Adult Dosing
Hunter Syndrome (Mucopolysaccharidosis II)
- 0.5 mg/kg diluted in 100 mL NaCl Solution over 1-3 hrs, once a week
- Start infusion at 8 mL/hr over 15 minutes. Increase rate of infusion by increments of 8 mL/hr if tolerated
- Infusion rate should not exceed 100 mL/hr
Note: Do not infuse with other products in the infusion tubing
Pediatric Dosing
Hunter Syndrome (Mucopolysaccharidosis II)
Children 
16 months
- 0.5 mg/kg diluted in 100 mL NaCl Solution over 1-3 hrs, once a week
- Start infusion at 8 mL/hr over 15 minutes. Increase rate of infusion by increments of 8 mL/hr if tolerated
- Infusion rate should not exceed 100 mL/hr
Note:
- Do not infuse with other products in the infusion tubing
- Safety and effectiveness in children <16 months of age have not been established
- Life-threatening anaphylactic reactions have been reported in patients during and up to 24 hrs following idursulfase infusion [US Black Box Warning]
- Anaphylaxis, manifesting as respiratory distress, hypoxia, hypotension, urticaria and/or throat or tongue angioedema has been reported with idursulfase use, irrespective of the treatment duration. Advise patients to seek immediate medical attention if signs and symptoms of anaphylaxis occur [US Black Box Warning]
- The incidence of hypersensitivity and serious adverse reactions is greater in Hunter syndrome patients
7 years with severe genetic mutations, including complete gene deletion, large gene rearrangement, nonsense, frameshift or splice site mutations - Patients experiencing an acute febrile or respiratory illness, or those with a compromised respiratory function have an increased risk of hypersensitivity reactions which may worsen their condition. Additional monitoring is required for these patients [US Black Box Warning]
- Patients at risk of fluid overload, or those with acute underlying respiratory illness or compromised cardiac and/or respiratory function necessitating fluid restriction should be treated with caution
Cautions: Use cautiously in
- Impaired respiratory function
- Acute illness
- Nursing mothers
Pregnancy Category:C
Breastfeeding: Safety unknown. Manufacturer advises caution.
