Dosing & Uses
Dosage Forms & Strengths
injectable solution
- 2mg/mL (as 6mg/3mL single-use vial)
Hunter Syndrome
Dosage Forms & Strengths
injectable solution
- 2mg/mL (as 6mg/3mL single-use vial)
Hunter Syndrome
<5 years: Safety and efficacy not established
≥5 years: 0.5 mg/kg IV qWeek; infuse over 1-3 hr
Dosing Considerations
Shown to improve walking capacity in patients aged ≥5 years
In patients aged 16 months to 5 yr, no data are available to demonstrate improvement in disease-related symptoms or long term clinical outcome; however, treatment has reduced spleen volume similarly to that of adults and children aged 5 yr and older
Adverse Effects
>10%
Pyrexia (63%)
Headache (59%)
Arthralgia (31%)
Limb pain (28%)
Pruritus (28%)
Hypertension (25%)
Malaise (22%)
Visual disturbance (22%)
Wheezing (19%)
Abscess (16%)
Chest wall musculoskeletal pain (16%)
Musculoskeletal dysfunction (16%)
Urticaria (16%)
AE resulting from injury (13%)
Anxiety (13%)
Iirritability (13%)
Atrial abnormality (13%)
Dyspepsia (13%)
Infusion site edema (13%)
Pruritic rash (13%)
Skin disorder (13%)
Superficial injury (13%)
Frequency Not Defined
Angioedema
Loss of conaciousness
Seizure
Cardiac arrhythmia
Cyanosis
Warnings
Black Box Warnings
Anaphylactoid reactions, which may be life threatening, have been observed in some patients during idursulfase infusions
Anaphylaxis, presenting as respiratory distress, hypoxia, hypotension, urticaria and/or angioedema of throat or tongue have been reported to occur during and after infusions, regardless of duration of the course of treatment
Appropriate medical support should be readily available when idursulfase is administered
Biphasic anaphylactic reactions have also been observed after idursulfase administration, and patients who have experienced anaphylactic reactions may require prolonged observation
Patients with compromised respiratory function or acute respiratory distress may be at risk of serious acute exacerbation of their respiratory compromise due to infusion reactions and require additional monitoring
Contraindications
None listed in the manufacturer's label
Cautions
Risk of anaphylactoid reactions; monitor patients with compromised respiratory function/acute respiratory disease
Patients with compromised respiratory function or acute febrile or respiratory illness at the time of infusion may be at higher risk of life-threatening complications from hypersensitivity reactions
Risk of hypersensitivity and antibody development is higher in individuals with seere genetic mutations (ie, complete gene deletion, large gene rearrangement, nonsense, frameshift or splice site mutations)
Caution when administering to patients susceptible to fluid overload, or patients with acute underlying respiratory illness or compromised cardiac and/or respiratory function for whom fluid restriction is indicated; risk of serious exacerbation of cardiac or respiratory status during infusions
Development of anti-idursulfase IgG antibodies reported in 51% of patients, which may increase incidence of infusion-related reactions
A registry has been created to track adverse effects, and monitor therapeutic responses during long-term treatment; may contact 1-866-888-0660 or www.eleprase.com
Pregnancy & Lactation
Pregnancy
There are no adequate and well-controlled studies in pregnant women; available data from a small number of postmarketing cases in pregnancy are insufficient to inform drug-associated risks for major birth defects, miscarriage, or adverse maternal or fetal outcomes
Animal data
- No evidence of adverse effects on pre- and post-natal development observed with twice weekly intravenous administration of drug to pregnant rats from gestation day 6 through lactation day 19 at about 4 times recommended human weekly dose of 0.5 mg/kg based on body surface area
Lactation
There are no data on presence of drug in human milk, effects on breastfed infant, or on milk production; drug was excreted in breast milk of lactating rats; drug present in animal milk, makes it likely that the drug will be present in human milk; consider developmental and health benefits of breastfeeding along with mother’s clinical need for therapy and any potential adverse effects on breastfed child from therapy or from 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.Pharmacology
Mechanism of Action
Replaces iduronate-2-sulfatase enzyme, which hydrolyzes the mucopolysaccharides dermatan sulfate and heparan sulfate in various cells. Respiratory, cardiac and mobility dysfunction are caused by the accumulation of these polysacharides. Physical changes and CNS involvement also occur.
Absorption
Peak plasma concentration: 1.1-1.5 mcg/mL
AUC: 169-206 min•mcg/mL
Distribution
Vd: 21-25 L
Elimination
Half-Life: 44-48 min
Clearance: 3-3.4 mL/min/kg
Administration
IV Incompatibilities
Do not infuse with other products in infusion tubing
IV Preparation
Dilute calculated volume of drug in 100 mL 0.9% NaCl; mix gently
Discard if not administered/refrigerated within 8 hr
Diluted solution may be stored in refrigerator for 48 hr
Do not use if solution is discolored
IV Administration
Infuse IV over 1-3 hr; not to exceed 8 hr
Initial infusion rate: 8 mL/hr x15 min
If well tolerated, may increase by 8 mL/hr x15 min up to 100 mL/hr
Infusion may be slowed and/or temporarily stopped or discontinued
Storage
Refrigerate vials at 2-8°C (36-46°F); protect from light
Do not freeze/shake
Images
Patient Handout
idursulfase intravenous
IDURSULFASE - INJECTION
(Eye-dur-SUL-fase)
COMMON BRAND NAME(S): Elaprase
WARNING: Idursulfase may rarely cause serious (sometimes fatal) allergic reactions that may happen while receiving idursulfase and for 24 hours after the infusion. A health care professional will monitor you closely during and after your infusion. Get medical help right away if you notice any signs of a serious allergic reaction, such as rash, itching/swelling (especially of the face/tongue/throat), severe dizziness, or trouble breathing. Your doctor may prescribe other medications (such as antihistamines, corticosteroids) to treat these side effects. Your infusion may also be slowed down or stopped depending on your symptoms.Before you start your infusion, tell your doctor if you have breathing problems or a fever. Breathing problems may get worse during the infusion. Your doctor may delay your infusion or monitor you for longer periods after your infusion.
USES: Idursulfase is used to treat a certain inherited condition (mucopolysaccharidosis type II-Hunter syndrome) that causes a buildup of a certain substance (glycosaminoglycan-GAG) in the body. Idursulfase works by breaking down GAGs in the body. This medication may improve how far you can walk.
HOW TO USE: Read the Patient Information Leaflet if available from your pharmacist before you start using idursulfase and each time you get an infusion. If you have any questions, ask your doctor or pharmacist.This medication is given by injection into a vein by a health care professional. It is given as directed by your doctor, usually once weekly. The first dose is injected slowly into a vein over 3 hours. Later doses may be given over a shorter time. The dosage and treatment schedule are based on your medical condition, weight, and response to treatment.Tell your doctor or nurse right away if you have any symptoms of infusion reactions such as headache, dizziness, flushing, fever, nausea, or vomiting. Your doctor may slow down or stop your treatment for some time if you get serious side effects. Your doctor may also prescribe other medications before each treatment to help prevent these side effects. Carefully follow your doctor's directions for all of your medications.Use this medication regularly to get the most benefit from it. To help you remember, mark the days on the calendar when you need to receive the medication.Tell your doctor if your condition gets worse.
SIDE EFFECTS: See also Warning section.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.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: See also Warning section.Before using idursulfase, 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: swelling, heart failure.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: Keep all medical and lab appointments.
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.
STORAGE: Not applicable. This medication is given in a hospital or clinic or doctor's office and will not be stored at home.
Information last revised March 2023. Copyright(c) 2023 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.
Formulary
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