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-864524-904875nATIONAL REPORT ON THE ISSUE AND USE OF IMMUNOGLOBULIN (Ig)Annual Report 2015-16With the exception of any logos and registered trademarks, and where otherwise noted, all material presented in this document is provided under a Creative Commons Attribution 3.0 Australia licence.The details of the relevant licence conditions are available on the Creative Commons website (accessible using the links provided) as is the full legal code for the CC BY 3.0 AU licence.The content obtained from this document or derivative of this work must be attributed as the National Blood Authority National Report on the Issue and Use of Immunoglobulin (Ig) Annual Report 2015-16.ISSN 1839-1079 (online version)This report is available online at Bag 8430Canberra ACT 2601Phone: 13 000 BLOOD (13000 25663)Email: data@.au.auContents TOC \o "1-3" \h \z \u List of Tables PAGEREF _Toc485894434 \h 4List of Figures PAGEREF _Toc485894435 \h 4Introduction PAGEREF _Toc485894436 \h 5Report Snapshot PAGEREF _Toc485894437 \h 7Methodology PAGEREF _Toc485894438 \h 8Data quality PAGEREF _Toc485894439 \h 910 Year Trends PAGEREF _Toc485894440 \h 10Demand Trends PAGEREF _Toc485894441 \h 10Financial Trends PAGEREF _Toc485894442 \h 11Demographics PAGEREF _Toc485894443 \h 13Patient Numbers PAGEREF _Toc485894444 \h 13Geographic Distribution PAGEREF _Toc485894445 \h 14Age PAGEREF _Toc485894446 \h 15Weight PAGEREF _Toc485894447 \h 16Expenditure PAGEREF _Toc485894448 \h 17Clinical Indications PAGEREF _Toc485894449 \h 19Ig issues by criteria chapter PAGEREF _Toc485894450 \h 19Ig issues by diagnostic groups PAGEREF _Toc485894451 \h 20Ig issues by condition PAGEREF _Toc485894452 \h 22Ig issues by clinical discipline PAGEREF _Toc485894453 \h 24Ig grams issued per 1,000 population PAGEREF _Toc485894454 \h 25Dosing PAGEREF _Toc485894455 \h 30IVIg and SCIg PAGEREF _Toc485894456 \h 32NHIg PAGEREF _Toc485894457 \h 39Appendix A – Background PAGEREF _Toc485894458 \h 41Appendix B – Acronyms and Glossary PAGEREF _Toc485894459 \h 46Acronyms PAGEREF _Toc485894460 \h 46Glossary of terms PAGEREF _Toc485894461 \h 47Appendix C – Clinical Discipline mapping table PAGEREF _Toc485894462 \h 49Appendix D – Dataset of Ig supply by state/territory 2015-16 PAGEREF _Toc485894463 \h 55Appendix E – Grams Ig Issued by State and Territory PAGEREF _Toc485894464 \h 70Appendix F – Unique Patients by Quarter and State and Territory PAGEREF _Toc485894465 \h 71Appendix G – System Source for Tables and Figures PAGEREF _Toc485894466 \h 72List of Tables TOC \h \z \c "Table" Table 1Growth in Ig grams issued since 2006-07 PAGEREF _Toc485894467 \h 10Table 2Percentage change in grams issued over time by state and territory PAGEREF _Toc485894468 \h 11Table 3Annual numbers of patients, treatment episodes and grams PAGEREF _Toc485894469 \h 13Table 4Basic numbers PAGEREF _Toc485894470 \h 13Table 5Issues of domestic Ig compared with imported Ig PAGEREF _Toc485894471 \h 18Table 6Ig issues (g) by Criteria chapter PAGEREF _Toc485894472 \h 19Table 7Ig issues by Criteria chapter (percentage) PAGEREF _Toc485894473 \h 19Table 8Ig grams issued for top 10 diagnostic groups over time PAGEREF _Toc485894474 \h 21Table 9Difference in grams issued for secondary hypogammaglobulinaemia (percentage) PAGEREF _Toc485894475 \h 21Table 10Patient numbers and age for the top 20 conditions PAGEREF _Toc485894476 \h 22Table 11Ig grams issued by clinical discipline PAGEREF _Toc485894477 \h 24Table 12Grams of Ig issued by state and territory PAGEREF _Toc485894478 \h 26Table 13Patient numbers by state and territory: chronic inflammatory demyelinating polyneuropathy PAGEREF _Toc485894479 \h 26Table 14Patient numbers by state and territory: common variable immunodeficiency disease PAGEREF _Toc485894480 \h 27Table 15Patient numbers by state and territory: myasthenia gravis PAGEREF _Toc485894481 \h 27Table 16Patient numbers by state and territory: chronic lymphocytic leukaemia PAGEREF _Toc485894482 \h 28Table 17Patient numbers by state and territory: multiple myeloma PAGEREF _Toc485894483 \h 28Table 18Ig issued per 1,000 population by state and territory for top 10 conditions PAGEREF _Toc485894484 \h 29Table 19Ig grams per kg weight per episode PAGEREF _Toc485894485 \h 31Table 20Patient numbers for products issued by state and territory in 2015-16 PAGEREF _Toc485894486 \h 33Table 21Grams of product issued by state and territory in 2015-16 PAGEREF _Toc485894487 \h 34Table 22Treatment episode numbers for products issued by state and territory in 2015-16 PAGEREF _Toc485894488 \h 35Table 23Patient numbers for products issued by diagnostic group in 2015-16 PAGEREF _Toc485894489 \h 36Table 24Grams of product issued by diagnostic group in 2015-16 PAGEREF _Toc485894490 \h 37Table 25Treatment episodes for product issued by diagnostic group in 2015-16 PAGEREF _Toc485894491 \h 38Table 26NHIg issued from 2011-12 to 2015-16 PAGEREF _Toc485894492 \h 39Table 27Grams of NHIg issued by state and territory PAGEREF _Toc485894493 \h 40Table 28Grams per 1,000 population of NHIg issued by state and territory PAGEREF _Toc485894494 \h 40List of Figures TOC \h \z \c "Figure" Figure 1Ten year trends in issues of Ig PAGEREF _Toc485894495 \h 10Figure 2Ten year trends in expenditure on Ig PAGEREF _Toc485894496 \h 12Figure 3Patients per 1,000 population 2015-16 PAGEREF _Toc485894497 \h 14Figure 4Grams of Ig per 1,000 population by state and territory over time PAGEREF _Toc485894498 \h 15Figure 5Patient age compared to average Australian age PAGEREF _Toc485894499 \h 15Figure 6Patient weights relative to Australian average PAGEREF _Toc485894500 \h 16Figure 7Ig expenditure as a proportion of the national blood budget PAGEREF _Toc485894501 \h 17Figure 8Ig grams issued by diagnostic group PAGEREF _Toc485894502 \h 20Figure 9Proportion of Ig used for top 10 diagnosis group PAGEREF _Toc485894503 \h 23Figure 10Ig issues by clinical discipline PAGEREF _Toc485894504 \h 24Figure 11Percentage Ig issues by clinical discipline for top 10 diagnosis groups by state and territory PAGEREF _Toc485894505 \h 25Figure 12Grams per episode by condition PAGEREF _Toc485894506 \h 30Figure 13NHIg Grams issued and grams issued per 1,000 population PAGEREF _Toc485894507 \h 39IntroductionImmunoglobulin products analysed in this report include intravenous immunoglobulin (IVIg, subcutaneous immunoglobulin (SCIg) and normal human immunoglobulin (NHIg). Aggregated data for IVIg and SCIg is referred to as immunoglobulin (Ig) unless specifically stated. NHIg is reported separately. Ig is a blood product derived from donated human blood. Ig products are used to treat a broad range of conditions, with applications in replacement and immune modulation therapy. This report provides an analysis of national data on national Ig supply in Australia in 2015-16, also considering trends in supply over the last ten years.In Australia it is estimated that over 99% of all Ig is supplied under national blood arrangements through contracts administered by the National Blood Authority (NBA). The NBA’s role is to coordinate national supply and demand planning for blood and blood products including supply risk management; purchasing blood and blood products on behalf of all Australian governments; developing and implementing national strategies to encourage better governance, promoting appropriate use of blood and blood products; and providing expert advice to support government policy development. Further background is at REF _Ref384557528 \h \* MERGEFORMAT Appendix A.The Criteria for the Clinical Use of Intravenous Immunoglobulin (IVIg) in Australia?(Criteria) identifies the conditions and circumstances for which the use of intravenous and subcutaneous immunoglobulin (SCIg) is funded under national blood arrangements. The Criteria was first published in 2008, and was updated in 2012. It classifies the 93 diagnostic groups described in the Criteria into those for which IVIg has an established therapeutic role (Chapter 5), has an emerging therapeutic role (Chapter 6) and those where IVIg has application in exceptional circumstances only (Chapter 7). IVIg is only supplied for these diagnostic groups unless purchased by a single state, hospital or individual (a Direct Order). Chapter 8 of the Criteria outlines those conditions for which IVIg should not be supplied, under national blood arrangements.In addition to the clinical and diagnostic criteria for access to immunoglobulin products, access to SCIg products is provided through an assurance framework for the appropriate use of the product. SCIg access rules are detailed on the NBA website at . Participation in the National SCIg program requires hospitals to establish their capability and capacity to manage a hospital-based SCIg program, where the hospital provides access to all resources and takes full accountability for the management and use of the product within defined governing requirements.Normal human immunoglobulin (NHIg) may only be supplied for two purposes; for the treatment of susceptible contacts of measles, hepatitis A, poliomyelitis and rubella, as directed by public health officials; or for the treatment of immunodeficiency conditions for which the product is indicated for patients for whom IVIg and SCIg are both contraindicated. NHIg access rules are detailed on the NBA website at products should be prescribed and dispensed in accordance with any applicable state or territory legislative requirements. In-hospital management of immunoglobulin products must also be in accordance with the National Safety and Quality Health Service (NSQHS) Standards, in particular Standards 1 and 7, and the Australian and New Zealand Society of Blood Transfusion (ANZSBT) Guidelines for the Administration of Blood Products and Guidelines for Pre-transfusion Laboratory Practice.Ig comprises a large proportion of blood expenditure each year. Demand for Ig continues to rise steadily, and Australian per 1000 population use of this product is one of the highest among western countries. Demand for Ig is met through domestic and imported Ig products. Domestic Ig is manufactured by CSL Behring using plasma collected from voluntary, non-remunerated Australian donations. Both domestic and imported Ig are distributed by the Australian Red Cross Blood Service (Blood Service), with the Blood Service also being responsible for collection of data on behalf of governments for product funded under the national blood arrangements.Australia is in a unique position to provide analysis and commentary on the use of Ig due to national supply arrangements. This report begins with an analysis of Ig supply over the last ten years, then considers patient demographics, expenditure on Ig, clinical indications for which Ig was supplied and finally analyses the dose prescribed for various conditions. The top ten diagnostic groups account for 88.4% of all Ig supplied in 2015-16, and for this reason specific analysis focuses on these groups.Report Snapshot20955021463016,331 patients6,398 new patientsMedian age 63 yearsPATIENTSTotal cost of $541.5 million49% of total blood budgetEXPENDITURE4.98 million grams issued208 grams per 1,000 population43% imported productIg USE016,331 patients6,398 new patientsMedian age 63 yearsPATIENTSTotal cost of $541.5 million49% of total blood budgetEXPENDITURE4.98 million grams issued208 grams per 1,000 population43% imported productIg USEMethodologyThe report uses data from two primary sources, as follows:Data collected by the Blood Service under contractual arrangements with the NBA on behalf of all Australian governments. This data is collected either when an order is placed for Ig, or is collected following the treatment where product is issued as imprest stock. The data is collected into the Blood Service’s Supply Tracking Analysis Recording System (STARS) database.Data collected by the NBA on the units Ig issued to Australian Health Providers (AHPs) and purchases from suppliers. This data is held in the NBA Integrated Data Management System (IDMS).Over the eight years between 2008-09 and 2015-16, data has been captured on 48,643 patients. Caveats relating to the quality of this data are outlined below.This report includes data on the supply of Normal Human Immunoglobulin (NHIg) for the past five years and Subcutaneous Immunoglobulin (SCIg) for 2013-15, as no SCIg product was available in Australia before 2013-14. In this report data for IVIg and SCIg is aggregated and referred to as immunoglobulin (Ig) unless specifically stated. NHIg is reported separately. The report includes some language that may be unique to the Australian environment. A list of acronyms and definitions used in this report is at REF _Ref384558524 \h \* MERGEFORMAT Appendix B.The Criteria groups together a number of conditions into one diagnostic group. For example, primary immunodeficiency disease is a diagnostic group in the Criteria, with this group incorporating the numerous separate conditions. In some cases the analysis will focus on the diagnostic group, while in other areas it will focus on the condition.Each condition has been classified according to its allocated clinical discipline. It is acknowledged that for some conditions this classification is somewhat arbitrary. For example, there are immunological conditions affecting the blood that could potentially be mapped to either immunology or haematology. Where there appears to be significant overlap between clinical disciplines, the condition was mapped as mixed. In the majority of cases, the condition was mapped to the speciality most likely to be responsible for patients with that condition, noting that this can vary. REF _Ref384558593 \h \* MERGEFORMAT Appendix C provides the mapping of condition to discipline.The summary of key items from the data file is provided for each condition at the state and territory level. The summary includes patient numbers, grams of Ig used for the condition, grams per treatment episode and grams per 1,000 population ( REF _Ref379893813 \h \* MERGEFORMAT Appendix D). The source used for each figure and table is provided at REF _Ref379897557 \h \* MERGEFORMAT Appendix G.Data qualityThere are some factors relating to data quality, which need to be considered when reading this report, as follows:The reconciliation of data held in STARS and IDMS indicates minor variances at a national level. In some cases these differences can be explained by product being ordered and recorded in STARS the month prior to product actually being issued to a patient.Not all data fields are completed for all patients. For example, of the total patients recorded since 2008 43,661 patients (90%) had weight data entered, but only 8,201 (17%) had their weight data change in a treatment following the first entry.The ABS population series 3201.0 (Population by Age and Sex, Australian States and Territories) ended in June 2010 and was replaced by Australian Demographic Statistics (cat. No 3101.0). Series 3201.0 was utilised as the denominator for population statistics for Ig annual reports before 2011-12.Care should be taken when interpreting the data relating to the smaller states and territories as one or two patients can overly influence the use compared to larger states. The five largest Australian states are New South Wales (NSW), Victoria (VIC), Queensland (QLD), South Australia (SA) and Western Australia (WA).There has been no adjustment for Ig used in one state or territory for patients residing in a different state or territory.A total of 1141 (2%) patients received product in more than one state and territory. For example, if a patient relocated from New South Wales to Victoria, they will be counted as a patient in both states. The national patient count only includes one count for each patient. This may result in the sum of the state and territory totals being greater than the national total.Patient numbers were first reported in 2008-09. A small number of patients who did not receive product funded under national blood arrangements have been excluded from the total patient count.A total of 4,423 (9%) patients had more than one condition over time. In these cases, a patient may be counted more than once in the data in this report, that is, the patient will be counted in the totals for each condition.The STARS data has age and weight data recorded at treatment dates (first reported in 200910). This data changes over time. Age data is based on the patient’s age at 1 January each year.Diagnosis group and conditions captured prior to the implementation of the Criteria were mapped to ensure that they were meaningfully represented, however information from previous years may not be directly comparable from 2008-09 forward. There is a small variance between disciplines by year due to mapping methodology.10 Year TrendsDemand TrendsIn 2015-16 a total of 4,982,503 grams of Ig was issued, representing an increase of 549,357 grams (12.4%) over 2014-15. Since 2006-07 there has been an on average 11.6% increase in Ig use, with the greatest proportion of that increase comprising imported products ( REF _Ref253556626 \h Figure 1).Figure SEQ Figure \* ARABIC 1Ten year trends in issues of IgTable 1Growth in Ig grams issued since 2006-072006-072007-082008-092009-102010-112011-122012-132013-142014-152015-16Growth from previous year14%13%11%12%11%11%11%11%10%12%Average Growth from 2006-077%8%10%11%12%13%14%15%16%Total grams per 1,000 population92102111121133145158173188208Increase in grams per 1,000 population over previous year12%11%8%10%10%9%9%9%9%11%There has been a steady increase in demand for Ig over the last ten years, with increases of 10-12% per annum for the last five years. While a small proportion of this increase may be attributable to population increases, there has also been a steady increase of 8-11% per annum in the use of Ig per ’000 population ( REF _Ref386097734 \h Table 1) since the introduction of the Criteria in 2008.A breakdown of the year on year change in grams issued by state and territory has been provided in REF _Ref386096440 \h Table 2. Queensland has been growing at the fastest rate, closely followed by Victoria and New South Wales. Further information about the breakdown of domestic and imported Ig by state over time can be found in REF _Ref386115776 \h \* MERGEFORMAT Appendix E.Table 2Percentage change in grams issued over time by state and territoryNSWVICQLDWASATASACTNT2006-0713%20%18%10%-11%30%12%-16%2007-0818%8%16%6%14%5%29%1%2008-0915%3%14%0%23%14%-14%54%2009-1013%11%15%-4%12%7%20%-18%2010-1111%10%16%10%-4%8%28%7%2011-1211%7%16%6%9%1%17%47%2012-1311%13%11%7%9%-6%12%21%2013-1410%11%12%6%15%14%1%12%2014-159%11%12%12%7%8%8%8%2015-1614%10%14%17%11%2%3%36%Financial TrendsThe increase in demand for Ig places a financial burden on the Australian health system. In Australia, the total cost of domestic Ig supply comprises the cost of the plasma collected by the Blood Service, plus the cost of purchase of the finished Ig product from the supplier (CSL Behring). Imported plasma is purchased at a total product cost only.Total expenditure on Ig (excluding plasma for fractionation) in 2015-16 was $296.4 million, an increase of $23.3 million (8.5%) over 2014-15 ( REF _Ref253557293 \h Figure 2). The increased expenditure predominately represents increases in demand.There also continues to be an increase in the price of plasma for fractionation due to the increased ratio of apheresis to whole blood plasma for fractionation being supplied, resulting in an increase in the cost of domestic Ig. Combined with expenditure for plasma for fractionation, Ig accounts for a total expenditure of $541.5 million (excluding hyperimmune plasma for fractionation).Figure SEQ Figure \* ARABIC 2Ten year trends in expenditure on IgDemographicsPatient NumbersA total of 16,331 patients were issued Ig under the national blood arrangements during 2015-16 for 159,041 treatment episodes. This represents a 9.0% increase in the number of patients since 2014-15. A summary of some patient numbers is provided in REF _Ref477773716 \h Table 3 and REF _Ref386098046 \h Table 4. A breakdown of unique patients by state and territory and quarter is provided in REF _Ref386115652 \h \* MERGEFORMAT Appendix F.Table 3Annual numbers of patients, treatment episodes and gramsYearPatientsTreatment EpisodesTotal Grams Issued2008-099,87077,2122,380,2572009-1010,53785,2992,655,1842010-1111,49293,8932,950,3712011-1212,127101,3883,271,3092012-1313,102110,1833,622,4332013-1413,981122,7914,021,8612014-1514,983140,8554,433,1462015-1616,331159,0414,982,503Table 4Basic numbers2015-16Total unique patient IDs with some weight data16,062Total unique patient IDs with an age recorded16,331Total unique patient IDs with a weight change918Total unique patient IDs with more than one state or territory248Total unique patient IDs with two states or territories229Total unique patient IDs with three or more states or territories19Total unique patient IDs with more than one condition451Total unique patient IDs with two conditions441Total unique patient IDs with three conditions10Total unique patient IDs with four or more conditions0Total unique patient IDs aged 93 or older63Note: The above table calculations relate to only 2015-16 patients unlike previous reports where it included multiple years of dataGeographic DistributionNationally, 0.7 patients per 1,000 population received Ig in 2015-16. This varied between states and territories, ranging from 0.4 in Western Australia to 0.9 in Queensland ( REF _Ref253558949 \h Figure 3). All states and territories show an increase in the number of patients per 1,000 population over the previous year.Details on the number of patients by condition are at REF _Ref379893813 \h \* MERGEFORMAT Appendix D.Figure SEQ Figure \* ARABIC 3Patients per 1,000 population 2015-16There is significant variation between jurisdictions in Ig use in grams per 1,000 population, ranging from 98.7 in the Northern Territory to 281.9 in Queensland ( REF _Ref393362413 \h \* MERGEFORMAT Figure 4). Rates for the smaller population states and territories must be viewed with some caution as there are many factors that could contribute to their different use patterns, such as patients travelling to larger states for specialist treatment. Comparing only the five largest Australian states, the variation in Ig use is 2.4 fold, ranging from 120.0 grams per 1,000 population in Western Australia to 281.9 grams per 1,000 population in Queensland. The reason for this inter-state and territory variation is unknown. The lower use may represent appropriate management and prescribing practices, or may represent a level of under-diagnosis.Prior to 2014-15 Western Australia had shown only slight increases in the number of grams issued per 1,000 population, while most states and territories have seen a continued strong increase in Ig issued per 1,000 population. However, in 2015-16 Western Australia had the highest increase in growth of Ig issued per 1,000 population (excluding NT). Western Australia does remain with the lowest Ig issued per 1,000 population regardless.Figure 4Grams of Ig per 1,000 population by state and territory over timeAgeThe distribution of estimated age is shown in REF _Ref253559300 \h Figure 5 where it is compared with the age distribution of the Australian population at December 2015. A bimodal peak can be seen in the patient population treated with Ig, with the majority of Ig recipients either being very young, or over 55. The ageing population is expected to place a greater burden on Ig demand into the future, with the proportion of the world’s population over 60 years expected to more than double between 2015 and 2050.Figure SEQ Figure \* ARABIC 5Patient age compared to average Australian ageWeightIg dosing is dependent on the weight of the patient. For many immune replacement conditions, the patient weight determines the initial dosing, with maintenance therapy titrated against IgG levels and the patient’s clinical response to therapy. However, for conditions where Ig is used for its immunomodulatory properties, the Criteria limits the dose that can be prescribed based on the patient weight alone.Figure 6Patient weights relative to Australian averageNote: The above figure calculations relate to only 2015-16 patients. REF _Ref380064918 \h Figure 6 compares the weight of Ig recipients in Australia and the Australian population. There is a higher proportion of patients treated with Ig less than 55kg relative to the proportion in the Australian population. The average weight of adult Ig patients (78.3 kg) is slightly higher than the average weight of an Australian adult (77.7 kg); this is a change from previous years where it has been lower suggesting that the Ig population is getting heavier. Given that studies suggest that 63% of Australians are overweight or obese, the similarity in weight profiles between Ig recipients and the Australian population suggests that a large proportion of Ig recipients may also be overweight. While the current Criteria provides for dosing based on body weight, some limited studies suggest that dosing on lean body weight (ideal body weight) may be more appropriate. A small pilot study in Western Australia focussing on a narrow range of conditions suggested reductions of Ig dose of between 2.4% and 4.2% were achieved using a lean body dosing methodology. However, this has not been published in peer review literature, was not a randomised controlled trial, and did not discuss whether there were differences in clinical outcomes between the two groups. With an increasingly obese population, we can expect increases in demand if total (rather than lean) body weight dosing is continued and following reviews conducted of the literature relating to lean body mass dosing this area should be considered for future research.It should be noted that care should be taken when analysing the weights, not all patients have weight recorded and for those that do the weight recorded may not be recent.ExpenditureIn 2015-16, Australian expenditure on Ig products was $296.4 million, with additional expenditure of $245.1 million on plasma for fractionation (excluding hyperimmune plasma for fractionation) collected by the Blood Service.The cost of Ig as a proportion of the national blood budget is shown at REF _Ref393369871 \h Figure 7. Ig is the second largest budget item, representing 27% of the total budget for blood and blood products. Combined with expenditure for plasma for fractionation, Ig accounts for 49% of the total blood budget, at a total expenditure of $541.5 million (excluding hyperimmune plasma for fractionation).Figure 7Ig expenditure as a proportion of the national blood budgetOf the Ig supplied under national blood arrangements in Australia, 57% (2,850,947 grams) was manufactured domestically and 43% (2,131,556 grams) was imported from overseas. This represents a 20.1% increase in product importation since 2013-14 (476,604 grams) ( REF _Ref254189034 \h Table 5). Domestic supply is driven by the amount of plasma for fractionation collected in Australia and this increased by 5.1% in 2015-16 over 2014-15. Intragam P (IVIg) and Evogam (SCIg) are Ig products manufactured domestically in 2015-16. The imported products available were Kiovig (IVIg), Octagam (IVIg), Privigen (IVIg), Flebogamma (IVIg), Hizentra (SCIg) and Gammanorm (SCIg). When a patient is allocated to receive one of the imported products it is the clinician’s choice as to which product they order. Supply of Octagam constituted 41% of the supply of imported Ig.Table 5Issues of domestic Ig compared with imported Ig?NSW?NSWVICQLDWASATASACTNTAUSDomestic Ig Intragam Pg986,685633,909754,161146,661161,28352,67752,5185,5892,793,483$(m)$62$40$47$9$10$3$3$0$175Evogamg17,8439,43117,0216,2396,3165308357,464$(m)$1$1$1$0$0$0$0$4Total Domesticg1,004,528643,340771,182152,900167,59953,20752,6015,5892,850,947$(m)$63$40$48$10$11$3$3$0$179Imported IgKiovigg144,640112,027130,70439,57765,5512,06816,5548,906520,025$(m)$9$7$8$2$4$0$1$1$31Octagamg364,569180,052237,90257,24617524,7784,514137869,372$(m)$22$11$14$3$0$1$0$0$52Gammanormg9,268?1,0515871,9141,0363,61417,470$(m)$1?$0$0$0$0$0$1Flebogammag79,29642,91742,69321,30812,3405,84187535205,305$(m)$4$2$2$1$1$0$0$0$9Privigeng119,105116,535162,26538,77021,62513,63013,7559,410495,095$(m)$5$5$7$2$1$1$1$0$22Hizentrag8,0832399,6612,1431,5606511,95224,289$(m)$0$0$1$0$0$0$0$1Total Importedg724,960451,770584,275159,631103,16548,00341,26418,4892,131,556$(m)$41$25$32$9$6$3$2$1$117Proportion of domestic to imported Igg %58%59%57%49%62%53%56%23%57%$(m) %61%62%60%53%65%56%59%27%61%Note: $(m) excludes the costs for plasma for fractionation.Clinical IndicationsIg issues by criteria chapterThe Criteria classifies conditions into four chapters based on the level of evidence supporting the use of Ig, as follows:Chapter 5, conditions for which IVIg has an established therapeutic roleChapter 6, conditions for which IVIg has an emerging therapeutic roleChapter 7, conditions for which IVIg has application in exceptional circumstances onlyChapter 8, conditions for which IVIg use is not indicated.Ig was predominately issued for conditions within Chapter 5 ( REF _Ref393362821 \h Table 6). The relative distribution by chapter has remained relatively stable since 2008, with a decrease in Ig issues for Chapter 8 conditions ( REF _Ref386098763 \h Table 7).Table 6Ig issues (g) by Criteria chapter2008-092009-102010-112011-122012-132013-142014-152015-16Chapter 51,990,5862,212,9142,505,3322,724,8093,025,4523,409,1003,785,6154,223,866Chapter 6345,176371,832397,231444,605453,352463,361494,489535,596Chapter 747,27561,92476,033101,287120,979148,581178,221216,927Chapter 83,3262,5502,5741,90939005Total2,386,3612,649,4622,981,3853,272,9303,599,8314,021,0424,458,3264,976,394Table 7Ig issues by Criteria chapter (percentage)2009-102010-112011-122012-132013-142014-152015-16Chapter 584%84%83%84%85%85%85%Chapter 614%13%14%13%12%11%11%Chapter 72%3%3%3%4%4%4%Chapter 8<1%<1%<1%<1%0%0%0%For conditions where Ig is used only in exceptional circumstances (Chapter 7), five diagnostic groups accounted for 54.1% of those issues. These conditions were Limbic Encephalitis – nonparaneoplastic (48,098g), Solid organ transplantation (other than kidney) (24,266g), Pyoderma gangrenosum (16,598g), Paraneoplastic syndromes (16,116g) and Devic disease (neuromyelitis optica) (12,477g). While use in these conditions represents a small proportion of total Ig use, closer examination may be warranted.Both Limbic Encephalitis – nonparaneoplastic and Pyoderma gangrenosum have approximately quadrupled in grams issued since 2012-13 and tripled in patient count.While Ig may be issued in life threatening situations prior to diagnosis or in situations where the diagnosis is unclear at the time of treatment, in 2015-16 there was one case where Ig was supplied for a condition not in the Criteria (excluding Direct Orders where alignment with the Criteria is not required as it is not funded under the national blood arrangements). However, data to support compliance with all aspects of qualifying criteria for each condition is not always collected.Ig issues by diagnostic groupsThe top ten diagnostic groups account for 88.4% of all Ig supplied, with the top three diagnostic groups accounting for 57.0%.Acquired hypogammaglobulinaemia secondary to haematological malignancies is the diagnostic group for which the greatest percentage of Ig was issued in 2015-16 (22.2%), closely followed by chronic inflammatory demyelinating polyneuropathy (21.5%). Primary immunodeficiency diseases accounted for 13.3% of total Ig use ( REF _Ref253562849 \h \* MERGEFORMAT Figure 8, REF _Ref386099026 \h \* MERGEFORMAT Table 8).Since 2011-12 there has been a greater than 14% increase in Ig issues for both acquired hypogammaglobulinaemia secondary to haematological malignancies and chronic inflammatory demyelinating polyneuropathy, and a 9.6% increase in issues for primary immunodeficiency diseases. This is compared with the 13% increase in Ig over this period for all conditions. This indicates that while Ig issues for acquired hypogammaglobulinaemia secondary to haematological malignancies and chronic inflammatory demyelinating polyneuropathy are growing at a high rate, primary immunodeficiency diseases are growing at a lower rate while remaining a high use diagnostic group. If these trends continue as they are, we would expect to see myasthenia gravis overtake primary immunodeficiency diseases in the next 7 years.Figure SEQ Figure \* ARABIC 8Ig grams issued by diagnostic groupTable 8Ig grams issued for top 10 diagnostic groups over time2011-122012-132013-142014-152015-16% ChangeAcquired hypogammaglobulinaemia secondary to haematological malignancies694,640771,071862,898982,7731,106,72112.6%Chronic inflammatory demyelinating polyneuropathy677,458758,271857,533974,2581,071,1359.9%Primary immunodeficiency diseases477,461509,364558,617614,781660,8167.5%Myasthenia gravis231,064257,966313,940348,336402,88115.7%Multifocal motor neuropathy192,109209,791239,314256,041293,45814.6%Inflammatory myopathies153,931188,362230,473249,229293,42217.7%ITP in adults162,098178,738186,640187,621210,09412.0%Secondary hypogammaglobulinaemia95,183106,484110,024126,561145,49715.0%Guillain-Barre syndrome95,359104,360108,929105,567124,69218.1%Specific antibody deficiency99,52197,74983,22083,38188,9946.7%Secondary hypogammaglobulinaemia falls into the top ten diagnostic groups. The increase in issues of secondary hypogammaglobulinaemia was largely in New South Wales between 2009-10 and 2012-13, however in the last 2 years QLD, VIC and WA have also had substantial increases ( REF _Ref462042382 \h Table 9). In NSW there has been a 431% increase between 2008-09 and 2015-16, associated with a concurrent increase in patient numbers (increase of 183%). The grams issued per patient has increased by 87%. However there has also been a large increase in grams per 1,000 population from 1.5 to 5.7.Table 9Difference in grams issued for secondary hypogammaglobulinaemia (percentage)2009-102010-112011-122012-132013-142014-152015-16NSW51%31%37%31%8%20%15%VIC17%35%30%4%-7%11%20%QLD-6%13%12%1%7%15%16%WA-14%-20%45%10%-24%6%38%SA88%0%-4%45%15%-9%-20%TAS16%41%-4%-8%-2%-3%-7%ACT29%-16%-66%-51%41%454%22%NT-1100%-67%330%-73%119%-81%Total11%21%20%12%3%15%15%Ig issues by conditionTable 10 provides an overview of the conditions that use the most Ig, including data on total Ig use, patient numbers and median birth year. These conditions account for 88.9% of all Ig supplied, with the top ten conditions accounting for 75.1%. This data is also replicated in REF _Ref386099392 \h Figure 9 for the top 10 conditions.Table SEQ Table \* ARABIC 10Patient numbers and age for the top 20 conditionsConditions (Top 20)Igg (% of total)Patientsn (% of total)Median AgeChronic inflammatory demyelinating polyneuropathy1,071,135 (22%)2,250 (14%)64Common variable immunodeficiency disease580,964 (12%)1,724 (11%)54Myasthenia gravis402,881 (8%)945 (6%)63Chronic lymphocytic leukaemia350,066 (7%)1,380 (8%)72Non-Hodgkin lymphoma332,148 (7%)1,308 (8%)68Multifocal motor neuropathy293,458 (6%)496 (3%)57Multiple myeloma275,685 (6%)1,177 (7%)71Polymyositis158,414 (3%)393 (2%)64Secondary hypogammaglobulinaemia (excludes haem malignancies)145,497 (3%)652 (4%)60Guillain-Barré syndrome124,692 (3%)727 (4%)55Kidney transplantation post-transplant100,556 (2%)533 (3%)50Other relevant haematological malignancies94,004 (2%)574 (4%)64ITP refractory80,807 (2%)349 (2%)64Specific antibody deficiency72,403 (1%)268 (2%)57ITP in specific circumstances (surgery, corticosteroids contraindicated, chronic ITP)70,571 (1%)193 (1%)59Dermatomyositis70,415 (1%)404 (2%)57Inclusion body myositis64,437 (1%)142 (1%)70HSCT - post48,266 (1%)345 (2%)52ITP with life-threatening haemorrhage48,098 (1%)188 (1%)66X linked agammaglobulinaemia37,968 (1%)110 (1%)24Figure 9Proportion of Ig used for top 10 diagnosis groupPopulation based data on Ig issues is particularly interesting for conditions where the majority of patients receive Ig as it can provide an estimation of disease prevalence. One condition for which Ig would be prescribed for the vast majority of diagnosed patients is common variable immunodeficiency disease.Ig was supplied for 1,724 patients with common variable immunodeficiency disease. The estimated prevalence of common variable immunodeficiency disease as measured by patients treated with Ig for this indication is 7.2 per 100,000 population (ranging from 3.7 to 16.0 per 100,000 population across Australian states and territories and 3.7 to 10.6 if ACT, NT and TAS are excluded).For common variable immunodeficiency disease, this estimate is higher than other studies suggest with estimates between 2 and 4 people per 100,000 population. The ability to calculate accurate prevalence estimates is important for health service planning. It should be noted that the prevalence estimate is for diagnosed and treated patients only.Ig issues by clinical disciplineThe number of grams of Ig issued categorised according to clinical discipline is shown in REF _Ref253563362 \h Figure 10. Some conditions are classified as mixed, in that they fall across more than one clinical discipline. Other conditions fall within a clinical discipline other than neurology, haematology or immunology, such as use in transplants or dermatology. These are considered under ‘Other’ in REF _Ref253563362 \h Figure 10. REF _Ref386099559 \h Table 11 replicates this data.Since 2011-12, there has been a 1.6 fold increase in Ig issues for neurological conditions, compared with a 1.4 fold increase for both haematological conditions and immunological conditions.Figure SEQ Figure \* ARABIC 10Ig issues by clinical disciplineTable 11Ig grams issued by clinical discipline2011-122012-132013-142014-152015-16Neurology1,460,7021,649,3581,916,7922,120,1112,407,995Haematology961,3661,026,1771,116,0371,234,8161,390,824Immunology656,179695,298746,828828,735885,933Other194,363228,947241,386274,664291,643There is significant variation across Australia in Ig use for each clinical discipline (as allocated). REF _Ref253563789 \h Figure 11 shows that in Western Australia issues for neurological conditions represent a greater proportion of total issues than for other states. The proportional use for immunological conditions is much lower in Queensland and Tasmania than other states, with use of Ig for neurological and haematological conditions prevailing in these two states. The reason for this inter-state and territory variation is unknown, but it may represent differences in clinical practice, differing patient populations with disease profiles, variable access to alternative therapies and/or differences due to the availability of specialist services across Australia.Figure SEQ Figure \* ARABIC 11Percentage Ig issues by clinical discipline for top 10 diagnosis groups by state and territoryIg grams issued per 1,000 populationThe amount of Ig issued per 1,000 population for each indication varies between state and territory. Complete data for conditions by state and territory can be found at REF _Ref379894780 \h \* MERGEFORMAT Appendix D and is summarised for the conditions with the highest usage of Ig. REF _Ref393303801 \h \* MERGEFORMAT Table 12 shows a breakdown of the proportion of Ig issued in each state and territory with a comparison to the proportion of the population in each state and territory.The highest variation between states and territories in Ig use per ’000 population is seen in Chronic Inflammatory Demyelinating Polyneuropathy and Non-Hodgkin lymphoma. In total, for the five largest states, there were a low number of Ig issues per ’000 population in South Australia and Western Australia respectively, and high use in Queensland. The reason for the significant variation between these states is unknown, and further studies may be required to ascertain the significance of this finding. Interestingly, the difference appears to be attributed to a greater number of patients, rather than higher dosing, with the dosing in South Australia and Western Australia being higher than Queensland for Chronic Inflammatory Demyelinating Polyneuropathy ( REF _Ref379894780 \h \* MERGEFORMAT Appendix D).Table 12Grams of Ig issued by state and territoryIg issued (g)Proportion of total Ig issuedProportion of Australian populationGrams per 1,000 populationNSW1,729,48834.7%32.0%225VIC1,095,11022%25.1%183QLD1,355,45727%20.1%282WA312,5316%10.9%120SA270,7645%7.1%159TAS101,2102%2.2%196ACT93,8652%1.0%385NT24,0780%1.6%61Total4,982,503100%100%208The following tables ( REF _Ref386100404 \h Table 13, REF _Ref386100414 \h Table 14, REF _Ref386100419 \h Table 15, REF _Ref386100426 \h Table 16 and REF _Ref386100433 \h Table 17) show the patient numbers for states and territories over time for specific conditions.Table 13Patient numbers by state and territory: chronic inflammatory demyelinating polyneuropathyChronic inflammatory demyelinating polyneuropathy2011-122012-132013-142014-152015-16NSW598652704772834VIC372421447464507QLD386485529580648WA99105108123130SA7380818193TAS3033373236ACT1722282732NT57<5815Australia1,5511,7531,9032,0542,250Note: The national patient count only includes one count for each patient. This may result in the sum of the state and territory totals being greater than the national total.Table 14Patient numbers by state and territory: common variable immunodeficiency diseaseCommon variable immunodeficiency disease2011-122012-132013-142014-152015-16NSW617650721793813VIC232241265276288QLD276311317338370WA6167788895SA102101110110116TAS2021252529ACT5458606663NT5<5<5<510Australia1,3231,4061,5431,6561,724Note: The national patient count only includes one count for each patient. This may result in the sum of the state and territory totals being greater than the national total.Table 15Patient numbers by state and territory: myasthenia gravisMyasthenia gravis2011-122012-132013-142014-152015-16NSW219235267297335VIC141177186199215QLD181199212245310WA3639514146SA1917141728TAS1710101116ACT1013141616NT<5Australia609671747818945Note: The national patient count only includes one count for each patient. This may result in the sum of the state and territory totals being greater than the national total.Table 16Patient numbers by state and territory: chronic lymphocytic leukaemiaChronic lymphocytic leukaemia2011-122012-132013-142014-152015-16NSW381394431483523VIC230225271290310QLD283297292318347WA4841456468SA7979867788TAS3131343432ACT2529303127NT55696Australia1,0601,0781,1791,2831,380Note: The national patient count only includes one count for each patient. This may result in the sum of the state and territory totals being greater than the national total.Table 17Patient numbers by state and territory: multiple myelomaMultiple myeloma2011-122012-132013-142014-152015-16NSW324378389425466VIC153157176215214QLD330346360365382WA1516202323SA1722242540TAS5147423940ACT1410101417NT<5<5<5<5Australia9019691,0121,1001,177Note: The national patient count only includes one count for each patient. This may result in the sum of the state and territory totals being greater than the national total.Table 18 shows the top 10 conditions by the Ig issued per 1,000 population by state and territory.Table 18Ig issued per 1,000 population by state and territory for top 10 conditionsConditionNSW VICQLDWASATASACTNTNationalFold Variation*Chronic inflammatory demyelinating polyneuropathy4741584023382527452.5Common variable immunodeficiency disease351625122115566242.9Myasthenia gravis18121951217188153.8Chronic lymphocytic leukaemia1717278510211175.5Non-Hodgkin lymphoma1211283916111149.4Multifocal motor neuropathy131011121791721121.7Multiple myeloma138202423701211.9Polymyositis75102925175.8Secondary hypogammaglobulinaemia (excludes haem malignancies)841121102067.4Guillain-Barré syndrome5655455251.5*The Fold Variation in REF _Ref406138226 \h \* MERGEFORMAT Table 18 is a measure describing difference in the Ig grams per 1,000 population between the state being issued the least to the state being issued the most, using only data from the five largest states. For example, a low value of 30 and a high value of 60 correspond to a fold variation of 2, or in common terms, a two-fold increase.DosingFigure SEQ Figure \* ARABIC 12Grams per episode by conditionThe data shows that there is significant variance in the dosing of the top 10 conditions by grams per episode; where dosing is calculated as number of grams administered in each episode ( REF _Ref386112223 \h \* MERGEFORMAT Figure 12). The definition of episode in the data is not uniform and therefore this data should be interpreted with caution. Variations are expected as the doses and frequency of dose varies as the underlying method for calculating the dose also varies. Also note that the Criteria requires the lowest possible dose to achieve the desired clinical outcome, so the ‘dose’ is not ‘mandated’ but rather suggested and guided to the lower end to achieve efficacy which may contribute to the differences in dosing between conditions. The dosing is stable compared to the 2014-15 year.Dosing in the neurological conditions is higher than for other conditions, as provided for in the Criteria. For dosing information for other conditions refer to REF _Ref379895385 \h \* MERGEFORMAT Appendix D.The grams per kilogram were calculated for each infusion episode ( REF _Ref386094763 \h Table 19). From this data it is difficult to assess whether the dosing strategy utilised was in accordance with that provided for under the Criteria. This is particularly difficult as the patient weight data is not updated for every episode and may change over time.Condition<=0.4 g/kg/episoden (%)0.4 – 0.99 g/kg/episoden (%)1 – 2 g/kg/episoden (%)>2 g/kg/episoden (%)No weight Datan(%)Chronic inflammatory demyelinating polyneuropathy9,151 (39%)12,766 (55%)1,101 (5%)27 (0%)372 (2%)Common variable immunodeficiency disease9,082 (47%)8,873 (46%)77 (0%)3 (0%)1,389 (7%)Myasthenia gravis3,891 (42%)4,926 (54%)271 (3%)5 (0%)86 (1%)Chronic lymphocytic leukaemia6,910 (58%)4,916 (41%)4 (0%)1 (0%)136 (1%)Non-Hodgkin lymphoma1,401 (26%)3,365 (62%)558 (10%)12 (0%)101 (2%)Multiple myeloma7,144 (62%)4,251 (37%)13 (0%)0 (0%)148 (1%)Multifocal motor neuropathy5,744 (61%)3,533 (38%)1 (0%)0 (0%)137 (1%)Polymyositis1,211 (35%)2,013 (58%)211 (6%)4 (0%)33 (1%)Secondary hypogammaglobulinaemia (excludes haem malignancies)419 (46%)432 (47%)45 (5%)21 (2%)3 (0%)Guillain-Barré syndrome2,931 (55%)2,256 (42%)72 (1%)0 (0%)51 (1%)Table SEQ Table \* ARABIC 19Ig grams per kg weight per episodeIVIg and SCIgIn March 2013, the JBC approved the introduction of SCIg under the national blood arrangements. The first phase of implementation was through hospital-based management arrangements, with no additional cost to patients, and further work will be undertaken to support supply of SCIg for other pathways of care. In 2015-16 the NBA established arrangements for supply of the following SCIg products:Evogam 16% 0.8g/5ml and 3.2g/20ml supplied by CSL Behring (Australia) Pty Ltd (domestic)Gammanorm 16% 1650mg/10ml and 3300mg/20ml supplied by Octapharma Australia Pty Ltd (imported)Kiovig 10% 1g/10ml, 2.5g/25ml, 5g/50ml, 10g/100ml and 20g/200ml supplied by Baxter Healthcare (imported)Hizentra 5% 1g/5ml, 2g/10ml, 4g/20ml and 10g/50ml supplied by CSL Behring (Australia) Pty Ltd (imported)In addition to the clinical and diagnostic criteria for access to immunoglobulin products, access to SCIg products is provided through an assurance framework for the appropriate use of the product. SCIg access rules are detailed on the NBA website at . Participation in the National SCIg program requires hospitals to establish their capability and capacity to manage a hospital-based SCIg program, where the hospital provides access to all resources and takes full accountability for the management and use of the product within defined governing requirements.These products are authorised and distributed by the Blood Service in the same manner as IVIg.Tables 20-22 show the patient numbers, grams issued andtreatment episodes, by state and territory for IVIg and SCIg products in 2015-16. Tables 23-25 show patient numbers, grams issued and treatment episodes by diagnostic group for IVIg and SCIg products in 2015-16.Table 20Patient numbers for products issued by state and territory in 2015-16?IVIgSCIg?StateFlebogamma 5 percentFlebogamma 10 percentIntragam PKiovig 10 percentOctagam 5 percentOctagam 10 percentPrivigen 10 percentSCIg EvogamSCIg gammanormSCIg Kiovig 10 percentHizentra 20 percentTotalNSW2352523,8534146104336737049?576,019VIC1111412,36033143021862858??<53,723QLD1311292,5054374304839046214<5514,276WA4845534108966115129<5?18916SA942665181<5?84338?10942TAS7211809189172<5<5?8340ACT?<5196488768<512?11296NT??4335<5?31????93AUS53862910,1661,5321,5781,2802,59425190<515616,331Note: The national patient count only includes one count for each patient. This may result in the sum of the state and territory totals being greater than the national total. In addition, each patient may have received multiple products, meaning the total number of patients for each state/territory may not match the total of the patient counts for each product.Table 21Grams of product issued by state and territory in 2015-16?IVIgSCIg?StateFlebogamma 5 percentFlebogamma 10 percentIntragam PKiovig 10 percentOctagam 5 percentOctagam 10 percentPrivigen 10 percentSCIg EvogamSCIg gammanormSCIg Kiovig 10 percentHizentra 20 percentTotalNSW36,13140,235991,716150,866221,856158,411113,15018,1569,230?8,3571,748,108VIC17,56221,525633,105114,407128,62258,169111,9909,435??2311,095,046QLD17,20919,125743,694134,087121,763124,627149,97517,4361,3931209,2381,338,667WA9,66610,800147,18639,82442,03016,23537,8956,045554?2,121312,355SA1,3787,050159,74767,499150?16,9206,2861,952?1,398262,379TAS1,2173,97553,3372,6285,65920,78012,4801121,096?623101,905ACT?93052,80316,9422,0153,48912,720773,706?1,54494,226NT??6,0879,192135?8,295????23,709AUS83,162103,6402,787,675535,444522,229381,711463,42557,54617,93112023,5124,976,394Table 22Treatment episode numbers for products issued by state and territory in 2015-16?IVIgSCIg?StateFlebogamma 5 percentFlebogamma 10 percentIntragam PKiovig 10 percentOctagam 5 percentOctagam 10 percentPrivigen 10 percentSCIg EvogamSCIg gammanormSCIg Kiovig 10 percentHizentra 20 percentTotalNSW1,05896931,4003,9916,0523,7983,1043,7981,668?1,20657,044VIC49765419,2272,8113,6171,5002,8662,110??3933,321QLD65052324,9263,9213,7963,7264,3942,255213241,12145,549WA1921794,17576574834663194448?4288,456SA331584,6791,4646?358896186?1057,885TAS31751,645661685703591265?803,071ACT?161,705315596629216519?1563,144NT??1772182?174????571AUS2,4612,57487,93413,55114,44810,00612,17810,0312,699243,135159,041Table 23Patient numbers for products issued by diagnostic group in 2015-16?IVIgSCIg?Diagnostic GroupFlebogamma 5 percentFlebogamma 10 percentIntragam PKiovig 10 percentOctagam 5 percentOctagam 10 percentPrivigen 10 percentSCIg EvogamSCIg gammanormSCIg Kiovig 10 percentHizentra 20 percentTotalAcquired hypogammaglobulinaemia secondary to haematological malignancies33464,1232091671753002810?274,711Chronic inflammatory demyelinating polyneuropathy129129881401450267608????2,250Primary immunodeficiency diseases10101,7134528294418667<51031,990Myasthenia gravis8571272169225150320????945Multifocal motor neuropathy232715711910252186????496Inflammatory myopathies365729413812984163????726ITP in adults6273466127115123296????1,168Secondary hypogammaglobulinaemia13135193033314514<5?10652Guillain-Barré syndrome48482197710685172????727Kidney transplantation316059427190116????410Specific antibody deficiency<5<5272512<592311?16314Note: Each patient may have received multiple products per diagnosis, so the total number of patients for each diagnostic group may not match the total of the patient counts for each product.Table 24Grams of product issued by diagnostic group in 2015-16?IVIgSCIg?Diagnostic GroupFlebogamma 5 percentFlebogamma 10 percentIntragam PKiovig 10 percentOctagam 5 percentOctagam 10 percentPrivigen 10 percentSCIg EvogamSCIg gammanormSCIg Kiovig 10 percentHizentra 20 percentTotalAcquired hypogammaglobulinaemia secondary to haematological malignancies3,3223,970940,02645,21735,21239,14329,3554,8901,927?3,6591,106,721Chronic inflammatory demyelinating polyneuropathy25,04024,225438,204161,461188,645109,705123,855????1,071,135Primary immunodeficiency diseases6751,420547,44012,7087,4938,6976,31045,25214,23512016,467660,816Myasthenia gravis12,18311,445119,20867,99384,45752,43555,160????402,881Multifocal motor neuropathy4,1357,62096,00060,15452,37824,28248,890????293,458Inflammatory myopathies4,9549,045116,03754,63146,83629,10532,815????293,422ITP in adults7,92511,28580,88625,72219,13621,84043,300????210,094Secondary hypogammaglobulinaemia893780114,9667,3327,0675,3644,0953,104482?1,415145,497Guillain-Barré syndrome7,0988,79533,27313,69919,18413,80828,835????124,692Kidney transplantation5,6007,6659,1748,42617,79222,56617,035????88,258Specific antibody deficiency22828575,7778702,7296758804,3011,287?1,96388,994Table 25Treatment episodes for product issued by diagnostic group in 2015-16?IVIgSCIg?Diagnostic GroupFlebogamma 5 percentFlebogamma 10 percentIntragam PKiovig 10 percentOctagam 5 percentOctagam 10 percentPrivigen 10 percentSCIg EvogamSCIg gammanormSCIg Kiovig 10 percentHizentra 20 percentTotalAcquired hypogammaglobulinaemia secondary to haematological malignancies9813132,9961,6561,2371,341982837180?46339,921Chronic inflammatory demyelinating polyneuropathy68056911,6633,9884,8972,5573,164????27,518Primary immunodeficiency diseases264218,9323602502791747,8752,193242,07632,231Myasthenia gravis3382793,3051,8282,4661,4421,581????11,239Multifocal motor neuropathy1101362,1921,2781,1835371,054????6,490Inflammatory myopathies1442253,0111,3821,312706846????7,626ITP in adults1871951,662555419448888????4,354Secondary hypogammaglobulinaemia33334,27527624720813951058?2766,055Guillain-Barré syndrome2382571,017409570430857????3,778Kidney transplantation289294396247663665764????3,318Specific antibody deficiency9102,915361042237809268?3164,526NHIgIn 2013–14, as a result of the introduction of SCIg as discussed above, demand for Normal Human Immunoglobulin (NHIg) reduced significantly by 18.8 per cent. CSL Behring (Australia) Pty Ltd produces NHIg from hyperimmune plasma specially collected by the Blood Service. The volume of product is limited by the availability of this specialised plasma, and by production scheduling arrangements in CSL Behring (Australia) Pty Ltd’s manufacturing facility.Demand for normal Ig NHIg further declined in 2014-15 as a result of implementation of the NHIg policy outlining the national position on access and use under the national blood arrangements.Normal human immunoglobulin (NHIg) may only be supplied for two purposes; for the treatment of susceptible contacts of measles, hepatitis A, poliomyelitis and rubella, as directed by public health officials; and for the treatment of immunodeficiency conditions for which the product is indicated for patients for whom IVIg and SCIg are both contraindicated. NHIg access rules are detailed on the NBA website at 26-28 and Figure 13 show the grams issued and the issues per 1,000 population by states and territories for either purpose listed above.Figure 13NHIg Grams issued and grams issued per 1,000 populationTable 26NHIg issued from 2011-12 to 2015-16Product2011-122012-132013-142014-152015-16Normal Immunoglobulin 2VI - 2ml (grams)879699654167112Normal Immunoglobulin 2VI - 5ml (grams)30,83630,46624,6495,4093,254Total (grams)31,71531,16525,3035,5763,366Grams Per 1,000 Population1.411.361.090.240.14Table 27Grams of NHIg issued by state and territory?2011-122012-132013-142014-152015-16NSW9,136 9,634 6,915 82 238 VIC6,323 6,903 6,747 2,278 489 QLD1,844 1,640 2,774 1,472 1,134 WA5,258 5,261 3,458 59 38 SA8,124 6,543 4,431 936 980 TAS182 344 272 154 43 ACT797 816 514 480 432 NT50 24 191 35 12 OTHER0 0 0 80 0 Australia30,867 30,325 24,597 4,981 2,922 Table 28Grams per 1,000 population of NHIg issued by state and territoryPer 1,000 Population2011-122012-132013-142014-152015-16NSW1.26 1.31 0.93 0.01 0.03 VIC1.13 1.22 1.17 0.39 0.08 QLD0.41 0.36 0.59 0.31 0.24 WA2.20 2.12 1.36 0.02 0.01 SA4.93 3.94 2.64 0.55 0.58 TAS0.36 0.67 0.53 0.30 0.08 ACT2.15 2.16 1.34 1.24 1.10 NT0.22 0.10 0.79 0.14 0.05 Appendix A – BackgroundFunding for IgIg supplied under national blood arrangements is funded 63% by the Commonwealth government, with the remaining 37% being funded by the state and territory to which the product is supplied.The CriteriaA process to review the Australian Health Ministers’ Advisory Council (AHMAC) (2000) guidelines commenced in 2004. A result was the approval of the first edition of the Criteria by Health Ministers in December 2007. The first edition of the Criteria was made available to clinicians on 3 March 2008 and applied to all new patients from that date. For patients already receiving Ig for an indication not listed as being funded under national blood arrangements, a six month transition period was allowed to enable treatment strategies to be reviewed, with the exception of IgG subclass deficiency, where grandfathering of the use of Ig was permitted under defined circumstances.The Criteria is a publication that describes the eligibility criteria that patients must meet to receive Ig that is funded by all Australian governments. Product is provided free of charge to all patients who have a condition meeting qualifying criteria for supply as outlined in the Criteria. The Criteria helps to ensure that Ig is accessed consistently across Australia for the treatment of patients whose health is likely to be improved with Ig therapy. The Criteria was developed using the best available medical evidence and expertise.As part of the process to implement the new Criteria, the NBA established a clarification process in November 2008. A consultation group was consulted on specific queries that arose in relation to interpretation of the Criteria. Consideration of the queries and comments resulted in some amendments to specific indications in the Criteria. The revisions were published on the NBA’s website in February 2009.A review of the Criteria commenced in 2010. A National Ig Criteria Review Working Group was established to oversee the 2010–11 Criteria review process. The Criteria second edition was made available to clinicians on 10 August 2012 and applied to all new patients from that date. For patients already receiving Ig for an indication where the specific eligibility criteria had changed, a six month transition period was allowed to enable treatment strategies to be reviewed, with the exception of IgG subclass deficiency patients, as described above.Supply of ProductImmunoglobulin is made from donated human plasma. The supply of Australian plasma is contracted by the NBA and importation of IVIg is a government policy position to ensure risk mitigation and continuity of supply. While the NBA makes sure there is enough Ig by importing this product, there is a finite international supply.There are two main ways Ig is available in Australia:Supply under national blood arrangementsIf the Ig is ordered to treat a medical condition which is funded under the Criteria then the product is supplied and funded under national blood arrangements. In this case the cost of the product is shared between the Commonwealth and the relevant state or territory.Orders for Ig under national blood arrangements are made to the Blood Service, which is contracted by the NBA as the authoriser and distributor of all Ig funded under these arrangements. In seeking authorisation, the requesting clinician will be asked to provide information to the Blood Service to establish that the request meets the Criteria. For ongoing conditions, the Criteria may specify review criteria to be applied in reviewing the patient to determine whether access to funded Ig will continue.In the role as authoriser of requests for Ig, the Blood Service maintains a database of requests, and provides data to the NBA which is used as a basis for reporting on the annual use of Ig in Australia.Direct order and other supply arrangementsIf the Ig is to treat a medical condition that is not funded under the Criteria, then the individual state or territory may approve the accessing of product under the Direct Order arrangements established by the NBA, or the product may be ordered directly from a commercial supplier of Ig. In this case the supply of the product is not funded under national blood arrangements, and the cost must be met in some other way.HistoryIn 2003-04 the NBA coordinated demand management activities for two products in short supply; Biostate (plasma-derived Factor VIII) and Intragam P (plasma-derived Ig). At all times, the NBA successfully met the blood and blood product needs of all Australian states and territories through intensive management of the product, via its contracts with the Blood Service and CSL Limited and the importation of substitutable products from overseas. The NBA arranged for an imported product to be purchased to make up for the shortfall, and this product was made available to patients in March 2004.In 2004-05 the NBA successfully negotiated a new Plasma Products Agreement with CSL Limited, which came into effect from 1 January 2005.In December 2004 the NBA also signed a Standing Offer contract with CSL Limited (for the supply of Sandoglobulin ?), as well as with Octapharma Australia Pty Ltd (for the supply of Octagam) for a two-year period in order to allow access to imported Ig as a contingency supply if and when needed to supplement shortfalls in the domestic Ig supply. The Ig Standing Offer comprised two components, a National Blood Supply component whereby imported Ig was procured by the NBA for use under the National Blood Agreement (i.e. for those conditions covered under the nationally agreed cost sharing arrangements) and a Jurisdictional Direct Order component which allowed approved recipients to access imported Ig for all other conditions.Ig had to be intensively managed again in 2004–05 due to ongoing increases in demand and indications for its clinical use for over 60 clinical syndromes and conditions.As part of a strategic solution to the shortage of Ig, governments purchased imported Ig (Sandoglobulin?) in 2003 and placed it in the National Reserve of Plasma Products. In order to optimise the use of the stocks in the National Reserve, the NBA in conjunction with states and territories, the Blood Service and CSL Limited, developed and implemented a plan to rotate the Sandoglobulin? stocks out of the National Reserve. This rotation commenced in October 2004.In 2005–06, the challenges in supply of domestic Ig required the NBA to adopt the same intensive product management arrangements as it had in 2004-05 with the continued rotation of Sandoglobulin?.In 2006-07 in order to ensure Ig remained available to all Australians, the NBA negotiated a further 12month extension to the Ig Standing Offer in December 2006. A procurement process for the renewal of the standing offer arrangements commenced in early 2007.Intensive product management was successfully undertaken in 2006–07 to avert a number of temporary and longer-term potential shortages, including shortages of Ig and plasma-derived Factor VIII.In 2007-08 the NBA commenced a procurement process for new contracts. The outcome of the procurement was the finalisation of a new fixed price contract with Octapharma Australia Pty Ltd for the supply of Octagam for three years under the National Blood Supply arrangement. Octagam and a CSL Ltd imported product, Sandoglobulin Liquid, were also supplied under Direct Order arrangements negotiated by the NBA.In 2008-09 the NBA continued imports of intravenous immunoglobulin to be able to meet domestic clinical demand.During 2009–10 the plasma fractionation arrangements were governed by the five-year Plasma Products Agreement between the NBA and CSL Limited, which expired on 31 December 2009, and a new CSL Australian Fractionation Agreement which took effect on 1 January 2010.The contract with Octapharma Australia Pty Ltd for the supply of Octagam was due to expire on 31 December 2010, with the NBA having an option to extend the contract by one year. In May 2010 the NBA moved to exercise the option to extend the contract with Octapharma Australia Pty Ltd, with improved value for money, for a further 12 months.A contract with CSL Limited for the supply of Sandoglobulin NF (nanofiltration) Liquid under the Direct Order arrangement expired at the end of December 2009.The NBA entered into a three-year contract with Lateral Grifols Pty Ltd for the supply of Flebogamma 5% DIF (dual inactivation plus nanofiltration) under Direct Orders, which commenced on 1 January 2010.During 2010-11 imported intravenous immunoglobulin continued to supplement domestic Ig production to meet clinical demand in Australia. In September 2010, Octapharma issued a nationwide voluntary recall of Octagam due to production concerns. To enable domestic demand to be met, the NBA invoked relevant clauses that had been included in the contract with Lateral Diagnostics to allow supply of Flebogamma through national blood arrangements (in addition to the Direct Orders supply). Lateral Diagnostics, working with the Spanish-based manufacturer of Flebogamma, Grifols S.A., responded rapidly and fully to the NBA’s additional requirements and this arrangement continued for the remainder of the year. The voluntary recall of Octagam was still in place in Australia at 30 June 2011.In 2011-12 CSL Limited experienced a decline in its immunoglobulin (IgG) yield. As a result of the reduction in yield, and other logistical factors, CSL Limited was unable to supply Intragam P 200ml from its working inventory against the full annual supply estimate amounts. The NBA also gave approval for CSL Limited to access the Minimum Product Inventory and the National CSL Reserve to augment supply. By the end of June 2012 CSL Limited had fully restocked the Minimum Product Inventory and the National CSL Reserve, although the NBA continued to carefully manage the planned supply of Intragam P in 2012-13.The Therapeutic Goods Administration (TGA), Australia’s national regulator for drugs and regulatory devices, approved the re-introduction of Octagam 5% in October 2011 following the voluntary recall of product in September 2010. The NBA worked with the Blood Service, Octapharma Australia Pty Ltd and Grifols Australia Pty Ltd to manage the transition of patients from Flebogamma 5% DIF under the national supply arrangements; this was achieved by March 2012.In October 2011 the NBA signed contracts for the supply of imported Ig with Octapharma Australia Pty Ltd for the supply of Octagam 5%. The new contract took effect on 1 January 2012. A 10% formulation of this product became available in July 2012; Baxter Healthcare Pty Ltd for the supply of Kiovig 10% from 1 January 2012 and with Grifols Australia Pty Ltd for a direct order contract operating until 31 December 2012 for the supply of Flebogamma 5% DIF. A new direct order contract for continued supply of Flebogamma 5% commenced on 1 January 2012.In 2012-13 two contracts were placed for supply of imported Ig under the national blood arrangements. The contracts commenced on 1 January 2012 for a period of three years with provision for a one year extension. The suppliers were Baxter Healthcare Pty Ltd and Octapharma Australia Pty Ltd.The NBA, on behalf of all Australian governments, completed a review of the adequacy of the current Ig authorisation and clinical governance arrangements. The aim of the review was to identify options for improvements in the management of Ig. The review also analysed the issues, benefits and risks of potentially including NHIg and subcutaneous immunoglobulin (SCIg) in the Ig management framework.The review identified significant variations in Ig management processes nationally, with process inefficiencies, under investment in integrated data systems and limited evidence of alternative therapies being considered before prescription. It also found variation in dosing, high prescription rates in some conditions compared to international rates of use, limited transparency of price implications and no accountability for cost with the prescriber.In March 2013, the Jurisdictional Blood Committee (JBC) considered the final report of the review and endorsed the NBA commencing work to implement five short term improvement projects recommended by the review. The five projects were to:describe the functional model for the authorisation and clinical governance arrangements, and formally allocate responsibility in each jurisdictionintroduce new management processes to include NHIg and SCIg in the Ig authorisation processimprove patient information to ensure patients are aware of the Criteria requirements for eligibility and ongoing therapycentralise hospital ordering and product management at the blood bank or pharmacy for improved management, and define when and how emergency stock should be manageddefine and deliver a package of information concerning Ig products and arrangements, particularly for junior medical and nursing staff.In March 2013, the JBC approved the introduction of SCIg under the national blood arrangements. The first phase of implementation was through hospital-based management arrangements, with no additional cost to patients, and further work was undertaken to support supply of SCIg for other pathways of care. Supply of SCIg commenced in September 2013, including both domestically manufactured and imported SCIg products.In 2013-14 the NBA established arrangements for supply of the following SCIg products:Evogam 16% 0.8g/5ml and 3.2g/20ml supplied by CSL Behring (Australia) Pty Ltd (domestic)Gammanorm 16% 1650mg/10ml and 3300mg/20ml supplied by Octapharma Australia Pty Ltd (imported)Kiovig 10% 1g/10ml, 2.5g/25ml, 5g/50ml, 10g/100ml and 20g/200ml supplied by Baxter Healthcare (imported)In 2015-16 the NBA established arrangements for supply of the following SCIg product:Hizentra 5% 1g/5ml, 2g/10ml, 4g/20ml and 10g/50ml supplied by CSL Behring (Australia) Pty Ltd (imported)During 2013-14 to 2015-16 the NBA made significant progress to implement new Ig authorisation and clinical governance arrangements. These arrangements aim to address a range of deficiencies identified in the 2012-13 review of the management of Ig, including:significant variations and inefficiencies in Ig management processes nationallyvariation in dosinghigh prescription rates in some conditions compared to international rates of uselimited transparency of price implicationsno accountability for cost with the prescriber.Key 2013-14 achievements included:development and approval by governments of the business cases describing the high level functional model for new authorisation and clinical governance arrangements and the associated supporting national databasedevelopment and implementation of new management processes for NHIg and SCIgestablishment of the National Ig Governance Advisory Committee.Key 2014-15 achievements included:establishment of a network of new national specialist advisory committees to assess and recommend changes to the arrangements for the supply of Ig in Australiapublication and implementation of new Ig Governance policies, a standardised patient treatment review process and revised formsreview of the Criteria for the clinical use of intravenous immunoglobulin in Australia (Criteria) for adaptation to the new information management system under development, BloodSTAR.Key 2015-16 achievements included:holding five meetings of the National Immunoglobulin Governance Advisory Committee (NIGAC), and approval by NIGAC of Specialist Working Group three year work plans for achieving key governance outcomesimplementation of the National Ig Governance framework processes in Western Australiapublication and implementation of the second edition of the Ig Governance policy to support the implementation of BloodSTARfinalisation of the online adaptation of Criteria for BloodSTARcommencement of a further review of the Criteria for the clinical use of intravenous immunoglobulin in Australia (the Criteria) with a view to implementing Version Three in 2017Specialist Working Groups for Neurology, Immunology, Haematology and Transplantation Medicine finalised the review of the medical conditions in Chapters 5 & 6 of the Criteria through regular teleconference meetingsApproval of revised Chapter 5 and 6 conditions by the JBC.For further information on the Ig Governance Program go to the NBA website at 31 December 2015 two contracts for the supply of imported Ig expired. The suppliers were Baxalta Australia Pty Ltd and Octapharma Australia Pty Ltd.In June 2015, the NBA successfully concluded a tender process for new contracts for the national supply of imported Ig to replace the current contracts which expired on 31 December 2015. The new contracts were awarded to CSL Behring Pty Ltd and Grifols Australia Pty Ltd.Appendix B – Acronyms and GlossaryAcronymsACTAustralian Capital TerritoryAHMACAustralian Health Ministers’ Advisory CouncilAHMCSee SCoHAHPAustralian Health ProvidersANCAAnti-neutrophil cytoplasmic antibody AUSAustraliaDODirect OrderHIVHuman immunodeficiency virusHSCTHematopoietic stem cell transplantationIDMSIntegrated Data Management SystemIgImmunoglobulin products including IVIg and SCIgITPIdiopathic thrombocytopenic purpuraIVIgIntravenous immunoglobulinJBCJurisdictional Blood CommitteeJDOJurisdictional Direct OrderNBANational Blood AuthorityNHIgNormal human immunoglobulinNIGACNational Immunoglobulin Governance Advisory CommitteeNSWNew South WalesNTNorthern TerritoryPANDASPaediatric autoimmune neuropsychiatric disorder associated with streptococcal infections QLDQueenslandSASouth AustraliaSCIgSubcutaneous ImmunoglobulinSCoHStanding Council of HealthSTARSSupply Tracking Analysis Recording SystemTASTasmaniaTGATherapeutic Goods AdministrationTSSToxic shock syndromeVICVictoriaWAWestern AustraliaGlossary of termsTerm DescriptionBlood productsProducts manufactured from human bloodBlood ServiceThe Australian Red Cross Blood ServiceClinical DisciplineClassification of the conditions according to the clinical specialityCondition Specific diagnoses within a diagnostic group. Also known as the primary diagnosis. In some instances the diagnostic group may be the same as the condition, for example – Myasthenia gravis is the condition and Diagnostic GroupCriteria for the clinical use of intravenous immunoglobulin in Australia (the Criteria)A document describing the conditions, indications and patient qualifying and review criteria for which Ig is funded under national blood arrangements by all Australian governmentsCriteria MetAn assessment that the patient eligibility requirements as defined in the Criteria for a particular condition have been achievedCriteria Not Met or Qualifying (Q) Criteria Not MetAn assessment that the patient eligibility requirements as defined in the Criteria for a particular condition have not been achievedDirect Orders (DO)Previously known as Jurisdictional Direct Orders (JDO). Arrangements implemented by the NBA with suppliers to facilitate the purchase of Ig for the treatment of conditions not satisfying the Criteria for the clinical use of IVIg in AustraliaDiagnostic GroupA grouping of clinical/medical conditions, as outlined in the Criteria. Also known as disease groupDisease GroupSee diagnostic groupFractionationA manufacturing process that separates blood plasma into specific protein fractionsImprest stockHealth provider orders of product for stock that is maintained at a certain levelIntravenous immunoglobulinAn immunoglobulin product derived from donated human plasma that is administered intravenouslyJurisdiction Any of the parties to the Australian National Blood Agreement, being the Australian Government and all state and territory governmentsMinimum Product InventoryThe minimum inventory of Ig held by CSL to meet contract obligationsNational Blood AgreementThe Agreement signed by all governments in 2003 that sets out the objectives for governments for the management of the Australian blood sectorNational blood arrangementsArrangements, including funding arrangements, established under the National Blood AgreementNational CSL ReserveThe reserve of inventory of Ig that CSL Behring manages on behalf of the NBA for contingency purposesNormal immunoglobulinAn immunoglobulin product derived from human plasma that is administered by intramuscular injection (as opposed to intravenous or sub-cutaneous injection)PlasmaThe liquid part of the blood containing antibodies and other proteinsPrimary diagnosisSee ‘condition’Subcutaneous immunoglobulinAn immunoglobulin product derived from donated human plasma that is administered subcutaneouslyTreatment episodeOne instance or episode of a treatment plan, for example a treatment plan may be made up of 4 episodes over 4 months with an episode occurring every 4 weeks OR 1 dose of transfused product every two weeks for 6 months would be 13 treatment episodesAppendix C – Clinical Discipline mapping tableConditionChapterDiagnostic GroupClinical DisciplineChronic lymphocytic leukaemiaChapter 5Acquired hypogammaglobulinaemia secondary to haematological malignanciesHaematologyMultiple myelomaChapter 5Acquired hypogammaglobulinaemia secondary to haematological malignanciesHaematologyNon-Hodgkin lymphomaChapter 5Acquired hypogammaglobulinaemia secondary to haematological malignanciesHaematologyOther relevant haematological malignanciesChapter 5Acquired hypogammaglobulinaemia secondary to haematological malignanciesHaematologyPost-haemopoietic stem cell transplantation (HSCT)Chapter 5Acquired hypogammaglobulinaemia secondary to haematological malignanciesHaematologyChronic inflammatory demyelinating polyneuropathyChapter 5Chronic inflammatory demyelinating polyneuropathyNeurologyGuillain-Barré syndromeChapter 5Guillain-Barré syndromeNeurologyDermatomyositisChapter 5Inflammatory myopathiesNeurologyInclusion body myositisChapter 5Inflammatory myopathiesNeurologyPolymyositisChapter 5Inflammatory myopathiesNeurologyIdiopathic thrombocytopenic purpura - AdultChapter 5ITP in adultsHaematologyITP associated with HIVChapter 5ITP in adultsHaematologyITP in pregnancyChapter 5ITP in adultsHaematologyITP in Specific circumstances (surgery, corticosteroids contraindicated, chronic ITP)Chapter 5ITP in adultsHaematologyITP RefractoryChapter 5ITP in adultsHaematologyITP with life-threatening haemorrhageChapter 5ITP in adultsHaematologyKawasaki diseaseChapter 5Kawasaki diseaseImmunologyLambert-Eaton myasthenic syndromeChapter 5Lambert-Eaton myasthenic syndromeNeurologyMultifocal motor neuropathyChapter 5Multifocal motor neuropathyNeurologyMultifocal motor neuropathy with persistent conduction blockChapter 5Multifocal motor neuropathyNeurologyMyasthenia gravisChapter 5Myasthenia gravisNeurologyNeonatal haemochromatosisChapter 5Neonatal haemochromatosisMixed - Haem/ImmunCommon variable immunodeficiency diseaseChapter 5Primary immunodeficiency diseasesImmunologyOther Primary ImmunodeficiencyChapter 5Primary immunodeficiency diseasesImmunologySevere combined ImmunodeficiencyChapter 5Primary immunodeficiency diseasesImmunologyTransient hypogammaglobulinaemia of infancyChapter 5Primary immunodeficiency diseasesImmunologyWiskott-Aldrich SyndromeChapter 5Primary immunodeficiency diseasesImmunologyX linked agammaglobulinaemiaChapter 5Primary immunodeficiency diseasesImmunologyStiff person syndromeChapter 5Stiff person syndromeNeurologyAcute disseminated encephalomyelitisChapter 6Acute disseminated encephalomyelitisNeurologyANCA (PR3 or MPO)-positive idiopathic rapidly progressive glomerulonephritisChapter 6ANCA-positive necrotising vasculitisImmunologyChurg-Strauss SyndromeChapter 6ANCA-positive necrotising vasculitisImmunologyMicroscopic polyangiitisChapter 6ANCA-positive necrotising vasculitisImmunologyWegener’s granulomatosisChapter 6ANCA-positive necrotising vasculitisImmunologyAutoimmune haemolytic anaemiaChapter 6Autoimmune haemolytic anaemiaHaematologyEvans syndromeChapter 6Evans syndromeHaematologyFoeto-maternal /neonatal alloimmune thrombocytopenia (Antenatal)Chapter 6Foeto-maternal /neonatal alloimmune thrombocytopeniaHaematologyFoeto-maternal /neonatal alloimmune thrombocytopenia (Neonatal)Chapter 6Foeto-maternal /neonatal alloimmune thrombocytopeniaHaematologyHaemophagocytic syndromeChapter 6Haemophagocytic syndromeHaematologyHSCT (for prevention of GvHD in high risk Allogeneic HSCT).Chapter 6HSCT (for prevention of GvHD in high risk Allogeneic HSCT).HaematologyIgM para-proteinaemic neuropathyChapter 6IgM para-proteinaemic neuropathyNeurologyITP in childrenChapter 6ITP in childrenHaematologyKidney transplantation – post-transplantChapter 6Kidney transplantationRenal specialistKidney transplantation – pre-transplantChapter 6Kidney transplantationRenal specialistKidney transplantation post-transplantChapter 6Kidney transplantationRenal specialistKidney transplantation pre-transplantChapter 6Kidney transplantationRenal specialistMultiple sclerosis - Severe relapse with no response to high dose methylprednisoloneChapter 6Multiple sclerosisNeurologyMultiple Sclerosis in PregnancyChapter 6Multiple sclerosisNeurologyMultiple Sclerosis in young patients severe/relapsing/remitting in whom other therapies have failedChapter 6Multiple sclerosisNeurologyOpsoclonus myoclonus ataxiaChapter 6Opsoclonus myoclonus ataxiaNeurologyBullous pemphigoidChapter 6PemphigoidImmunologyCicatricial pemphigoidChapter 6PemphigoidImmunologyPemphigus foliaceusChapter 6PemphigusImmunologyPemphigus vulgarisChapter 6PemphigusImmunologyPost transfusion purpuraChapter 6Post transfusion purpuraHaematologySecondary hypogammaglobulinaemia (excludes haem malignancies)Chapter 6Secondary hypogammaglobulinaemiaMixedIgG subclass deficiency EXISTING patients onlyChapter 6Specific antibody deficiencyImmunologySpecific antibody deficiencyChapter 6Specific antibody deficiencyImmunologyIgG subclass deficiency. Existing patient with suppurative lung diseaseChapter 6Specific antibody deficiencyImmunologyToxic epidermal necrolysis/Steven Johnson SyndromeChapter 6Toxic epidermal necrolysis/Steven Johnson SyndromeImmunologyToxic Shock Syndrome (TSS) - StaphylococcalChapter 6Toxic shock syndromeImmunologyToxic Shock Syndrome (TSS) - StreptococcalChapter 6Toxic shock syndromeImmunologyAcute leukaemia in childrenChapter 7Acute leukaemia in childrenHaematologyAutoimmune congenital heart blockChapter 7Autoimmune congenital heart blockImmunologyAutoimmune diabetic neuropathyChapter 7Autoimmune diabetic neuropathyNeurologyAutoimmune neutropeniaChapter 7Autoimmune neutropeniaHaematologyAutoimmune uveitisChapter 7Autoimmune uveitisImmunologyCatastrophic antiphospholipid syndromeChapter 7Catastrophic antiphospholipid syndromeImmunologyCoagulation factor inhibitorsChapter 7Coagulation factor inhibitorsHaematologyDevic disease (neuromyelitis optica)Chapter 7Devic disease (neuromyelitis optica)NeurologyDiabetic AmyotrophyChapter 7Diabetic AmytrophyNeurologyEpidermolysis bullosa acquisitaChapter 7Epidermolysis bullosa acquisitaDermatologyEpilepsy (rare childhood cases)Chapter 7Epilepsy (rare childhood cases)NeurologyGraves ophthalmopathyChapter 7Graves ophthalmopathyImmunologyHaemolytic disease of the newbornChapter 7Haemolytic disease of the newbornHaematologyHaemolytic transfusion reactionChapter 7Haemolytic transfusion reactionHaematologyHashimoto encephalopathyChapter 7Hashimoto enecephalopathyNeurologyHIV in childrenChapter 7HIV in childrenImmunologyLimbic encephalitis-nonparaneoplasticChapter 7Limbic encephalitis-nonparaneoplasticNeurologyMyocarditis in childrenChapter 7Myocarditis in childrenMixedPANDAS/tic disordersChapter 7PANDAS/tic disordersNeurologyLimbic encephalitis-paraneoplasticChapter 7Paraneoplastic syndromesNeurologyParaneoplastic cerebellar degeneration (Yo antibodies)Chapter 7Paraneoplastic syndromesNeurologyParaneoplastic Subacute Sensory NeuropathyChapter 7Paraneoplastic syndromesNeurologyParaneoplastic syndromesChapter 7Paraneoplastic syndromesNeurologyPotassium channel antibody-associated encephalopathyChapter 7Potassium channel antibody-associated encephalopathyNeurologyPure red cell aplasiaChapter 7Pure red cell aplasiaHaematologyPure white cell aplasiaChapter 7Pure white cell aplasiaHaematologyPyoderma gangrenosumChapter 7Pyoderma gangrenosumDermatologyRasmussen SyndromeChapter 7Rasmussen SyndromeNeurologyScleromyxedemaChapter 7ScleromyxedemaMixedSepsis - neonatalChapter 7Sepsis - neonatalPaediatricianSjogren’s syndromeChapter 7Sjogren’s syndromeImmunologySjogren's SyndromeChapter 7Sjogren’s syndromeImmunologySolid Organ - HeartChapter 7Solid organ transplantation (other than kidney)- totalOrgan specialistSolid Organ - Heart/LungChapter 7Solid organ transplantation (other than kidney)- totalOrgan specialistSolid Organ - LiverChapter 7Solid organ transplantation (other than kidney)- totalOrgan specialistSolid Organ - LungChapter 7Solid organ transplantation (other than kidney)- totalOrgan specialistSolid Organ - OtherChapter 7Solid organ transplantation (other than kidney)- totalOrgan specialistSolid Organ - PancreasChapter 7Solid organ transplantation (other than kidney)- totalOrgan specialistTransplant - Solid OrganChapter 7Solid organ transplantation (other than kidney)- totalOrgan specialistTransplants - Allogeneic stem cell or bone marrowChapter 7Solid organ transplantation (other than kidney)- totalOrgan specialistSusac syndromeChapter 7Susac syndromeNeurologySystemic Capillary Leak SyndromeChapter 7Systemic Capillary Leak SyndromeImmunologyAcute optic neuritisChapter 8Acute optic neuritisNeurologyAcute rheumatic feverChapter 8Acute rheumatic feverMixedAdrenoleukodystrophyChapter 8AdrenoleukodystrophyNeurologyAmegakaryocytic thrombocytopeniaChapter 8Amegakaryocytic thrombocytopeniaHaematologyAntiphospholipid syndrome (non obstetric)Chapter 8Antiphospholipid syndrome (non obstetric)MixedAplastic anaemia/pancytopeniaChapter 8Aplastic anaemia/pancytopeniaHaematologyAsthmaChapter 8AsthmaMixedAtopic dermatitis/eczemaChapter 8Atopic dermatitis/eczemaDermatologyAutism – young adultsChapter 8Autism – young adultsMixedAutologous haemopoietic stem cell transplantationChapter 8Autologous haemopoietic stem cell transplantationHaematologyBehcet's diseaseChapter 8Behcet's diseaseImmunologyCardiac surgery with bypass – prophylaxisChapter 8Cardiac surgery with bypass – prophylaxisMixedCongestive cardiac failureChapter 8Congestive cardiac failureMixedCrohn’s diseaseChapter 8Crohn’s diseaseMixedDiamond Blackfan syndromeChapter 8Diamond Blackfan syndromeHaematologyFemale infertilityChapter 8Female infertilityMixedGlomerulonephritis – IgA nephritisChapter 8Glomerulonephritis – IgA nephritisMixedHaemolytic uraemic syndromeChapter 8Haemolytic uraemic syndromeHaematologyHenoch-Schonlein purpuraChapter 8Henoch-Schonlein purpuraMixedHIV/AIDS – adultChapter 8HIV/AIDS – adultMixedIdiopathic dilated cardiomyopathyChapter 8Idiopathic dilated cardiomyopathyMixedLinear IgA diseaseChapter 8Linear IgA diseaseDermatologyLupus cerebritisChapter 8Lupus cerebritisMixedLupus nephritisChapter 8Lupus nephritisMixedMotor neuron disease/amyotrophic lateral sclerosisChapter 8Motor neuron disease/amyotrophic lateral sclerosisNeurologyMyalgic encephalomyelitisChapter 8Myalgic encephalomyelitisNeurologyNarcolepsy/cataplexyChapter 8Narcolepsy/cataplexyNeurologyNephrotic syndromeChapter 8Nephrotic syndromeMixedObsessive compulsive disordersChapter 8Obsessive compulsive disordersMixedPolyneuropathy of critical illnessChapter 8Polyneuropathy of critical illnessNeurologyRecurrent foetal loss (with or without antiphospholipid syndrome)Chapter 8Recurrent foetal loss (with or without antiphospholipid syndrome)MixedRheumatoid arthritisChapter 8Rheumatoid arthritisMixedSepsis (other than neonatal sepsis)Chapter 8Sepsis (other than neonatal sepsis)MixedSickle cell diseaseChapter 8Sickle cell diseaseHaematologySystemic lupus erythematosusChapter 8Systemic lupus erythematosusMixedUlcerative colitisChapter 8Ulcerative colitisMixedJDO issueJDO ChapterJDOJDOAcute Idiopathic DysautomiaNAPre 2008 CriteriaNeurologyAlloimmune Neutropenia In InfancyNAPre 2008 CriteriaHaematologyAlloimmune Thrombocytopenia NeonatalNAPre 2008 CriteriaHaematologyAutoimmune ThrombocytopenicNAPre 2008 CriteriaHaematologyCutaneous VasculitisNAPre 2008 CriteriaMixedHypogammaglobulinaemiaNAPre 2008 CriteriaImmunologyHypogammaglobulinaemia UnclassifiedNAPre 2008 CriteriaImmunologyImmunological Miscellaneous, No diagnosis recordedNAPre 2008 CriteriaImmunologyMiscellaneousNAPre 2008 CriteriaMixedMyelopathy due to HTLV-1NAPre 2008 CriteriaImmunologyNecrotising MyelitisNAPre 2008 CriteriaMixedOther Lymphoproliferative / HypogammaglobulinaemiaNAPre 2008 CriteriaHaematologyPaediatric MyocarditisNAPre 2008 CriteriaMixedSensory neuropathy associated with anti-Hu antibodiesNAPre 2008 CriteriaNeurologySeptic thrombocytopeniaNAPre 2008 CriteriaHaematologyStills Disease - AdultsNAPre 2008 CriteriaImmunologyTrauma - BurnsNAPre 2008 CriteriaMixedAppendix D – Dataset of Ig supply by state/territory 2015-16Condition?NSWVICQLDWASATASACTNTNationalChapter 5??????????Chronic inflammatory demyelinating polyneuropathyPatients834507648130933632152,250Grams363,767248,735277,894104,92040,00819,4139,8436,5571,071,135Grams/Episode393834644539324239Grams per 1,000 Population474158402338252745Chronic lymphocytic leukaemiaPatients5233103476888322761,380Grams134,56372,77490,92112,91521,2038,5517,1881,953350,066Grams/Episode302826263130313428Grams per 1,000 Population1812195121718815Common variable immunodeficiency diseasePatients813288370951162963101,724Grams265,16696,612122,26730,88734,9257,64521,8881,574580,964Grams/Episode212422182028173721Grams per 1,000 Population35162512211556624DermatomyositisPatients7149351412570193Grams21,42715,05617,9526,0752,9943,4503,618070,571Grams/Episode35413945375855039Grams per 1,000 Population334227903Guillain-Barré syndromePatients23119615971411811<5727Grams40,83033,73526,25712,1716,6032,5792,017501124,692Grams/Episode333432333334343633Grams per 1,000 Population565545525HSCT - postPatients168795719176<5<5345Grams19,55210,26912,3462,4921,8391,5351449048,266Grams/Episode271928233226483025Grams per 1,000 Population323<113<1<12Inclusion body myositisPatients484833<515<50<5142Grams18,10824,92914,1645436,29031209364,437Grams/Episode37373549412404737Grams per 1,000 Population243<14<10<13ITP associated with HIVPatients00000<500<5Grams0000027500275Grams/Episode00000920092Grams per 1,000 Population00000<100<1ITP in pregnancyPatients231816<56<50067Grams3,6872,9623,5556365044800011,824Grams/Episode5274367172320049Grams per 1,000 <1<1<1<1<1<100<1ITP in specific circumstances (surgery, corticosteroids contraindicated, chronic ITP)Patients1179013423327<5<5404Grams20,16712,35928,0263,8125,1217128014070,415Grams/Episode506138496065807046Grams per 1,000 Population326131<1<13ITP refractoryPatients97204156376412<55574Grams18,56730,37824,7514,40512,9531,69046080294,004Grams/Episode495936696365516250Grams per 1,000 Population255283134ITP with life-threatening haemorrhagePatients14114101310<5<5<5194Grams25,8642,1401,8031,2321,5477063828233,576Grams/Episode475436687735647148Grams per 1,000 Population3<1<1<1<1<1211Kawasaki diseasePatients140103563021889375Grams5,1514,6041,7911,3051,08035945929715,046Grams/Episode303028364745312731Grams per 1,000 Population<1<1<1<1<1<111<1Lambert-Eaton myasthenic syndromePatients7<510<5000020Grams2,7212,2834,856650000010,510Grams/Episode45453550000040Grams per 1,000 Population<1<11<10000<1Multifocal motor neuropathyPatients18998108384311135496Grams100,74760,20454,72032,22429,1364,7226,4945,213293,458Grams/Episode444338675434535745Grams per 1,000 Population13101112179172112Multiple myelomaPatients46621438223404017<51,177Grams102,72647,72198,4114,4607,53311,9552,81466275,685Grams/Episode303026293332292229Grams per 1,000 Population1382024237<112Myasthenia gravisPatients33521531046281616<5945Grams128,034100,633130,10722,0078,4185,2198,125339402,881Grams/Episode353634503536495736Grams per 1,000 Population171727851021117Neonatal haemochromatosisPatients5<500<50<5010Grams3531,335001201,26002,960Grams/Episode3283006060059Grams per 1,000 Population<1<100<1030<1Non-Hodgkin lymphomaPatients38827750438633515<51,308Grams95,51363,791136,2167,82515,4898,4564,496363332,148Grams/Episode292925273025303327Grams per 1,000 Population121128391611114Other primary immunodeficiencyPatients483410814<5<5<5121Grams12,74010,7252,4741,6652,56188822879032,072Grams/Episode11181616915102213Grams per 1,000 Population22<1<122<131Other relevant haematological malignanciesPatients230711502939129<5533Grams38,76415,67630,6783,6247,9321,9341,661288100,556Grams/Episode282925192828212426Grams per 1,000 Population536154414PolymyositisPatients163591091042<56<5393Grams57,09329,14948,8304,57115,1901,0862,137359158,414Grams/Episode354738444437493639Grams per 1,000 Population7510292517Severe combined ImmunodeficiencyPatients71317<5<500039Grams1,7432,3114,44013560008,636Grams/Episode992017600013Grams per 1,000 Population<1<1<1<1<1000<1Stiff person syndromePatients251321<50<50062Grams13,4874,83011,61486501,3380032,134Grams/Episode433837390370040Grams per 1,000 Population2<12<103001Wiskott-Aldrich syndromePatients<5<50<500005Grams33465067800001,176Grams/Episode336026000011Grams per 1,000 Population<1<10<10000<1X linked agammaglobulinaemiaPatients325017650<5<5110Grams10,79116,8895,9331,6981,815024160037,968Grams/Episode2520171031052419Grams per 1,000 Population131<110<122Chapter 5 TotalPatients4,9982,90935857037712722397613,328Grams1,501,589910,5631,150,002261,791223,15782,66873,79120,3064,223,866Grams/Episode313329393433274131Grams per 1,000 Population19615223910113116018883176Chapter 6??????????Acute disseminated encephalomyelitisPatients282023<50<50<575Grams5,2913,5573,279200323016012,630Grams/Episode3837372002708037Grams per 1,000 Population<1<1<1<10<10<1<1ANCA (PR3 or MPO)-positive idiopathic rapidly progressive glomerulonephritisPatients<5<58000<5014Grams4501,0901,90700017503,622Grams/Episode30444300018039Grams per 1,000 Population<1<1<1000<10<1Autoimmune haemolytic anaemiaPatients45382478<500125Grams7,2725,8253,4939362,3761800020,081Grams/Episode4158314177360045Grams per 1,000 Population<1<1<1<11<100<1Bullous pemphigoidPatients16<56<5000028Grams11,0871,8204,236660000017,803Grams/Episode47673644000045Grams per 1,000 Population1<1<1<10000<1Churg-Strauss syndromePatients0<500<5000<5Grams017500140000315Grams/Episode0250014000039Grams per 1,000 Population0<100<1000<1Cicatricial pemphigoidPatients<5<57<5<50<5021Grams3,6841,8002,6021,8803,76004,035017,761Grams/Episode886745134700122078Grams per 1,000 Population<1<1<1<120100<1Evans syndromePatients0<50<50000<5Grams016001950000355Grams/Episode080065000071Grams per 1,000 Population0<10<10000<1Foeto-maternal /neonatal alloimmune thrombocytopenia (Antenatal)Patients<556<5<5<50021Grams2,2684,3854,6832,4842,1752400016,235Grams/Episode7669787162600071Grams per 1,000 Population<1<1<1<11<100<1Foeto-maternal /neonatal alloimmune thrombocytopenia (Neonatal)Patients95<55<5<5<5023Grams3321327636099Grams/Episode343333303Grams per 1,000 Population<1<1<1<1<1<1<10<1Haemophagocytic syndromePatients23138<5<500049Grams2,1561,5891,1653975600005,867Grams/Episode354438369300040Grams per 1,000 Population<1<1<1<1<1000<1HSCT (for prevention of GvHD in high risk Allogeneic HSCT).Patients<50000000<5Grams48000000048Grams/Episode24000000024Grams per 1,000 Population<10000000<1IgG subclass deficiency EXISTING patients onlyPatients022<5<5<5<50031Grams03,20742351549948005,097Grams/Episode028212331220027Grams per 1,000 Population0<1<1<1<1200<1IgG subclass deficiency. Existing patient with suppurative lung diseasePatients16190<5<5<50042Grams5,5454,22105194777320011,494Grams/Episode302902325290029Grams per 1,000 Population<1<10<1<1100<1IgM para-proteinaemic neuropathyPatients391237<56<50098Grams13,1022,87314,8612,8741,5907670036,066Grams/Episode3735346832430037Grams per 1,000 Population2<131<11002ITP in childrenPatients2842481016<55<5152Grams2,1981,8422,1322491,605435462248,947Grams/Episode293227233124311229Grams per 1,000 Population<1<1<1<1<1<11<1<1Kidney transplantation post-transplantPatients6317558172212<5<5349Grams8,03047,68814,3523,1222,0884,38353061680,807Grams/Episode223020382246447727Grams per 1,000 Population183118133Kidney transplantation pre-transplantPatients32248<55<5<5072Grams2,1342,8101,05232577916518707,451Grams/Episode27141681315547020Grams per 1,000 Population<1<1<1<1<1<1<10<1Microscopic polyangiitisPatients00<5<50000<5Grams001682880000456Grams/Episode002424000024Grams per 1,000 Population00<1<10000<1Multiple sclerosis - severe relapse with no response to high dose methylprednisolonePatients5<550<500013Grams1,3546241,3090200003,307Grams/Episode35243402000032Grams per 1,000 Population<1<1<10<1000<1Multiple sclerosis in pregnancyPatients<50<5000005Grams657026700000924Grams/Episode370300000034Grams per 1,000<10<100000<1Multiple sclerosis in young patients severe/relapsing/remitting in whom other therapies have failedPatients170<5000<5019Grams4,028034800015004,526Grams/Episode3003500025030Grams per 1,000 Population<10<1000<10<1Opsoclonus myoclonus ataxiaPatients126<5<5<500027Grams2,1079272551682,0080005,465Grams/Episode271615135400027Grams per 1,000 Population<1<1<1<11000<1Pemphigus foliaceusPatients<5<5<5000006Grams2,1575401,121000003,818Grams/Episode4960350000045Grams per 1,000 Population<1<1<100000<1Pemphigus vulgarisPatients1558<5<50<5034Grams10,4042,1756,1832,23864001,615023,254Grams/Episode537053971600124060Grams per 1,000 Population1<11<1<1040<1Post transfusion purpuraPatients0<5<500000<5Grams06927000000339Grams/Episode069450000048Grams per 1,000 Population0<1<100000<1Secondary hypogammaglobulinaemia (excludes haem malignancies)Patients2691251973414158<5652Grams57,59122,35151,5625,4052,4565,26083439145,497Grams/Episode262523161833223924Grams per 1,000 Population841121102<16Specific antibody deficiencyPatients10539495019<58<5268Grams28,03710,31213,77312,0565,4535032,14912272,403Grams/Episode18232315142424918Grams per 1,000 Population42353<15<13Toxic epidermal necrolysis/Steven Johnson syndromePatients1921<5<5<5<5<5<553Grams2,6492,92410851024349060307,014Grams/Episode5561912849163601557Grams per 1,000 Population<1<1<1<1<1<1<1<1<1Transplant - Solid OrganPatients0<5000000<5Grams0162000000162Grams/Episode02700000027Grams per 1,000 Population0<1000000<1TSS - staphylococcalPatients13328<5<5<5<5<563Grams1,7943,8511,0613691824419237,532Grams/Episode6977665398164370Grams per 1,000 Population<1<1<1<1<1<1<1<1<1TSS - streptococcalPatients3127349<5<5<50109Grams4,2484,3544,5721,21913290587015,202Grams/Episode70938687334553079Grams per 1,000 Population<1<1<1<1<1<110<1Wegeners granulomatosisPatients0<5<50<50006Grams06504903240001,023Grams/Episode0282502700028Grams per 1,000 Population0<1<10<1000<1Chapter 6 TotalPatients7906285431681175438102,315Grams178,320132,001134,84936,29027,39814,76310,982994535,596Grams/Episode293127242836463429Grams per 1,000 Population23222814162928422Condition?NSWVICQLDWASATASACTNTNationalChapter 7??????????Acute leukaemia in childrenPatients012000<50013Grams085500012000975Grams/Episode033000240031Grams per 1,000 Population0<1000<100<1Autoimmune congenital heart blockPatients<50<500000<5Grams6060000012Grams/Episode606000006Grams per 1,000 Population<10<100000<1Autoimmune neutropeniaPatients<5<5<500<50<56Grams447375150008001,1162,168Grams/Episode507530004008664Grams per 1,000 Population<1<1<100<105<1Autoimmune uveitisPatients<50<500000<5Grams473017500000648Grams/Episode470350000043Grams per 1,000 Population<10<100000<1Catastrophic antiphospholipid syndromePatients<5<55<5000<513Grams482181813149000801,705Grams/Episode409137210001636Grams per 1,000 Population<1<1<1<1000<1<1Coagulation factor inhibitorsPatients<5<56<5<500013Grams6352003,120784950004,528Grams/Episode4510089393300067Grams per 1,000 Population<1<1<1<1<1000<1Devic disease (neuromyelitis optica)Patients2656<5<5<5<5042Grams7,6671,5381,1371,625260150100012,477Grams/Episode323526125293020034Grams per 1,000 Population<1<1<1<1<1<1<10<1Diabetic AmyotrophyPatients<5<5<5000009Grams1,4044851,023000002,912Grams/Episode2632280000028Grams per 1,000 Population<1<1<100000<1Epidermolysis bullosa acquisitaPatients000<5<50<50<5Grams0002,8869002,00604,982Grams/Episode0007490087079Grams per 1,000 Population0001<1050<1Epilepsy (rare childhood cases)Patients<57<56<500020Grams6962,4968941,3472520005,685Grams/Episode414343364200041Grams per 1,000 Population<1<1<1<1<1000<1Graves ophthalmopathyPatients00<5<500005Grams002,36045000002,810Grams/Episode005490000057Grams per 1,000 Population00<1<10000<1Haemolytic disease of the newbornPatients26201111130<5<586Grams1,0683,9621923425101835,636Grams/Episode23511224303331Grams per 1,000 Population<1<1<1<1<10<1<1<1Haemolytic transfusion reactionPatients<5<5000000<5Grams200110000000310Grams/Episode505500000052Grams per 1,000 Population<1<1000000<1Hashimoto encephalopathyPatients<5<5<5<5000010Grams1,3379463301,65500004,268Grams/Episode29323057000037Grams per 1,000 Population<1<1<1<10000<1Limbic Encephalitis (nonparaneoplastic)Patients544170510<56<5188Grams12,3558,16621,9139701,5833682,19455048,098Grams/Episode342932463726563733Grams per 1,000 Population215<1<1<1622Limbic Encephalitis (Paraneoplastic)Patients14915<5<50<5046Grams2,3131,1902,982490687045308,114Grams/Episode2729363833024031Grams per 1,000 Population<1<1<1<1<1010<1Myocarditis in childrenPatients6116<5<500029Grams4871920454300001,055Grams/Episode7262018800020Grams per 1,000 Population<1<1<1<1<1000<1PANDAS/tic disordersPatients<5<5<5<50<5006Grams2447921711200120001,447Grams/Episode41361711200600045Grams per 1,000 Population<1<1<1<10<100<1Paraneoplastic cerebellar degenerationPatients<50<5<5<5<5008Grams1000180850175274001,579Grams/Episode200305335300039Grams per 1,000 Population<10<1<1<1<100<1Paraneoplastic cerebellar degeneration (Yo antibodies)Patients<500<5<5<5005Grams1600066535256001,116Grams/Episode40006035320047Grams per 1,000 Population<100<1<1<100<1Paraneoplastic Subacute Sensory NeuropathyPatients6500<5<50<517Grams1,3079410048010506553,488Grams/Episode233800283508232Grams per 1,000 Population<1<100<1<103<1Paraneoplastic syndromesPatients0<5<50<5000<5Grams01501,24004290001,819Grams/Episode0304303300039Grams per 1,000 Population0<1<10<1000<1Potassium channel antibody-associated encephalopathyPatients125<5<5<5<50025Grams4,5161,0276409201,1481,170009,421Grams/Episode3337409238330037Grams per 1,000 Population<1<1<1<1<1200<1Pure red cell aplasiaPatients10<58<5<5<50024Grams2,4672213,3946101701,560008,422Grams/Episode39374512285400044Grams per 1,000 Population<1<1<1<1<1300<1Pyoderma gangrenosumPatients81700<500027Grams4,68710,141001,77000016,598Grams/Episode8158006800064Grams per 1,000 Population<12001000<1Rasmussen SyndromePatients11<5<50<50<5019Grams4,0675511,3030715049507,131Grams/Episode373136055038038Grams per 1,000 Population<1<1<10<1010<1ScleromyxedemaPatients6<5<5<5<50<5014Grams3,3672,3722403501,50502707,861Grams/Episode4739803579027047Grams per 1,000 Population<1<1<1<1<10<10<1Sjogren's SyndromePatients10<550<50<5022Grams3,8114891,51001,08802,80009,698Grams/Episode313337068058040Grams per 1,000 Population<1<1<10<1070<1Solid organ - heartPatients95<5<50<50016Grams1,2959994951500132003,071Grams/Episode244335750440032Grams per 1,000 Population<1<1<1<10<100<1Solid organ - heart/lungPatients<5<5<5000007Grams345547324000001,216Grams/Episode5820270000026Grams per 1,000 Population<1<1<100000<1Solid organ - liverPatients8<5<50000011Grams77796000000846Grams/Episode439200000038Grams per 1,000 Population<1<1<100000<1Solid organ - lungPatients315513<55<500106Grams4,99310,4731,8855648611400018,915Grams/Episode3027204029350027Grams per 1,000 Population<12<1<1<1<100<1Solid organ - otherPatients0<5<500000<5Grams0535100000104Grams/Episode087000007Grams per 1,000 Population0<1<100000<1Solid organ - pancreasPatients<50000000<5Grams1150000000115Grams/Episode38000000038Grams per 1,000 Population<10000000<1Susac syndromePatients7080000015Grams6,14803,146000009,294Grams/Episode510460000049Grams per 1,000 Population<10<100000<1Systemic Capillary Leak syndromePatients<5<5<5000<509Grams6722,4963,8790001,36008,407Grams/Episode278990000105077Grams per 1,000 Population<1<1<100030<1Chapter 7 TotalPatients270224188496115216820Grams68,19952,48253,81614,27511,8244,4759,4532,404216,927Grams/Episode353737574135565738Grams per 1,000 Population991157924109Chapter 8??????????Sepsis (other than neonatal sepsis)Patients0000000<5<5Grams000000055Grams/Episode000000055Grams per 1,000 Population0000000<1<1Chapter 8 TotalPatients0000000<5<5Grams000000055Grams/Episode000000055Grams per 1,000 Population0000000<1<1TotalPatients6,0193,72342769169423402969316,331Grams1,748,1081,095,0461,338,667312,355262,379101,90594,22623,7094,976,394Grams/Episode313329373333304231Grams per 1,000 Population22818327812015419724097208Note: The national patient count only includes one count for each patient. This may result in the sum of the state and territory totals being greater than the national total.Appendix E – Grams Ig Issued by State and Territory??NSWVICQLDWASATASACTNT2005-06?Imported Ig76,36852,097134,4757,76515,30013,6088,165?Domestic Ig452,565361,665219,633152,127109,51533,83721,7748,0042006-07?Imported Ig103,27088,39879,39320,57718,37511,0657,170?Domestic Ig493,172407,244337,301155,82192,95850,58326,4706,7322007-08?Imported Ig105,633111,01085,05538,44518,41611,74016,875Domestic Ig599,126423,170400,144148,986108,59652,75527,3936,8252008-09?Imported Ig249,905131,228171,36742,89527,60419,96514,200?Domestic Ig562,320417,574383,865143,628128,51153,74522,84110,5032009-10?Imported Ig252,416101,930200,26416,24831,24417,11011,550?Domestic Ig668,526507,038439,089162,963143,28561,68633,2258,6102010-11?Imported Ig136,72893,835107,79830,10827,3838,84311,90080Domestic Ig887,016577,260631,545167,745139,29676,19745,5409,0992011-12?Imported Ig265,995144,284183,43559,90035,77512,13814,70830Domestic Ig874,995570,969674,277150,294145,13473,49152,44613,4402012-13?Imported Ig467,371321,085361,65492,91472,61316,43626,6489,551Domestic Ig804,375484,680589,662132,108123,81064,30548,4806,7442013-14Imported Ig469,174312,713291,46070,70987,90124,06930,62610,429Domestic Ig934,478584,561771,037168,295138,87667,77653,7236,0362014-15Imported Ig593,045416,868458,189111,570107,34341,60832,19912,861Domestic Ig930,412579,560735,658155,977135,79557,98759,2104,8632015-16Imported Ig724,960451,770584,275159,631103,16548,00341,26418,489Domestic Ig1,004,528643,340771,182152,900167,59953,20752,6015,589Appendix F – Unique Patients by Quarter and State and TerritoryYearQuarterNSWVICQLDWASATASACTNTAUST2009-10Q12,4341,3671,644380400183112236,508Q22,4961,3781,667356440177109206,619Q32,5541,3861,682353395183102156,640Q42,6021,4511,752371413189120226,8892010-11Q12,6921,4921,839376420197143227,148Q22,7811,5331,886394394205132217,315Q32,7521,5321,884376396211130157,262Q42,7911,6221,946385417197142237,4962011-12Q12,9211,6582,047407419199142277,794Q22,9711,6282,115413428206137227,898Q32,9491,5902,150401430203150237,860Q42,9611,6322,215405458202154298,0192012-13Q13,1071,7512,391449449205168328,494Q23,1391,8092,360436462196171268,557Q33,2111,7532,298410454183164338,465Q43,3091,8212,378425463187170368,7372013-14Q13,4061,8902,472435506204181369,081Q23,4281,9712,510472481209172369,237Q33,4401,9522,583454502213188309,317Q43,5502,0422,660513493215188349,6532014-15Q13,7132,1502,7635185452381894110,099Q23,7252,1692,7195215062282023210,057Q33,7332,1612,7725105302151912510,096Q43,8462,2492,8685145552232023110,4402015-16Q14,1012,3543,0265545872342024611,033Q24,1032,3463,0675835912251983811,081Q34,1612,3583,0735835952261974111,164Q44,2632,4003,1326026012272075011,424Appendix G – System Source for Tables and Figures TOC \h \z \c "Table" Table 1Growth in Ig grams issued since 2006-07IDMSTable 2Percentage change in grams issued over time by state and territoryIDMSTable 3Annual numbers of patients, treatment episodes and gramsSTARS & IDMSTable 4Basic numbersSTARSTable 5Issues of domestic Ig compared with imported IgIDMSTable 6Ig issues (g) by Criteria chapterSTARSTable 7Ig issues by Criteria chapter (percentage)STARSTable 8Ig grams issued for top 10 diagnostic groups over timeSTARSTable 9Difference in grams issued for secondary hypogammaglobulinaemia (percentage)STARSTable 10Patient numbers and age for the top 20 conditionsSTARSTable 11Ig grams issued by clinical disciplineSTARSTable 12Grams of Ig issued by state and territoryIDMSTable 13Patient numbers by state and territory: chronic inflammatory demyelinating polyneuropathySTARSTable 14Patient numbers by state and territory: common variable immunodeficiency diseaseSTARSTable 15Patient numbers by state and territory: myasthenia gravisSTARSTable 16Patient numbers by state and territory: chronic lymphocytic leukaemiaSTARSTable 17Patient numbers by state and territory: multiple myelomaSTARSTable 18Ig issued per 1,000 population by state and territory for top 10 conditionsSTARSTable 19Ig grams per episodeSTARSTable 20Patient numbers for products issued by state and territory in 2015-16STARSTable 21Grams of product issued by state and territory in 2015-16STARSTable 22Treatment episode numbers for products issued by state and territory in 2015-16STARSTable 23Patient numbers for products issued by diagnostic group in 2015-16STARSTable 24Grams of product issued by diagnostic group in 2015-16STARSTable 25Treatment episode numbers for product issued by diagnostic group in 2015-16STARSTable 26NHIg issued from 2011-12 to 2015-16IDMSTable 27Grams of NHIg issued from by state and territoryIDMSTable 28Grams per 1,000 population of NHIg issued by state and territoryIDMS TOC \h \z \c "Figure" Figure 1Ten year trends in issues of IgIDMSFigure 2Ten year trends in expenditure on IgIDMSFigure 3Patients per 1,000 population 2015-16STARSFigure 4Grams of Ig per 1,000 population by state and territory over timeIDMSFigure 5Patient age compared to average Australian ageSTARSFigure 6Patient weights relative to Australian averageSTARSFigure 7Ig expenditure as a proportion of the national blood budgetIDMSFigure 8Ig grams issued by diagnostic groupSTARSFigure 9Proportion of Ig used for top 10 diagnosis groupSTARSFigure 10Ig issues by clinical disciplineSTARSFigure 11Ig issues by clinical discipline for top 10 conditions by state and territorySTARSFigure 12Grams per episode by conditionSTARSFigure 13NHIg Grams issued and grams issued per 1,000 populationIDMS REF _Ref379893813 \h \* MERGEFORMAT Appendix D – Dataset of Ig supply by state/territory 2015-16………………………………………………………………………………………………………………………………………..…STARS REF _Ref393440546 \h Appendix E – Grams Ig Issued by State and Territory …………………………………………………………………………………………………………………………………………….……..…..IDMS REF _Ref393440549 \h Appendix F – Unique Patients by Quarter and State and Territory ……………………………………………………………………………………………………………………………….....STARS ................
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