Naglazyme (galsulfase)

• Naglazyme (galsulfase) is an enzyme used to treat some of the symptoms of a genetic condition called Maroteaux-Lamy syndrome (mucopolysaccharidosis).
• Naglazyme improves walking and stair-climbing capacity. Naglazyme is indicated for patients with Mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome).

• headache,
• joint pain,
• eye redness,
• nausea,
• vomiting,
• stomach pain,
• diarrhea,
• runny or stuffy nose,
• sore throat,
• cough,
• ear pain,
• rash or itching, or
• injection site reactions (pain, redness, swelling, or other irritation)

Recommended Dose

• The recommended dosage regimen of Naglazyme is 1 mg per kg of body weight administered once weekly as an intravenous infusion.
• Pretreatment with antihistamines with or without antipyretics is recommended 30 to 60 minutes prior to the start of the infusion.
• The total volume of the infusion should be delivered over a period of time of no less than 4 hours.
• Naglazyme should be diluted with 0.9% Sodium Chloride Injection, USP, to a final volume of 250 mL and delivered by controlled intravenous infusion using an infusion pump.
• The initial infusion rate should be 6 mL per hour for the first hour.
• If the infusion is well tolerated, the rate of infusion may be increased to 80 mL per hour for the remaining 3 hours.
• The infusion time can be extended up to 20 hours if infusion reactions occur.
• For patients 20 kg and under or those who are susceptible to fluid volume overload, physicians may consider diluting Naglazyme in a volume of 100 mL.
• The infusion rate (mL per hour) should be decreased so that the total infusion duration remains no less than 4 hours.
• Each vial of Naglazyme provides 5 mg of galsulfase (expressed as protein content) in 5 mL of solution and is intended for single use only.
• Do not use the vial more than one time.
• The concentrated solution for infusion must be diluted with 0.9% Sodium Chloride Injection, USP, using aseptic techniques.
• Prepare Naglazyme using low-protein-binding containers and administer the diluted Naglazyme solution to patients using a low-protein-binding infusion set equipped with a low-protein-binding 0.2 µm in-line filter.
• There is no information on the compatibility of diluted Naglazyme with glass containers.

• Available data from case reports and postmarketing experience with Naglazyme use in pregnant women are insufficient to evaluate for a drug-associated risk of major birth defects, miscarriage, or adverse maternal or fetal outcomes.
• There are no data on the presence of galsulfase in human milk, the effects on the breastfed infant, or the effects on milk production. The developmental and health benefits of breastfeeding should be considered along with the mother's clinical need for Naglazyme and any potential adverse effects on the breastfed infant from Naglazyme or from the underlying maternal condition.