Vpriv

VPRIV treatment should be supervised by a physician experienced in the management of patients with Gaucher disease. Posology The recommended dose is 60 Units/kg administered every other week. Dose adjustments can be made on an individual basis based on achievement and maintenance of therapeutic goals.

Clinical studies have evaluated doses ranging from 15 to 60 Units/kg every other week. Doses higher than 60 Units/kg have not been studied. Patients currently treated with imiglucerase enzyme replacement therapy for type 1 Gaucher disease may be switched to VPRIV, using the same dose and frequency.

1). 2). 2). Paediatric population Twenty of the 94 patients (21%) who received velaglucerase alfa during clinical studies were in the paediatric and adolescent age range (4 to 17 years). 1 for further information). Data on home infusion in the paediatric population by the patient or a patient’s caregiver are very limited.

The safety and efficacy of velaglucerase alfa in children below the age of 4 years have not yet been established. No data are available. Method of administration For intravenous infusion use only. To be administered as a 60-minute intravenous infusion.

22 μm filter. e. administration by the patient’s caregiver or by the patients themselves) may be considered for patients who have received at least three infusions and who tolerated their infusions well. The decision to have a patient move to home infusion should be made after evaluation and recommendation by the treating physician.

Self-administration in particular should be closely monitored by the treating physician and should occur in the presence of a responsible adult. The treating physician has to make sure that the one who will administer velaglucerase alfa in the home setting is appropriately trained.

Dose and infusion rate should remain constant while at home, and not be changed without supervision of the treating physician. Appropriate medical support, including personnel adequately trained in emergency measures, should be readily available when velaglucerase alfa is administered.

4). Subsequent infusions may need to occur in a clinical setting. 6.

1%). 4%). 4 for further information). The only adverse reaction leading to discontinuation of treatment was an infusion-related reaction. Tabulated list of adverse reactions Adverse reactions reported in patients with type 1 Gaucher disease are listed in Table 1.

Information is presented by system organ class and frequency according to MedDRA convention. Frequency is defined as very common (1/10), common (1/100 to <1/10), and uncommon (≥1/1 000 to <1/100). Within each frequency grouping, adverse reactions are presented in order of decreasing seriousness.

6 Table 1: Adverse reactions reported with VPRIV in patients with type 1 Gaucher disease System organ class Adverse reaction Very common Common Uncommon Immune system disorders hypersensitivity reactions (includes dermatitis allergic and anaphylactic*/anaphylactoid reactions) Nervous system disorders headache, dizziness Eye disorders vision blurred* Cardiac disorders tachycardia Respiratory, thoracic and mediastinal disorders dyspnoea* Vascular disorders hypertension, hypotension, flushing Gastrointestinal disorders abdominal pain/abdominal pain upper nausea vomiting* Skin and subcutaneous tissue disorders rash, urticaria, pruritus* Musculoskeletal and connective tissue disorders bone pain, arthralgia, back pain General disorders and administration site conditions infusion-related reaction, asthenia/fatigue, pyrexia/body temperature increased chest discomfort* Investigations activated partial thromboplastin time prolonged, neutralizing antibody positive *Adverse reactions derived from post-marketing reports Description of selected adverse reactions Vomiting In some cases vomiting can be serious and severe.

Vomiting most often occurs during the infusion and up to 24 hours after the infusion. Other special populations Elderly population (≥ 65 years) The safety profile of VPRIV in clinical studies involving patients aged 65 years and above was similar to that observed in other adult patients.

Traceability In order to improve the traceability of biological medicinal products, the name and batch number of the administered medicinal product should be clearly recorded. Hypersensitivity Hypersensitivity reactions, including symptoms consistent with anaphylaxis, have been reported in patients in clinical studies and in post-marketing experience.

The majority of hypersensitivity reactions usually occur up to 12 hours post infusion. The most frequently reported symptoms of 4 hypersensitivity include nausea, rash dyspnoea, back pain, chest discomfort (including chest tightness), urticaria, arthralgia, and headache.

Infusion-related reactions An infusion-related reaction is defined as any adverse drug reaction occurring within 24 hours after the initiation of velaglucerase alfa infusion. Infusion-related reactions (IRR) were the most commonly observed adverse reactions in patients treated in clinical studies.

An IRR often appears as a hypersensitivity reaction. The most frequently reported symptoms of hypersensitivity include nausea, rash, dyspnoea, back pain, chest discomfort (including chest tightness), urticaria, arthralgia, and headache.

Symptoms consistent with anaphylaxis have been reported in patients in clinical studies and in post-marketing experience. Apart from symptoms associated with hypersensitivity reactions IRRs might show as fatigue, dizziness, pyrexia, blood pressure increase, pruritus, vision blurred, or vomiting.

In treatment-naïve patients, the majority of infusion-related reactions occurred during the first 6 months of treatment. Prevention and management of infusion related reactions including hypersensitivity reactions The management of infusion-related reactions should be based on the severity of the reaction, and include slowing the infusion rate, treatment with medicinal products such as antihistamines, antipyretics and/or corticosteroids, and/or stopping and resuming treatment with increased infusion time.

1.