VPRIV

What it is

VPRIV is velaglucerase alfa ghu supplied in 400 U/vial (10 mg). It is a powder for solution for infusion, supplied as a sterile, white to off-white, preservative-free lyophilised powder in single-use vials.

Approved indications

— Long-term enzyme replacement therapy for paediatric and adult patients with type 1 Gaucher disease.

Dosing overview

The recommended dose is 60 U/kg administered every other week as a 60-minute intravenous infusion. Dose adjustments can be made on an individual basis based on achievement and maintenance of therapeutic goals. Patients currently being treated with other enzyme replacement therapy for type 1 Gaucher disease may be switched to VPRIV using the same dose and frequency.

Key safety warnings

Hypersensitivity reactions including symptoms consistent with anaphylaxis have been reported in patients in clinical studies and in post-marketing experience. As with any intravenous protein product, hypersensitivity reactions are possible, therefore appropriate medical support should be readily available when VPRIV is administered. Infusion-related reactions were the most commonly reported adverse reactions, occurring in approximately 62% (58/94) of patients treated with VPRIV in clinical studies. Most of the infusion-related reactions were mild. The most commonly observed symptoms of infusion-related reactions were: headache, dizziness, hypotension, hypertension, nausea, fatigue/asthenia, and pyrexia/body temperature increased. In treatment-naïve patients, the majority of infusion-related reactions occurred during the first six months of treatment with VPRIV. The management of infusion-related reactions should be based on the severity of the reaction, and include slowing the infusion rate, treatment with medications such as antihistamines, antipyretics and/or corticosteroids, and/or stopping and resuming treatment with increased infusion time. Patients with respiratory symptoms should be evaluated for the presence of pulmonary hypertension.

Contraindications

Hypersensitivity to the active substance or to any of the excipients.

Regulatory history

VPRIV was first listed on the ARTG as ARTG 180965 on 29 February 2012. The TGA approved VPRIV for long-term enzyme replacement therapy in paediatric and adult patients with type 1 Gaucher disease associated with specific clinical manifestations, based on a positive assessment of its quality, safety, and efficacy.