Prepare the patient
Explain the procedure
Before infusion, explain the procedure to the patient and/or parent.
Over time, patients and their caregivers will become familiar with the weekly infusion routine. During the first visit, however, you will want to go over each step.
Obtain vital signs and weight
Prior to infusion, you will need to obtain the patient’s vital signs, including:
- Blood pressure
- Pulse oximetry (Sp02) level
- Weight (prior to dose preparation for accurate dosing)
For dosing, convert the patient’s weight from pounds to kilograms: (X lbs / 2.2 = X kg (or X lbs x 0.4536 = X kg)
Assess respiratory status, health history
To determine whether the patient is able to undergo the infusion, do the following:
- Assess the patient’s respiratory function
- Review recent health history
- Note any changes in the patient’s baseline health and respiratory function
- Be aware that fever and respiratory symptoms may mask infusion-associated reactions
- Consider postponing infusions in patients with acute febrile or respiratory illness
Medications recommended prior to NAGLAZYME® (galsulfase) administration
Prior to IV insertion and 30 to 60 minutes before beginning infusion, the patient should be premedicated with antihistamines (with or without antipyretics) because of the potential for infusion-associated reactions.
Sleep apnea is common in patients with MPS VI. Be aware that antihistamine pretreatment may increase the risk of apneic episodes.
Obtain IV access
It is important to make sure the vein is accessed before mixing NAGLAZYME® (galsulfase) into the saline solution.
After completing venipuncture:
- Draw blood work if needed, and flush the line with normal saline
- Consider using plain 0.9% Sodium Chloride Injection, USP, with no drug added as the primary line, at the rate specified by the physician
- Keeps the vein open prior to NAGLAZYME infusion
- Provides immediate use in case of an infusion-associated reaction