Elaprase (idursulfase)

Idursulfase is administered by IV infusion only. The specific activity of idursulfase is 41-77 units/mg, with 1 unit defined as the amount of activity that results in the hydrolysis of 1 mcmol of heparin disaccharide substrate per hour under specified assay conditions.

Idursulfase is administered by IV infusion only. Prior to infusion, the calculated dose of the commercially available concentrated solution containing 2 mg of idursulfase per mL (6 mg/3 mL per vial) must be diluted in 100 mL of 0.9% sodium chloride for injection according to the manufacturer's instructions. The initial infusion rate should be 8 mL/hour for the first 15 minutes. If the infusion is well tolerated, the rate may be increased in increments of 8 mL/hour every 15 minutes in order to administer the entire volume within the desired time, to a maximum rate of 100 mL/hour. The total volume of infusion may be administered in 1-3 hours; however, it should not exceed 8 hours. Infusion rate may be decreased and/or temporarily stopped or discontinued for the particular visit if infusion-related reactions occur. During administration, the diluted solutions of the drug should be filtered through a 0.2-mcm filter. The manufacturer states that idursulfase infusions should not be infused simultaneously through the same IV line with other drugs.

Commercially available idursulfase concentrate for IV infusion should be stored at 2-8degreesC, protected from light, and should not be frozen or shaken. The vials of concentrated solution should be inspected visually for particulate matter and discoloration prior to use. The solution should not be used if it is discolored or particulate matter is present.

The calculated volume of idursulfase to provide the appropriate dose should be withdrawn from the appropriate number of vials and diluted in 100 mL of 0.9% sodium chloride injection. The solution in the infusion bag should be mixed gently and should not be shaken. Diluted solutions should be administered immediately (with the infusion completed within 8 hours) or may be stored refrigerated at 2-8degreesC for up to 48 hours, or, alternatively, must be used within 8 hours at room temperature. Commercially available idursulfase concentrate for IV infusion does not contain preservatives; vials are for single use only. The manufacturer's labeling should be consulted for additional information on dilution and administration of idursulfase.

No dosing information is available for this tab.

No formal drug interaction studies have been performed to date.

There are no contraindication interactions for this drug.

There are no severe interactions for this drug.

There are no moderate interactions for this drug.

The manufacturer states that there are no known contraindications to the use of idursulfase.

There are no contraindication contraindications for this drug.

There are no severe contraindications for this drug.

• Acute respiratory failure
• Asthma
• Chronic obstructive pulmonary disease
• Heart failure

Adverse effects reported in at least 10% of patients receiving idursulfase in a clinical trial include pyrexia, headache, arthralgia, limb pain, pruritus, hypertension, malaise, visual disturbance, wheezing, abscess, chest wall musculoskeletal pain, musculoskeletal dysfunction, urticaria, anxiety/irritability, atrial abnormality, dyspepsia, infusion site edema, injury, pruritic rash, skin disorder, and superficial injury.

In clinical studies, the most frequent serious adverse effects related to idursulfase were hypoxic episodes. The most common infusion-related reactions were headache, fever, cutaneous reactions, and hypertension.

More Frequent

• Fever
• Headache disorder

Less Frequent

• Urticaria

Rare or Very Rare

• Acute respiratory failure
• Anaphylaxis
• Angioedema
• Arthralgia
• Cardiac arrhythmia
• Cyanosis
• Hypotension
• Hypoxia
• Infection
• Localized edema
• Pneumonia
• Pulmonary thromboembolism
• Respiration changes
• Seizure disorder
• Wheezing

More Frequent

• Chills
• Cough
• Diarrhea
• Flushing
• Pruritus of skin
• Skin rash
• Vomiting

Less Frequent

• Myalgia
• Nausea
• Tachycardia

No enhanced messaging available.

In clinical studies, safety and efficacy of idursulfase have been evaluated in pediatric patients 5 years of age and older. Clinical response to idursulfase was similar in children, adolescents, and adults.

Category C. (See Users Guide)

Not known whether idursulfase is distributed into milk. Since many drugs are distributed into milk, caution is advised if the drug is administered to nursing women.

Clinical studies of idursulfase did not include patients 65 years of age and older; it is not known whether geriatric patients respond differently than younger individuals.