Idursulfase

Trade Names:Elaprase- Solution for injection 2 mg/mL

Pharmacology

Provides exogenous enzyme for uptake into cellular lysosomes.

Pharmacokinetics

Absorption

C _max is about 1.5 mcg/mL after 1 wk and about 1.1 mcg/mL after 27 wk. The AUC after 1 wk and 27 wk is about 206 and 169 min•mcg/mL, respectively.

Elimination

The t _½ after 1 wk and 27 wk is approximately 44 and 48 min, respectively. Total body Cl after 1 wk and 27 wk is about 3 and 3.4 mL/min/kg, respectively.

Indications and Usage

Treatment of patients with Hunter syndrome (mucopolysaccharidosis [MPS] ΙΙ).

Contraindications

None known.

Dosage and Administration

IV 0.5 mg/kg every week by IV infusion.

General Advice

• Dilute in 100 mL of sodium chloride 0.9%.
• Each vial is for single use only.
• Use of an infusion with a 0.2 micrometer filter is recommended.
• Administer total volume over a period of 1 to 3 h. If a longer infusion time is required because of infusion reactions, the infusion time should not exceed 8 h. However, the infusion rate may be slowed, temporarily stopped, or discontinued for that visit if infusion reactions occur.
• The initial infusion rate should be 8 mL/h for the first 15 min. Then, if infusion is tolerated, the rate may be increased by 8 mL/h at 15-min intervals.
• At no time should the infusion rate exceed 100 mL/h.
• Do not infuse with other products in the infusion tubing.

Storage/Stability

Refrigerate at 36° to 46°F. Protect from light. Do not freeze or shake. If immediate use after reconstitution is not possible, the diluted solution can be refrigerated at 36° to 46°F for up to 48 h or must be administered within 8 h if held at room temperature.

Drug Interactions

None well documented.

Laboratory Test Interactions

None well documented.

Adverse Reactions

Cardiovascular

Hypertension (25%); atrial abnormality (13%).

CNS

Headache (59%); malaise (22%); anxiety, irritability (13%).

Dermatologic

Pruritus (28%); urticaria (16%); pruritic rash, skin disorder (13%).

EENT

Visual disturbances (22%).

GI

Dyspepsia (13%).

Local

Infusion site edema (13%).

Musculoskeletal

Arthralgia (31%); limb pain (28%); chest wall musculoskeletal pain, musculoskeletal dysfunction (16%).

Respiratory

Wheezing (19%).

Miscellaneous

Pyrexia (63%); anti-idursulfase immunoglobulin G antibodies (51%); abscess (16%); superficial injury (13%).

Precautions

Pregnancy

Category C .

Lactation

Undetermined.

Children

Safety and efficacy not established in children younger than 5 yr of age.

Concomitant illness

Consider delaying infusion in patients with concomitant acute respiratory and/or febrile illness.

Overdosage

Symptoms

No experience with overdosage in humans is available.

Patient Information

• Encourage patient to participate in the Hunter Outcome Survey.

Copyright © 2009 Wolters Kluwer Health.