AVALGLUCOSIDASE ALFA-NGPT INFUSION		version 2/27/2025		

Appointment Requests 	

	
	Interval	Defer Until	Duration	
	☑	Referral to IV Infusion Room	Every 2 weeks	S	 treatments	
		Status: Future, Expected: S, Expires: S+365, Clinic Performed
	Urgency of Scheduling: Routine		

Nursing Orders 	

	
	Interval	Defer Until	Duration	
	☐	Insert Peripheral IV or Access Portacath	Every visit	S	Until discont'd	
		No details available for preview		
	☐	Vital signs - T/P/R/BP Per Unit Protocol	Every visit	S	Until discont'd	
		No details available for preview		
	☐	dextrose 5% flush syringe 10 mL	Every visit	S	Until discont'd	
		10 mL, intravenous, PRN, line care, Starting when released, Until Discontinued
	Do NOT push (IVP) the flush		
	☐	dextrose 5% infusion 250 mL	Every visit	S	Until discont'd	
		250 mL, intravenous, at 30 mL/hr, continuous, Starting when released, Until Discontinued
	To keep vein open / flush bag		
	☐	HEParin, porcine injection 500 Units	Every visit	S	Until discont'd	
		500 Units, intra-catheter, once PRN, line care, Starting when released, Until Discontinued
	Heparin flush for Implanted Vascular Access Device Maintenance.		
	☐	Discontinue IV	Every visit	S	Until discont'd	
		No details available for preview		

Pre-Medications 	

	
	Interval	Defer Until	Duration	
	☐	acetaminophen (TYLENOL) tablet 650 mg	Every visit	S	Until discont'd	
		650 mg, oral, once, Starting when released
	Maximum of 4 grams of acetaminophen per day from all sources. (Cirrhosis patients maximum: 2 grams per day from all sources).		
	☐	diphenhydrAMINE (BENADRYL) tablet 25 mg	Every visit	S	Until discont'd	
		25 mg, oral, once, Starting when released		
	☐	methylPREDNISolone sodium succinate (Solu-MEDROL) injection 40 mg	Every visit	S	Until discont'd	
		40 mg, intravenous, once, Starting when released		

Medications 	

	
	Interval	Defer Until	Duration	
	☐	avalglucosidase alfa-ngpt (NEXVIAZYME) 20 mg/kg in dextrose 5% (Total Volume) infusion (RESTRICTED)	Every 2 weeks	S	Until discont'd	
		20 mg/kg, intravenous, every 14 days, Starting S As Scheduled, Until Discontinued
	Infuse through a separate line via an inline, low-protein-binding, 0.2-micronfilter.
	Actual body weight =30 kg (20 mg/kg/dose):  Initiate IV infusion at 1mg/kg/hour. If tolerated, gradually increase the infusion rate every 30 minutes as follows: 3 mg/kg/hour, then 5 mg/kg/hour, then 7 mg/kg/hour.Then, maintain infusion rate at 7 mg/kg/hour until the infusion is complete(total IV infusion duration: ~4 to 5 hours).
	
	For Patients GREATER / EQUAL to 30 kg --> Dose: 20 mg/kg - Duration: 4 to 5 hours
	For Patients LESS than 30 kg --> Dose: 40 mg/kg - Duration: 7 hours		

Hypersensitivity 	

	
	Interval	Defer Until	Duration	
	☐	Nursing communication	PRN	S	Until discont'd	
		In the event of a mild-to-moderate hypersensitivity reaction or a moderate IAR, consider 
	temporarily holding or slowing the infusion rate and initiating appropriate medical treatment. 
	If symptoms: 
	
	• Persist despite temporarily holding the infusion, wait at least 30 minutes for symptoms 
	to resolve before deciding to stop the infusion for the day 
	
	• Subside, resume the infusion for 30 minutes at half the rate at which the reaction 
	occurred, and subsequently increase the infusion rate by 50% for 15 to 30 minutes. 
	If symptoms do not recur, increase the infusion rate to the rate at which the reaction 
	occurred and consider continuing to increase the rate in a stepwise manner		
	☐	acetaminophen (TYLENOL) tablet 650 mg	PRN	S	Until discont'd	
		650 mg, oral, every 4 hours PRN, mild pain (score 1-3), Starting when released, Until Discontinued
	Maximum of 4 grams of acetaminophen per day from all sources. (Cirrhosis patients maximum: 2 grams per day from all sources).		
	☐	diphenhydrAMINE (BENADRYL) tablet 25 mg	PRN	S	Until discont'd	
		25 mg, oral, every 6 hours PRN, itching, Starting when released, Until Discontinued		
	☐	diphenhydrAMINE (BENADRYL) injection 25 mg	PRN	S	Until discont'd	
		25 mg, intravenous, once PRN, for minor infusion reaction, Starting when released, Until Discontinued		
	☐	diphenhydrAMINE (BENADRYL) injection 50 mg	PRN	S	Until discont'd	
		50 mg, intravenous, once PRN, for severe infusion reaction, Starting when released, Until Discontinued		
	☐	epINEPHrine (ADRENALIN) injection 1 mg/mL 0.5 mg	PRN	S	Until discont'd	
		0.5 mL, subcutaneous, once PRN, anaphylaxis, Starting when released, Until Discontinued		
	☐	hydrocortisone sodium succinate (Solu-CORTEF) injection 100 mg	PRN	S	Until discont'd	
		100 mg, intravenous, once PRN, for infusion reaction, Starting when released, Until Discontinued		
	☐	promethazine (PHENERGAN) tablet 25 mg	PRN	S	Until discont'd	
		25 mg, oral, every 6 hours PRN, nausea, vomiting, Starting when released, Until Discontinued		
	☐	sodium chloride 0.9% infusion 250 mL	PRN	S	Until discont'd	
		250 mL, intravenous, once PRN, Hydration, Starting when released, Until Discontinued