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Assemble Needed Supplies

View video: Preparing equipment and supplies


  • IV infusion pump
  • Suction device
  • Oxygen setup
  • Pulse oximeter
  • Emergency medication

Proper storage of NAGLAZYME® (galsulfase) vials

NAGLAZYME® (galsulfase) is supplied as a sterile, nonpyrogenic, colorless to pale yellow, clear to slightly opalescent solution.

Each 5-mL, single-use vial provides 5 mg of galsulfase (expressed as protein content).

Please observe the following procedures for proper storage of NAGLAZYME vials1:

  • Store NAGLAZYME vials under refrigeration at 2ºC to 8ºC (36ºF to 46ºF)
  • NAGLAZYME contains no preservatives and should be used immediately following preparation
  • Prepared NAGLAZYME must be refrigerated at 2ºC to 8ºC (36ºF to 46ºF) and administered within 48 hours from the time of preparation to completion of administration

Supplies needed for dilution of NAGLAZYME

You will need the following supplies for NAGLAZYME dilution:

  • NAGLAZYME 5-mL, single-use vials
  • 0.9% Sodium Chloride Injection, USP, infusion bag (100 mL or 250 mL)
  • Syringes for dilution
  • 18-gauge needles without filtering devices for dilution
  • Low-protein-binding straight IV tubing (no Volutrol or Buretrol)
  • In-line, low-protein-binding 0.2-μm filter
  • Additional supplies per institutional protocols

Recommended equipment for NAGLAZYME administration

For a NAGLAZYME infusion you will need the following equipment:

  • IV infusion pump
  • Wall suction (or portable suction machine)
  • Oxygen setup
  • Pulse oximeter
  • Emergency medication, such as diphenhydramine, systemic corticosteroids, and epinephrine, to treat potential reactions
    • As with all medications, be sure to check the expiration date before administering

Step 2: Prepare the patient »


  1. NAGLAZYME [package insert]. Novato, CA: BioMarin Pharmaceutical Inc; 2019.


Life-threatening anaphylactic reactions and severe allergic reactions have been observed in some patients during NAGLAZYME infusions and up to 24 hours after infusion. If these reactions occur, immediate discontinuation of NAGLAZYME is recommended and appropriate medical treatment should be initiated, which may include resuscitation, epinephrine, administering additional antihistamines, antipyretics or corticosteroids. In patients who have experienced anaphylaxis or other severe allergic reactions during infusion with NAGLAZYME, caution should be exercised upon rechallenge; appropriately trained personnel and equipment for emergency resuscitation (including epinephrine) should be available during infusions.

As with other enzyme replacement therapies, immune-mediated reactions, including membranous glomerulonephritis have been observed. In clinical trials, nearly all patients developed antibodies as a result of treatment with NAGLAZYME; however, the analysis revealed no consistent predictive relationship between total antibody titer, neutralizing or IgE antibodies, and infusion-associated reactions, urinary glycosaminoglycan (GAG) levels, or endurance measures.

Caution should be exercised when administering NAGLAZYME to patients susceptible to fluid volume overload because congestive heart failure may result. Consider a decreased total infusion volume and infusion rate when administering NAGLAZYME to these patients.

Consideration to delay NAGLAZYME infusion should be given when treating patients who present with an acute febrile or respiratory illness. Sleep apnea is common in MPS VI patients and antihistamine pretreatment may increase the risk of apneic episodes. Evaluation of airway patency should be considered prior to the initiation of treatment. Patients using supplemental oxygen or continuous positive airway pressure (CPAP) during sleep should have these treatments readily available during infusion in the event of an infusion reaction, or extreme drowsiness/sleep induced by antihistamine use.

Pretreatment with antihistamines with or without antipyretics is recommended prior to the start of infusion to reduce the risk of infusion reactions. If infusion reactions occur, decreasing the infusion rate, temporarily stopping the infusion, or administering additional antihistamines and/or antipyretics is recommended.

During infusion, serious adverse reactions included laryngeal edema, apnea, pyrexia, urticaria, respiratory distress, angioedema, and anaphylactoid reaction; severe adverse reactions included urticaria, chest pain, rash, abdominal pain, dyspnea, apnea, laryngeal edema, and conjunctivitis. The most common adverse events (≥10%) observed in clinical trials in patients treated with NAGLAZYME were rash, pain, urticaria, pyrexia, pruritus, chills, headache, nausea, vomiting, abdominal pain and dyspnea. The most common adverse reactions requiring interventions are infusion-related reactions.

Spinal/cervical cord compression is a known and serious complication that is expected to occur during the natural course of MPS VI. Signs and symptoms of spinal/cervical cord compression include back pain, paralysis of limbs below the level of compression, and urinary or fecal incontinence. Patients should be evaluated for spinal/cervical cord compression prior to initiation of NAGLAZYME to establish a baseline and risk profile. Patients treated with NAGLAZYME should be regularly monitored for the development or progression of spinal/cervical cord compression and be given appropriate clinical care.

To report SUSPECTED ADVERSE REACTIONS, contact BioMarin Pharmaceutical Inc. at 1-888-906-6100, or FDA at 1-800-FDA-1088 or

Please see full Prescribing Information.

NAGLAZYME® (galsulfase) is indicated for patients with mucopolysaccharidosis VI (MPS VI; Maroteaux-Lamy syndrome). NAGLAZYME has been shown to improve walking and stair-climbing capacity.