Infuse NAGLAZYME® (galsulfase)
Equipment setup
Following are the steps for setting up the equipment for infusing NAGLAZYME. If you are using plain saline as a primary line, connect the NAGLAZYME tubing at the port closest to the patient.
- Prime the IV tubing with NAGLAZYME solution
- Attach a 0.2-μm in‐line filter to the end of the tubing and slowly prime the filter
- Prime tubing and filter SLOWLY to prevent foaming
- Be sure the drug administration tubing and filter are primed with drug solution (not plain saline). This will ensure that the correct volume is delivered in the first hour of infusion
Infusion rate
Use an infusion pump for NAGLAZYME administration.
Infuse the total volume of NAGLAZYME solution over no less than 4 hours as follows:
- 2.5% of the total NAGLAZYME solution volume in the first hour
- 97.5% of the total volume over the next 3 hours
Infusion rate examples
For 250-mL volume:
6 mL/hour for the first hour. If the infusion is well tolerated, the infusion rate can be increased to 80 mL/hour for approximately 3 hours.
For 120-mL volume:
3 mL/hour for the first hour. If the infusion is well tolerated, the infusion rate can be increased to 39 mL/hour for approximately 3 hours.
Note: Patients and families often ask if the infusion can be administered in a shorter amount of time. For maximum safety and efficacy, do not administer NAGLAZYME at a faster rate than recommended.
Patient monitoring during NAGLAZYME infusion
What to monitor
Monitor vital signs:
- Prior to infusion
- Every hour during the infusion especially after increases in infusion rate
- At infusion completion
- Prior to discharge
What to look for
When monitoring vital signs, look for signs of infusion‐associated reactions (IARs), such as:
- Increase or decrease in heart rate
- Increase or decrease in respiratory rate
- Decrease in oxygen saturation (pulse oximetry)
- Increase or decrease in temperature
Other mild reactions (including shivering, rashes, or swelling of the lips) may progress rapidly if not treated.
Be prepared for IARs
The first IARs occurred as late as 146 weeks in the clinical studies. Therefore it is important that:
- A physician be available or accessible by phone or pager
- The patient be monitored closely for IAR signs and symptoms
- Emergency procedures be in place in the event a severe IAR occurs
- Patients and/or parents be educated and encouraged to report IAR symptoms
- This is especially important for parents of younger patients who may not be able to self‐report IAR symptoms
NAGLAZYME IARs1,2
- IARs occurred in 33 of 59 patients treated with NAGLAZYME
- Initial reactions occurred as early as week 1 and as late as week 146
- The most frequent serious adverse events related to the use of NAGLAZYME occurred during infusions and included laryngeal edema, apnea, pyrexia, urticaria, respiratory distress, angioedema, and anaphylactoid reaction
- Severe symptoms included urticaria, chest pain, rash, dyspnea, apnea, laryngeal edema, and conjunctivitis
- The most common symptoms of IARs included:
- Pyrexia, chills, rash, urticaria, dyspnea, nausea, vomiting, pruritus, erythema, abdominal pain, hypertension, and headache
- Respiratory distress, chest pain, hypotension, angioedema, conjunctivitis, tremor, and cough were also reported
- Because of the potential for IARs, patients should receive antihistamines with or without antipyretics prior to infusion
- Despite these measures, 23 of 33 patients experienced recurrent IARs
- Symptoms typically abated with slowing or interruption of infusion and additional antihistamines, antipyretics, and occasionally steroids
Special safety considerations for patients with airway obstruction
- Patients with highly compromised upper airway disease warrant close monitoring during infusions
- Sleep apnea is common in MPS VI patients and antihistamine pretreatment may increase the risk of apneic episodes
- Caution should be exercised when administering prophylactic antihistamines as patients may have airway difficulty during deep sleep
- Use of continuous positive airway pressure (CPAP) and bilevel positive airway pressure (BiPAP) during infusion should be considered in patients with sleep apnea who are using positive airway pressure machines
- Evaluation of airway patency should be considered prior to initiation of treatment due to the increased risk of sleep apnea
Manage IARs
If an IAR occurs, stop the infusion promptly. Then:
- Assess and appropriately manage the patient’s symptoms
- Consider administration of additional antihistamines, antipyretics, and possibly corticosteroids
If symptoms subside, consider restarting the infusion at a slower rate (eg, half the rate at which the IAR occurred).
Subsequent infusions may be managed with:
- A slower rate of infusion
- Additional prophylactic antihistamines
- Antipyretics
- Possibly prophylactic corticosteroids
The physician should evaluate the risks and benefits of readministering NAGLAZYME following a severe hypersensitivity or anaphylactic reaction.
Note: Caution should be exercised if epinephrine use is being considered in patients with MPS VI, due to increased prevalence of coronary artery disease.
Please see Important Safety Information below and full Prescribing Information for more information.
Postinfusion procedures
After the infusion is complete:
- Run normal saline to clear NAGLAZYME solution from tubing
- Obtain postinfusion vital signs
- Observe the patient for a postinfusion period specified by the treating physician
- Discard and dispose of infusion‐related materials in accordance with your institutional policies
Downloadable infusion resources for you and your patients »