Idursulfase Dosage

Medically reviewed by Drugs.com. Last updated on Nov 24, 2023.

Applies to the following strengths: 2 mg/mL

Usual Adult Dose for Mucopolysaccharidosis Type II

0.5 mg/kg IV once a week

Comment:

Clinical studies did not include patients older than 31 years and it is therefore unknown if they respond differently than younger patients.

Use: For the treatment of Hunter syndrome (Mucopolysaccharidosis II [MPS II])

16 months or older:
0.5 mg/kg IV once a week

Use: For the treatment of Hunter syndrome (MPS II)

Data not available

Data not available

US BOXED WARNING:

RISK OF ANAPHYLAXIS:
Life-threatening anaphylactic reactions have occurred in some patients during and up to 24 hours after infusions. Anaphylaxis presenting as respiratory distress, hypoxia, hypotension, urticaria, and/or angioedema of throat or tongue have been reported to occur during and after infusions, regardless of duration of course of treatment.
Patients should be observed closely during and after administration of this drug and healthcare providers should be prepared to manage anaphylaxis. Patients should be informed of the signs and symptoms of anaphylaxis and they should seek immediate medical care should symptoms occur. Patients with compromised respiratory function or acute respiratory disease may be at risk of serious acute exacerbation of their respiratory compromise due to hypersensitivity reactions and require additional monitoring.

Safety and efficacy have not been established in patients younger than 16 months.

Consult WARNINGS section for additional precautions

Data not available

Administration advice:

For IV infusion only
Administer the diluted solution using a low protein binding infusion set with an in-line, low-protein-binding 0.2 micrometer filter; do not administer with other products in infusion tubing.
Infuse over 3 hours; some patients may require longer infusion times if hypersensitivity reactions occur, but do not exceed 8 hours.
May gradually reduce to a 1 hour infusion if no hypersensitivity reactions are observed
Use an initial infusion rate of 8 mL/hr for the first 15 minutes; if well tolerated, may increase rate by 8 mL/hr increments every 15 minutes; do not exceed 100 mL/hour.
May slow, temporarily stop, or discontinue infusion for that visit if hypersensitivity reaction occurs

Storage requirements:

Vials should be stored in the carton at 2C to 8C (36F to 46F) to protect from light; do not freeze or shake.
Do not store diluted solution at room temperature other than during infusion.
Use diluted solution immediately; if cannot use immediately, may store for up to 24 hours at 2C to 8C (36F to 46F).

Reconstitution/preparation techniques:

IV compatibility:

General:

Walking capacity has been shown to improve in patients 5 years and older.
There is no data available to demonstrate improvement in disease-related symptoms or long term clinical outcome in patients 16 months to 5 years; however, there is evidence of reduced spleen volume similarly to that of patients 5 years and older.

Monitoring:

Hypersensitivity: Signs/symptoms of hypersensitivity reaction (during and after infusion)

Patient advice:

Seek medical attention immediately if signs or symptoms of hypersensitivity or anaphylaxis occur.
You are encouraged to participate in the Hunter Outcome Survey and should call 1-866-888-0660 for more information.