ARCHIVED - Evaluation of The Prion Diseases Program
1.0 Introduction and Context
1.1 Program Description
Orientation, Rationale and Policy Context
Prion diseases, also known as Transmissible Spongiform Encephalopathies (TSEs), comprise a number of lethal, transmissible neurodegenerative conditions affecting both humans (Creutzfeldt-Jakob disease, CJD) and animals (Bovine Spongiform Encephalopathy, BSE, in cattle; scrapie in sheep and goats; and Chronic Wasting Disease, CWD, in deer and elk). The Prion Diseases Program (PDP) of the National Microbiology Laboratory (NML), Public Health Agency of Canada (PHAC) delivers national surveillance, laboratory services and research for human and animal prion diseases.
The unconventional infective agent of prion diseases (called a prion) is neither a virus nor a bacterium, but rather a misshapen form of a normal host protein. Over the past 20 years this central characteristic – and the many unusual and difficult features that derive from it – have required public health systems to develop novel, specialized capacities for surveillance, science and technology to deal effectively with the significant challenges posed to both human and animal health by prion diseases.
One important example of such a difficult feature is the fact that these infective agents appear to arise de novo in humans and probably animals, via recurrent spontaneous misfolding of the prion protein. Another is the ability of prion agents to resist complete inactivation by disinfection treatments that would be sterilizing for most viruses and bacteria. Yet another is the capacity of prion agents, despite their lack of genetic material (DNA or RNA), to vary and adapt, allowing formation of “strains” within a host species and change in virulence properties after transmission to a new host species.
Although relatively obscure for many years, prion diseases began attracting broad public attention in the mid-1980s, following emergence of a large feedborne epizootic of BSE in the United Kingdom (UK) and Europe. Variant Creutzfeldt-Jakob disease (vCJD), a new human prion disease that emerged in the mid-1990s and has affected over 200 primarily younger people in 11 countries to date, was almost certainly caused by exposure to BSE-contaminated food. Four probable cases of secondary (human to human) transmission via blood transfusion have also been reported since 2004. One case of vCJD was confirmed in Canada in 2002, in an individual who had resided in the UK during the peak years of BSE incidence there.
Although the threat posed by vCJD and possibly other zoonotic human prion diseases has led to intensive public health response, so-called “classical” forms of CJD also occur universally at the rate of 1-2 cases per million population per year. Classical CJD carries many of the same risks of onward infectious transmission as do zoonotic human prion diseases; e.g. through surgery, transplantation and other medical procedures. Thus, despite their relative rarity human prion diseases command ongoing effort with regard to surveillance and risk management, even if zoonotic disease threats are not immediate.
To meet this challenge, the PDP (previously called the Host Genetics and Prion Diseases – HGPD) was established in 1998 by the Laboratory Centre for Disease Control (LCDC) as a new infectious-diseases program through policy decisions laid out in the Health Canada Blood Safety Program. The main purpose of this program, which followed the 1997 Final Report of the Krever Commission of Inquiry on the Blood System in Canada, was to meet regulatory and public-health responsibilities in relation to known and emerging infectious diseases transmissible through blood transfusion. April 1, 2008 marked the 10th anniversary of the creation of the PDP. Resources and Management Profile
Funding obtained through the Blood Safety Program initially covered the period April 1, 1998 – March 31, 2003. A project plan submitted by LCDC in March 1999 specified a PDP resource envelope finishing the 5-year project period at $1.860M (O&M) and 19 FTEs ongoing (A-base). Given the management structure of LCDC at that time, funding was distributed between two “sub-projects” – one situated with the Bureau of Infectious Diseases in Ottawa and the other with the Bureau of Microbiology in Winnipeg – with Director-level program leadership situated with the Ottawa arm of the program. This structure persisted until October 2005, at which time directorship was relocated to Winnipeg and a process of program updating and reintegration was initiated.
Additional A-base resources of $0.600M (O&M) and 4 FTEs, specifically for the Winnipeg-based laboratory arm of the PDP, were allotted in 2001. Following discovery of indigenous BSE in Canada in May 2003, between 2004 and 2009 funds were received to support BSE Research and Risk Assessment through an interdepartmental program designed to respond to Bovine Spongiform Encephalopathy (BSE) in the amount of $0.778M (O&M) annually. This money was initially for two years, was extended several times and will now be sunsetting in March 2009.
In 2007-08, actual expenditures of the PDP were slightly over three million dollars with staffing of approximately 24 full time equivalents (FTEs) broken down by salaries and O&M as follows:
- Salaries: $1.541 million
- O&M: $1.537 million
In addition to these amounts, there has been significant leveraging of research funds through external and internal (biotechnology R&D) grants for research.
It is worth noting that the management structure, funding inputs, and even specific activities and objectives of the PDP have changed over time. This has partly resulted from internal factors such as structural changes in the federal Health Portfolio such as:
- relocation of the Bureau of Microbiology from Ottawa to Winnipeg (1998); • branch- and directorate-level realignment of Health Canada (2000);
- creation of PHAC (2004); and
- relocation of program leadership from Ottawa to Winnipeg (2005).
The program’s configuration and priorities have also been influenced by the evolving landscape of risk for human and animal prion diseases, for example:
- consensus (1999) that classical CJD is a small or negligible risk to blood safety;
- implementation (1999) of a geographic blood donor-deferral scheme for vCJD;
- declining incidence of vCJD in the UK since 2000;
- emergence of BSE in Canada (2003); and
- confirmation of transfusion transmissibility of vCJD (2004).
1.2 Previous Health Canada Evaluation
A summative evaluation of the Health Canada Blood Safety Program was planned to be presented to the Minister of Health by 2003. However, no formal evaluation of the Blood Safety Program as a whole, or the surveillance/public health portion of it that eventually came under the auspices of the PHAC (50% of total resources), including the PDP, was completed.
HC and PHAC did complete a formative evaluation of the Bovine Spongiform Encephalopathy I and II initiative between December 2005 and June 2006 (with publication in April 2007). This evaluation drew the following key conclusions:
- there is a rationale and relevance to the BSE Initiatives and a continued need for these activities;
- the BSE activities have been supported by sound science;
- while there was general consensus regarding the appropriateness of the design of the BSE initiatives, both the time frame of the Initiatives and the lack of an inter-departmental and intra-branch coordination/communication strategy were identified as significant issues impacting the implementation of the initiatives;
- the implementation of the BSE initiatives was acceptable; and
- progress has been made towards the achievement of expected BSE II immediate and intermediate outcomes.
The four recommendations resulting from the evaluation were related to:
- Increasing key stakeholder interview involvement in the summative evaluation;
- Strengthening stakeholder involvement in BSE initiatives;
- Enhancing internal/external coordination and communication; and
- Implementing performance measurement and logic model adjustments.
This evaluation of the PDP represents the first review of the program as a whole and over the life of the initiative.
1.3 Structure of the Report
This report contains the following sections:
- Section 2 summarizes the evaluation methodology, including the evaluation framework, data collection methods and limitations of the evaluation;
- Section 3 presents the findings and conclusions related to the evaluation issue of relevance of the system;
- Section 4 presents the findings and conclusions related to the evaluation issue of success of the system;
- Section 5 outlines the findings and conclusions related to the design and delivery of the Prion Disease Program; and
- Section 6 summarizes the conclusions and the recommendations resulting from the evaluation.
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