VPRIV

VPRIV- velaglucerase alfa injection, powder, lyophilized, for solution
Shire Human Genetics Therapies, Inc.

VPRIV™ (velaglucerase alfa for injection)

1 INDICATIONS AND USAGE

VPRIV (velaglucerase alfa for injection) is a hydrolytic lysosomal glucocerebroside-specific enzyme indicated for long-term enzyme replacement therapy (ERT) for pediatric and adult patients with type 1 Gaucher disease.

2 DOSAGE AND ADMINISTRATION

2.1 Recommended Dose

The recommended dose is 60 Units/kg administered every other week as a 60-minute intravenous infusion.

Patients currently being treated with imiglucerase for type 1 Gaucher disease may 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.

Dosage adjustments can be made based on achievement and maintenance of each patient’s therapeutic goals. Clinical studies have evaluated doses ranging from 15 Units/kg to 60 Units/kg every other week.

VPRIV should be administered under the supervision of a healthcare professional.

2.2 Preparation and Administration Instructions

Use aseptic technique

VPRIV is a lyophilized powder, which requires reconstitution and dilution, and is intended for intravenous infusion only. VPRIV contains no preservatives and vials are single-use only. Discard any unused solution. VPRIV should be prepared as follows:

Determine the number of vials to be reconstituted based on the individual patient’s weight and the prescribed dose. Follow the instructions in Table 1 for reconstitution.

Table 1: Reconstitution Instructions
200 Units/vial 400 Units/vial
Volume of Sterile Water for Injection, USP, for reconstitution 2.2 mL 4.3 mL
Concentration after reconstitution 100 Units/mL 100 Units/mL
Withdrawal volume 2 mL 4 mL

Upon reconstitution, mix vials gently. DO NOT SHAKE. Prior to further dilution, visually inspect the solution in the vials; the solution should be clear to slightly opalescent and colorless; do not use if the solution is discolored or if foreign particulate matter is present. Withdraw the calculated volume of drug from the appropriate number of vials and dilute the total volume required in 100 mL of 0.9% sodium chloride solution suitable for IV administration. Mix gently. DO NOT SHAKE.

VPRIV should be administered over 60 minutes. VPRIV should not be infused with other products in the same infusion tubing as the compatibility in solution with other products has not been evaluated. The diluted solution should be filtered through an in-line low protein-binding 0.2µm filter during administration.

As VPRIV contains no preservatives, once reconstituted the product should be used immediately. If immediate use is not possible, the reconstituted or diluted product may be stored for up to 24 hours at 2 to 8°C (36 to 46°F). Do not freeze. Protect from light. The infusion should be completed within 24 hours of reconstitution of vials.

3 DOSAGE FORMS AND STRENGTHS

VPRIV is a sterile, white to off-white, lyophilized powder for reconstitution with Sterile Water for Injection, USP, to yield a final concentration of 100 Units/mL.

VPRIV is available as 200 Units and 400 Units single-use vials.

4 CONTRAINDICATIONS

None.

5 WARNINGS AND PRECAUTIONS

5.1 Hypersensitivity Reactions

Hypersensitivity reactions have been reported in patients in clinical studies with VPRIV [see Adverse Reactions (6.1) ]. As with any intravenous protein product, hypersensitivity reactions are possible, therefore appropriate medical support should be readily available when VPRIV is administered. If a severe reaction occurs, current medical standards for emergency treatment are to be followed.

Treatment with VPRIV should be approached with caution in patients who have exhibited symptoms of hypersensitivity to the active ingredient or excipients in the drug product or to other enzyme replacement therapy.

5.2 Infusion-related Reactions

Infusion-related reactions were the most commonly observed adverse reactions in patients treated with VPRIV in clinical studies. The most commonly observed symptoms of infusion-related reactions were: headache, dizziness, hypotension, hypertension, nausea, fatigue/asthenia, and pyrexia. Generally the infusion-related reactions were mild and, in treatment-naïve patients, onset occurred mostly during the first 6 months of treatment and tended to occur less frequently with time.

The management of infusion-related reactions should be based on the severity of the reaction, e.g. slowing the infusion rate, treatment with medications such as antihistamines, antipyretics and/or corticosteroids, and/or stopping and resuming treatment with increased infusion time.

Pre-treatment with antihistamines and/or corticosteroids may prevent subsequent reactions in those cases where symptomatic treatment was required. Patients were not routinely pre-medicated prior to infusion of VPRIV during clinical studies.

6 ADVERSE REACTIONS

6.1 Clinical Studies Experience

The data described below reflect exposure of 94 patients with type 1 Gaucher disease who received VPRIV at doses ranging from 15 Units/kg to 60 Units/kg every other week in 5 clinical studies. Fifty-four (54) patients were naïve to ERT and received VPRIV for 9 months and 40 patients switched from imiglucerase to VPRIV treatment and received VPRIV for 12 months [see Clinical Studies (14) ]. Patients were between 4 and 71 years old at time of first treatment with VPRIV, and included 46 male and 48 female patients.

The most serious adverse reactions in patients treated with VPRIV were hypersensitivity reactions [see Warnings and Precautions (5.1) ].

The most commonly reported adverse reactions (occurring in ≥10% of patients) that were considered related to VPRIV are shown in Table 2. The most common adverse reactions were infusion-related reactions.

Because clinical trials are conducted under widely varying conditions, adverse reaction rates observed in the clinical trials of a drug cannot be directly compared to rates in the clinical trials of another drug and may not reflect the rates observed in practice.

Table 2: Adverse Reactions Observed in ≥ 10% of Patients with Type 1 Gaucher Disease Treated with VPRIV
*
Denotes any event considered related to and occurring within up to 24 hours of VPRIV infusion
System Organ Class Preferred Term Naïve to ERTN = 54 Switched from imiglucerase to VPRIVN = 40
Number of Patients (% )
Nervous system disorders
Headache 19 (35.2) 12 (30)
Dizziness 12 (22.2) 3 (7.5)
Gastrointestinal disorders
Abdominal pain 10 (18.5) 6 (15)
Nausea 3 (5.6) 4 (10)
Musculoskeletal and connective tissue disorders
Back pain 9 (16.7) 7 (17.5)
Joint pain (knee) 8 (14.8) 3 (7.5)
Infections and infestations
Upper respiratory tract infection 17 (31.5) 12 (30)
Investigations
Activated partial thromboplastin time prolonged 6 (11.1) 2 (5)
General disorders and administration site conditions
Infusion-related reaction * 28 (51.9) 9 (22.5)
Pyrexia 12 (22.2) 5 (12.5)
Asthenia/Fatigue 7 (13) 5 (12.5)

Less common adverse reactions affecting more than one patient (>3% in the treatment-naïve group and >2% in patients switched from imiglucerase to VPRIV treatment) were bone pain, tachycardia, rash, urticaria, flushing, hypertension, and hypotension.

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