GALSULFASE IS GIVEN BY INTRAVENOUS INFUSION IN A DOSE OF 1 MG/KG ONCE A WEEK. INFUSION REACTIONS ARE COMMON AND PATIENTS SHOULD BE PRE-TREATED WITH ANTIHISTAMINES WITH OR WITHOUT ANTIPYRETICS. GALSULFASE SHOULD BE RECONSTITUTED TO A FINAL VOLUME OF 250 ML IN SODIUM CHLORIDE 0.9% AND GIVEN USING AN INFUSION PUMP. THE INITIAL INFUSION RATE SHOULD BE 6 ML/HOUR FOR THE FIRST HOUR, WHICH MAY THEN BE INCREASED TO 80 ML/HOUR IF WELL TOLERATED. THE TOTAL INFUSION TIME SHOULD BE AT LEAST 4 HOURS TO MINIMISE THE RISK OF INFUSION REACTIONS, BUT MAY BE EXTENDED TO UP TO 20 HOURS, AND INTERRUPTED IF NECESSARY, IN THE EVENT OF INFUSION REACTIONS. PATIENTS WEIGHING 20 KG AND UNDER MAY BE SUSCEPTIBLE TO FLUID OVERLOAD AND A FINAL RECONSTITUTED VOLUME OF 100 ML SHOULD BE CONSIDERED, IN WHICH CASE, THE INFUSION RATE SHOULD BE DECREASED ACCORDINGLY SO THAT THE TOTAL INFUSION TIME DOES NOT EXCEED 4 HOURS.
Administration:
ALLERGIC SYMPTOMS IF OCCURRED ON ADMINISTRATION THEY TYPICALLY ABATED WITH SLOWING OR TEMPORARY INTERRUPTION OF THE INFUSION AND ADMINISTRATION OF ADDITIONAL ANTIHISTAMINES, ANTIPYRETICS, AND OCCASIONALLY CORTICOSTEROIDS. MOST PATIENTS WERE ABLE TO COMPLETE THEIR INFUSIONS. SUBSEQUENT INFUSIONS WERE MANAGED WITH A SLOWER RATE OF NAGLAZYME ADMINISTRATION, TREATMENT WITH ADDITIONAL PROPHYLACTIC ANTIHISTAMINES, AND, IN THE EVENT OF A MORE SEVERE REACTION, TREATMENT WITH PROPHYLACTIC CORTICOSTEROIDS.