IV 20 mg/kg once every 2 wk infused incrementally at an initial rate of 1 mg/kg/hr & gradually increased by 2 mg/kg/hr every 30 min if there are no signs of infusion-associated reactions (IARs), until max rate 7 mg/kg/hr is reached.
Consider discontinuation & initiate appropriate medical treatment in case of severe hypersensitivity or anaphylactic reactions; immune-mediated reactions. Greater risk for IARs in patients w/ acute illness eg, pneumonia, sepsis at the time of infusion. IARs may occur at any time during or up to 2 hr after infusion, & w/ higher infusion rates. Closely monitor patients w/ advanced Pompe disease during administration. Development of IgG & IgE Abs. Monitor for signs & symptoms of systemic immune-mediated reactions involving skin & other organs. Perform periodic urinalysis among patients w/ high IgG Ab titres. May affect the ability to drive & use machines. Patients w/ renal or hepatic impairment. Pregnancy & lactation.
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