Dosage & Administration
The recommended dosage is 0.5 mg per kg of body weight administered once every week as an intravenous infusion .
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Elaprase Prescribing Information
Life-threatening anaphylactic reactions have occurred in some patients during and up to 24 hours after ELAPRASE infusions. Anaphylaxis, presenting as respiratory distress, hypoxia, hypotension, urticaria and/or angioedema of throat or tongue have been reported to occur during and after ELAPRASE infusions, regardless of duration of the course of treatment. Closely observe patients during and after ELAPRASE administration and be prepared to manage anaphylaxis. Inform patients of the signs and symptoms of anaphylaxis and have them seek immediate medical care should symptoms occur. Patients with compromised respiratory function or acute respiratory disease may be at risk of serious acute exacerbation of their respiratory compromise due to hypersensitivity reactions and require additional monitoring [see Warnings and Precautions (5.1, 5.3) and Adverse Reactions (6)].
ELAPRASE is indicated for patients with Hunter syndrome (Mucopolysaccharidosis II, MPS II). ELAPRASE has been shown to improve walking capacity in patients 5 years and older.
In patients 16 months to 5 years of age, no data are available to demonstrate improvement in disease-related symptoms or long term clinical outcome; however, treatment with ELAPRASE has reduced spleen volume similarly to that of adults and children 5 years of age and older.
The safety and efficacy of ELAPRASE have not been established in pediatric patients less than 16 months of age [see Use in Specific Populations (8.4)].
Recommended Dose
The recommended dosage regimen of ELAPRASE is 0.5 mg per kg of body weight administered once weekly as an intravenous infusion.
Parenteral drug products should be inspected visually for particulate matter and discoloration prior to administration, whenever solution and container permit.
Preparation Instructions
Prepare and use ELAPRASE according to the following steps using aseptic technique:
- Determine the total volume of ELAPRASE to be administered and the number of vials needed based on the patient's weight and the recommended dose of 0.5 mg/kg.
Round up to the next whole vial to determine the total number of vials needed. Remove the required number of vials from the refrigerator to allow them to reach room temperature.Patient's weight (kg) × 0.5 mg per kg of ELAPRASE ÷ 2 mg per mL =
Total mL of ELAPRASETotal mL of ELAPRASE ÷ 3 mL per vial = Total number of vials - Before withdrawing the ELAPRASE solution from the vial, visually inspect each vial for particulate matter and discoloration. The ELAPRASE solution should be clear to slightly opalescent and colorless. Do not use if the solution is discolored or if there is particulate matter in the solution. Do not shake the ELAPRASE solution.
- Withdraw the calculated volume of ELAPRASE from the appropriate number of vials.
- Add the calculated volume of ELAPRASE solution to a 100 mL bag of 0.9% Sodium Chloride Injection, USP for intravenous infusion.
- Mix gently. Do not shake the solution.
Administration Instructions
Administer the diluted ELAPRASE solution to patients using a low-protein-binding infusion set equipped with a low-protein-binding 0.2 micrometer (µm) in-line filter.
The total volume of infusion should be administered over a period of 3 hours, which may be gradually reduced to 1 hour if no hypersensitivity reactions are observed. Patients may require longer infusion times if hypersensitivity reactions occur; however, infusion times should not exceed 8 hours. The initial infusion rate should be 8 mL per hour for the first 15 minutes. If the infusion is well tolerated, the rate of infusion may be increased by 8 mL per hour increments every 15 minutes. The infusion rate should not exceed 100 mL per hour. The infusion rate may be slowed, temporarily stopped, or discontinued for that visit in the event of hypersensitivity reactions [see Warnings and Precautions (5.1)]. ELAPRASE should not be infused with other products in the infusion tubing.
Storage and Stability
ELAPRASE does not contain preservatives; therefore, after dilution with saline, the infusion bags should be used immediately. If immediate use is not possible, the diluted solution should be stored refrigerated at 2°C to 8°C (36°F to 46 °F) for up to 24 hours. Other than during infusion, do not store the diluted ELAPRASE solution at room temperature. Any unused product or waste material should be discarded and disposed of in accordance with local requirements.
Injection: 6 mg/3 mL (2 mg/mL) in single-use vials
Pregnancy
Risk Summary
There are no adequate and well-controlled studies with ELAPRASE use in pregnant women. Available data from a small number of postmarketing cases with ELAPRASE use in pregnancy are insufficient to inform drug-associated risks for major birth defects, miscarriage, or adverse maternal or fetal outcomes. In an animal reproduction study, no evidence of adverse effects on pre- and post-natal development was observed with twice weekly intravenous administration of idursulfase to pregnant rats from gestation day 6 through lactation day 19 at about 4 times the recommended human weekly dose of 0.5 mg/kg based on body surface area (see Data).
The estimated background risk of major birth defects and miscarriage for the indicated population is unknown. All pregnancies have a background risk of birth defect, loss, or other adverse outcomes. In the U.S. general population, the estimated background risk of major birth defects and miscarriage in clinically recognized pregnancies is 2% to 4% and 15% to 20%, respectively.
Data
Animal Data
In a pre- and post-natal development study, idursulfase was administered to pregnant rats twice weekly, intravenously, from gestation day 6 through lactation day 19. No significant adverse effects on pre- and post-natal development of the offspring were observed at twice weekly intravenous doses up to 12.5 mg/kg (about 4 times the recommended human weekly dose of 0.5 mg/kg based on body surface area).
Lactation
Risk Summary
There are no data on the presence of idursulfase in human milk, the effects on the breastfed infant, or the effects on milk production. Idursulfase was excreted in breast milk of lactating rats (see Data). When a drug is present in animal milk, it is likely that the drug will be present in human milk. The developmental and health benefits of breastfeeding should be considered along with the mother's clinical need for ELAPRASE and any potential adverse effects on the breastfed child from ELAPRASE or from the underlying maternal condition.
Data
Idursulfase was excreted in breast milk of lactating rats at a concentration higher (4- to 5-fold) than the maximum blood concentration, when administered as a single intravenous dose up to 2.5 mg/kg. The concentration of drug in animal milk does not necessarily predict the concentration of drug in human milk.
Pediatric Use
Clinical trials with ELAPRASE were conducted in 96 patients with Hunter syndrome, ages 5 to 31 years old, with the majority of the patients in the pediatric age group (median age 15 years old). In addition, an open-label, uncontrolled clinical trial was conducted in 28 patients with Hunter syndrome, ages 16 months to 7.5 years old. Patients 16 months to 5 years of age demonstrated reduction in spleen volume that was similar to that of adults and children 5 years and older. However, there are no data to support improvement in disease-related symptoms or long term clinical outcome in patients 16 months to 5 years of age [see Clinical Studies (14)].
The safety and effectiveness of ELAPRASE have not been established in pediatric patients less than 16 months of age.
Geriatric Use
Clinical studies of ELAPRASE did not include patients older than 31 years of age. It is not known whether older patients respond differently from younger patients.
None.