agalsidase beta

Absorption: IV administration results in complete bioavailability.

Distribution: Widely distributed.

Metabolism/Excretion: Unknown.

Half-Life: 45–102 min.

Time/Action Profile ⬆ ⬇

ROUTE	ONSET	PEAK	DURATION
IV	unknown	end of infusion	2 wk

Contraind./Precautions ⬆ ⬇

Contraindicated in:

None noted.

Use Cautiously in:

Compromised cardiac function (may have ↑ risk of infusion reactions);
OB: Use during pregnancy only if potential maternal benefit justifies potential fetal risk;
Lactation: Use while breastfeeding only if potential maternal benefit justifies potential risk to infant;
Pedi: Children <2 yr (safety and effectiveness not established).

Adv. Reactions/Side Effects ⬆ ⬇

CV: edema, hypotension, cardiomegaly, hypertension

Derm: pallor

GI: dyspepsia, nausea

GU: testicular pain

MS: skeletal pain, arthrosis

Neuro: anxiety, depression, dizziness, headache

Resp: bronchospasm

Misc: infusion reactions , rigors, fever, HYPERSENSITIVITY REACTIONS (INCLUDING ANAPHYLAXIS)

Interactions ⬆ ⬇

Drug-drug:

None reported.

Route/Dosage ⬆ ⬇

IV (Adults and Children ≥2 yr): 1 mg/kg every 2 wk.

Availability ⬆ ⬇

Lyophilized powder for injection: 5 mg/vial; 35 mg/vial

Assessment ⬆ ⬇

Assess for infusion reactions (fever, feeling hot or cold, dyspnea, nausea, flushing, headache, fatigue, pruritus, pain in extremity, hypertension, chest pain, throat tightness, abdominal pain, dizziness, tachycardia, nasal congestion, diarrhea, peripheral edema, myalgia, urticaria, bradycardia, and drowsiness). Pretreat patients with antipyretics. If an infusion reaction occurs, regardless of pretreatment, decrease infusion rate, temporarily stop the infusion, and 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 for signs and symptoms of hypersensitivity reactions (swelling of the face, mouth, and throat; bronchospasm; hypotension; generalized urticaria; dysphagia; rash; dyspnea; flushing; chest discomfort; pruritus; nasal congestion). If anaphylactic or severe hypersensitivity reactions occur, immediately discontinue infusion and start emergency treatment. Consider the risks and benefits of readministering following severe hypersensitivity reactions.
Monitor cardiac function during treatment in patients with previously compromised cardiac function.

Lab Test Considerations:

Testing for IgE antibodies are available for patients who experienced hypersensitivity reactions. There are no marketed tests for antibodies against Fabrazyme. If testing is warranted, contact Genzyme Corporation at 1-800-745-4447. Patients who have tested positive for Fabrazyme-specific IgE antibody have been rechallenged with Fabrazyme using a rechallenge protocol.

Implementation ⬆ ⬇

IV Administration:

Administer antihistamines, antipyretics, and/or corticosteroids prior to infusion, depending on individual status.
Administer in a health care setting with appropriate medical monitoring and supportive measures (cardiopulmonary resuscitation equipment) available during administration.
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. Reconstitution: 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 hr..
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 ⬆ ⬇

Explain purpose and side effects of agalsidase beta to patient.
Advise patient and caregivers to readPatient Information before starting therapy.
Advise patient to notify health care professional of all Rx or OTC medications, vitamins, or herbal products being taken and to consult health care professional before taking other medications.
Instruct patient to notify health care professional of any signs and symptoms of an infusion reaction.
Teach patient the signs and symptoms of an hypersensitivity reaction; if any occur, seek immediate medical care.
Advise patient to notify a health care professional if a dose is missed.
Inform patients that a registry has been established to better understand the variability and progression of Fabry disease and to monitor the long-term effects of Fabrazyme. Encourage patients to participate; participation is voluntary. For information, visit www.registrynxt.com or call 800-745-4447, ext. 15500.
Rep: Advise females of reproductive potential to notify health care professional if pregnancy is planned or suspected or if breastfeeding. Encourage pregnant women and women of reproductive potential to enroll in Fabry patient registry to monitor the effect of Fabrazyme on pregnant women and their offspring.

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Pronunciation ⬇

Classifications ⬆ ⬇

Indications ⬆ ⬇

Action ⬆ ⬇

Pharmacokinetics ⬆ ⬇