ALDURAZYME

Dosage and administration of Aldurazyme

The recommended dosage regimen of Aldurazyme is 0.58 mg/kg of body weight, and it should be administered once weekly as an intravenous (IV) infusion. Healthcare professionals recommend pretreatment with antipyretics and antihistamines for 60 minutes before starting the infusion.

• Instruction for use
  Prepare and use Aldurazyme according to the following steps:
  One should not use the Vial more than one time. The concentrated solution for infusion must be diluted in 0.9% Sodium Chloride Injection. One should prepare Aldurazyme using PVC containers and administer it with a PVC infusion set equipped with an in-line, low protein binding 0.2 micrometer (µm) filter.
• One should 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 equation:
  Patient’s weight (kg) x 1 mL/kg of Aldurazyme = Total mL Aldurazyme
  Total mL Aldurazyme ÷ 5 mL per Vial = Total number of Vials.
• You should round up to the nearest whole Vial. One should remove the required number of vials from the refrigerator to allow them to reach room temperature. Do not heat or microwave vials.
• One should inspect each Vial before withdrawing the Aldurazyme from the Vial for particulate matter and discoloration. The Aldurazyme solution should be clear to slightly opalescent and colorless to pale yellow( few translucent particles may be present). One should not use it if the solution is discolored or if there is particulate matter in the solution.
• One should withdraw and discard a volume of the 0.9% Sodium Chloride Injection from the infusion bag equal to the volume of Aldurazyme concentrate to be added.
• One should slowly withdraw the calculated volume of Aldurazyme from the appropriate number of vials using caution to avoid excessive agitation. One should not use a filter needle, as this may cause agitation. Agitation may denature the medicine, rendering it biologically inactive.
• You should slowly add the Aldurazyme solution to the 0.9% Sodium Chloride Injection, using care to avoid agitation of the solutions. Do not use a filter needle.
• One should gently rotate the infusion bag to ensure proper distribution of Aldurazyme and not shake the solution.
• One should deliver the entire infusion volume 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.

Note: 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.

Overdosage
There is no experience with overdoses of Aldurazyme. If someone takes an overdose, consult the doctor immediately and keep the patient under observation.

Side effects of Aldurazyme

• The common side-effects of Aldurazyme in at least 10% of patients 6 years and older are rash, upper respiratory tract infection, injection site reaction, hyperreflexia, paresthesia, and vein disorder.
• The common side-effects of Aldurazyme in at least 10% of patients under 6 years of age are: pyrexia, chills, increased blood pressure, tachycardia, and decreased oxygen saturation.

Warnings and Precautions

Anaphylaxis and Allergic Reactions
Healthcare professionals observed Anaphylaxis and severe allergic reactions in patients during or up to 3 hours after Aldurazyme infusions. If anaphylactic or other severe allergic reactions occur, immediately discontinue the infusion of Aldurazyme and initiate appropriate medical treatment.

Acute Respiratory Complications Associated with Administration

Patients with an acute febrile or respiratory illness at the time of Aldurazyme infusion may be at greater risk for infusion reactions. One should carefully consider the patient’s clinical status before administration of Aldurazyme and consider delaying Aldurazyme infusion.

Sleep apnea is common in MPS I patients. One should consider evaluation of airway patency before initiation of treatment with Aldurazyme. Patients using supplemental oxygen or continuous positive airway pressure (CPAP) during sleep should have these treatments readily available during infusion in the event of an infusion reaction or extreme drowsiness/sleep induced by antihistamine use.

Risk of Acute Cardiorespiratory Failure

One should exercise caution when administering Aldurazyme to patients susceptible to fluid overload or patients with acute underlying respiratory illness or compromised cardiac and respiratory function for whom fluid restriction is indicated. One should make available appropriate medical support and monitoring measures during Aldurazyme infusion. Some patients may require prolonged observation times based on the patient’s individual needs.

Patients should receive antipyretics and antihistamines before infusion because of the potential for infusion reactions. If an infusion reaction occurs, regardless of pretreatment, decreasing the infusion rate, temporarily stopping the infusion, or administering additional antipyretics and antihistamines may ameliorate the symptoms.

Handling and storage of Anaphylaxis

Aldurazyme is supplied as a sterile solution in clear Type I glass 5 mL vials (2.9 mg Laronidase per 5 mL).

• One should refrigerate the medicine at 2°C to 8°C (36°F to 46°F).
• Do not freeze or shake the solution.
• Do not use Aldurazyme after the expiration date on the Vial.