imiglucerase

Contraindicated in:

Hypersensitivity.

Use Cautiously in:

Lactation: Use while breastfeeding only if potential maternal benefit justifies potential risk to infant.

Adv. Reactions/Side Effects ⬆ ⬇

CV: hypotension

Derm: pruritus, rash

GI: abdominal discomfort, nausea

GU: decreased urinary frequency

Neuro: dizziness, headache

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

Interactions ⬆ ⬇

Drug-drug:

None reported.

Availability ⬆ ⬇

Lyophilized powder for injection: 400 units/vial

Route/Dosage ⬆ ⬇

IV (Adults and Children ): Initial range of 2.5 units/kg 3 times weekly to 15–60 units/kg every 1–2 wk. Evaluate dosage every 6 mo for possible reduction.

US Brand Names ⬆ ⬇

Action ⬆ ⬇

Prevents the accumulation of glucocerebrosides in cells. Replaces glucocerebrosidases that are deficient in type 1 Gaucher disease.

Therapeutic effects:

Improvement in symptoms of Gaucher disease (anemia, thrombocytopenia, bone disease, splenomegaly, and hepatomegaly).

Classifications ⬆ ⬇

Therapeutic Classification: replacement enzyme

Pharmacokinetics ⬆ ⬇

Absorption: IV administration results in complete bioavailability.

Distribution: Widely distributed.

Metabolism/Excretion: Excreted mainly by the kidneys.

Half-Life: 3.6–10.4 min.

Time/Action Profile ⬆ ⬇

(improvement in symptoms)

ROUTE	ONSET	PEAK	DURATION
IV	unknown	unknown	unknown

Patient/Family Teaching ⬆ ⬇

Explain purpose and the importance of treatment at least every 4 wk to the patient. Imiglucerase helps control the symptoms but does not cure Gaucher disease. Lifelong therapy may be required.
Advise patient 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.
Teach patient the signs and symptoms of hypersensitivity reactions, and infusion-associated reactions; if any occur, seek immediate medical care.
Advise patient to notify a health care professional if a dose is missed.
Rep: Advise females of reproductive potential to notify health care professional if pregnancy is planned or suspected or if breastfeeding. There is a pregnancy exposure registry that monitors pregnancy outcomes in women exposed to imiglucerase during pregnancy. Pregnant women exposed to imiglucerase and health care providers are encouraged to contact the Gaucher patient registry at 1-800-745-4447, ext. 15500, or visit www.registrynxt.com.
Emphasize the importance of follow-up examinations and lab tests.

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Indications ⬇

Contraind./Precautions ⬆ ⬇