Myozyme

MYOZYME- alglucosidase alfa injection, powder, lyophilized, for solution
Genzyme Corporation

WARNING: ANAPHYLAXIS, SEVERE ALLERGIC AND IMMUNE MEDIATED REACTIONS and RISK OF CARDIORESPIRATORY FAILURE

Life-threatening anaphylactic, severe allergic and immune mediated reactions have been observed in some patients during MYOZYME® infusions. Therefore, appropriate medical support should be readily available when MYOZYME is administered. [see Warnings and Precautions (5.1) ]
Risk of Cardiorespiratory Failure Patients with compromised cardiac or respiratory function may be at risk for serious acute exacerbation of their cardiac or respiratory compromise due to infusion reactions, and require additional monitoring. [see Warnings and Precautions (5.3) ]

1 INDICATIONS AND USAGE

MYOZYME® (alglucosidase alfa) [see Description (11)]is a lysosomal glycogen-specific enzyme indicated for use in patients with Pompe disease (GAA deficiency). MYOZYME has been shown to improve ventilator-free survival in patients with infantile-onset Pompe disease as compared to an untreated historical control, whereas use of MYOZYME in patients with other forms of Pompe disease has not been adequately studied to assure safety and efficacy [see Clinical Studies (14) ].

2 DOSAGE AND ADMINISTRATION

2.1 Recommended Dose

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

2.2 Instructions for Use

MYOZYME does not contain any preservatives. Vials are single-use only. Any unused product should be discarded.

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. See Table 1 below for the rate of infusion at each step, expressed as mL/hr based on the recommended infusion volume by patient weight.

Table 1: Recommended Infusion Volumes and Rates
Patient Weight Range (kg) Total infusion volume (mL)

Step 1

1 mg/kg/hr

(mL/hr)

Step 2

3 mg/kg/hr

(mL/hr)

Step 3

5 mg/kg/hr

(mL/hr)

Step 4

7 mg/kg/hr

(mL/hr)
1.25 — 10 50 3 8 13 18
10.1 — 20 100 5 15 25 35
20.1 — 30 150 8 23 38 53
30.1 — 35 200 10 30 50 70
35.1 — 50 250 13 38 63 88
50.1 — 60 300 15 45 75 105
60.1 — 100 500 25 75 125 175
100.1 — 120 600 30 90 150 210
120.1 — 140 700 35 105 175 245
140.1 — 160 800 40 120 200 280
160.1 — 180 900 45 135 225 315
180.1 — 200 1000 50 150 250 350

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