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The recommended dosage of Fabrazyme is 1 mg/kg body weight infused every two weeks as an intravenous (IV) infusion. Patients should receive antipyretics prior to infusion.

The initial IV infusion rate should be no more than 0.25 mg/min (15 mg/hr). The infusion rate may be slowed in the event of infusion reactions. After patient tolerance to the infusion is well established, the infusion rate may be increased in increments of 0.05 to 0.08 mg/min (increments of 3 to 5 mg/hr) with each subsequent infusion. For patients weighing < 30 kg, the maximum infusion rate should remain at 0.25 mg/min (15 mg/hr). For patients weighing ≥ 30 kg, the administration duration should not be less than 1.5 hours (based on individual patient tolerability).

Patients who have had a positive skin test to Fabrazyme or who have tested positive for anti-Fabrazyme IgE may be successfully re-challenged with Fabrazyme. The initial re-challenge administration should be a low dose at a lower infusion rate, e.g., 1/2 the therapeutic dose (0.5 mg/kg) at 1/25 the initial standard recommended rate (0.01 mg/min).

Once a patient tolerates the infusion, the dose may be increased to reach the approved dose of 1 mg/kg and the infusion rate may be increased by slowly titrating upwards (doubled every 30 minutes up to a maximum rate of 0.25 mg/min), as tolerated.

Cerezyme® (imiglucerase for injection) is administered by intravenous infusion over 1–2 hours. Dosage should be individualized to each patient.

Initial dosages range from 2.5 U/kg of body weight 3 times a week to 60U/kg once every 2 weeks. 60 U/kg every 2 weeks is the dosage for which the most data are available. Disease severity may dictate that treatment be initiated at a relatively high dose or relatively frequent administration.

Dosage adjustments should be made on an individual basis and may increase or decrease, based on achievement of therapeutic goals as assessed by routine comprehensive evaluations of the patient’s clinical manifestations.

60 Units/kg administered every other week as a 60-minute intravenous infusion.

Patients currently being treated with imiglucerase for Gaucher disease can be switched to VPRIV. Patients previously treated on a stable dose of imiglucerase are recommended to begin treatment with VPRIV at that same dose when they switch from imiglucerase to VPRIV.

Physicians can make dosage adjustments based on achievement and maintenance of each patient’s therapeutic goals. Clinical trials have evaluated doses ranging from 15 Units/kg to 60 Units/kg every other week.

The recommended dose is 60 Units/kg of body weight administered once every 22 weeks as a 60–120 minute intravenous infusion.

Patients currently being treated with imiglucerase for Type 1 Gaucher disease can be switched to ELELYSO. Patients previously treated on a stable dose of imiglucerase are recommended to begin treatment with ELELYSO at that same dose when they switch from imiglucerase to ELELYSO.

Dosage adjustments can be made based on achievement and maintenance of each patient’s therapeutic goals. Clinical studies have evaluated dose ranges from 11 Units/kg to 73 Units/kg every other week.

The initial infusion rate should be 11.3 mL/min. After patient tolerability to the infusion rate is established, the rate of infusion may be increased to 2.3 mL/min. The total volume of the infusion solution should be delivered over a period of no less than 1 hour.

The recommended dosage regimen of Myozyme is 20 mg/kg body weight administered every 2 weeks as an intravenous infusion. The total volume of infusion is determined by the patient’s body weight and should be administered over approximately 4 hours.

Infusions should be administered in a step-wise manner using an infusion pump. The initial infusion rate should be no more than 1 mg/kg/hr. The infusion rate may be increased by 2 mg/kg/hr every 30 minutes, after patient tolerance to the infusion rate is established, until a maximum rate of 7 mg/kg/hr is reached. Vital signs should be obtained at the end of each step. If the patient is stable, Myozyme may be administered at the maximum rate of 7 mg/kg/hr until the infusion is completed. The infusion rate may be slowed and/or temporarily stopped in the event of infusion reactions.

The recommended dosage of Lumizyme is 20 mg/kg body weight administered every 2 weeks as an intravenous infusion.

The total volume of infusion is determined by the patient’s body weight and should be administered over approximately 4 hours. Infusions should be administered in a step-wise manner using an infusion pump.

The initial infusion rate should be no more than mg/kg/hr. The infusion rate may be increased by 2 mg/kg/hr every 30 minutes, after patient tolerance to the infusion rate is established, until a maximum rate of 7 mg/kg/hr is reached. Vital signs should be obtained at the end of each step. If the patient is stable, Lumizyme may be administered at the maximum rate of 7 mg/kg/hr until the infusion is completed. The infusion rate may be slowed or temporarily stopped in the event of infusion reactions.

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.

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.

The recommended dosage regimen of Elaprase is 0.5 mg/kg of body weight administered every week as an intravenous infusion.

Elaprase is a concentrated solution for intravenous infusion.

The total volume of infusion may be administered over a period of 1 to 3 hours. Patients may require longer infusion times due to infusion reactions; however, infusion times should not exceed 8 hours.

The initial infusion rate should be 8 mL/hr for the first 15 minutes. If the infusion is well tolerated, the rate may be increased by 8 mL/hr increments at 15 minute intervals in order to administer the full volume within the desired period of time. However, at no time should the infusion rate exceed 100 mL/hr. The infusion rate may be slowed and/or temporarily stopped, or discontinued for that visit, based on clinical judgment, if infusion reactions were to occur. Elaprase should not be infused with other products in the infusion tubing.

The recommended dosage regimen of Naglazyme is 1 mg per kg of body weight administered once weekly as an intravenous infusion.

Pretreatment with antihistamines with or without antipyretics is recommended 30 to 60 minutes prior to the start of the infusion.

The total volume of the infusion should be delivered over a period of time of no less than 4 hours. The initial infusion rate should be 6 mL per hour for the first hour. If the infusion is well tolerated, the rate of infusion may be increased to 80 mL per hour for the remaining 3 hours. The infusion time can be extended up to 20 hours if infusion reactions occur.

For patients 20 kg and under or those who are susceptible to fluid volume overload, physicians may consider diluting Naglazyme in a volume of 100 mL. The infusion rate (mL per min) should be decreased so that the total infusion duration remains no less than 4 hours.