Version July 2025
Hypersensitivity reactions, including anaphylaxis:
Patients treated with enzyme replacement therapies have experienced life-threatening hypersensitivity reactions, including anaphylaxis. Anaphylaxis has occurred during the early course of enzyme replacement and after extended duration of therapy. Initiate galsulfase in a healthcare setting with appropriate medical monitoring and support measures, including access to cardiopulmonary resuscitation equipment. If a severe hypersensitivity reaction (eg, anaphylaxis) occurs, discontinue galsulfase and immediately initiate appropriate medical treatment, including use of epinephrine. Inform patients of the symptoms of life-threatening hypersensitivity reactions, including anaphylaxis and to seek immediate medical care should symptoms occur.
Dosage guidance:
Safety: Premedicate with antihistamines with or without antipyretics and/or corticosteroids 30 to 60 minutes prior to start of infusion. Initiate in a health care setting that includes cardiopulmonary monitoring and can provide resuscitation if necessary.
Dosing: Manufacturer recommends rounding dose to the nearest whole vial (5 mg) to prepare infusion.
Mucopolysaccharidosis (MPS) VI:
Infants: Limited data available: IV: 1 or 2 mg/kg/dose once weekly (Ref).
Children and Adolescents: IV: 1 mg/kg/dose once weekly.
Children and Adolescents: There are no dosage adjustments provided in the manufacturer's labeling.
Children and Adolescents: There are no dosage adjustments provided in the manufacturer's labeling.
(For additional information see "Galsulfase (recombinant human N-acetylgalactosamine-4 sulfatase): Drug information")
Dosage guidance:
Clinical considerations: Premedicate with antihistamines (with/without antipyretics) 30 to 60 minutes prior to the start of the infusion. Initiate in a health care setting with appropriate medical monitoring and support measures, including CPR equipment, readily available during administration.
Mucopolysaccharidosis VI (MPS VI): IV: 1 mg/kg/dose once weekly.
There are no dosage adjustments provided in the manufacturer's labeling.
There are no dosage adjustments provided in the manufacturer's labeling.
The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified. Adverse reactions reported in children, adolescents, and adults.
>10%:
Cardiovascular: Chest pain (16%), hypertension (11%)
Dermatologic: Skin rash (21%)
Gastrointestinal: Abdominal pain (47%), gastroenteritis (11%), umbilical hernia (11%)
Hypersensitivity: Infusion-related reaction (56%)
Immunologic: Antibody development (98%)
Nervous system: Absent reflexes (11%), chills (21%), malaise (11%), pain (32%)
Neuromuscular & skeletal: Arthralgia (42%)
Ophthalmic: Conjunctivitis (21%), corneal opacity (11%)
Otic: Auditory impairment (11%), otalgia (42%)
Respiratory: Dyspnea (21%), nasal congestion (11%), pharyngitis (11%)
Frequency not defined:
Hypersensitivity: Angioedema, nonimmune anaphylaxis, severe hypersensitivity reaction, type III hypersensitivity reaction (including membranous glomerulonephritis)
Respiratory: Apnea, laryngeal edema, respiratory distress
Postmarketing:
Cardiovascular: Bradycardia, hypotension, shock, tachycardia
Dermatologic: Erythema of skin, pallor
Hematologic & oncologic: Thrombocytopenia
Hypersensitivity: Anaphylaxis
Nervous system: Paresthesia
Renal: Kidney disease (membranous)
Respiratory: Bronchospasm, cyanosis, hypoxia, respiratory failure, tachypnea
Miscellaneous: Fever
There are no contraindications listed in the manufacturer's labeling
Concerns related to adverse effects:
• Hypersensitivity: Galsulfase may cause severe hypersensitivity reactions, including anaphylaxis (sometimes life-threatening). Some reactions included shock, respiratory distress, dyspnea, bronchospasm, laryngeal edema, and hypotension have been reported during and within 24 hours after infusion. Immediate treatment for hypersensitivity reactions should be available during administration. Discontinue treatment immediately if signs or symptoms of anaphylaxis or severe allergic reactions occur; use caution upon rechallenge.
• Immune-mediated reactions: Severe type III immune-mediated reactions (eg, membranous glomerulonephritis) have occurred; monitor patients closely. Consider discontinuation of treatment if signs or symptoms occur; use caution with readministration (some patients have been successfully re-challenged with close supervision).
• Infusion reactions: Infusion-related reactions have been commonly reported; may be sporadic and/or severe. Serious/severe infusion reactions have included laryngeal edema, apnea, respiratory distress, dyspnea, angioedema, and anaphylactoid reaction, pyrexia, chest pain, rash, urticaria, and conjunctivitis; other reactions included chills, nausea, vomiting, pruritus, erythema, abdominal pain, hypertension, hypotension, headache, tremor, and cough. Reactions began as early as week 1 and as late as week 146 of treatment. Many patients experienced recurrent infusion reactions during multiple infusions, though not always in consecutive weeks. Patients should be premedicated with antihistamines and/or antipyretics prior to infusion; evaluate airway prior to therapy (due to possible effects of antihistamine use). Symptoms typically abated with slowing or temporary interruption of the infusion and administration of additional antihistamines, antipyretics, and, occasionally, corticosteroids. Most patients were able to complete their infusions. Subsequent infusions were managed with a slower rate of administration, treatment with additional prophylactic antihistamines, and, in the event of a more severe reaction, treatment with prophylactic corticosteroids. Discontinue treatment immediately and initiate appropriate treatment if severe reaction occurs; use caution with readministration (consider the risks/benefits of readministering following a severe reaction).
Disease-related concerns:
• Acute febrile/respiratory illness: Consider delaying treatment in patients with an acute febrile or respiratory illness.
• Patients at risk for fluid overload: Use with caution in patients who are at risk of fluid overload (patients ≤20 kg, underlying respiratory disease, or compromised cardiopulmonary function); may cause heart failure. Monitor patients closely.
• Sleep apnea: Use with caution in patients with sleep apnea (sleep apnea is common in patients with MPS VI); antihistamine pretreatment may increase the risk of apneic episodes. Evaluate airway patency prior to initiation of treatment; apnea treatment options should be readily available during the infusion and with antihistamine premedication.
• Spinal/cervical cord compression: Worsening and new-onset spinal/cervical cord compression (SCC) has been reported (SCC with resultant myelopathy is a known and anticipated serious complication of MPS VI). Monitor patients for signs and symptoms of SCC (eg, back pain, limb paralysis below the level of compression, urinary and fecal incontinence) and provide appropriate clinical care.
Special populations:
• Adults: Studies did not include patients >29 years of age.
Dosage form specific issues:
• Polysorbate 80: Some dosage forms may contain polysorbate 80 (also known as Tweens). Hypersensitivity reactions, usually a delayed reaction, have been reported following exposure to pharmaceutical products containing polysorbate 80 in certain individuals (Isaksson 2002; Lucente 2000; Shelley 1995). Thrombocytopenia, ascites, pulmonary deterioration, and renal and hepatic failure have been reported in premature neonates after receiving parenteral products containing polysorbate 80 (Alade 1986; CDC 1984). See manufacturer's labeling.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution, Intravenous [preservative free]:
Naglazyme: 1 mg/mL (5 mL) [contains mouse (murine) and/or hamster protein, polysorbate 80]
No
Solution (Naglazyme Intravenous)
1 mg/mL (per mL): $588.24
Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.
Excipient information presented when available (limited, particularly for generics); consult specific product labeling.
Solution, Intravenous:
Naglazyme: 1 mg/mL (5 mL) [contains mouse (murine) and/or hamster protein, polysorbate 80]
Note: Premedicate with antihistamines with or without antipyretics 30 to 60 minutes prior to start of infusion. Initiate infusion in a health care setting that includes cardiopulmonary monitoring and can provide resuscitation if necessary.
Parenteral: IV infusion: Infuse over ≥4 hours via an infusion pump and PVC infusion set with in-line, low-protein-binding 0.2 micrometer filter. Infusion time may be extended to up to 20 hours if infusion reactions occur.
Infusion volume 250 mL: Infuse at 6 mL/hour for the first hour; if well-tolerated, increase rate to 80 mL/hour for the remaining 3 hours.
Infusion volume 100 mL: Use a lower rate than those recommended for the 250 mL volume to infuse over at least 4 hours.
Rate adjustment for infusion-related reactions or hypersensitivity:
Mild to moderate infusion-related reactions: Slowing of infusion rate or temporary interruption of the infusion has been shown to treat symptoms; additional doses of antihistamines, antipyretics, and occasionally corticosteroids may be necessary. For subsequent infusions, initiate with a slower infusion rate along with premedication regimen.
Anaphylaxis or severe hypersensitivity or infusion-related reaction: Discontinue therapy immediately and initiate appropriate medical treatment. If the decision is made to readminister following a risk versus benefit assessment, appropriately trained personnel and emergency medications should be immediately available and patients monitored closely for recurrence. For subsequent infusions following severe infusion-related reactions, a corticosteroid should be added to the premedication regimen.
IV: Infuse over at least 4 hours; infusions of a 250 mL total volume should begin at a rate of 6 mL/hour for the first hour, if tolerated, may increase the rate of infusion to 80 mL/hour for the remaining 3 hours. May extend infusion time up to 20 hours if infusion reactions occur. For patients requiring fluid restriction, solutions of 100 mL may be used with the rate adjusted to infuse over at least 4 hours. Administer using infusion pump and PVC (low protein-binding) infusion set with a low protein-binding 0.2 micrometer in-line filter.
Premedicate with antihistamines (with or without antipyretics) 30 to 60 minutes prior to infusion. In case of infusion-related reactions, decrease or temporarily discontinue infusion or administer additional antihistamines, antipyretics, and possibly corticosteroids. Discontinue immediately if severe reaction occurs. Patients requiring supplemental oxygen or CPAP during sleep should have these treatments readily available in case of infusion-related reaction or antihistamine-induced drowsiness.
Prior to reconstitution, store intact vials at 2°C to 8°C (36°F to 46°F). Do not freeze. Do not shake. Protect from light. Following dilution in NS, use immediately. May store under refrigeration if used within 48 hours from the time of preparation to the completion of infusion. Do not store solution for infusion at room temperature. Allow vials to reach room temperature prior to dilution. Do not keep vials at room temperature >24 hours prior to dilution. Do not heat or microwave vials.
Treatment of mucopolysaccharidosis VI (MPS VI, Maroteaux-Lamy syndrome) for improvement of walking and stair-climbing capacity (FDA approved in pediatric patients [age not specified] and adults).
None known.
Note: Interacting drugs may not be individually listed below if they are part of a group interaction (eg, individual drugs within “CYP3A4 Inducers [Strong]” are NOT listed). For a complete list of drug interactions by individual drug name and detailed management recommendations, use the drug interactions program
There are no known significant interactions.
Data related to the use of galsulfase in pregnancy are limited (Stewart 2016). Based on 17 pregnancies identified in the MPS VI Clinical Surveillance Program and case reports, an increased risk of major birth defects or adverse pregnancy outcomes has not been observed.
Pregnancy may exacerbate symptoms of mucopolysaccharidosis VI, leading to adverse maternal and fetal outcomes.
Signs/symptoms of hypersensitivity (including anaphylaxis), infusion-related reactions (during infusion and for 24 hours following infusion); vital signs; presence of infusion-related reactions (chills, pruritus, urticaria, dyspnea, rash, shortness of breath, nausea, abdominal pain). Patients with mucopolysaccharidosis (MPS) VI should be monitored for signs and symptoms of spinal/cervical cord compression (eg, back pain, paralysis of limbs below the level of compression, urinary and fecal incontinence). In patients susceptible to fluid overload (eg, weight less than 20 kg, underlying acute respiratory illness, compromised cardiac and/or respiratory function), monitor cardiac and respiratory function since congestive heart failure may occur. Evaluation of airway patency should be considered prior to start of treatment and patients using supplemental oxygen or CPAP during sleep should have these treatments available during infusion.
Galsulfase is a recombinant form of N-acetylgalactosamine 4-sulfatase, produced in Chinese hamster cells. A deficiency of this enzyme leads to accumulation of the glycosaminoglycan dermatan sulfate in various tissues, causing progressive disease which includes decreased growth, skeletal deformities, upper airway obstruction, clouding of the cornea, heart disease, and coarse facial features. Exogenous replacement of this enzyme has been shown to improve mobility and physical function (measured by walking and stair-climbing).
Note: Data based on mixed patient population of children ≥5 years and adults <29 years.
Distribution: Vz: Week 1: 103 mL/kg (range: 56 to 323 mL/kg); Week 24: 69 mL/kg (range: 59 to 2,799 mL/kg)
Half-life elimination: Week 1: Median 9 minutes (range: 6 to 21 minutes); Week 24: Median 26 minutes (range: 8 to 40 minutes)
Molecular weight: ~56,000.
