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Lumizyme

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Lumizyme

Lumizyme

INDICATIONS

LUMIZYME (alglucosidase alfa) [see DESCRIPTION] is a lysosomal glycogen-specific enzyme indicated for patients 8 years and older with late (non-infantile) onset Pompe disease (acid a-glucosidase (GAA) deficiency) who do not have evidence of cardiac hypertrophy. The safety and efficacy of LUMIZYME have not been evaluated in controlled clinical trials in infantileonset patients, or in late (non-infantile) onset patients less than 8 years of age [see Use in Specific Populations].

DOSAGE AND ADMINISTRATION

Recommended Dose

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

Instructions For Use

LUMIZYME does not contain any preservatives. Vials are single-use only. Discard any unused product.

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, 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. 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 1mg/kg/hr (mL/hr) Step 2 3mg/kg/hr (mL/hr) Step 3 5mg/kg/hr (mL/hr) Step 4 7mg/kg/hr (mL/hr)
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 1,000 50 150 250 350

Reconstitution, Dilution, And Administration

LUMIZYME should be reconstituted, diluted and administered by a healthcare professional.

Use aseptic technique during preparation. Do not use filter needles during preparation.

  1. 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 (68 kg) x dose (20 mg/kg) = patient dose (1,360 mg)
    1,360 mg divided by 50 mg/vial = 27.2 vials; therefore, 28 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).
  2. Reconstitute each LUMIZYME vial by slowly injecting 10.3 mL of Sterile Water for Injection, USP to the inside wall of each vial. Each vial will yield a concentration of 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.
  3. The reconstituted LUMIZYME solution should be protected from light.
  4. 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.
  5. LUMIZYME should be diluted in 0.9% Sodium Chloride for Injection, USP, immediately after reconstitution, to a final LUMIZYME concentration of 0.5 to 4 mg/mL. See Table 1 for the recommended total infusion volume based on patient weight.
  6. Slowly withdraw the reconstituted solution from each vial. Avoid foaming in the syringe.
  7. Remove airspace from the infusion bag to minimize particle formation due to the sensitivity of LUMIZYME to air-liquid interfaces.
  8. Add the reconstituted LUMIZYME 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.
  9. Gently invert or massage the infusion bag to mix. Do not shake.
  10. Administer LUMIZYME using an in-line low protein binding 0.2 ^m filter.
  11. Do not infuse LUMIZYME in the same intravenous line with other products.

LUMIZYME does not contain any preservatives. Vials are single-use only. Discard any unused product.

HOW SUPPLIED

Dosage Forms And Strengths

LUMIZYME is supplied as a sterile, nonpyrogenic, white to offwhite, 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.

Storage And Handling

LUMIZYME 50 mg vials are supplied as a sterile, nonpyrogenic, white to off-white lyophilized cake or powder. LUMIZYME is supplied in single-use, clear Type I glass 20 mL (cc) vials. The closure consists of a siliconized butyl stopper and an aluminum seal with a plastic flip-off cap.

Store LUMIZYME under refrigeration between 2° to 8°C (36° to 46°F). Do not use LUMIZYME after the expiration date on the vial.

The reconstituted and diluted solution should be administered without delay. If immediate use is not possible, the reconstituted and diluted solution is stable for up to 24 hours at 2° to 8°C (36° to 46°F). Storage of the reconstituted solution at room temperature is not recommended. The reconstituted and diluted LUMIZYME solution should be protected from light. Do not freeze or shake.

LUMIZYME does not contain any preservatives. Vials are singleuse only. Discard any unused product.

NDC 58468-0160-1 (Carton of one single-use vial)
NDC 58468-0160-2 (Carton of ten single-use vials)

LUMIZYME is manufactured and distributed by: Genzyme Corporation, 500 Kendall Street, Cambridge, MA 02142, 1-800-745-4447 (phone). Revised: Nov 2013

Last reviewed on RxList: 7/14/2014
This monograph has been modified to include the generic and brand name in many instances.

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