"The U.S. Food and Drug Administration today approved Dotarem (gadoterate meglumine) for use in magnetic resonance imaging (MRI) of the brain, spine and associated tissues of patients ages 2 years and older.
Dotarem is a gadolinium-based"...
ALDURAZYME® (laronidase) is indicated for patients with Hurler and Hurler-Scheie forms of Mucopolysaccharidosis I (MPS I) and for patients with the Scheie form who have moderate to severe symptoms. The risks and benefits of treating mildly affected patients with the Scheie form have not been established.
ALDURAZYME has been shown to improve pulmonary function and walking capacity. ALDURAZYME has not been evaluated for effects on the central nervous system manifestations of the disorder.
DOSAGE AND ADMINISTRATION
The recommended dosage regimen of ALDURAZYME is 0.58 mg/kg of body weight administered once weekly as an intravenous (IV) infusion. Pretreatment is recommended 60 minutes prior to the start of the infusion and may include antihistamines, antipyretics, or both [see Instructions for Use below and WARNINGS AND PRECAUTIONS].
Each vial of ALDURAZYME provides 2.9 milligrams (mg) of laronidase in 5.0 milliliters (mL) of solution and is intended for single use only. Do not use the vial more than one time. The concentrated solution for infusion must be diluted with 0.9% Sodium Chloride Injection, USP, to a final volume of 100 mL or 250 mL, using aseptic techniques. The final volume of the infusion is determined by the patient's body weight. Patients with a body weight of 20 kg or less should receive a total volume of 100 mL. Patients with a body weight greater than 20 kg should receive a total volume of 250 mL. For patients with underlying cardiac or respiratory compromise and weighing up to 30 kg, physicians may consider diluting ALDURAZYME in a volume of 100 mL and administering at a decreased infusion rate [see Instructions for Use below, WARNINGS AND PRECAUTIONS and ADVERSE REACTIONS].
Instructions for Use
Prepare and use ALDURAZYME according to the following steps. Use aseptic techniques. Prepare ALDURAZYME using low-protein-binding containers and administer with a low-protein-binding infusion set equipped with an in-line, low-protein-binding 0.2 micrometer (μm) filter. There is no information on the compatibility of diluted ALDURAZYME with glass containers.
- Determine the number of vials to be diluted based on the
patient's weight and the recommended dose of 0.58 mg/kg, using the following
Patient's weight (kg) x 1 mL/kg of ALDURAZYME = Total mL ALDURAZYME
Total mL ALDURAZYME ÷ 5 mL per Vial = Total number of Vials.
- Round up to the next whole vial. Remove the required number of vials from the refrigerator to allow them to reach room temperature. Do not heat or microwave vials.
- Before withdrawing the ALDURAZYME from the vial, visually inspect each vial for particulate matter and discoloration. The ALDURAZYME solution should be clear to slightly opalescent and colorless to pale yellow. Some translucency may be present in the solution. Do not use if the solution is discolored or if there is particulate matter in the solution.
- Withdraw and discard a volume of the 0.9% Sodium Chloride Injection, USP, from the infusion bag, equal to the volume of ALDURAZYME concentrate to be added.
- Slowly withdraw the calculated volume of ALDURAZYME from the appropriate number of vials using caution to avoid excessive agitation. Do not use a filter needle, as this may cause agitation. Agitation may denature ALDURAZYME, rendering it biologically inactive.
- Slowly add the ALDURAZYME solution to the 0.9% Sodium Chloride Injection, USP, using care to avoid agitation of the solutions. Do not use a filter needle.
- Gently rotate the infusion bag to ensure proper distribution of ALDURAZYME. Do not shake the solution.
- The entire infusion volume (100 mL for patients weighing 20 kg or less and 250 mL for patients weighing greater than 20 kg) should be delivered over approximately 3 to 4 hours. The initial infusion rate of 10 μg/kg/hr may be incrementally increased every 15 minutes during the first hour, as tolerated, until a maximum infusion rate of 200 μg/kg/hr is reached. The maximum rate is then maintained for the remainder of the infusion (2-3 hours), as outlined in Tables 1 and 2.
- Administer the diluted ALDURAZYME solution to patients using a low-protein-binding infusion set equipped with a low-protein-binding 0.2 μm in-line filter.
Table 1: Incremental Rates for 100 mL ALDURAZYME® Infusion
(For use with Patients Weighing 20 kg or Less)
|Infusion Rate||Criteria for Increasing Infusion Rate|
|2 mL/hr x 15 minutes (10 μg/kg/hr)||Obtain vital signs, if stable then increase the rate to...|
|4 mL/hr x 15 minutes (20 μg/kg/hr)||Obtain vital signs, if stable then increase the rate to.|
|8 mL/hr x 15 minutes (50 μg/kg/hr)||Obtain vital signs, if stable then increase the rate to.|
|16 mL/hr x 15 minutes (100 μg/kg/hr)||Obtain vital signs, if stable then increase the rate to.|
|32 mL/hr x ~3 hours (200 μg/kg/hr)||For the remainder of the infusion.|
Table 2: Incremental Rates for 250 mL ALDURAZYME® Infusion
(For use with Patients Weighing Greater than 20 kg)
|Infusion Rate||Criteria for Increasing Infusion Rate|
|5 mL/hr x 15 minutes (10 μg/kg/hr)||Obtain vital signs, if stable then increase the rate to...|
|10 mL/hr x 15 minutes (20 μg/kg/hr)||Obtain vital signs, if stable then increase the rate to.|
|20 mL/hr x 15 minutes (50 μg/kg/hr)||Obtain vital signs, if stable then increase the rate to.|
|40 mL/hr x 15 minutes (100 μg/kg/hr)||Obtain vital signs, if stable then increase the rate to.|
|80 mL/hr x ~3 hours (200 μg/kg/hr)||For the remainder of the infusion.|
ALDURAZYME does not contain any 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° to 8°C (36° to 46°F) for up to 36 hours. Other than during infusion, room temperature storage of diluted solution is not recommended. Any unused product or waste material should be discarded and disposed of in accordance with local requirements.
ALDURAZYME must not be administered with other medicinal products in the same infusion. The compatibility of ALDURAZYME in solution with other products has not been evaluated.
Dosage Forms And Strengths
ALDURAZYME is supplied as a sterile solution in 5 mL vials (2.9 mg per 5 mL).
Storage And Handling
ALDURAZYME is supplied as a sterile solution in single-use, clear Type I glass 5 mL vials, containing 2.9 mg laronidase per 5 mL solution. The closure consists of a siliconized butyl stopper and an aluminum seal with a plastic flip-off cap.
NDC 58468-0070-1, 5 mL vial
Refrigerate vials of ALDURAZYME at 2° to 8°C (36° to 46°F). Do not freeze or shake. Protect from light. Do not use ALDURAZYME after the expiration date on the vial. This product contains no preservatives.
BioMarin Pharmaceutical Inc., Novato, CA 94949. Distributed by: Genzyme Corporation, 500 Kendall Street, Cambridge, MA 02142. Revised April 2013.This monograph has been modified to include the generic and brand name in many instances.
Last reviewed on RxList: 4/18/2013
Additional Aldurazyme Information
Report Problems to the Food and Drug Administration
You are encouraged to report negative side effects of prescription drugs to the FDA. Visit the FDA MedWatch website or call 1-800-FDA-1088.
Find out what women really need.