enzyme
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Adverse Reactions/Side Effects
Central nervous system
- edema (most frequent)
- hypotension (most frequent)
- testicular pain
- skeletal pain (most frequent)
- infusion reactions
- rigors (most frequent)
Drug-Drug interactionNone noted.
- 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 DiagnosesDeficient knowledge, related to medication regimen (Patient/Family Teaching)
- 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.
- 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.
- Reduction of globotriaosylceramide deposition in capillary endothelium of the kidney and certain other cell types.