PracticeUpdate Conference Series ISN WCN 2019

A simple blood test may explain unexplained end-stage renal disease

TEST

Test for Fabry disease in your patients with chronic kidney disease. A simple blood test, fully funded by Sanofi Genzyme, can be arranged.

Download your blood collection form or request your free DBS testing kit www.sanofigenzymeonline.com.au/pathologyforms/pdf

While Fabry disease is rare, it may be more common within a Fabry family. 1 Test the family. Fabry disease is a X-linked genetic condition with a high transmission rate – testing the family of a patient with Fabry disease may identify affected relatives early.

TREAT WITH FABRAZYME ® (agalsidase beta)

Fabrazyme 1mg/kg every other week is indicated for long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease ( α -galactosidase deficiency) 2

>10 YEARS OF EVIDENCE 3

Long-term use of Fabrazyme can slow the progression of the serious, life-threatening complications of Fabry disease. 3

PBS Information: This product is not listed on the PBS.This product is listed on the Life Saving Drugs Program.

Fabrayzme ® is a registered trademark of Sanofi Genzyme Corporation. sanofi-aventis Australia pty ltd trading as Sanofi Genzyme ABN 31 008 558 807. 12-24 Talavera Road, Macquarie Park, NSW 2113. GZANZ.FABR.19.01.0007a. Date of preparation June 2019. References: 1. Desnick RJ and Brady RO. J Pediatr 2004;144:S20-S26. 2. FABRAZYME Product Information. 3. Germain DP, et al. J Med Genet 2015;0:1–6. MINIMUM PRODUCT INFORMATION: FABRAZYME ® (agalsidase beta – powder for concentrate for solution for infusion). INDICATION: Long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease ( α -galactosidase deficiency). DOSAGE AND ADMINISTRATION: 1 mg/kg once every 2 weeks as an intravenous infusion. Initial infusion rate ≤ 0.25 mg/min (15 mg/hr) to minimise potential infusion-associated reactions. After patient tolerance is established, the rate may be incrementally increased with subsequent infusions. Reconstitute and dilute prior to use – see full PI. Does not contain preservatives. Use within 24 hours of infusion preparation. CONTRAINDICATIONS: Hypersensitivity to the active or any of the excipients. PRECAUTIONS: Anaphylactoid reactions, hypersensitivity, development of IgG antibodies, skin reactivity, infusion reactions, pregnancy (category B2), lactation, hepatic impairment. No clinical studies in children < 8 years (consider treatment after careful risk/benefit analysis) or in patients > 65 years. Therapy should only be initiated/continued under the ongoing supervision of a physician with experience and training in the treatment of Fabry disease. See full PI. INTERACTIONS: No interaction data. Theoretical risk of inhibited intracellular α -galactosidase activity when administered with chloroquine, amiodarone, benoquin, gentamicin. Do not mix with other medicines in the same infusion. ADVERSE EFFECTS: Infusion-associated reactions: chills, pyrexia, temperature change sensation, hypertension, nausea, vomiting, flushing, paraesthesia, fatigue, pain, headache, chest pain, pruritus, urticaria, dyspnoea, dizziness, pallor, somnolence and tachycardia. Others: malaise, musculoskeletal pain, oedema, rhinitis, rhinorrhoea and oxygen saturation decreased/hypoxia. Anaphylactoid reactions: localised angioedema, generalised urticaria, bronchospasm and hypotension. See full PI. NAME OF SPONSOR: Genzyme Australasia Pty Ltd, 12-24 Talavera Road, Macquarie Park, NSW 2113. Please review full Product Information before prescribing. Full Product Information is available from sanofi-aventis australia pty ltd at http://www.guildlink.com.au/gc/ws/sw/pi.cfm?product=swpfabra or 1800 818 806.