DOSAGE AND ADMINISTRATION
Recommended Dose
The recommended dosage regimen of MYOZYME is 20 mg/kg
body weight administered every 2 weeks as an intravenous
infusion.
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 |
Reconstitution, dilution and administration
MYOZYME should be reconstituted, diluted and
administered by a health care professional.
Use aseptic technique during
preparation. Do not use filter needles during
preparation.
-
Determine the number of vials to be reconstituted
based on the individual patient's weight
and the recommended dose of 20 mg/kg.
Patient weight (kg) x
dose (mg/kg) = patient dose (in mg)
Patient
dose (in mg) divided by 50 mg/vial = number of vials
to reconstitute. If the number of vials
includes a fraction, round up to the next whole
number.
Example: Patient weight (16 kg) x
dose (20 mg/kg) = patient dose (320 mg)
320
mg divided by 50 mg/vial = 6.4 vials; therefore, 7
vials should be reconstituted
Remove the
required number of vials from the refrigerator and
allow them to reach room temperature prior to
reconstitution (approximately 30 minutes).
-
Reconstitute each MYOZYME vial by
slowly
injecting 10.3 mL of Sterile Water for
Injection, USP to the inside wall of each
vial. Each vial will yield 5
mg/mL. The total extractable dose per vial
is 50 mg per 10 mL. Avoid forceful impact
of the water for injection on the powder and avoid
foaming. This is done by slow drop-wise
addition of the water for injection down the inside
of the vial and not directly onto the lyophilized
cake. Tilt and roll each vial gently. Do
not invert, swirl, or shake.
-
The reconstituted MYOZYME solution should be
protected from light.
-
Perform an immediate visual inspection on the
reconstituted vials for particulate matter and
discoloration. If upon immediate
inspection opaque particles are observed or if the
solution is discolored do not use. The
reconstituted solution may occasionally contain some
alglucosidase alfa particles (typically less than 10
in a vial) in the form of thin white strands or
translucent fibers subsequent to the initial
inspection. This may also happen following
dilution for infusion. These particles
have been shown to contain alglucosidase alfa and
may appear after the initial reconstitution step and
increase over time. Studies have shown
that these particles are removed via in-line
filtration without having a detectable effect on the
purity or strength.
-
MYOZYME should be diluted in 0.9% Sodium
Chloride for Injection, USP, immediately after
reconstitution, to a final MYOZYME concentration of
0.5 to 4 mg/mL. See Table 1 for the recommended total
infusion volume based on patient weight.
-
Slowly withdraw the reconstituted solution from
each vial. Avoid foaming in the syringe.
-
Remove airspace from the infusion bag to minimize
particle formation due to the sensitivity of MYOZYME
to air-liquid interfaces.
-
Add the reconstituted MYOZYME solution slowly and
directly into the sodium chloride
solution. Do not add directly into
airspace that may remain within the infusion
bag. Avoid foaming in the infusion bag.
-
Gently invert or massage the infusion bag to
mix. Do not shake.
-
Administer MYOZYME using an in-line low protein binding 0.2 µm filter.
MYOZYME should not be infused in the same
intravenous line with other products.
Myozyme does not contain any preservatives. Vials are single-use only. Discard any unused product.
DOSAGE FORMS AND STRENGTHS
MYOZYME is supplied as a sterile, nonpyrogenic, white to
off-white, lyophilized cake or powder for reconstitution with Sterile
Water for Injection, USP to yield a concentration of 5 mg/mL; and then
further diluted with 0.9% Sodium Chloride for Injection, USP for
intravenous infusion.
Single use vials are available in 50 mg dosage only.
|
