Spleen Australia. Immunisation for people with a non-functioning spleen. Vaccine schedules National and Victorian. 29 th July 2017

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1 Spleen Australia Immunisation for people with a non-functioning spleen. Vaccine schedules National and Victorian Penelope Jones RN Grad Dip Epi Manager Spleen Australia 29 th July 2017

2 Meningococcal W Spleen room pin board Capnocytophaga canimorsus SunHerald 03/01/2007 Strep pneumoniae

3 Outline Cases Why a Clinical Registry Recommendations Summary numbers Take home messages

4 Case 1-40 year old man husband and father of 3 young boys 24hr history of lethargy, chills Collapse at home Ambulance - Cardiac arrest Admission Alfred CPR, adrenalin, IV fluids, antibiotics shock, cyanosed, unreactive pupils Intubated, inotropes, vasopressin, antibiotics Dead within 5 hours of admission

5 Case 1 - Intensive Care Purpura fulminans fatal thrombotic disorder bruising, blood spots associated with DIC

6 Investigations Blood cultures x 3 - negative at 24hrs Blood film - diplococci in neutrophils and Howell Jolly Bodies Blood - positive PCR Streptococcus pneumoniae Further history Case 1 continued Splenectomy for trauma 20 yrs prior No preventive measures

7 Case 1 Blood Film: positive PCR Streptococcus pneumoniae Diplococci bacteria within neutrophils Howell-Jolly Bodies in the erythrocytes

8 1 Case 2-6 year old girl Coroner s report June 2013 Admitted to QLD Hospital referred by GP Was known to have had a splenectomy (HS)?Viral infection no one seems to have seriously considered that Lilli was also at risk of developing an OPSI No blood cultures done in early admission Late reaction to results of bacterial sepsis SYSTEM FAILURE where action could have been taken earlier, which may have prevented her death occurring.

9 Case 3 Father of 6 children & husband quadruple amputations following severe pneumococcal sepsis **Hospitalised for 7 months** Concord Hospital NSW 49 year old male Cost >$300K (Hospital admission)

10 Background phagocytes remove encapsulated bacteria Pneumococcal & meningococcal *Streptococcus pneumoniae Blood film Neisseria meningitidis Others Haemophilus Influenzae Group B streptococcus Capnocytophaga canimorsus

11 Background Identifying people with asplenia/hyposplenism 1. History of operation 2. Howell-Jolly Bodies on morphology

12 Splenic Artery Embolisation Spleen salvaging

13 Background Functions of the spleen FILTER Re-cycling of blood cells & removing RBCs (expiry ~120 days) Bacteria, viruses & toxic substances - filtered out of blood by WBCs IMMUNE SYSTEM / STORAGE Stores platelets and WBCs - monocytes Opsonization Largest unit of mononuclear phagocyte system (RES)

14 Background At time of pancreatectomy Gas Infections abscess in spleen

15 Background- Asplenia/hyposplenism Some medical conditions associated with hyposplenism Congenital cyanotic heart disease Coeliac Disease Sickle cell disease SLE Rheumatoid disease Splenic infarctions Splenic irradiation HIV infection Graft v Host Disease

16 Background Overwhelming Post Splenectomy Infection (OPSI) Risk difficult to quantify Estimates 3-5% lifetime risk * (1:20) 38-80% mortality * (morbidity?) Incidence % per year * Risk is considered high in first 2 years Case reports OPSI up to 30 yrs post splenectomy S.pneumoniae 50-90% of infections * Clin Micro Infect COST of OPSI ~ $50-$280K

17 Background Risk of sepsis (OPSI) varies Age young people more at risk Indication splenectomy (trauma v malignancies) Underlying medical conditions Lack of knowledge (of infection risk)!!

18 OPSI still occurring Best practice preventive strategies are known BUT Advice not given (medical staff unaware of risk/recent guidelines) Advice forgotten Quality of advice varies No reminders

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20 Spleen Australia Clinical Service and Registry for people without a functioning spleen Case + Finding Registration Education Clinical Follow Up SPLEEN REGISTRY 7,051 Resource Development Research

21 Australia pop ~24 million Estimated 25,000 people with asplenia Or hyposplenism?? NZ collaboration Spleen Australia s collaborating states cover 10.3 million people

22 Annual referrals to Spleen Australia 10 Dec Dec QLD & Tas join 1000 Patient numbers Vic state funding Years

23 Ascertainment of cases No mandatory reporting what would it take? Anatomical pathologists Medical referrals (specialists, GPs) Medical records (Alfred/RMH) Opportunistic referrals Word of mouth (self referrals)

24 Resolve quandries (reassure) Liaise with GP Liaise with specialists/ hospitals Kit Reminders (Ongoing contact) Children and Adult Patients Travel advice NES *EDUCATE +/- relative VACCINES Confirm Sourcing ANTIBIOTICS

25 Spleen Australia s strategies for reducing sepsis Education Signs & symptoms of bacterial infection Alert cards Travel, animal bites, ticks, educate GPs Other pregnancy/breastfeeding, OCP or HRT Translated documents (Greek, Chinese, Arabic, Vietnamese) Vaccinations (Immunisation Handbook 10 th Edn) Pneumococcus Meningococcus Haemophilus Influenzae type b Annual influenza Antibiotics (Therapeutic Guidelines) Prophylaxis penicillin (allergic - roxithromycin) at least 3 years Emergency supply 3gm amoxycillin

26 Education kit items Spleen Australia

27 Extra items in kit for children

28 Sent to Patient Local doctor Referring doctor Specialists Treating hospital PLEASE enter the dates of vaccines the patient has received as per this report onto to GP immunisation history!

29 Reminders Annual newsletter & Apple device App

30 Invasive pneumococcal disease Epidemiology Strep. pneumoniae infection - major cause of VPD worldwide 90 strains of Streptococcus pneumoniae Nasopharynx of healthy carriers ,640 cases in Australia (7.2 cases per 100,000 pop) 6 times higher Indigenous Australians 23vPPV funded 65+ & Aboriginal & Torres Strait Islanders 50+ PREVENAR 13 (13 serotypes) PNEUMOVAX 23 (23 serotypes) (Pfizer) (Merck) 13vPCV 23vPPV

31 Invasive meningococcal disease Neisseria meningitidis 13 serogroups of N.meningitidis Six serogroups (A, B, C, W, X and Y) majority of cases worldwide 2002 to 2015 main serogroup in Australia was serogroup B Notifications of serogroup W (MenW) increased nearly 5-fold between 2014 and MenW main serogroup notified in Australia

32 Vaccines - types Polysaccharide eg. 23vPPV and 4vMenPV short term protection (few years) less immunogenic in children aged <2 Polysaccharide antigens induce antibodies without involvement of T-lymphocytes T-cell lymphocyte involvement necessary for long-term immune memory without it, protection short-lived and immunity wanes (?revaccination) Conjugate higher-quality & longer-term immunity, esp in children <2 years eg. Haemophilus influenzae type b - HIBERIX, PEDVAXHIB Neisseria meningitidis ( A, C, W135 & Y) - MENACTRA, MENVEO, NIMENRIX Streptococcus pneumoniae (13vPCV 19A & 6A) - SYNFLORIX & PREVENAR 13

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34 NOT previously vaccinated Page 1 Hib if in doubt give Annual influenza 2 doses 4/52 apart if 1 st dose ever

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36 Have had previous vaccines page 2

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38 Paediatric medical recommendations

39 Medical recommendations for patients after Splenic artery embolisation Additional test - IgM memory B cells

40 Spleen Australia Database n= 7051 Splenectomy n = 6350 (90%) Functional Hyposplenism n = 315 (5%) Recommendations same as splenectomised patient Retained spleen function Splenic artery embolisation n=386 (5%) Spleen salvaging procedure >300 patients waitlisted (states not funding Registry)

41 Patients on database and reasons for referral n= No spleen function Spleen function

42 Children aged 0-18 years on database n= Number of patients male female Ages

43 Number of patients

44 Cost of Spleen Vaccines Private Chemists (initial doses and 8 week boosters) Pneumococcal Pneumo conjugate (Prevenar 13) $110 Pneumo polysaccharide (Pneumovax23) $36 (PBS) Meningococcal 4vMenCV (Menactra/Menveo) need 2 $160 MenB (Bexsero) need 2 $260 Haemophilus Influenzae Hib $36 (PBS) TOTAL ~$600 Hospital pharmacy costs as outpatient (except Men B) The PBS co-contribution price of $5.90 (health card) or $36.10 (no health card) **Free if an inpatient

45 Salient patient story in our 2015 newsletter

46 LESSONS LEARNED LEGAL ISSUE Australian Family Physician - June 2010 Dr Sara Bird Medico-legal and advisory unit, MDA Case: 42 yr unwell, mother of 3 children, presented to ED, MCA, no meds, no vaccinations, smoker +++ DIED after 30 mins of arrival in ED Family sued 1. Hospital - failed to vaccinate post op - educate and offer follow up 2. GP - failed to vaccinate - slow to diagnose IPD Settled out of court

47 Patients admitted to ICU with OPSI notified to Spleen Australia Sept/Oct 2016 Case Indication for splenectomy Years post splenectomy OPSI & serotype Vaccination Prophylaxis Mrs. MD Hereditary Spherocytosis 66 S. pneumoniae epidural abscess 23B Pneumovax 2004, 2010 Nil Mrs. JM 7581 Hereditary Spherocytosis 56 S. pneumoniae meningitis 23B Pneumovax 2002 Nil Mrs. HD Hodgkin Lymphoma 25 S. pneumoniae thoracic mycotic aneurysm 23B Unclear Nil Mr. BD Trauma 41 S. Pneumoniae infective endocarditis, septic arthritis 9N (23vPPV) Nil Nil

48 Does the spleen registry work? Rate of invasive pneumococcal and invasive meningococcal disease Pre-registration Post- registration 150 per 100,000 patient years 36 per 100,000 patient year Registration with Registry 69% reduction in risk of infection Incidence rate ratio 0.31, 95% CI: 0.12, 0.83 p= invasive infections prevented per year

49 Frequently asked questions We can answer them! 1. Zostavax see newsletter 2015 (YES if not immunosuppressed ) 2. Risk of meningococcal disease (see 2016 newsletter all newsletters on website) MenBV gaps b/n doses (not a concern but make sure 2 doses are given) Definitely give ACWY vaccines 3. Antibiotics lifelong in people >65yrs? (?probably) 4. Vaccine errors eg given out of order, given too soon, concern with chemo, multiple 23vPPV doses (you need our help!) 5. Cannot afford vaccines prioritising which ones 6. Flu shot quadrivalent & 2 doses if receiving for 1 st time 7. Travel advice (malaria) 8. DVT risk 9. Pregnancy/breastfeeding and vaccines (RWH ) 10. Intragam infusions & timing of vaccines

50 Take home messages Not having a spleen is a serious health issue Preventative measures work esp Educations, education, education Registration available if live in Vic, Tas & QLD If registered patient & health care providers have access to our support SPLEEN AUSTRALIA prevents OPSI spleenregistry@alfred.org.au

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52 Acknowledgements Victoria, Tasmania and Queensland Departments of Health Immunisation nurses A/Prof Denis Spelman & Dr Ian Woolley Nurses: Julia McNamara, Nigel Pratt, Cate Bunn, Kate Wall Prof Allen Cheng & Prof Karin Leder (epidemiologists) Louise Grannell (pharmacist) A/Prof Jim Buttery & A/Prof Mike Starr & Nigel Crawford (Melb Paeds) Anatomical Pathologists BMedSci & medical students Participating patients (all unique!!) Alfred hospital s Dept of Infectious Diseases Alfred Health GPs & clinic nurses & hospital doctors who refer patients to us and follow up these vulnerable children and adult