AUCKLAND CITY HOSPITAL AUCKLAND

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AUCKLAND CITY HOSPITAL AUCKLAND IMMUNOLOGY NURSING AT AUCKLAND CITY HOSPITAL AUCKLAND NEW ZEALAND - AOTEAROA SANCHIA VOS CLINICAL NURSE SPECIALIST

21 DISTRICT HEALTH BOARDS Population of 4. 2 million-http://www. stats Population of only 4.2 million whose health needs are managed by 21 district health boards, making it a difficult task to calculate the number of patients with PID.

GEOGRAPHICAL AREA SERVED Consultants: 3 part-time Fellow: 1 full-time Registrar: 1 full-time CNS: 1 full-time RN research: 1 part-time Dietician: 1 part-time North Island more heavily populated with + 3million people, over 1 million reside in Auckland.

The Day Ward One of our CVID patients receiving IVIG replacement therapy

PATIENTS ON Ig REPLACEMENT Out of our total patient population of 45 with PID, 37 have CVID.(82%) the remaining 18% include patients with Brutons, Goods syndrome, Churg-strauss, Hyper IgE and IgG Subclass deficiency. Our patients are mainly female (67%) with a predominant age group of 30 – 40 years.

INTRAGAM®P (Human immunoglobulin) Pooled plasma donated by NZ blood donors (Unique to NZ; plasma from other countries is not used to make the NZ product) Sent to CSL Bioplasma in Australia for processing and returned to NZ Resulting product is a sterile preservative free solution; 90% IgG, trace of IgA and IgM. Licensed in NZ as replacement therapy in PID & certain disease conditions e.g. Guillain-Barre syndrome, ITP.

IMMUNOGLOBULIN REPLACEMENT THERAPY Calculated at 400mg/kg body weight 74% intravenous IgG (IVIG) 3-4 weekly infused through peripheral vein via electronic infusion pump no after hour service work time lost stigma – visible evidence – impact choice of venous access 21% home subcutaneous infusions (SCIG) Graseby syringe drivers CSL Bioplasma trial in progress 5% home IVIG infusions Intravenous Infusions are given via venous access using a peripheral vein via an electronic infusion pump. No after hour service. Patients loose time off from work, do not want visible evidence of venous access.

ADVERSE EVENTS Rare: Often related to infusion rate: Observed events: patient with anti-IgA antibodies; risk of haemolysis Often related to infusion rate: infusions commenced at 60ml/hour, increased every 15 min by 60ml if patient tolerant to a maximum rate of 240ml/hour those demonstrating side-effects rate not more than 150ml/hour premedication with antihistamine and paracetamol effective ( or NSAID) Observed events: hypertension, back pain, rigor and headache Reported: lethargy and mild backache 12 to 24 hrs post infusion Patients tend to take a mild analgesic e.g. paracetamol and an anti-histamine prior to their infusion.

SUPPORT GROUPS The Immune Deficiencies Foundation of New Zealand (member of IPOPI) www.idfnz.org.nz

ACKNOWLEDGMENTS A sincere thank you to Dr Penny Fitzharris (Clinical Director) and Dr Miriam Hurst (Immunology Fellow).

REFERENCES/RESOURCES www.stats.govt.nz www.nzblood.co.nz