Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology,...
-
Upload
elaine-pole -
Category
Documents
-
view
214 -
download
0
Transcript of Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology,...
![Page 1: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/1.jpg)
Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006
Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta
![Page 2: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/2.jpg)
Disclosure
Honoraria:
Bayer, Baxter, Sanofi, Astra-Zeneca, Novo-Nordisk, Aventis-Behring, ZLB, Pharmacia, Pfizer - all go towards studentships
Studentships:
Pharmacia, Baxter, Sanofi, Novo-Nordisk, Bayer
Travel Support:
Bayer, Baxter, Astra-Zeneca, Novo-Nordisk, Pharmacia, Canadian Hemophilia Society, Canadian Hereditary AngioEdema Society, Canadian Immunodeficiency Patient Organization, Alberta Association of Radiologists,
Research Funding: Bayer, Baxter, Novartis, NovoNordisk, CHS, Health Canada/Public Health Agency of Canada.
![Page 3: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/3.jpg)
Outline of my presentation
Rare Blood Disorder Initiative
What is it?
What is needed?
How do we get it?
Treatment surveillance
Networks in place in Canada
Sample collection/testing
Inhibitors, HIV
BBPSP
![Page 4: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/4.jpg)
Rare Blood Disorders Initiative
What is it?
It is: specialized care for rare disorders including:
specialized diagnostics
specialized management - immunizations, …
specialized tracking of outcomes
surveillance
![Page 5: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/5.jpg)
Rare Blood Disorders InitiativeHemophilia
Untreated Hemophilia - median survival of 25
Optimal management - near normal life span
Currently we have close to optimal management
Available:
Clinic for early diagnosis, treatment, tracking, surveillance
![Page 6: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/6.jpg)
Rare Blood Disorders InitiativeSickle Cell Anemia
Untreated Sickle cell anemia - median survival of 25
Optimal management - near normal life span
Currently we have something in between.
Needs:
Better and earlier diagnosis, treatment
Better tracking
Better transfusion management
Better chelation management
![Page 7: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/7.jpg)
Exchange transfusionSickle Cell Anemia
Exchange TransfusionMore effective to lower % of sickle cells (<30%)Less iron overloadRequires specialized equipment and staff
![Page 8: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/8.jpg)
Rare Blood Disorders InitiativeHereditary AngioEdema
Undiagnosed HAE common
Mistreated HAE common - antihistamines, steroids, adrenalin don’t work
Optimal management - near normal life span
Currently we have something in between.
Needs:
Better and earlier diagnosis, treatment
Better tracking
Home management
![Page 9: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/9.jpg)
Home RxHemophilia, HAE, PID
![Page 10: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/10.jpg)
Rare Blood Disorders InitiativePrimary Immnodeficiency
Undiagnosed PID common
Mistreated PID common
Optimal management - near normal life span
Currently we have something in between.
Needs:
Better and earlier diagnosis, treatment
Better tracking
Home management - IgSC
![Page 11: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/11.jpg)
Kazatchkine, M. D. et al. N Engl J Med 2001;345:747-755
The IgG MoleculeHypogqammaglobulinemias
![Page 12: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/12.jpg)
IgIV OutcomesHeadache, Aseptic Meningitis
Allergy, anaphylaxis
Acute Renal Failure in the elderly, diabetes
Blood Borne Pathogens - hepatitis C in mid 1990s
IgSC Immunoglobulin
16% IgSC solutions licensed in Europe (ZLB, CSL), IgIm preparation licensed here (Talecris)
Unlicensed (SAP) vs “Off-label” use of IgIM or IGIV for SC
![Page 13: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/13.jpg)
Subgam - Patient Subgam - Patient Satisfaction SurveySatisfaction Survey After 6 months:
All patients(n=43)
P rior SCIG(n=14)
P rior IVIG(n=29)
Adults (>19 y)(n=23)
Children (<12y) (n=15)
Teenagers(12-19 y) (n=5)
0
10
20
30
40
50
60
70
Perc
enta
ge o
f patients
Much more More Same Not as much
![Page 14: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/14.jpg)
IVIG/SCIG Cost Comparison IVIG/SCIG Cost Comparison ((£)£)
Year 1
•Adult Drug Cost•Routine
Consultations and Tests
•Patient Training•Syringe Drivers•Maintenance
•Total Year 1
SCIG Home Based
10,752
673365
1,762804
14,356
IVIG Hospital Based
10,752
673n/an/a
4,812
16,237
![Page 15: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/15.jpg)
Rare Blood Disorders InitiativeBone Marrow Failure
Undiagnosed mild disease common, but there is associated morbidity
Specific treatment not readily available except in trials
Currently we have something in between.
Needs:
Better and earlier diagnosis, treatment
Better tracking
Better transfusion management
Better chelation therapy - ?Exjade
![Page 16: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/16.jpg)
Rare Blood Disorders Initiative
What is needed?
A clinic with dedicated staff consisting of:
1. nurse, admin assistant, data entry person, physician
2. space - offices, stuff, storage, computers
3. Oversight - Multidisciplinary group (patients)
![Page 17: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/17.jpg)
Rare Blood Disorders Initiative
How do we get it?
Lobbying - governments don’t listen to phsycians, so patients must lobby on their own behalf
Organize
meet, talk, argue, reach consensus
establish policy - Rx, safety, …
Network of Rare Blood Disorder Organizations,
![Page 18: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/18.jpg)
FVIII Concentrates purified from up to 100,000 donors
Hemophilia & HIV
![Page 19: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/19.jpg)
QuickTime™ and aTIFF (LZW) decompressor
are needed to see this picture.
http://www.hc-sc.gc.ca/english/protection/krever/
![Page 20: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/20.jpg)
Transmissible Spongiform Encephalopathies (TSEs)
Rodent TSEHamster, Mouse
Sheep TSEScrapie
Bovine TSEBSE
Cat TSEFSE
Mink TSETME
Deer, Elk TSECWD
Human TSEClassical CJD, GSS, FFI, SPKuru, New Variant CJD
� Normal host protein
� Proteinase K sensitivity
� 33–35 kD MW
PrPC PrPSC
� Associated with infectivity
� Proteinase K partial resistance
� 33–35 kD MW
![Page 21: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/21.jpg)
Treatment surveillance
Networks in place in Canada
Quebec Blood Surveillance Network
Health Canada
AHCDC - BBPSP
Sample collection/testing
Inhibitors, HIV
BBPSP - Blood Borne Pathogens Surveillance Project
![Page 22: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/22.jpg)
QuickTime™ and aTIFF (Uncompressed) decompressor
are needed to see this picture.
![Page 23: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/23.jpg)
Canadian Hemophilia Assessment and Resource Management information System (CHARMS) & NACHC
Product distribution(CBS)
Clinical outcomes(clinic)
CBS, HQ
CenterPoint server, Hamilton
Genotyping(AHCDC)Sample archive
Health CanadaManufacturersCBS, QBS
Provincial Governments
Drug CompanyPalm Pilot systems
Adverseevents
Productuse/outcomes
![Page 24: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/24.jpg)
PDA systemsViews- Messages
Q ui ckTi me™ and a TI FF (LZW) decompressor are needed to see thi s pi cture.
Dialog - Bayer (formerly HemaScan) • Began as a PDA based inventory system• Barcodes generated by system• Now includes internet module• Currently in trial in Hamilton, Ste. Justine, Edmonton• Data management Arrowhead
Advoy - Baxter
• Began as a web based management system• Will use consensus barcodes• Now includes PDAs• Currently in trial in Toronto, Edmonton• Data management 3rd party
![Page 25: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/25.jpg)
Machine Readable Labels
![Page 26: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/26.jpg)
![Page 27: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/27.jpg)
Freezer Map
![Page 28: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/28.jpg)
Freezer Map
![Page 29: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/29.jpg)
The Inventory Database
QuickTime™ and aPhoto - JPEG decompressor
are needed to see this picture.
![Page 30: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/30.jpg)
Bruce Ritchie
![Page 31: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/31.jpg)
Bruce Ritchie
QuickTime™ and aMotion JPEG OpenDML decompressor
are needed to see this picture.
QuickTime™ and aMotion JPEG OpenDML decompressor
are needed to see this picture.
![Page 32: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/32.jpg)
Bruce Ritchie
![Page 33: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/33.jpg)
Bruce Ritchie
![Page 34: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/34.jpg)
Gene Therapy for point mutations
• Genotyping• Chimeroplasty
![Page 35: Rare Blood Disorder Initiative, Blood Surveillance Sept 30, 2006 Bruce Ritchie Hematology, University of Alberta, Edmonton, Alberta.](https://reader035.fdocuments.in/reader035/viewer/2022062620/551a0bc355034619378b4c9c/html5/thumbnails/35.jpg)