idursulfase

Absorption: IV administration results in complete bioavailability.

Distribution: Unknown.

Metabolism/Excretion: Unknown.

Half-Life: 44–48 min.

Time/Action Profile ⬆ ⬇

ROUTE	ONSET	PEAK	DURATION
IV	rapid	end of infusion	unknown

Contraind./Precautions ⬆ ⬇

Contraindicated in:

None.

Use Cautiously in:

Compromised respiratory function/acute respiratory disease (increased risk of infusion reactions);
Severe genetic mutations (↑ risk of serious adverse reactions);
Patients at risk for fluid overload, acute respiratory disorder, cardiac or respiratory illness (↑ risk of compromised cardiac or respiratory function);
OB: Use during pregnancy only if potential maternal benefit outweighs potential fetal risk;
Lactation: Use while breastfeeding only if potential maternal benefit outweighs risk to infant;
Pedi: Children <16 mo (safety not established).

Adv. Reactions/Side Effects ⬆ ⬇

CV: hypertension

Derm: cutaneous reactions

GI: dyspepsia

Local: infusion site swelling

MS: arthralgia

Neuro: headache, malaise

Misc: fever, ANAPHYLAXIS

Interactions ⬆ ⬇

Drug-drug:

None reported.

Route/Dosage ⬆ ⬇

IV (Adults and Children ≥5 yr): 0.5 mg/kg weekly.

Availability ⬆ ⬇

Solution for injection: 2 mg/mL

Assessment ⬆ ⬇

Observe for signs and symptoms of anaphylaxis (rash, pruritus, laryngeal edema, wheezing) during and for 24 hr after infusion. Keep epinephrine, an antihistamine, corticosteroids, and resuscitation equipment close by in case of anaphylactic reaction.
Monitor for infusion-related reactions (headache, fever, rash, pruritus, erythema, urticaria, hypertension. Most reactions are managed with antihistamines and/or corticosteroids prior to or during infusions, slowing rate of infusion, and/or early discontinuation if reaction is serious.

Implementation ⬆ ⬇

Patients with compromised respiratory function or acute respiratory disease are at increased risk of life-threatening infusion reactions. Consider delaying infusion in patients with concomitant acute respiratory and/or febrile illness.
Intermittent Infusion: Determine number of vials required for the dose ordered. If number of vials includes a fraction, round up to next whole number. Vials are for single use; discard remaining medication. Diluent: Dilute each vial in 100 mL of 0.9% NaCl. Use an infusion set with a 0.2 micrometer filter for administration. Mix gently; do not shake solution. Solution is clear to slightly opalescent; do not administer solutions that are discolored or contain particulate matter. Solution may be stored in refrigerator for up to 48 hr. Discard solution of not administered or refrigerated within 8 hr.
Rate: Administer at 8 mL/hr for first 15 min. If well tolerated, may increase by 8 mL/hr at 15 min intervals to administer total volume within desired time. Do not exceed 100 mL/hr. May slow, temporarily stop, or discontinue for that visit if infusion reactions occur. Administer total infusion over 1–3 hr. May require longer times if infusion reactions occur; should not exceed 8 hr.
Y-Site Incompatibility: Do not administer other medications or solutions in same tubing as idursulfase.

Patient/Family Teaching ⬆ ⬇

Explain purpose of infusion to patient and parents.
Inform patient of potential for life-threatening anaphylactic reactions. Advise patient to notify health care professional immediately if signs and symptoms of anaphylactic reactions occur.
Advise female patient to notify health care professional if pregnancy is planned or suspected, or if breastfeeding.
Inform patient that a Hunter Outcome Survey was established to evaluate long term treatments. For information, visit www.elaprase.com or call 1-866-888-0660.

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Pronunciation ⬇

Classifications ⬆ ⬇

Indications ⬆ ⬇

Action ⬆ ⬇

Pharmacokinetics ⬆ ⬇