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ARCHIVED - Evaluation of The Prion Diseases Program

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Annexes

Annex A

Steering Committee

The members of the Steering Committee for the evaluation are listed below.

  • Graham Tipples Director, Surveillance and Reference Services
  • Hank Krueger Director, Business Operations, Public Health Agency of Canada
  • Stephanie Czub Head, National BSE Reference Centre, CFIA
  • Jane Buxton Physician Epidemiologist, BCCDC
  • Jan Trumble-Waddell Senior Advisor, Chief Public Health Officer, Public Health Agency of Canada

Annex B

Evaluation Framework

Evaluation Issues Evaluation Questions Indicators Data Sources
1. Relevance 1.1 Is there a clear and relevant vision and are there clear objectives for activities under PDP?
  • Clear mission and vision statements
  • Ongoing business planning process
  • Evidence of management use of science-based relationship to the global risk context to develop program vision, objectives and activities
  • Policy documentation
  • Program business plan and annual reports
  • External audit report (2005)
  • Reference Services peer review (2006)
  • Staff interviews
  • Staff presentations
  • Senior management interviews
1.2 How has the mandate changed since program inception and what internal and external factors have contributed to the identified changes?
  • Differences in mandate, vision, objectives of PDP over time
  • Identified internal and external factors
  • Policy documentation
  • Program business plan and annual reports
  • Staff Interviews
  • Senior management interviews
1.3 Does the initiative continue to be consistent with the PHAC mandate and government-wide priorities?
  • Degree of integration into NML Strategic and Business Plans
  • Fit with PHAC 5-year Strategic Plan
  • Contribution to PHAC Report on Plans and Priorities
  • Fit with Government of Canada priorities
  • Program documentation,
  • Staff interviews
  • Senior management interviews
  • NML Strategic and Business Plans
  • PHAC DPR, RPP, MRRS
  • PHAC 5-year Strategic Plan
1.4 Is there a continued need for the program? Do current client needs and risk environment indicate a continued need?
  • Current risk context for prion diseases
  • Projections of future course for existing prion disease epidemics (vCJD; BSE; CWD; scrapie)
  • Client service uptake (physicians)
  • Perceived utility of services
  • Collaborations (e.g., PrioNet Canada)
  • Sufficient capacities elsewhere? (e.g., provincial labs; academic sector)
  • Other jurisdictions identification of continued need for similar programs (USA; Europe)
  • Statistics and epidemiology for human and animal prion diseases (Canada; international)
  • Risk assessments for various routes of prion transmission
  • International recognition of program contributions
  • Staff interviews
  • Client interviews
  • Stakeholder interviews
  • Documentation from similar programs in other jurisdictions (audits, plans, reports)
2. Success 2.1 Are activities being implemented as planned and producing the expected outputs? (See Logic Model)
  • Achievement of CJDSS activities and outputs:
  • CJD surveillance statistics, CJD database, publications, support and consultation, expert advice and consultation, international liaison, 1-800 number
  • Achievement of CJD Reference Services and Applied Research activities and outputs:
  • Reference test service delivery, improved existing laboratory methods, new test evaluations, quality and performance standards, publications,  reports, presentations
  • Achievement of Pathobiology Research activities and outputs:
  • Studies, leads for novel approaches to laboratory testing, publications, presentations, case reports,  intellectual property
  • Achievement of National Biorepository activities and outputs:
  • Disease-specific collections, healthy population collections, service delivery for use of HBM
  • Policy documentation
  • Physical inspection and tour of laboratories and surveillance office
  • CJD Surveillance System website External audit report (2005)
  • Reference Services peer review (2006)
  • ISO 17025 audit reports (Standards Council of Canada): 2006; 2008
  • Program business plans and annual reports
  • Staff Interviews
  • Senior management interviews
  • Client interviews
  • Stakeholder Interviews
  • Staff presentations, Publications
  • Advisory documents
  • Collaborations
2.2 Have the expected outcomes and reach been achieved through program activities? (See Logic Model)
  • Audits against international performance benchmarks for similar programs in other jurisdictions
  • Successful public health interventions (e.g., Canadian vCJD)
  • Use of surveillance data to support risk assessment for CJD in Canada
  • Impact of publications and presentations
  • Solicitation and uptake of advice and information by other government departments
  • Provincial/Territorial public health collaborations
  • Business Plans, Annual Reports, Reviews
  • Staff Interviews
  • Senior management interviews
  • Client interviews
  • Stakeholder Interviews
  • Staff presentations
  • Scientific databases (e.g., Scopus; Science Citation Index)
  • Professional honours and awards
  • External audit report (2005)
  • Reference Services peer review (2006)
  • Advisory documents
  • Collaborations
2.3 Have there been any unexpected outcomes?
  • Identified unexpected outcomes
  • Staff Interviews
  • Management Interviews
  • Stakeholder Interviews
  • Document Review – annual reports, publications
3. Design and Delivery 3.1 Are there appropriate management and decision-making structures in place to meet the objectives?
  • Well-defined governance structure
  • Clear lines of reporting
  • Definition of responsibilities for issues at levels of program, NML, PHAC and Health Portfolio
  • Explicit roles and responsibilities
  • Timely business and financial planning and reporting
  • Quality management system
  • Human resources strategy and processes
  • Performance monitoring and review processes
  • Program policy documents
  • Staff interviews
  • Senior management interviews
  • Program business plan
  • NML and PHAC Strategic Plans
  • ISO accreditation documents
  • Organization charts
  • Performance review documents
3.2 Are there effective partnerships / linkages to external programs in place and do they support the delivery of the program?
  • Functioning agreements with external clients and stakeholders for communication, data sharing and service delivery
  • Alignment of partnership activities with program objectives and NML and PHAC priorities
  • Active linkages to other PHAC programs to achieve common goals and address unmet needs
  • Documentation on roles, organization, agreements, partner interactions and other arrangements (MOUs, proposals, joint publications)
  • Staff interviews
  • Stakeholder interviews
  • Client interviews
3.3 Has there been an assessment and strategic use of lessons learned?
  • Processes to collect and use performance information
  • Appropriate changes in approach or activities based on learning
  • Performance assessments
  • Annual reports
  • Peer reviews
  • Response to recommendations
  • Review of management processes and planning/reporting documents
  • Staff interviews
  • Senior management interviews
3.4 Are the resources adequate to achieve the expected outputs and outcomes?
  • Level of resources over time
  • Identified gaps and shortfalls
  • Unmet outputs and outcomes
  • Financial records
  • Staff interviews
  • Management interviews
3.5 Are there alternative means of achieving these same program objectives that might be more efficient or effective?
  • Existence of similar capacities in Canadian public health environment
  • Advantages/disadvantages of locating program in federal sector
  • Assessment of alternative delivery models
  • Assessment of opportunities to enhance program by collaboration
  • Staff Interviews
  • Client interviews
  • Stakeholder Interviews
  • International benchmarks
  • Web review of other models

Annex C - Logic Model

Annex C – Logic Model

Annex D

List of Documents and Key Web Sites Reviewed
Title Date Author
Program Documents
Project Management Plan: Capacity to conduct surveillance and laboratory investigation of prion diseases: epidemiology and surveillance 1998 LCDC
Project Management Plan: Winnipeg Human and Animal Prion Reference Laboratory 1998 LCDC
National Microbiology Laboratory, Business Plan 06/07 HGPD Apr 13 2006 PDP
Annual Report Template for National Microbiology Laboratory Programs Reporting Period April 1 2006 – March 31 2007 2007 PDP
Annual Report Template for National Microbiology Laboratory Programs Reporting Period April 1 2005 – March 31 2006 2006 PDP
Annual Report / Business Plan for National Centres HGPD Program, Reporting Period April 1 2005 – March 31 2006 Evaluation Form 2006 PDP
Canadian Public Health Laboratory Network, Reference Centre Advisory Subcommittee: Annual Report/Business Plan for National Centres, Evaluation Form    
PDP Organization Chart Jan 19 2007 PDP
The Canadian Creutzfeldt-Jakob Disease Surveillance System (CJDSS): Purposes, Functions and Basic Results (presentation) Nov 2006 M Coulthart
Report: UK Prion Laboratory Scan Oct 1998 M Coulthart
EUROCJD Surveillance Audit Template Mar 2008 M Coulthart
International Audit of the National Canadian CJD Surveillance System Apr 2005 University of Edinburgh
Canadian Creutzfeldt-Jakob Disease Surveillance System, Five-Year Cumulative Report 1998-2003 Apr 2005 PHAC
PHAC Documents
Reports on Plans and Priorities Various PHAC
Departmental Performance Reports Various PHAC
Reference Documents
WHO Guidelines on Tissue Infectivity Distribution in Transmissible Spongiform Encephalopathies
http://www.who.int/bloodproducts/TSEPUBLISHEDREPORT.pdf		 2006 WHO
WHO Manual for Surveillance of Human Transmissible Spongiform Encephalopathies, including Variant Creutzfeldt-Jakob Disease http://whqlibdoc.who.int/publications/2003/9241545887.pdf 2003 WHO
Other
Health Canada Policy on the Approval and Management of Adjunct Professorships 2007 HC

A special meeting of the Steering Committee was held on September 12, 2008 to elicit expert input into the key areas of success and relevance of the PDP. The objectives of the session were to:

  • provide expert opinion on the continued need for the Prion Diseases Program (PDP) within PHAC, given client needs and the current risk environment;
  • provide expert opinion on the degree of Program success achieved to date in the areas of surveillance, reference services and research; and
  • discuss future directions that may guide PDP design going forward.

The following are the key program success and opportunities for improvement that were identified during the workshop discussions.

Successes

  • The CJD Surveillance System appears to be effective, finding the expected number of cases when compared to international benchmarks. The Canadian system compares favourably to the European model and is more comprehensive than the US system.
  • While PHAC involvement in primary diagnosis and data collection of disease surveillance is unique with respect to human prion diseases, the rarity and specialized expertise required to diagnose indicates a need for ongoing federal involvement.
  • Reference services appear to be fulfilling the required functions with good high quality work and ISO Accreditation in place.
  • Quality research is taking in place evidenced by peer-reviewed publications, and other outputs.
  • It is viewed as very important to maintain research capacity to ensure expertise within PHAC.
  • Collaborations with other government departments and other organizations on research are effective.

Opportunities for Improvement

  • Surveillance system could be making better use of data in terms of publishing and sharing.
  • There is a need for the surveillance system to become better linked with provinces and territories.
  • The Program relies heavily on a single individual and succession planning should be considered.
  • Improvements (e.g. quicker turnaround times, new tests, better integration, etc) in the reference services could be made.
  • Productivity of the PDP research activities is less than other NML programs in terms of peer reviewed publications.
  • The research activities could be more clearly linked with the PHAC mandate, research priorities developed and promulgated. Research activities could be better integrated with the CJDSS.
  • The recent relocation of the basic pathobiology research out of the PDP raises questions of integration. Other mechanisms may need to be considered to ensure alignment of research priorities.
  • Stable funding for research needs to be maintained.

Annex G

Activities and Outputs Identified in Annual Planning and Reporting

2005 – 2006 and 2006 - 2007
Activity, Outputs Status Source
CJDSS
A. CJDSS Operations
Core Activities:
  • Management of over 200 inquiries and approximately 100 formal referrals annually, involving clinical and laboratory investigation of suspect, possible, probable and confirmed CJD in Canada.
Ongoing annually CJD-SS cumulative report pg 9, 32
  • Securing of informed consent for autopsy, laboratory testing, record review, interview, etc. for all referrals to CJDSS
Ongoing annually CJD-SS cumulative report pg 8
  • On-site medical-record recovery and family interviews for approximately 30 definite and probable cases of CJD annually.
Ongoing annually CJD-SS cumulative report pg 9
  • Responses to numerous requests for current information on CJD from government officials, elected representatives, government departments and agencies, physicians, affected families, journalists and the general public.
Ongoing CJD-SS cumulative report pg 39
  • Collection, management and reporting of national data on incidence, prevalence and diversity of human prion diseases in Canada, according to procedural standards and performance benchmarks set by international CJD surveillance organizations (EUROCJD) and public health organizations (WHO)
Published   In progress Published C.3.2. Abstracts 2005 CJD-SS cumulative report pg 14, 40
Annual Report Template 05-06 – Coulthart pg 24
  • Management of liaison and applications for annually renewed ethics approvals for CJD surveillance with approximately 60 Institutional Review Boards across Canada.
Ongoing annually CJD-SS cumulative report pg 8
Non-core Activities:
  • Delineation and ongoing optimization of CJDSS operations within the Canadian legal environment, including information-sharing with provincial Chief Medical Officers of Health as well as local and regional public health officials, while jointly satisfying the requirements of effective surveillance and the needs of participating patients, families, physicians and other healthcare providers.
Incomplete to date International Audit 2005 – pg 2
  • Continuous reassessment of the structure and operation of CJDSS, in the context of ongoing global developments in the science and epidemiology of CJD.
In progress CJD-SS cumulative report pg 39
  • Update of CJDSS Surveillance Protocol, in light of current status and recent developments. (2006 – 2007 only)
In progress NML Annual Report 2007-2008, Module B: Protocol revision is in progress; not yet submitted to Health Canada REB
B. Reference services
Core Activities:
  • Laboratory testing (neuropathology; biochemistry; molecular genetics) to directly support clinical investigations of human prion diseases.
 Ongoing, annually
“Commended” 		CJD-SS cumulative report pg 28
International Audit 2005 pg 3
Non-core Activities:
  • Development of procedures and documentation for laboratory accreditation under ISO 17025.
In progress Annual Report Template 05-06 Coulthart pg 20
  • Development of an electronic laboratory information management system (LIMS) to support CJD reference services.
Incomplete Annual Report Template 05-06 Coulthart pg 22
  • Successful internal audit on ISO documentation and workflow for 14-3-3 assay (Standard Operating Procedures; personnel records; quality control, etc.).
 In progress CJD-SS cumulative report pg 11, 27
  • Successful external audit by Standards Council of Canada (November 2006) for 14-3-3 assay. (2006 – 2007 only)
Done Annual Report Template 08-09 p.22 confirm when it was achieved
  • Under the auspices of National Microbiology Laboratory IT infrastructure, development of electronic Laboratory Information Management System (LIMS) to support HGPD laboratory reference services.
Incomplete Annual Report Template 05-06 Coulthart pg 22
C. Advice and education
Core Activities:
  • Provision of expert advice to Health Canada regulators and policy-makers on risks posed by human and animal prion diseases in Canada, particularly in the context of blood safety.
Ongoing CJD-SS cumulative report pg 39
  • Prompt investigations of and support for prion-related health events such as inadvertent exposure to CJD through surgical procedures (e.g., transplantation).
Ongoing CJD-SS cumulative report pg 21
  • Responses to inquiries from general public, via toll-free telephone line (1-888-489-2999).
Ongoing PHAC CJD website lists 1-800.
NML Annual Report 2005-2006 and 2006-2007 both indicate the provision of 1-800 services
  • Numerous presentations and publications
Ongoing CJD-SS cumulative report pg 40
Technical Research in Prion Diseases
Core Activities:
  • Studies of existing and novel biological markers to: (i) reliably distinguish, using in vitro tests, between clinical-stage prion disease and many other conditions with clinical presentations consistent with CJD; and (ii) detect and confirm the presence of prion infection in preclinical stages.
 Published   Complete C.3.2 Abstracts 2005  
  • Development of rodent and primate models for prion diseases affecting, or potentially affecting, humans and animals
Complete  
  • Genetic and phenotypic analyses of typical and atypical Canadian BSE cases, to study epidemiology of the disease in Canada (in collaboration with the CFIA).
Published C.3.2 Abstracts 2006  
  • Genetic and phenotypic analysis of CWD in native Canadian cervid species, to study epidemiology of the disease in Canada (collaboration with the CFIA)
Complete NML Annual Report 2008-2009, Module D – S. Booth, Project Title: Biomarker discovery for the diagnosis of prion diseases.
  • Continuous development of improved versions of existing techniques for laboratory analysis; e.g., obtaining genetic information from atypical specimens, such as formalin-fixed and/or microdissected tissues, or extracellular nucleic acids found in serum and CSF.
 Published   Done C.3.1 List of Peer Reviewed Publications  
Non-core Activities:
  • Participation in formation of a Canadian network for therapeutic trials of CJD.
    Documentation not found
  • Participation in the formation of a Canadian research network (Network of Centres of Excellence) for prion diseases, PrioNet Canada (established December 2005). (2005 – 2006 only)
Done Director M. Coulthart was a founder of PrioNet (PrioNet website)
  • Participation in the operation of a Canadian research network (Network of Centres of Excellence) for prion diseases, PrioNet Canada (established December 2005). (2006 – 2007 only)
Done M. Coulthart and S. Booth are Full Scientific Members
Pathobiology Research in Prion Diseases
  • Implementation of genomics technology platforms for identification of molecular pathways for prion-induced neurodegeneration and potential targets for treatment.
In Progress NML Annual Report 2008-2009 Module D – S. Booth, Project Title: Identification of MiRNA Driven Regulatory Circuits Involved in Prion-Induced Neurodegeneration
  • Implementation of proteomics technology platform for identification of biomarkers to aid in diagnosis of prion diseases.
In Progress NML Annual Report 2008-2009 Module D – S. Booth, Project Title: The use of proteomics for surrogate markers of prion infection
  • Implementation of laser capture microdissection technology platform for enhanced resolution of pathobiologic changes in prion-infected cells and tissues.
  Documentation not found
  • Implementation of reverse genetics technology platforms for identification of host gene products essential for prion pathogenesis
In Progress NML Annual Report 2008-2009 Module D – D. Knox, Project Title: A Genetic “Knock-Down” Approach to Identify Host Cellular Factor Essential for Infectious Agent Replication and Pathogenesis
  • Development of mouse models for strain-typing of existing and novel prion diseases.
In Progress NML Annual Report 2008-2009 Module D – S. Booth, Project Title: Investigation of protein - protein interactions from scrapie infected mice brain tissue using NAPPA
  • Proteomic analysis of rodent, bovine, and cervid urine (collaboration with the Canadian Food Inspection Agency and the German National BSE reference laboratory.)
In Progress NML Annual Report 2007-2008 Module D – D. Knox, Project Title: The Identification of Molecular Biomarkers in Bovine Urine
Sept 2008 – news story re: success in the project
  • Studies of genetic and epigenetic correlates and risk factors for CJD, BSE and other prion diseases.
  NML Annual Report 2006-2007 Module B Manuscript in preparation describing results of biochemical and genetic studies of Canadian-born cases of BSE, 2003-2008 (Poster presented at PrioNet Canada  annual scientific meeting (Toronto, Ontario Feb 2008)
Technical plan under development for genetic epidemiology studies of sporadic CJD
  • Studies of mechanisms of neurotoxicity and cell death in prion diseases.
Done NML Annual Report 2006-2007 Module D (S. Booth) Title: Dissection of the molecular pathways leading to neuronal cell death in prion induced neurodegeneration
2007-2008
Goal#1  Develop an enhanced public health network for human prion diseases in Canada, incorporating the Canadian Creutzfeldt-Jakob Disease Surveillance System (CJDSS).
Activities / Outputs Status Source
1.1 Complete the establishment of an integrated PHAC Prion Diseases Program, and its management transition from CIDPC to NML by:
  • preparing a unified business plan and costing for an integrated PHAC Prion Diseases Program;
 Done 2006-07 NML Business Plan completed
  • completing staffing actions and secure related office accommodations; and
 In progress NML Annual Report 2007-2008 Module B: Staffing and office space issues partially resolved
  • completing the setup and operationalization of contracts for neuropathology reference laboratory services, funeral transport services, field nurses and medical consultants.
Done NML Annual Report 2007-2008 Module B
1.2 Continue operations of the CJDSS, to detect, characterize and report all cases of human prion diseases in Canada through:
  • providing expert consultation and logistic support to physicians on diagnosis and handling of suspected CJD cases;
 Done NML Annual Report 2007-2008 Module B
  • conducting detailed epidemiological and demographic investigations on confirmed cases of CJD;
 Done NML Annual Report 2007-2008 Module B
  • maintaining monthly reporting of confirmed cases of CJD to PHAC and EUROCJD; and
 Done NML Annual Report 2007-2008 Module B
  • providing information and support for families participating in CJDSS.
Done NML Annual Report 2007-2008 Module B
1.3 Update and reconstitute the study protocol for CJDSS by:
  • bringing medical, scientific and public-health context and rationale up to date;
 Done NML Annual Report 2007-2008 Module B
  • preparing / redesigning CJD-related consent forms, protocols, questionnaires, pamphlets, etc.
In progress Protocol revision is in progress; Re-design of questionnaire is in progress
  • submitting revised protocol to Health Canada Research Ethics Board for review and once approved, circulate to currently participating Canadian Institutional Review Boards.
In progress NML Annual Report 2007-2008 Module B: not yet submitted to Health Canada REB.
1.4 Improve linkages and communication between CJDSS and provincial and regional public health authorities by:
  • providing practical input for developing PHAC legislation governing the handling of personal health information within public health; and
 In progress NML Annual Report 2007-2008 Module B: Federal/Provincial sharing of personal health information in relation to CJD has been discussed during drafting of PHAC legislation and regulations
  • continuing consultation with provincial Chief Medical Officers of Health regarding optimization of communication between CJDSS and provincial public health authorities.
In progress NML Annual Report 2007-2008 Module B: Discussion with Chief Medical Officers of Health continues
1.5 Conduct publication, outreach and education activities for human prion diseases by:
  • preparing peer-reviewed manuscripts to publish results from CJDSS;
 In progress NML Annual Report 2007-2008 Module B: Several peer-reviewed manuscripts describing 10-year findings of CJDSS are in development
  • conducting information sessions in Canadian centres of medical education;
 In progress NML Annual Report 2007-2008 Module B: Half-day information session successfully conducted at McMaster University, April 20 2007; return invitation has been extended for Fall 2008
  • improving PHAC web-based informational resources;
 In progress NML Annual Report 2007-2008 Module B
  • reinforcing CJD surveillance by strengthening links with patient advocacy and support groups; and
 In progress NML Annual Report 2007-2008 Module B: Connection with CJD Foundation established through presentation at CJD2007 conference in Vancouver November 2007; invited for return engagement at CJD2008, Washington DC July 2008
  • responding to requests for interviews with communications media.
Ongoing NML Annual Report 2007-2008 Module B: Numerous (>10) media interviews conducted
1.6 Provide expert consultation and advice on risks of human prion diseases in Canada by:
  • assisting, consulting and advising provincial and regional public-health authorities and briefing the Chief Public Health Officer and Minister of Health regarding CJD-related issues; and
 Ongoing NML Annual Report 2007-2008 Module B: External consultations provided as needed on CJD infection-control questions to hospital-based stakeholders
Presentation delivered to Infection Control Practitioners conference (Sudbury, March 2008)
Internal consultations provided as needed for CJD-related issues such as F/P/T information sharing
  • maintaining an up-to-date, scientifically-detailed awareness of the global epidemiologic situation regarding prion disease and serving on expert advisory committees for prion research or other considerations.
In progress NML Annual Report 2007-2008 Module B: Attended and presented at scientific conferences (EUROCJD May 2007; CJD2007 November 2007; PrPCanada February 2008) Continued participation on APRI Surveillance and Control Advisory Committee; CSA prion decontamination standards committee
Goal#2  Contribute to a reduction in impact of human prion diseases through laboratory reference services for the surveillance and clinical investigation of human prion diseases.
Activities / Outputs Status Source
2.1 Maintain and improve service delivery for existing laboratory testing platforms for CJD by:
  • delivering laboratory reference services for neuropathology examination, molecular genetic analysis and biochemical testing on patients referred to CJDSS;
 Ongoing NML Annual Report 2007-2008 Module B: Full reference services offered for biochemical, molecular genetic and neuropathology analyses (see Section C.1.2 for statistics)
  • enhancing the specificity and sensitivity of CJD diagnosis by adding cerebrospinal fluid protein markers;
 In progress NML Annual Report 2007-2008 Module B: Technical validation of CSF tau, P-tau and S100B protein assays complete; test deployment is planned for  2008-09
  • enhancing genetic diagnostic services by developing a reliable method for sequencing the human prion-protein gene from formaldehyde-fixed tissue specimens;
 In progress NML Annual Report 2007-2008 Module B: Technical validation study of PRNP sequencing from formaldehyde-fixed brain tissues is ongoing
  • supporting diagnostic and epidemiologic studies by acquiring and validating methods for biochemical sub-typing of disease-associated prion protein in human brain tissue;
 In progress NML Annual Report 2007-2008 Module B: Methods for biochemical subtyping of disease-associated prion protein in human brain tissue have been acquired and validated using a proficiency panel of previously typed materials
  • achieving ISO accreditation for additional laboratory reference-services component of the Prion Diseases Program; and
 In progress NML Annual Report 2007-2008 Module B: ISO 17025 accreditation was achieved for human PRNP sequencing (SCC audit: Winter 2008)
  • providing education and technical advice to physicians and external laboratories on optimal use of clinical testing services for CJD.
Ongoing NML Annual Report 2007-2008 Module B: Advice is provided to physicians on request as needed, regarding optimal use of clinical testing services for CJD
2.2 Develop "next-generation" assay platforms for known and candidate biological markers of clinical-stage prion disease by:
  • developing high-capacity platforms for quantitative assays of protein markers of CJD and other dementias by mass spectrometry and immuno-detection; and
 In progress NML Annual Report 2007-2008 Module B: Methodology and instrumentation for fluorescence-based quantitative Western blotting have been acquired
Technical plan for mass spectrometry-based protein marker assays developed
  • acquiring new technologies for ultra sensitive assays of disease-associated prion protein in body fluids.
In progress NML Annual Report 2007-2008 Module B: Technical plan to acquire recently developed technologies for ultrasensitive assays of disease-associated prion protein in body fluids under development
Goal#3  Improved understanding of the biology of prion diseases and their infectious agents.
Activities / Outputs Status Source
3.1 Continue studies of genomics, proteomics, cell biology, strain typing and infectivity in prion disease models, including gene expression in animal models of TSE. In progress (June 2004- Dec 2008) NML Annual Report 2006-2007 Module D (S. Booth) Title: Primary transmission of BSE, CJD, and CWD into mice; strain-typing and model development
3.2 Undertake transmission studies of Chronic Wasting Disease (CWD) to nonhuman primates, to assess risk and determine characteristics of possible human forms of CWD. Discontinued NML Annual Report 2007-2008: Module B: Active participation in this long-term project discontinued due to loss of PHAC BSE Research Program funding
3.3 Continue transmission studies in mice of Canadian BSE, vCJD and CWD cases to establish and study rodent models of forms of prion disease important in Canadian public health and publish a related manuscript. In progress NML Annual Report 2007-2008: Module D (S. Booth) Title: Biomarker discovery for the diagnosis of prion diseases
3.4 Continue studies of novel preclinical biomarkers for prion disease by developing and applying cutting-edge proteomic and biochemical assay platforms and performing genomic analysis of cells that act as reservoirs of prion infectivity. In progress NML Annual Report 2007-2008: Module D (S. Booth) Title: Identification of MiRNA Driven Regulatory Circuits Involved in Prion-Induced Neurodegeneration
3.5 Continue studies of genetic epidemiology of human and animal prion diseases and publish related results and case reports. Done PDP Publication list

Annex H

Analysis of Achievement of Outcomes

1. Outcomes Related to Annual Reporting
1.1 2005 – 2006 and 2006 - 2007
Outcomes Findings
Creutzfeldt-Jakob disease surveillance system (CJDSS):
Assessment and mitigation of risks to Canadians from prion infection through blood, food, tissues, organs, medical procedures and dentistry, by performing active surveillance for all forms of human prion diseases (CJD) in Canada.
  • The PDP CJDSS maintains a broad range of contacts to identify infection (Hospitals, physicians, etc.) Footnote 11
  • Active surveillance ongoing
  • Surveillance finding expected number of cases in Canada annually
Technical research in prion diseases
Development of faster, simpler, more economical and more accurate methods and strategies for detection and diagnosis of prion diseases, to improve the effectiveness of CJD surveillance, prevention and control
  • PDP reference lab ISO accredited in 2006
  • Usage of CJD-related test services by province is approximately proportional to populationFootnote 12
Improved understanding of prion-transmission risks in Canada.
  • PDP research on prion transmission ongoingFootnote 13
  • Focus group comments indicated that PDP research includes improved understanding of transmission risks
Strengthening of Canadian capacity for collaborative prion disease research, to deal more effectively with emerging challenges to human and animal health on national and international levels
  • PDP research increasing number and variety of collaborations nationally and internationally
  • Focus group and interview comments indicated that research scientists highly regarded among their peers nationally and internationally.
Pathobiology research in prion diseases
Contribute to enhanced scientific understanding of basic biological mechanisms, processes and behaviour of prions and prion diseases, to support and improve Canadian capacity for their diagnosis, treatment, surveillance, risk assessment, prevention and control
  • PDP Reference List demonstrates increased scientific understanding
  • PDP reference list demonstrates increasing number of peer-reviewed publications, speaking engagements etc. though according to the Steering Committee focus group PDP is not yet comparable to other, similar research groups within the NML.
1.2 2007 – 2008
Outcomes Findings
Develop an enhanced public health network for human prion diseases in Canada, incorporating the CJDSS
  • Not well developed yet, plan for future. Plans may include:
    • CPHLN: not sure how to collaborate, maybe just keep informed, exchange of information, better communication, translation of results, bodies of information 
    • Better links with the provinces
  • Better communication with other organizations – pathologists, geneticists etc
Provide laboratory reference services for surveillance and clinical investigation of human prion diseases
  • Reference services ongoingFootnote 14
  • Physicians and healthcare professionals interviewed indicated general satisfaction with the reference services provided with the notable exception of the turnaround times for neuropathology tests
  • PDP program enabling communication between labs and epidemiologists established in 2007Footnote 15
Improved understanding of the biology of prion diseases and their infectious agents
  • Research scientists externally funded projects focused on improving the understanding of prion biology and their infectious agentsFootnote 16
  • Focus group comments indicate work continuing in both areas.
2. Outcomes Related to PDP Logic Model
Developed for PDP Evaluation in May 2008
Outcomes Findings
Short Term Outcomes
Understanding of incidence and diversity of human prion diseases in Canada
  • CJDSS tracks incidence and diversity of prion diseasesFootnote 17
  • Active surveillance ongoing
  • Surveillance finding expected number of cases in Canada annually
Timely intervention for CJD-related health events
  • Interviews report timely intervention
  • Health care professionals satisfied with timeliness except for neuropathology results
Expert technical support for investigation of suspected CJD
  • Interviews report that experts are available and provide valued technical support
  • Referring physicians lauded the work of the Nurse Consultants
  • Nurse consultant interviews indicated that access to and support from reference services and principal investigators was good.
Improved physician awareness of CJD
  • Good network of neuropathologists for identification of suspected cases
  • Increased CJD awareness among physicians referring cases to CJDSSFootnote 18
  • Physicians interviewed indicated that awareness had improved
Improved scientific understanding of prion diseases
  • PDP reference list demonstrates increasing number of peer-reviewed publications, speaking engagements etc. though according to the Steering Committee focus group PDP is not yet comparable to other, similar research groups within the NML.
  • Research scientists increasingly successful in winning external funding with a growing list of collaborators and funding agencies to continue their work
New opportunities for technical improvements
  • PDP reference list, Section F: Intellectual Property, patent pending relating to a technical improvement for a test.
  • Multiple interviewees report that tests used in other Prion-related laboratories have not been integrated into the reference services’ offering.
  • International proficiency testing for the periodic evaluation of 14-3-3 recommended though negotiations to implement have not been successfulFootnote 19
Long Term Outcomes
Effective, active surveillance and public health intervention for all human prion diseases in Canada
  • Active surveillance is ongoing
  • Canadian surveillance is finding the number of CJD cases expected – in the range of internationally predicted frequency of cases (1 – 2 per million per year)
  • The readily available 14-3-3 test serves to cast a wide net for referrals in Canada.
Improved awareness of CJD
  • Physician interview responses indicate that awareness has improved
  • Interviewees report that capacity and resources limit activities to promote awareness
Dissemination of pathobiology research
  • PDP capacity for dissemination of research is limited.
  • PDP reference list demonstrates increasing number of peer-reviewed publications, speaking engagements etc. though according to the Steering Committee focus group PDP is not yet comparable to other, similar research groups within the NML.
Technical improvements
  • Tests and improvements validated in other labs have not been incorporated

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