Prion Disease Transmitted Through Bodily Fluids

A new form of Creutzfeldt-Jakob disease (CJD) in the mid-1990s, as a result of the Bovine Spongiform Encephalopathy (BSE) epidemic in the United Kingdom, has increased the concern of transmissible spongiform encephalopathies as a risk to human health and has already affected public health policy worldwide.

It is assumed that the new mutation of CJD (vCJD) results from the consumption of meat products from cattle infected with BSE and that there is a relation of the incidence of vCJD to the incidence of BSE in the countries where the disease has occurred. Since 1996, over 140 cases of vCJD have occurred in UK, seven in France and one each in Ireland, Italy, USA and Canada. CJD is closely related to Alzheimer’s disease. In fact, doctors cannot distinguish the two without guessing. Both are forms of prion disease, which also are known as transmissible spongiform encephalopathy (TSE).

Policies related to vCJD and the potential risk of human to human transmission are based on three main factors:

  • an unknown number of individuals who might be infected with the BSE agent;
  • presence of the pathological prion protein in many peripheral tissues from vCJD terminal patients and
  • evidence of experimental transmission in animals from blood of rodents and sheep infected with vCJD and BSE respectively.

There is increasing concern about the troubling possibility that blood or blood products, vaccines and other pharmaceutical products could spread the agent of variant CJD (vCJD) worldwide, especially in countries where BSE has not yet been reported. Bovine derived materials involved in the production of vaccines and other pharmaceutical products could represent a way of potential transmission of the disease.

Moreover, the possibility that human blood and plasma could be a vehicle for the transmission and spread of the disease have led to a number of donor deferral policies aimed at minimizing the risk of accepting a blood donor who might be incubating the human form of BSE.

prions disease and Alzheimer's disease and Creutzfeldt-Jakob disease

In addition, blood fractionated products such as albumin are used as stabilizers in the production of vaccines and recombinant pharmaceutical products. There is, therefore, a need to ensure that regulatory authorities with limited resources can have reliable information when making their risk assessment and evaluation of product safety to prevent the transmission of TSE to human via biological and pharmaceutical products.

A WHO Consultation was held in February 2003 to update the WHO Recommendations on Medicinal Products in relation to Human TSEs which were prepared in 1997, following a WHO Consultation on the same subject.

This Consultation complemented other important efforts of WHO in the follow up of the scientific and epidemiological developments in TSEs such as the Joint WHO/FAO/OIE Technical Consultation on BSE organized by the WHO Department of Communicable Disease Surveillance and Response (CSR) and the activities of the “Working Group on International Reference Materials for Diagnosis and Study of TSEs”, established in 1999 as a scientific forum to advance development of diagnostic tests based on available research methods and their application in health technology and pharmaceuticals.

The primary aim of this Consultation was to provide evidence-based information to medicines regulatory authorities of Member States, specially to those where BSE has not yet been reported, with regard to risk assessment, precautionary and control measures of medicinal products.

The Recommendations of the Consultation form the basis of the WHO Guidance Document to support regulatory decisions by National Regulatory Authorities in developing countries. Based on scientific information available, a tissue infectivity category was developed for the first time, that serves as a global basis for the development of risk assessment models for biological and pharmaceutical products derived from human or animal tissues or body fluids in relation to the transmission of TSE agents.

Prions are associated with TSEs. The operative word is “transmissible.” TSEs include Alzheimer’s disease, Creutzfeldt-Jakob disease, Parkinson’s disease, Huntington’s disease, scrapie, chronic wasting disease and mad cow disease. The disease has killed many species of mammals including dolphins. Victims permanently contaminate the world around them with their bodily fluids. Once contaminated with prions, items cannot be sterilized.

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Gary Chandler is a prion expert. He is the CEO of Crossbow Communications, author of several books and producer of documentaries about health and environmental issues around the world. Chandler is connecting the dots to the global surge in neurodegenerative disease, including Alzheimer’s disease, Parkinson’s disease, Creutzfeldt-Jakob disease, chronic wasting disease and other forms of prion disease.