galsulfase (Rx)

Brand and Other Names:Naglazyme
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Dosing & Uses

AdultPediatric

Dosage Forms & Strengths

injectable solution

  • 1mg/mL

Mucopolysaccharidosis VI

1 mg/kg IV infusion qWeek  

Pretreat with antihistamines with or without antipyretics 30-60 minutes before infusion

Dosage Forms & Strengths

injectable solution

  • 1mg/mL

Mucopolysaccharidosis VI

<5 years: Safety & efficacy not established

>5 years: 1 mg/kg IV infusion qWeek  

Pretreat with antihistamines with or without antipyretics 30-60 minutes before infusion

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Adverse Effects

>10%

Abdominal pain (53%)

Ear pain (42%)

Conjunctivitis (21%)

Dyspnea (21%)

Rigors (21%)

Chest pain (16%)

Pharyngitis (16%)

Areflexia (11%)

Face edema (11%)

Gastroenteritis (11%)

HTN (11%)

Increased corneal opacification (11%)

Malaise (11%)

Nasal congestion (11%)

Umbilical hernia (11%)

Frequency Not Defined

Bronchospasm

Erythema

Tachycardia

Thrombocytopenia

Tachypnea

Shock

Cyanosis

Hypoxia

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Warnings

Contraindications

None listed in the manufacturer's label

Cautions

Anaphylaxis and severe allergic reactions have been observed during and up to 24 hr after infusion; some reactions were life-threatening and included anaphylaxis, shock, respiratory distress, dyspnea, bronchospasm, laryngeal edema, and hypotension

Type III immune complex-mediated reactions, including membranous glomerulonephritis, observed with enzyme replacement therapies

Caution in patients susceptible to fluid volume overload (eg, weight ≤20 kg, acute underlying respiratory illness, compromised cardiac and/or respiratory function) because CHF may result

Because of the potential for infusion reactions, patients should receive antihistamines with or without antipyretics prior to infusion; infusion reactions may still occur despite of premedication; decrease rate of infusion if it occurs or discontinue immediately if reaction is severe; use caution with readministration

Sleep apnea is common in patients with MPS VI and antihistamine pretreatment may increase the risk of apneic episodes

Spinal or cervical cord compression (SCC) with resultant myelopathy is a known and serious complication of MPS VI; SCC is expected to occur in the natural history of the disease, including in patients on galsulfase; there are postmarketing reports of onset or worsening of SCC requiring decompression surgery

Consider postponing treatment in patients with acute febrile or respiratory illness

Excess agitation of solutioni prior to or after dilution may denature and inactivate galsulfase

A registry has been created to track adverse effects, and monitor therapeutic responses during long-term treatment; may contact 1-800-983-4587 or at www.naglazyme.com/en/clinical-resources/surveillance-program.aspx

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Pregnancy & Lactation

Pregnancy

Available data from case reports and postmarketing experience with use in pregnant women are insufficient to evaluate for a drug-associated risk of major birth defects, miscarriage, or adverse maternal or fetal outcomes

Clinical considerations

  • Pregnancy can exacerbate preexisting clinical manifestations of MPS and lead to adverse pregnancy outcomes for both mother and fetus

Lactation

There are no data on the presence of galsulfase in human milk, the effects on the breastfed infant, or the effects on milk production

Consider developmental and health benefits of breastfeeding along with the mother’s clinical need and any potential adverse effects on the breastfed infant or from the underlying maternal condition

Pregnancy Categories

A: Generally acceptable. Controlled studies in pregnant women show no evidence of fetal risk.

B: May be acceptable. Either animal studies show no risk but human studies not available or animal studies showed minor risks and human studies done and showed no risk.

C: Use with caution if benefits outweigh risks. Animal studies show risk and human studies not available or neither animal nor human studies done.

D: Use in LIFE-THREATENING emergencies when no safer drug available. Positive evidence of human fetal risk.

X: Do not use in pregnancy. Risks involved outweigh potential benefits. Safer alternatives exist.

NA: Information not available.

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Pharmacology

Mechanism of Action

Recombinant N-acetylgalactosamine-4-sulfatase enzyme replacement that prevents the accumulation of the glycosaminoglycan dermatan sulfate in various tissues that could cause progressive disease including decreased growth, skeletal deformities, clouding of the cornea, upper airway obstruction, coarse facial features, and heart disease

Absorption

Peak plasma concentration: 0.8 mcg/mL (Week 1); 1.5 mcg/mL (Week 24)

AUC: 2.3 mcg⋅hr/mL (Week 1); 4.3 mcg⋅hr/mL (Week 24)

Distribution

Vd: 56-323 mL/kg (Week 1); 59-2799 mL/kg (Week 24)

Elimination

Clearance: 4.7-10.5 mL/kg/min (Week 1); 1.1-55.9 mL/kg/min (Week 24)

Half-life: 9 min (Week 1); 26 min (Week 24)

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Administration

IV Incompatibilities

Do not infuse with other products in the infusion tubing

Compatibility of solution with other products has not been evaluated

IV Compatibilities

0.9% NaCl

IV Preparation

Dilute galsulfase with 0.9% NaCl, to a final volume of 250 mL and delivered by IV infusion using an infusion pump; do not use a filter needle to add drug to solution

Consider reducing infusion volume to 100 mL for pt <20 kg and susceptible to fluid overload (no need to remove NS from bag to accommodate galsulfase volume)

Visually inspect each vial for particulate matter and discoloration; diluted solution should be clear to slightly opalescent and colorless to pale yellow

Gently rotate the infusion bag; do not shake the solution

Use low-protein-binding containers and administer diluted solution to patients using a low-protein-binding infusion set equipped with a low-protein-binding 0.2 micron in-line filter

There is no information on compatibility of diluted solutions with glass containers

IV Administration

Pretreatment with antihistamines with or without antipyretics is recommended 30-60 minutes before the infusion

Infused over no less than 4 hr using infusion pump (reduce infusion rate if <250 mL used to maintain at least 4 hr infusion time)

Initial rate 6 mL/hr, may be increase to 80 mL/hr if well-tolerated

Infusion time may be increase up to 20 hr if infusion reactions occur

Storage

Unopened vials

  • Refrigerate at 2-8ºC (36-46ºF)
  • Do not freeze or shake
  • Protect from light; product contains no preservatives

Diluted solution

  • If immediate use is not possible, refrigerate at 2-8ºC (36-46ºF) for up to 48 hr (from the time of dilution to completion of administration)
  • Other than during infusion, do not store diluted solution at room temperature
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Images

No images available for this drug.
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Patient Handout

Patient Education
galsulfase intravenous

GALSULFASE - INJECTION

(gal-SUL-fase)

COMMON BRAND NAME(S): Naglazyme

USES: Galsulfase is used to treat a certain rare genetic problem (Maroteaux-Lamy syndrome - MLS). Galsulfase replaces a certain natural substance that is missing in people with MLS.This medication improves how far you can walk and the number of stairs you can climb up. Galsulfase does not correct the genetic problem, and treatment must be continued for life.

HOW TO USE: This medication is given slowly into a vein as directed by your doctor, usually over 4 hours once a week. The dosage is based on your weight, medical condition, and response to treatment.If you are using this medication at home, galsulfase will be shipped to your home. A nurse will come to your home to give you the treatment and to monitor you afterward. Do not shake the medication. Galsulfase should be colorless or pale yellow. Before using, check this product visually for particles or discoloration. If either is present, do not use it. Discard any unused prepared medication. Do not save for later use. Learn how to store and discard medical supplies safely.Galsulfase may cause side effects (such as fever, chills, rash, shortness of breath, nausea/vomiting, increased blood pressure, headache, chest pain, dizziness, weakness/tiredness, joint pain, flushing, coughing) while it is being given and for a short time after the infusion has stopped. Your doctor may decide to stop or slow down the medication infusion for a while if this happens. Tell your healthcare professional right away if you have any of these symptoms. Your doctor may direct you to take other medications (such as antihistamines, drugs for fever, or corticosteroids such as prednisone) to treat your reaction and before your next treatment(s) to prevent these reactions from happening again.Use this medication regularly to get the most benefit from it. To help you remember, use it on the same day each week.Tell your doctor if your condition gets worse.

SIDE EFFECTS: See also How to Use section.Ear pain, eye redness/itching/discharge, or stomach/abdominal pain may occur. If any of these effects last or get worse, tell your doctor or pharmacist promptly.Remember that this medication has been prescribed because your doctor has judged that the benefit to you is greater than the risk of side effects. Many people using this medication do not have serious side effects.Tell your doctor right away if you have any serious side effects, including: vision/hearing changes, back pain, inability to move arms/legs, inability to control urine/stool, signs of kidney problems (such as change in the amount of urine).A very serious allergic reaction to this drug is rare. However, get medical help right away if you notice any symptoms of a serious allergic reaction, including: rash, itching/swelling (especially of the face/tongue/throat), severe dizziness, trouble breathing.This is not a complete list of possible side effects. If you notice other effects not listed above, contact your doctor or pharmacist.In the US -Call your doctor for medical advice about side effects. You may report side effects to FDA at 1-800-FDA-1088 or at www.fda.gov/medwatch.In Canada - Call your doctor for medical advice about side effects. You may report side effects to Health Canada at 1-866-234-2345.

PRECAUTIONS: Before using galsulfase, tell your doctor or pharmacist if you are allergic to it; or if you have any other allergies. This product may contain inactive ingredients, which can cause allergic reactions or other problems. Talk to your pharmacist for more details.Before using this medication, tell your doctor or pharmacist your medical history, especially of: heart problems (such as heart failure), lung problems.Before having surgery, tell your doctor or dentist about all the products you use (including prescription drugs, nonprescription drugs, and herbal products).During pregnancy, this medication should be used only when clearly needed. Discuss the risks and benefits with your doctor.It is unknown if this medication passes into breast milk. Consult your doctor before breast-feeding.

DRUG INTERACTIONS: Drug interactions may change how your medications work or increase your risk for serious side effects. This document does not contain all possible drug interactions. Keep a list of all the products you use (including prescription/nonprescription drugs and herbal products) and share it with your doctor and pharmacist. Do not start, stop, or change the dosage of any medicines without your doctor's approval.

OVERDOSE: If someone has overdosed and has serious symptoms such as passing out or trouble breathing, call 911. Otherwise, call a poison control center right away. US residents can call their local poison control center at 1-800-222-1222. Canada residents can call a provincial poison control center.

NOTES: Do not share this medication with others.

MISSED DOSE: It is important to get each dose of this medication as scheduled. If you miss a dose, ask your doctor or pharmacist right away for a new dosing schedule. Do not double the dose to catch up.

STORAGE: Before mixing, store the vial in the refrigerator. Protect from light and freezing.Once it has been mixed, consult the product instructions and your pharmacist for storage details. Discard any unused portion. Keep all medications away from children and pets.Do not flush medications down the toilet or pour them into a drain unless instructed to do so. Properly discard this product when it is expired or no longer needed. Consult your pharmacist or local waste disposal company.

Information last revised August 2021. Copyright(c) 2022 First Databank, Inc.

IMPORTANT: HOW TO USE THIS INFORMATION: This is a summary and does NOT have all possible information about this product. This information does not assure that this product is safe, effective, or appropriate for you. This information is not individual medical advice and does not substitute for the advice of your health care professional. Always ask your health care professional for complete information about this product and your specific health needs.

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Formulary

FormularyPatient Discounts

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The above information is provided for general informational and educational purposes only. Individual plans may vary and formulary information changes. Contact the applicable plan provider for the most current information.

Tier Description
1 This drug is available at the lowest co-pay. Most commonly, these are generic drugs.
2 This drug is available at a middle level co-pay. Most commonly, these are "preferred" (on formulary) brand drugs.
3 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs.
4 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
5 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
6 This drug is available at a higher level co-pay. Most commonly, these are "non-preferred" brand drugs or specialty prescription products.
NC NOT COVERED – Drugs that are not covered by the plan.
Code Definition
PA Prior Authorization
Drugs that require prior authorization. This restriction requires that specific clinical criteria be met prior to the approval of the prescription.
QL Quantity Limits
Drugs that have quantity limits associated with each prescription. This restriction typically limits the quantity of the drug that will be covered.
ST Step Therapy
Drugs that have step therapy associated with each prescription. This restriction typically requires that certain criteria be met prior to approval for the prescription.
OR Other Restrictions
Drugs that have restrictions other than prior authorization, quantity limits, and step therapy associated with each prescription.
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Medscape prescription drug monographs are based on FDA-approved labeling information, unless otherwise noted, combined with additional data derived from primary medical literature.