- Long-term enzyme replacement therapy for pediatric and adult patients with confirmed diagnosis of Mucopolysaccharidosis VI (MPS VI: N-acetylgalactosamine 4-sufatase deficiency; Maroteaux-Lamy syndrome)
- Available as 1 mg/ mL preservative free 5 mL vial
- Refrigerate vials at 2 C to 8 C. DO NOT freeze
- Remove required vials from the refrigeration to allow them to reach room temperature. Do not allow vials to remain at room temperature longer than 24 hours prior to dilution
- DO NOT SHAKE VIAL. Gently rotate the infusion bag to ensure proper distribution
- Avoid excessive agitation by slowly withdrawing galsulfase. Do not use a filter needle
- Do not use any vials exhibiting particulate matter or discoloration. Galsulfase should be clear to slightly opalescent and colorless to pale yellow
- Diluted product should be used immediately. If not used immediately, refrigerate at 2C to 8C no longer than 24 hours followed by 24 hours at room temperature during administration (48 hours from time of preparation to completion of administration)
- Solutions Compatible: 0.9% Sodium Chloride
- Incompatible: Do not infuse with other products
(For approved routes of administration by nursing personnel, refer to Policy for the Administration of Intravenous Medications.)
|IV Intermittent Infusion||YES - DO NOT SHAKE VIAL OR BAG
Infuse through infusion set equipped with a 0.2 micron in-line filter
|IV Continuous Infusion||NO|
(For neonatal dosages, refer to Neonatal IV Drug Manual.)
- 1 mg/kg once weekly IV
- round to closest whole vial (5mg)
- Most common adverse reactions: pyrexia, rash, pruritus, urticaria, chills/rigors, nausea, headache, abdominal pain, vomiting, and dyspnea.
- Serious, but less common, adverse reactions included laryngeal edema, apnea, pyrexia, urticarial, respiratory distress, angioedema, asthma, and anaphylactoid reaction.
- Infusion-associated reactions (IAR):
- Very common: pyrexia, chills/rigors, rash, urticaria and dyspnea.
- Less common: pruritus, vomiting, abdominal pain, nausea, hyper- or hypotension, headache, chest pain, erythema, cough, angioedema, respiratory distress, tachypnea, tremor, conjunctivitis, malaise, bronchospasm, and arthralgia
- Uncommon, but serious: shock, anaphylaxis, bronchospasm, and respiratory failure.
- Stop infusion promptly upon IAR. Consider restarting at slower rate once symptoms subside. The infusion rate can be extended up to 20 hours if infusion reaction occurs.
- Agitation of the vial or reconstituted bag may denature galsulfase, rendering it biologically inactive. DO NOT SHAKE VIAL OR BAG.
- Pretreatment with antihistamine (e.g. diphenhydramine) with or without antipyretics (e.g. acetaminophen) is recommended 30 to 60 minutes prior to the start of infusion.
- Extremely expensive, handle with care.
NAGLAZYME [Product Monograph]. Toronto, ON: BioMarin Pharmaceutical Inc; 2013.