Microsoft word - uk replagal pi mar 2012 final.doc

Replagal (agalsidase alfa) Prescribing
Information: Please consult the Summary
patient receiving Replagal. Extensive renal of Product Characteristics (SmPC) before Presentation: Concentrate solution for IV
infusion. 1ml of concentrate for solution for infusion contains 1mg of agalsidase alfa. Indication: Long-term enzyme intracellular a-galactosidase activity.
replacement therapy in patients with a
Side effects: Most reported adverse
Dosage and administration: Replagal
effects have been mild to moderate. Very 0.2mg/kg body weight by IV infusion over common (>1/10 patients): headache, pain/discomfort, fatigue; common (>1/100, <1/10 patients): peripheral oedema, month study with Replagal administered at tinnitus, tinnitus aggravated, tachycardia, 0.2mg/kg, this dose regimen is suggested for children between 7–18 years of age. Contraindications: Hypersensitivity to the
active substance or any of the excipients. throat secretion, rhinorrhoea, diarrhoea, Warnings and precautions: 13.7% of
vomiting, abdominal pain/discomfort, acne, adult patients receiving Replagal in clinical erythema, pruritus, rash, livedo reticularis, trials had idiosyncratic infusion-related musculoskeletal discomfort, myalgia, back reactions (generally within 2–4 months of [after 1 year] has been reported as well). fatigue, feeling hot, feeling cold, asthenia, Four of 17 paediatric patients >7 years of chest pain, chest tightness, influenza-like age and 3 of 8 paediatric patients <7 years experienced at least one infusion reaction decreased corneal reflex; uncommon (>1/1000, <1/100 patients): parosmia, angioneurotic oedema, urticaria, sensation saturation. Not known (since derived from attention immediately. The infusion can be temporarily interrupted (for 5–10 minutes) cardiac arrhythmias (atrial fibrillation, until symptoms subside. If severe allergic heart failure, hypotension, hyperhidrosis. initiate appropriate treatment. A review of Infusion related reactions reported in the arrhythmias (atrial fibrillation, ventricular events in patients with pre-existing cardiac manifestations of Fabry disease. Patients myocardial ischemia, and heart failure in may develop IgG antibodies to the protein. patients with Fabry disease involving the A low titre antibody response was seen in hyperhidrosis, and hypotension. The most negative throughout. In paediatric patients >7 yrs of age, 1/16 male patients tested pyrexia, flushing, headache, nausea, and antibodies. No increase in the incidence of Package quantity and price: Vials of 5ml
patient. In paediatric patients <7 yrs of (containing 3.5ml concentrate) in a pack age, 0/7 male patients tested positive for size of 1 vial. Price: £1049.94 for one 5ml IgG anti-agalsidase alfa antibodies. No IgE Pharmaceutical precautions: Store in a
Legal category: POM. Further information
Marketing authorisation number and
holder: EU/1/01/189/001-006. Shire
from the marketing authorisation holder.
Date of preparation: 30th March 2012
Item code: UK/HG/REP/12/0015
Adverse events should be reported. Reporting forms and information can be found at www.mhra.gov.uk/yellowcard. Adverse events should also be reported to Shire Human Genetic Therapies on +44 (0)1256 894000 or faxed on +44 (0)1256 894715 or emailed to GlobalPharmacovigilance@shire.com

Source: http://www.replagal.co.uk/resources/UK_PI.pdf