Impact of Being Placed at Risk of Creutzfeldt-Jakob Disease: A Qualitative Study of Blood Donors to Variant CJD Cases and Patients Potentially Surgically Exposed to CJD
Gillian Elam a Katie Oakley b Nicky Connor b Patricia Hewitt c Hester J.T. Ward e
Syed M.A. Zaman b Yimmy Chow b Theresa M. Marteau d
a University College London, Mortimer Market Centre, b Health Protection Agency Centre for Infections,
c NHS Blood and Transplant, Colindale Centre, and d Health Psychology Section, Psychology Department,
King’s College London, Guy’s Campus London, London , and e National CJD Research and Surveillance Unit,
University of Edinburgh, Edinburgh , UK
The study objective was to describe the emotional and behavioural responses to Creutzfeldt-Jakob disease (CJD) risk notification. Methods: A qualitative study using 11 participants’ interviews, which were analysed thematically with Framework Analysis. Participants: Six participants purposively selected from people exposed to surgical instruments used previously on patients with or at risk of CJD (any type; n = 60), and 5 participants from a cohort of blood donors to patients who subsequently developed variant CJD (n = 110). Results: Notification was initially a shocking event, but with no lasting emotional impact. Those notified were convinced they were at extremely low risk of CJD and coped by not thinking about the information. Disclosure outside the immediate family was limited by fears of stigma. All expressed concern about the possibility of onward transmission and agreed notification was appropriate. Individual adherence to public health precautions varied from those who did nothing, apart from not donating blood, to those who consistently followed all advice given. This variation was in-formed by an assumption that information was always shared among health professionals. Conclusions: Factors contributing to minimising emotional distress following notification of CJD risk were evident. We found little evidence of sustained emotional distress. However, implementation of behaviours to minimise onward transmission, particularly in health care settings, was variable – this requires further investigation.
Introduction The CJD Incidents Panel advises on the risk of Creutzfeldt-Jakob disease (CJD) being spread between patients through health care  , and has advised that people exhibiting a 1% or greater risk (above that of the general UK population) of CJD should be informed and asked to follow public health advice to reduce the risk of transmission ( table 1 ).
The risk of CJD transmission through surgery is uncertain, depending on tissue infectivity and the efficacy of standard decontamination processes [2, 3] . Six cases of surgical transmission of sporadic CJD and none of vari-ant CJD (vCJD) have been reported  . There are 4 reports of vCJD infection following blood transfusion [5– 8] , and 1 following plasma product treatment  .
Risk notification aims for openness with individuals, and asks them to follow public health advice to reduce the risk of onward transmission. They are asked not to donate blood, tissues or organs, to inform their family, and to inform clinicians prior to undergoing invasive procedures  . We report here the first study to describe the impact of CJD risk notifications. A qualitative approach was used, informed by the principles of grounded theory. This places an emphasis on understanding phenomena from the perspective of those directly affected in terms of their own framework, language and experiences. The study was conducted to identify which further data should be collected in a larger quantitative populationbased study.
Statement of Principle Findings
Overall, participants felt it was correct to inform them about their CJD risk, as their wish to protect others outweighed the personal adverse effects of the news.
We found little evidence of adverse emotional consequences following notification of CJD risk status after intervals ranging from less than 6 months to over 5 years. Notification of these groups appears to have little impact on their everyday lives.
Most participants did not dwell on the possibility of CJD. Some anticipated negative reactions from health professionals and people outside their immediate family. This, and fear of mistreatment, may have deterred some from informing clinicians of their risk.
The notification appeared to have effectively prevented people at risk of CJD from volunteering to donate blood or organs, complementing the measure of asking CJD risk questions to all potential donors. The notification seemed less effective at prompting communication of CJD risk status to clinicians. This failure related to uncertainty about relevance of the risk status in different clinical situations, and perception that it would be communicated directly between clinicians.
snip...see full text ;
A VERY IMPORTANT FACTOR that must be weighed in on, HEALTH INSURANCE COMPANIES. IF there is not a provision, clause, that stipulates that being placed AT RISK OF CREUTZFELDT JAKOB DISEASE, would NOT blackball you i.e. MARK you, that NOT in any way can discriminate against you because of the disease, that NOT in any way would jeopardize or enhance cost for health insurance for anyone being placed 'AT RISK'.
Thursday, August 12, 2010
Seven main threats for the future linked to prions
The TSE road map defining the evolution of European policy for protection against prion diseases is based on a certain numbers of hypotheses some of which may turn out to be erroneous. In particular, a form of BSE (called atypical Bovine Spongiform Encephalopathy), recently identified by systematic testing in aged cattle without clinical signs, may be the origin of classical BSE and thus potentially constitute a reservoir, which may be impossible to eradicate if a sporadic origin is confirmed.
***Also, a link is suspected between atypical BSE and some apparently sporadic cases of Creutzfeldt-Jakob disease in humans. These atypical BSE cases constitute an unforeseen first threat that could sharply modify the European approach to prion diseases.
Rural and Regional Affairs and Transport References Committee
The possible impacts and consequences for public health, trade and agriculture of the Government’s decision to relax import restrictions on beef Final report June 2010
2.66 Dr Fahey also told the committee that in the last two years a link has been established between forms of atypical CJD and atypical BSE. Dr Fahey said that: They now believe that those atypical BSEs overseas are in fact causing sporadic Creutzfeldt-Jakob disease. They were not sure if it was due to mad sheep disease or a different form. If you look in the textbooks it looks like this is just arising by itself. But in my research I have a summary of a document which states that there has never been any proof that sporadic Creutzfeldt-Jakob disease has arisen de novo—has arisen of itself. There is no proof of that. The recent research is that in fact it is due to atypical forms of mad cow disease which have been found across Europe, have been found in America and have been found in Asia. These atypical forms of mad cow disease typically have even longer incubation periods than the classical mad cow disease.50
Wednesday, March 31, 2010
Atypical BSE in Cattle
To date the OIE/WAHO assumes that the human and animal health standards set out in the BSE chapter for classical BSE (C-Type) applies to all forms of BSE which include the H-type and L-type atypical forms. This assumption is scientifically not completely justified and accumulating evidence suggests that this may in fact not be the case. Molecular characterization and the spatial distribution pattern of histopathologic lesions and immunohistochemistry (IHC) signals are used to identify and characterize atypical BSE. Both the L-type and H-type atypical cases display significant differences in the conformation and spatial accumulation of the disease associated prion protein (PrPSc) in brains of afflicted cattle. Transmission studies in bovine transgenic and wild type mouse models support that the atypical BSE types might be unique strains because they have different incubation times and lesion profiles when compared to C-type BSE. When L-type BSE was inoculated into ovine transgenic mice and Syrian hamster the resulting molecular fingerprint had changed, either in the first or a subsequent passage, from L-type into C-type BSE.
In addition, non-human primates are specifically susceptible for atypical BSE as demonstrated by an approximately 50% shortened incubation time for L-type BSE as compared to C-type. Considering the current scientific information available, it cannot be assumed that these different BSE types pose the same human health risks as C-type BSE or that these risks are mitigated by the same protective measures.
This study will contribute to a correct definition of specified risk material (SRM) in atypical BSE. The incumbent of this position will develop new and transfer existing, ultra-sensitive methods for the detection of atypical BSE in tissue of experimentally infected cattle.
14th ICID International Scientific Exchange Brochure -
Final Abstract Number: ISE.114
Session: International Scientific Exchange
Transmissible Spongiform encephalopathy (TSE) animal and human TSE in North America update October 2009
Bacliff, TX, USA
An update on atypical BSE and other TSE in North America. Please remember, the typical U.K. c-BSE, the atypical l-BSE (BASE), and h-BSE have all been documented in North America, along with the typical scrapie's, and atypical Nor-98 Scrapie, and to date, 2 different strains of CWD, and also TME. All these TSE in different species have been rendered and fed to food producing animals for humans and animals in North America (TSE in cats and dogs ?), and that the trading of these TSEs via animals and products via the USA and Canada has been immense over the years, decades.
12 years independent research of available data
I propose that the current diagnostic criteria for human TSEs only enhances and helps the spreading of human TSE from the continued belief of the UKBSEnvCJD only theory in 2009. With all the science to date refuting it, to continue to validate this old myth, will only spread this TSE agent through a multitude of potential routes and sources i.e. consumption, medical i.e., surgical, blood, dental, endoscopy, optical, nutritional supplements, cosmetics etc.
I would like to submit a review of past CJD surveillance in the USA, and the urgent need to make all human TSE in the USA a reportable disease, in every state, of every age group, and to make this mandatory immediately without further delay. The ramifications of not doing so will only allow this agent to spread further in the medical, dental, surgical arena's. Restricting the reporting of CJD and or any human TSE is NOT scientific. Iatrogenic CJD knows NO age group, TSE knows no boundaries. I propose as with Aguzzi, Asante, Collinge, Caughey, Deslys, Dormont, Gibbs, Gajdusek, Ironside, Manuelidis, Marsh, et al and many more, that the world of TSE Transmissible Spongiform Encephalopathy is far from an exact science, but there is enough proven science to date that this myth should be put to rest once and for all, and that we move forward with a new classification for human and animal TSE that would properly identify the infected species, the source species, and then the route.
Saturday, June 25, 2011
Transmissibility of BSE-L and Cattle-Adapted TME Prion Strain to Cynomolgus Macaque
"BSE-L in North America may have existed for decades"
Sunday, June 26, 2011
Risk Analysis of Low-Dose Prion Exposures in Cynomolgus Macaque
Thursday, June 23, 2011
Experimental H-type bovine spongiform encephalopathy characterized by plaques and glial- and stellate-type prion protein deposits
Wednesday, June 15, 2011
Galveston, Texas - Isle port moves through thousands of heifers headed to Russia, none from Texas, Alabama, or Washington, due to BSE risk factor
Thursday, July 14, 2011
Valley Farm Meats (DBA Strasburg Provision, Inc) Issues Precautionary Recall for Beef Products Due to Possible Contamination with Prohibited Materials SRM
Ohio Department of Agriculture and Ohio Department of Health
Monday, June 20, 2011 2011
Annual Conference of the National Institute for Animal Agriculture ATYPICAL NOR-98 LIKE SCRAPIE UPDATE USA
Thursday, June 2, 2011
USDA scrapie report for April 2011 NEW ATYPICAL NOR-98 SCRAPIE CASES Pennsylvania AND California
Monday, June 27, 2011
Comparison of Sheep Nor98 with Human Variably Protease-Sensitive Prionopathy and Gerstmann-Sträussler-Scheinker Disease
Wednesday, July 06, 2011
Swine Are Susceptible to Chronic Wasting Disease by Intracerebral Inoculation
JULY 2011 UPDATE
Monday, June 27, 2011
Zoonotic Potential of CWD: Experimental Transmissions to Non-Human Primates
UPDATED DATA ON 2ND CWD STRAIN
Wednesday, September 08, 2010
CWD PRION CONGRESS SEPTEMBER 8-11 2010
Wednesday, January 5, 2011
ENLARGING SPECTRUM OF PRION-LIKE DISEASES Prusiner Colby et al 2011
David W. Colby1,* and Stanley B. Prusiner1,2
----- Original Message -----
From: David Colby
Sent: Tuesday, March 01, 2011 8:25 AM
Subject: Re: FW: re-Prions David W. Colby1,* and Stanley B. Prusiner1,2 + Author Affiliations
Dear Terry Singeltary,
Thank you for your correspondence regarding the review article Stanley Prusiner and I recently wrote for Cold Spring Harbor Perspectives. Dr. Prusiner asked that I reply to your message due to his busy schedule. We agree that the transmission of CWD prions to beef livestock would be a troubling development and assessing that risk is important. In our article, we cite a peer-reviewed publication reporting confirmed cases of laboratory transmission based on stringent criteria. The less stringent criteria for transmission described in the abstract you refer to lead to the discrepancy between your numbers and ours and thus the interpretation of the transmission rate. We stand by our assessment of the literature--namely that the transmission rate of CWD to bovines appears relatively low, but we recognize that even a low transmission rate could have important implications for public health and we thank you for bringing attention to this matter.
Warm Regards, David Colby
David Colby, PhDAssistant ProfessorDepartment of Chemical EngineeringUniversity of Delaware
re-ENLARGING SPECTRUM OF PRION-LIKE DISEASES Prusiner Colby et al 2011 Prions
CWD to cattle figures CORRECTION
I believe the statement and quote below is incorrect ;
"CWD has been transmitted to cattle after intracerebral inoculation, although the infection rate was low (4 of 13 animals [Hamir et al. 2001]). This finding raised concerns that CWD prions might be transmitted to cattle grazing in contaminated pastures."
Please see ;
Within 26 months post inoculation, 12 inoculated animals had lost weight, revealed abnormal clinical signs, and were euthanatized. Laboratory tests revealed the presence of a unique pattern of the disease agent in tissues of these animals. These findings demonstrate that when CWD is directly inoculated into the brain of cattle, 86% of inoculated cattle develop clinical signs of the disease.
"although the infection rate was low (4 of 13 animals [Hamir et al. 2001])."
shouldn't this be corrected, 86% is NOT a low rate. ...
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518
Thanks so much for your updates/comments. We intend to publish as rapidly as possible all updates/comments that contribute substantially to the topic under discussion.
re-Prions David W. Colby1,* and Stanley B. Prusiner1,2 + Author Affiliations
1Institute for Neurodegenerative Diseases, University of California, San Francisco, San Francisco, California 94143 2Department of Neurology, University of California, San Francisco, San Francisco, California 94143 Correspondence: email@example.com
snip...full text ;
Wednesday, January 5, 2011
ENLARGING SPECTRUM OF PRION-LIKE DISEASES Prusiner Colby et al 2011 Prions
David W. Colby1,* and Stanley B. Prusiner1,2
what is Alzheimer's anyway ?
strictly NOT private and confidential $$$
Saturday, January 22, 2011
Alzheimer's, Prion, and Neurological disease, and the misdiagnosis there of, a review 2011
Friday, September 3, 2010
Alzheimer's, Autism, Amyotrophic Lateral Sclerosis, Parkinson's, Prionoids, Prionpathy, Prionopathy, TSE
Thursday, December 23, 2010
Alimentary prion infections: Touch-down in the intestine, Alzheimer, Parkinson disease and TSE mad cow diseases $ The Center for Consumer Freedom
Monday, May 23, 2011
Atypical Prion Diseases in Humans and Animals 2011
Top Curr Chem (2011)
# Springer-Verlag Berlin Heidelberg 2011
Michael A. Tranulis, Sylvie L. Benestad, Thierry Baron, and Hans Kretzschmar
Saturday, March 5, 2011
MAD COW ATYPICAL CJD PRION TSE CASES WITH CLASSIFICATIONS PENDING ON THE RISE IN NORTH AMERICA
Tuesday, June 14, 2011
Clinical research in CJD at a U.S. clinical prion research center: CJD Quinacrine Study results and improved diagnosis of prion disease
Tuesday, April 26, 2011
sporadic CJD RISING Text and figures of the latest annual report of the NCJDRSU covering the period 1990-2009 (published 11th March 2011)
Tuesday, March 29, 2011
TRANSMISSIBLE SPONGIFORM ENCEPHALOPATHY EXPOSURE SPREADING VIA HOSPITALS AND SURGICAL PROCEDURES AROUND THE GLOBE
Wednesday, June 29, 2011
TSEAC Meeting August 1, 2011 donor deferral ...
Saturday, June 18, 2011
Self-propagation and transmission of misfolded mutant SOD1 Prion or Prion-like phenomenon?
SEE TONNAGE OF MAD COW FEED IN COMMERCE IN 2007 ALONE, A DECADE POST PARTIAL AND VOLUNTARY MAD COW FEED BAN ;
Saturday, November 6, 2010
TAFS1 Position Paper on Position Paper on Relaxation of the Feed Ban in the EU Berne, 2010 TAFS
INTERNATIONAL FORUM FOR TRANSMISSIBLE ANIMAL DISEASES AND FOOD SAFETY a non-profit Swiss Foundation
Archive Number 20101206.4364 Published Date 06-DEC-2010 Subject PRO/AH/EDR> Prion disease update 2010 (11)
PRION DISEASE UPDATE 2010 (11)
Terry S. Singeltary Sr. P.O. Box 42 Bacliff, Texas USA 77518 firstname.lastname@example.org