NEUROSURGERY AND CREUTZFELDT-JAKOB DISEASE Health Law, Ethics, and Human Rights The Disclosure Dilemma

NEUROSURGERY AND CREUTZFELDT-JAKOB DISEASE Health Law, Ethics, and Human Rights The Disclosure Dilemma



NEUROSURGERY AND CREUTZFELDT-JAKOB DISEASE

The most vexing large-scale adverse events involve potential injuries that cannot be definitively diagnosed and have no treatment. A patient who underwent neurosurgery in Denver was found several weeks later at autopsy to have died of classic sporadic Creutzfeldt-Jakob disease. This diagnosis was not suspected at the time of surgery. Instruments used in this patient’s surgery were later used in surgery involving six other patients.17

Creutzfeldt-Jakob disease is a transmissible spongiform encephalopathy, a rare prion disease that causes rapid, fatal progression of neurologic symptoms. Iatrogenic transmission of Creutzfeldt-Jakob disease has been associated with neurosurgical procedures and corneal transplantation.18,56 The incubation period for surgical exposure to Creutzfeldt-Jakob disease ranges from 6 months to 20 years or more.17,57 Once symptoms appear, patients die within about 1 year.58 Creutzfeldt-Jakob disease is diagnosed by postmortem examination of the brain. Estimates of risk range from 1 in 100,000 to 1 in 1 million.58 Iatrogenic transmission is estimated to account for less than 1% of cases of transmissible spongiform encephalopathy.12 Prevention of surgical transmission of Creutzfeldt-Jakob disease is possible but cumbersome and costly. Although normal disinfection methods are ineffective against prions, available guidelines for infection control involve the sequestering, incinerating, or high-intensity sterilizing of neurosurgical instruments if transmissible spongiform encephalopathy is suspected.18,59

This event is among the most rare and challenging type of large-scale adverse event, since it is difficult to know whether any harm has occurred until decades after the exposure. Thus, a duty to tell the truth might be outweighed by a duty of nonmaleficence. The fear and worry that could accompany disclosure may constitute a greater and more permanent harm in the case of Creutzfeldt-Jakob disease than in other large-scale adverse events without any corresponding benefit, given the remote chance of transmission of this disease and the lack of diagnostic and treatment options.

The rationale for disclosure is also compelling. There is a professional duty to disclose because contracting the disease, even decades later, is a severe, deadly harm. Arguably, a patient has a right to this information. Should Creutzfeldt-Jakob disease develop in the patient, the harm is intensified by the sense of betrayal if the patient learns that providers have kept the risk of the development of a terminal, incurable disease a secret. If a potentially infected patient has subsequent neurosurgery, additional patients may be at risk for exposure, thereby amplifying harm. Disclosure would also allow patients to access testing and treatment that become available in the future,16 and it would demonstrate to the community that the hospital puts the interests of its patients first, even if the benefits to the patient are small and there is a risk of litigation.

Thus, when the institution has no testing or treatment to offer patients and the disclosure is associated with a risk of creating clinically significant long-term harm, a true ethical dilemma exists. Although, on balance, we believe that disclosure is often warranted, there is also greater ethical justification for nondisclosure in this case than in the other types of large-scale adverse events. The challenge of disclosing large-scale adverse events related to prion diseases is an important topic for further analysis.


snip...


INSUFFICIENT DISINFECTION OF ENDOSCOPES


Some large-scale adverse events are caused by deviations from standards of practice,31 but often they are not. Perhaps the most common large-scale adverse events involve insufficient disinfection of equipment. At the University of Washington Medical Center, one step in a six-step endoscope disinfection process failed. The faulty machine was running several minutes too fast. Two months later, when the malfunction was detected, the hospital corrected the flaw and identified approximately 600 patients who were exposed to incompletely cleaned endoscopes.11 Scientists could not calculate the increased risk posed by omitting one cleaning step, but they thought it was remote and indistinguishable from the baseline risk of contracting bloodborne pathogens from an endoscope (estimated to be 1 in 1.8 million).54,55 Despite the extremely low risk of infection, the hospital considered the rationale for disclosure to be compelling and sent letters to all affected patients. The event was reported in a front-page story in the Seattle Times.11

The hospital devoted considerable time and resources to developing and implementing a process for following up on the disclosure. It enlisted its organizational ethics consultants and patient relations department, set up a hotline to answer concerned patients’ questions, referred interested patients to physicians, and provided free follow-up testing. No cases of infection with bloodborne pathogens were identified and no lawsuits were filed.

snip...

see full text ;


Health Law, Ethics, and Human Rights The Disclosure Dilemma — Large-Scale Adverse Events

NEJM September 1, 2010 Topics: Medical Ethics Denise M. Dudzinski, Ph.D., Philip C. Hébert, M.D., Ph.D., Mary Beth Foglia, R.N., Ph.D., and Thomas H. Gallagher, M.D.


http://healthpolicyandreform.nejm.org/?p=12337&query=TOC


http://www.nejm.org/doi/full/10.1056/NEJMhle1003134?ssource=hcrc


IN my opinion, there should be NO Disclosure Dilemma when it comes to a transmissible disease as with CJD. The ethical reasons of health discloser law should NOT have anything to do with an adverse event that may harm many more from further transmission from the 1st adverse event, from a disease that if risk factor is not validated, the risk from further infectivity of exposure from the friendly fire and or pass it forward modes of transmission and possible death is very real. IT should be _law_ to inform those others that are _adversely_ exposed, and possible death there from. Hiding behind confidentiality laws on any adverse events (negligence), just to hide this adverse event from the public and media should be illegal. IF not, and they go on to have other medical procedures in the years and or decades to come, how many more will become exposed, and possibly die there from ??? THEN who's responsibility will it be for those further infected ??? IF not reported, no one, and that's the whole purpose. kinda like the SSS of the USDA et al, i.e. shoot, shovel, and shut the hell up. ...just my opinion...TSS


Friday, August 13, 2010

Creutzfeldt-Jakob disease (CJD) biannual update 13 August 2010 UK Iatrogenic CJD Incidents Report

http://creutzfeldt-jakob-disease.blogspot.com/2010/08/creutzfeldt-jakob-disease-cjd-biannual.html


Thursday, August 12, 2010

USA Blood products, collected from a donor who was at risk for vCJD, were distributed July-August 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/08/usa-blood-products-collected-from-donor.html


Thursday, July 08, 2010

Nosocomial transmission of sporadic Creutzfeldt-Jakob disease: results from a risk-based assessment of surgical interventions Public release date: 8-Jul-2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/07/nosocomial-transmission-of-sporadic.html


Wednesday, June 02, 2010

CJD Annex H UPDATE AFTER DEATH PRECAUTIONS Published: 2 June 2003 Updated: May 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/06/cjd-annex-h-update-after-death.html


Tuesday, May 11, 2010

Current risk of iatrogenic Creutzfeld-Jakob disease in the UK: efficacy of available cleaning chemistries and reusability of neurosurgical instruments

http://creutzfeldt-jakob-disease.blogspot.com/2010/05/current-risk-of-iatrogenic.html


Tuesday, March 16, 2010

Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Part 4 REVISED FEB. 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/03/transmissible-spongiform-encephalopathy.html


Saturday, January 16, 2010

Evidence For CJD TSE Transmission Via Endoscopes 1-24-3 re-Singeltary to Bramble et al Evidence For CJD/TSE Transmission Via Endoscopes

From Terry S. Singletary, Sr flounder@wt.net

1-24-3

http://creutzfeldt-jakob-disease.blogspot.com/2010/01/evidence-for-cjd-tse-transmission-via.html


Friday, November 20, 2009

SaBTO Advisory Committee on the Safety of Blood, Tissues and Organs Summary of the Eighth Meeting, 27 October 2009

http://vcjdtransfusion.blogspot.com/2009/11/sabto-advisory-committee-on-safety-of.html


Sunday, May 10, 2009

Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)

http://tseac.blogspot.com/2009/05/meeting-of-transmissible-spongiform.html


Friday, July 24, 2009

UW Hospital and Clinics Addresses Creutzfeldt-Jakob Disease Risk

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/uw-hospital-and-clinics-addresses.html


http://creutzfeldt-jakob-disease.blogspot.com/2009/07/uw-hospital-warning-53-patients-about.html


Friday, July 17, 2009

Revision to pre-surgical assessment of risk for vCJD in neurosurgery and eye surgery units Volume 3 No 28; 17 July 2009

http://creutzfeldt-jakob-disease.blogspot.com/2009/07/revision-to-pre-surgical-assessment-of.html


Monday, August 17, 2009

Transmissible Spongiform Encephalopathy Agents: Safe Working and the Prevention of Infection: Annex J,K, AND D Published: 2009

http://creutzfeldt-jakob-disease.blogspot.com/2009/08/transmissible-spongiform-encephalopathy.html


Wednesday, August 20, 2008

Tonometer disinfection practice in the United Kingdom: A national survey

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/tonometer-disinfection-practice-in.html


Tuesday, August 12, 2008

Biosafety in Microbiological and Biomedical Laboratories Fifth Edition 2007 (occupational exposure to prion diseases)

http://creutzfeldt-jakob-disease.blogspot.com/2008/08/biosafety-in-microbiological-and.html


Saturday, August 14, 2010

BSE Case Associated with Prion Protein Gene Mutation (g-h-BSEalabama) and VPSPr PRIONPATHY

(see mad cow feed in COMMERCE IN ALABAMA...TSS)

http://prionpathy.blogspot.com/2010/08/bse-case-associated-with-prion-protein.html


Wednesday, July 28, 2010

re-Freedom of Information Act Project Number 3625-32000-086-05, Study of Atypical BSE UPDATE July 28, 2010

http://bse-atypical.blogspot.com/2010/07/re-freedom-of-information-act-project.html


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.


http://www.prionetcanada.ca/detail.aspx?menu=5&dt=293380&app=93&cat1=387&tp=20&lk=no&cat2


Wednesday, March 31, 2010

Atypical BSE in Cattle

http://bse-atypical.blogspot.com/2010/03/atypical-bse-in-cattle-position-post.html


Friday, August 27, 2010

NEW ATYPICAL NOR-98 SCRAPIE CASE DETECTED IDAHO NOW 5 CASES DOCUMENTED 2010

http://nor-98.blogspot.com/2010/08/new-atypical-nor-98-scrapie-case.html


Tuesday, August 03, 2010

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein

http://creutzfeldt-jakob-disease.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html


Monday, August 9, 2010

Variably protease-sensitive prionopathy: A new sporadic disease of the prion protein or just more PRIONBALONEY ?

http://prionunitusaupdate2008.blogspot.com/2010/08/variably-protease-sensitive-prionopathy.html


Wednesday, August 18, 2010

Incidence of CJD Deaths Reported by CJD-SS in Canada as of July 31, 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/08/incidence-of-cjd-deaths-reported-by-cjd.html


Monday, August 9, 2010

National Prion Disease Pathology Surveillance Center Cases Examined (July 31, 2010)

(please watch and listen to the video and the scientist speaking about atypical BSE and sporadic CJD and listen to Professor Aguzzi)

http://prionunitusaupdate2008.blogspot.com/2010/08/national-prion-disease-pathology.html


CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER


>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<


http://www.recordandoalinda.com/index.php?option=com_content&view=article&id=19:cjd-english-info&catid=9:cjd-ingles&Itemid=8



>>> Up until about 6 years ago, the pt worked at Tyson foods where she worked on the assembly line, slaughtering cattle and preparing them for packaging. She was exposed to brain and spinal cord matter when she would euthanize the cattle. <<<


http://creutzfeldt-jakob-disease.blogspot.com/2010/03/irma-linda-andablo-cjd-victim-she-died.html



CJD TEXAS 38 YEAR OLD FEMALE WORKED SLAUGHTERING CATTLE EXPOSED TO BRAIN AND SPINAL CORD MATTER

http://cjdtexas.blogspot.com/2010/03/cjd-texas-38-year-old-female-worked.html


Thursday, July 08, 2010

GLOBAL CLUSTERS OF CREUTZFELDT JAKOB DISEASE - A REVIEW 2010

http://creutzfeldt-jakob-disease.blogspot.com/2010/07/global-clusters-of-creutzfeldt-jakob.html


Thursday, August 19, 2010

REPORT ON THE INVESTIGATION OF THE SEVENTEENTH CASE OF BOVINE SPONGIFORM ENCEPHALOPATHY (BSE) IN CANADA

http://bseusa.blogspot.com/2010/08/report-on-investigation-of-seventeenth.html


Thursday, August 19, 2010

SCRAPIE CANADA UPDATE Current as of 2010-07-31 The following table lists sheep flocks and/or goat herds confirmed to be infected with scrapie in Canada in 2010.

Current as of: 2010-07-31

http://nor-98.blogspot.com/2010/08/scrapie-canada-update-current-as-of.html


Friday, May 14, 2010

Prion Strain Mutation Determined by Prion Protein Conformational Compatibility and Primary Structure

Published Online May 13, 2010 Science DOI: 10.1126/science.1187107 Science Express Index

http://chronic-wasting-disease.blogspot.com/2010/05/prion-strain-mutation-determined-by.html


Thursday, June 03, 2010

Prion Strain Mutation and Selection John Collinge MEDICINE

http://chronic-wasting-disease.blogspot.com/2010/06/prion-strain-mutation-and-selection.html


P35

ADAPTATION OF CHRONIC WASTING DISEASE (CWD) INTO HAMSTERS, EVIDENCE OF A WISCONSIN STRAIN OF CWD

Chad Johnson1, Judd Aiken2,3,4 and Debbie McKenzie4,5 1 Department of Comparative Biosciences, University of Wisconsin, Madison WI, USA 53706 2 Department of Agriculture, Food and Nutritional Sciences, 3 Alberta Veterinary Research Institute, 4.Center for Prions and Protein Folding Diseases, 5 Department of Biological Sciences, University of Alberta, Edmonton AB, Canada T6G 2P5

The identification and characterization of prion strains is increasingly important for the diagnosis and biological definition of these infectious pathogens. Although well-established in scrapie and, more recently, in BSE, comparatively little is known about the possibility of prion strains in chronic wasting disease (CWD), a disease affecting free ranging and captive cervids, primarily in North America. We have identified prion protein variants in the white-tailed deer population and demonstrated that Prnp genotype affects the susceptibility/disease progression of white-tailed deer to CWD agent. The existence of cervid prion protein variants raises the likelihood of distinct CWD strains. Small rodent models are a useful means of identifying prion strains. We intracerebrally inoculated hamsters with brain homogenates and phosphotungstate concentrated preparations from CWD positive hunter-harvested (Wisconsin CWD endemic area) and experimentally infected deer of known Prnp genotypes. These transmission studies resulted in clinical presentation in primary passage of concentrated CWD prions. Subclinical infection was established with the other primary passages based on the detection of PrPCWD in the brains of hamsters and the successful disease transmission upon second passage. Second and third passage data, when compared to transmission studies using different CWD inocula (Raymond et al., 2007) indicate that the CWD agent present in the Wisconsin white-tailed deer population is different than the strain(s) present in elk, mule-deer and white-tailed deer from the western United States endemic region.

http://www.istitutoveneto.it/prion_09/Abstracts_09.pdf


CWD FIRST AND SECOND PASSAGE TRANSMISSION TO CATTLE

Susceptibility of Cattle to First-passage Intracerebral Inoculation with Chronic Wasting Disease Agent from White-tailed Deer

A. N. Hamir1, J. M. Miller1, R. A. Kunkle1, S. M. Hall2 and J. A. Richt1 + Author Affiliations

1National Animal Disease Center, ARS, USDA, Ames, IA 2Pathobiology Laboratory, National Veterinary Services Laboratories, Ames, IA Dr. A. N. Hamir, National Animal Disease Center, ARS, USDA, 2300 Dayton Avenue, PO Box 70, Ames, IA 50010 (USA). E-mail: ahamir@nadc.ars.usda.gov. Abstract Fourteen, 3-month-old calves were intracerebrally inoculated with the agent of chronic wasting disease (CWD) from white-tailed deer (CWDwtd) to compare the clinical signs and neuropathologic findings with those of certain other transmissible spongiform encephalopathies (TSE, prion diseases) that have been shown to be experimentally transmissible to cattle (sheep scrapie, CWD of mule deer [CWDmd], bovine spongiform encephalopathy [BSE], and transmissible mink encephalopathy). Two uninoculated calves served as controls. Within 26 months postinoculation (MPI), 12 inoculated calves had lost considerable weight and eventually became recumbent. Of the 12 inoculated calves, 11 (92%) developed clinical signs. Although spongiform encephalopathy (SE) was not observed, abnormal prion protein (PrPd) was detected by immunohistochemistry (IHC) and Western blot (WB) in central nervous system tissues. The absence of SE with presence of PrPd has also been observed when other TSE agents (scrapie and CWDmd) were similarly inoculated into cattle. The IHC and WB findings suggest that the diagnostic techniques currently used to confirm BSE would detect CWDwtd in cattle, should it occur naturally. Also, the absence of SE and a distinctive IHC pattern of CWDwtd and CWDmd in cattle suggests that it should be possible to distinguish these conditions from other TSEs that have been experimentally transmitted to cattle.

http://vet.sagepub.com/content/44/4/487


second passage is even worse ;

Experimental Second Passage of Chronic Wasting Disease (CWDmule deer) Agent to Cattle

A. N. Hamir, R. A. Kunkle, J. M. Miller, J. J. Greenlee and J. A. Richt Agricultural Research Service, United States Department of Agriculture, National Animal Disease Center, 2300 Dayton Avenue, P.O. Box 70, Ames, IA 50010, USA

Summary

To compare clinicopathological findings in first and second passage chronic wasting disease (CWDmule deer) in cattle, six calves were inoculated intracerebrally with brain tissue derived froma first-passageCWD-affected calf in an earlier experiment. Two uninoculated calves served as controls. The inoculated animals began to lose both appetite and weight 10–12 months later, and five subsequently developed clinical signs of central nervous system (CNS) abnormality. By 16.5 months, all cattle had been subjected to euthanasia because of poor prognosis. None of the animals showed microscopical lesions of spongiform encephalopathy (SE) but PrPres was detected in their CNS tissues by immunohistochemistry (IHC) and rapid Western blot (WB) techniques. Thus, intracerebrally inoculated cattle not only amplified CWD PrPres from mule deer but also developed clinicalCNSsigns in the absence of SElesions.This situation has also been shown to occur in cattle inoculated with the scrapie agent. The study confirmed that the diagnostic techniques currently used for diagnosis of bovine spongiformencephalopathy (BSE) in theUS would detect CWDin cattle, should it occur naturally. Furthermore, it raised the possibility of distinguishing CWDfromBSE in cattle, due to the absence of neuropathological lesions and to a distinctive multifocal distribution of PrPres, as demonstrated by IHC which, in this study, appeared to be more sensitive than the WB technique. Published by Elsevier Ltd. Keywords: cattle; chronic wasting disease (CWD); deer; transmissible spongiform encephalopathy (TSE)

snip...

Discussion CWD, like all other TSEs, is characterized by a long incubation period, which in deer is seldom less than 18 months (Williams and Young, 1992). In an experimental study of cattle inoculated intracerebrally with CWD from mule deer (first passage), amplification of PrPres was demonstrated in only five of 13 (38%) cattle, after incubation periods that ranged from 23 to 63 months (Hamir et al., 2001a, 2005a). In contrast, all inoculated cattle in the present study were positive for PrPres within 16.5 months. This increased attack rate with shorter incubation periods probably indicates adaptation of the CWDmule deer agent to a new host.

snip...

The uniform susceptibility, relatively short incubation, and absence of microscopical lesions in cattle given CWD brain material passaged once through cattle resembled findings in cattle inoculated intracerebrally with the scrapie agent (Cutlip et al., 1997). In that experiment, 100% of cattle died 14–18 months after inoculation with material from the first cattle-passage of a US strain of the scrapie agent; none showed microscopical lesions and all were positive for PrPres.

http://ddr.nal.usda.gov/bitstream/10113/34009/1/IND43787291.pdf



Thursday, August 12, 2010

Seven main threats for the future linked to prions

http://prionpathy.blogspot.com/2010/08/seven-main-threats-for-future-linked-to.html


http://prionpathy.blogspot.com/




TSS

Revision to pre-surgical assessment of risk for vCJD in neurosurgery and eye surgery units Volume 3 No 28; 17 July 2009

Volume 3 No 28; 17 July 2009

HPR Home Archives 2009 news

Revision to pre-surgical assessment of risk for vCJD in neurosurgery and eye surgery units

Pandemic flu: UK situation at 16 July 2009

Revision to pre-surgical assessment of risk for vCJD in neurosurgery and eye surgery units

Patients are routinely assessed prior to undergoing surgical or neuro-endoscopy procedures to determine whether they may have an increased risk of CJD infection. If this is the case, special infection control precautions should be followed [1].

This pre-surgical assessment process has been revised to fully take account of the latest knowledge of variant CJD (vCJD). Now patients undergoing high risk surgery [2] or neuro-endoscopy should be assessed to identify those who have received transfusions from 80 or more donors since 1980. Any patients so identified have an increased risk of vCJD, and special infection control procedures should be followed. It is estimated that around 50 patients will be identified in this way in the UK each year.

Hospitals are being asked to collate blood transfusion histories for these patients, and conduct risk assessments for patients with uncertain or incomplete transfusion histories. Patients who are found to have an increased risk of vCJD will need to be informed via the HPA Centre for Infections CJD section, which is coordinating the implementation of the revised guidance, and their GP.

The vCJD risk to these patients is uncertain, and depends on the prevalence of subclinical vCJD infection among blood donors, the infectivity in the blood of infected donors, and the number of donors the patients have received blood from.

The HPA's CfI-CJD section should be informed of any patients who are identified prior to high risk surgery or neuro-endoscopy as having received blood from 80 or more donors. Health Protection Units have been asked to ensure that infection control teams are aware of the revised guidance.

Information for healthcare workers and patients is available at:


http://www.hpa.org.uk/vCJDpresurgicalassessment



References/notes

1. Department of Health. "Assessment to be carried out before surgery and/or endoscopy to identify patients with, or at increased risk of, CJD or vCJD" (Annex J of ACDP TSE Working Group guidance).

2. High risk surgery is defined as surgery involving any of the following organs or tissues (high risk tissues): brain, spinal cord, dura mater, cranial nerves (specifically the entire optic nerve and only the intracranial components of the other cranial nerves), cranial nerve ganglia, posterior eye (specifically the posterior hyaloid face, retina, retinal pigment epithelium, choroid, subretinal fluid, optic nerve) and pituitary gland.

Pandemic flu: UK situation at 16 July 2009

The latest HPA Weekly Pandemic Flu Update [1] notes the following developments as at 16 July:

GP consultation rates in England for individuals presenting with flu-like illness showed increased rates, well above the threshold level for normal seasonal flu activity; the under-fives and 5-14 year olds are the age groups predominantly affected; the majority of cases continue to be mild, but there had been 26 deaths in England as at 16 July; and HPA estimated that there were 55,000 new cases of swine flu during the previous week (within a possible range of 30,000 to 85,000). Following the move away from laboratory testing for confirmation of swine flu cases to clinical diagnosis [2], the level of influenza in the community is being monitored using a range of surveillance mechanisms. One of these is the collaborative project between the HPA and the University of Nottingham Division of Primary Care known as QSurveillance®. This is a not-for-profit network over 3,300 general practices covering a total population of almost 22 million patients (more than 25% of the UK population). In the week-ending 12 July, the flu-like illness weekly consultation rate recorded by QSurveillance® reached 86.8 per 100,000, higher than the peak activity in winter 08/09 (see figure 2 in the HPA Weekly Pandemic Flu Update for 16 July [1]).

Given that laboratory testing is no longer routinely recommended, figure 1 (below) graphically illustrates how primary care surveillance such as QSurveillance® is providing a good comparator to laboratory confirmation, showing daily reporting of laboratory confirmed cases alongside daily primary care surveillance reports for flu-like illness.

Figure 1: Comparison of daily laboratory confirmed H1N1v case reports and daily QSurveillance® reports of influenza-like illness, UK*

* Data source: QSurveillance (daily data). Database version 1. Copyright QRESEARCH 2009.

Department of Health guidance: the National Pandemic Flu Service

In view of the very high demand for primary care, NHS Direct and A and E services in many areas, the Department of Health announced plans to activate, subject to rigorous testing, the National Pandemic Flu Service before the end of July [3].

References

1. "Weekly pandemic flu update (16 July 2009)", (HPA press release of 16 July 2009). HPA website: National Press Releases.

2. “Treatment approach announced for pandemic flu”, Health Protection Report [serial online] 2009; 3 (26): news. Available at:


http://www.hpa.org.uk/hpr/archives/2009/news2609.htm#h1n1



3. Department of Health. “National Pandemic Flu Service”, 17 July 2009



http://www.hpa.org.uk/hpr/archives/2009/news2809.htm



Pre-surgical risk assessment for variant Creutzfeldt-Jakob disease (vCJD) risk in neurosurgery and eye surgery units


Hospitals should already be using a questionnaire in Annex J of the ACDP TSE Working Group Infection Control guidance to find out whether any patients who are about to undergo any surgery or endoscopy may be at increased risk of being infected with CJD. If a patient is found to have an increased risk of CJD prior to their surgery or endoscopy then special infection control precautions may need to be taken.

Annex J of the TSE Infection Control guidance has recently been revised, and now advises that patients who are due to have high risk surgery [1] or neuro-endoscopy should be asked an additional question: whether they have received transfusions of blood or blood components from 80 or more donors since 1980. This is because these patients may have an increased risk of being infected with variant CJD (vCJD).

On 16 July 2009 the HPA wrote to the chief executives of NHS trusts asking them to ensure that the guidance is implemented. Detailed information and tools for implementing the guidance can be downloaded from the links below.

If you have any queries about the implementation of the guidance, please contact the HPA Centre for Infections CJD Section at mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000391/!x-usc:mailto:cjd@hpa.org.uk or on 020 8327 6074/6411.

Background information on this new pre-surgical assessment is contained in this Letter to chief executives - July 2009 (PDF, 73 KB) written to all hospitals in England.

The new version of Annex J of the TSE Infection Control Guidance contains new question for patients undergoing high risk surgery and neuro-endoscopy. These questions should be used to assess patients' CJD risk factors.

Clinicians carrying out the new pre-surgical assessment should read Pre-surgical assessment Information for healthcare staff - July 2009 (PDF, 163 KB) This vCJD Algorithm for per-surgical roles - July 2009 (PDF, 28 KB) shows suggested roles and responsibilities for infection control teams, surgical teams and blood transfusion specialists.

Information on patients' transfusion histories should be collected using the Highly transfused vCJD risk assessment form - July 2009 (Word Document, 328 KB) This form is also available as a vCJD risk assessment spreadsheet - July 2009 (Excel Spreadsheet, 2.7 MB). This may help calculate the number of blood donors to a patient. The form may be posted or emailed to the HPA Centre for Infections CJD Section mhtml:%7B33B38F65-8D2E-434D-8F9B-8BDCD77D3066%7Dmid://00000391/!x-usc:mailto:cjd@hpa.org.uk.

Blood transfusion laboratories may wish to use this draft Letter to other blood laboratories - July 2009 (Word Document, 31 KB) when collecting transfusion information from other hospitals.

Pre-surgical assessment teams and patients may wish to read this vCJD Information for presurgical patients - July 2009 (PDF, 29 KB) about this new pre-surgical assessment.

[1] High risk surgery is defined as surgery involving any of the following organs or tissues (high risk tissues): brain, spinal cord, cranial nerves (specifically the entire optic nerve and only the intercranial components of the other cranial nerves), cranial nerve ganglia, posterior eye (specifically the posterior hyaloid face, retina, retinal pigment epithelium, choroid, subretinal fluid, optic nerve) and pituitary gland.

Letter to chief executives - July 2009 (PDF, 73 KB) Added/updated: 16 July 2009



http://www.hpa.org.uk/webc/HPAwebFile/HPAweb_C/1247469060207



Pre-surgical assessment Information for healthcare staff - July 2009 (PDF, 163 KB) Added/updated: 16 July 2009



http://www.hpa.org.uk/webc/HPAwebFile/HPAweb_C/1247469061870



vCJD Algorithm for per-surgical roles - July 2009 (PDF, 28 KB) Added/updated: 16 July 2009



http://www.hpa.org.uk/webc/HPAwebFile/HPAweb_C/1247469062057



Highly transfused vCJD risk assessment form - July 2009 (Word Document, 328 KB) Added/updated: 16 July 2009



http://www.hpa.org.uk/webc/HPAwebFile/HPAweb_C/1247469062225



Letter to other blood laboratories - July 2009 (Word Document, 31 KB) Added/updated: 16 July 2009



http://www.hpa.org.uk/webc/HPAwebFile/HPAweb_C/1247469062420



vCJD Information for presurgical patients - July 2009 (PDF, 29 KB) Added/updated: 16 July 2009



http://www.hpa.org.uk/webc/HPAwebFile/HPAweb_C/1247469062586



vCJD risk assessment spreadsheet - July 2009 (Excel Spreadsheet, 2.7 MB) Added/updated: 16 July 2009



http://www.hpa.org.uk/webc/HPAwebFile/HPAweb_C/1247728926790



full text ;



http://www.hpa.org.uk/webw/HPAweb&Page&HPAwebAutoListName/Page/1247469069188



http://www.hpa.org.uk/webc/HPAwebFile/HPAweb_C/1247728926790




Saturday, May 23, 2009

Latest results of HPA study on vCJD-related abnormal prion proteins in extracted tonsil



http://creutzfeldt-jakob-disease.blogspot.com/2009/05/latest-results-of-hpa-study-on-vcjd.html




Wednesday, August 20, 2008

Tonometer disinfection practice in the United Kingdom: A national survey



http://creutzfeldt-jakob-disease.blogspot.com/2008/08/tonometer-disinfection-practice-in.html



Wednesday, January 02, 2008

Risk factors for sporadic Creutzfeldt-Jakob disease



http://creutzfeldt-jakob-disease.blogspot.com/2008/01/risk-factors-for-sporadic-creutzfeldt.html



Sunday, December 16, 2007

Risk factors for sporadic Creutzfeldt-Jakob disease



http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-factors-for-sporadic-creutzfeldt.html



Monday, December 31, 2007

Risk Assessment of Transmission of Sporadic Creutzfeldt-Jakob Disease in Endodontic Practice in Absence of Adequate Prion Inactivation



http://creutzfeldt-jakob-disease.blogspot.com/2007/12/risk-assessment-of-transmission-of.html



Eye procedure raises CJD concerns November 19, 2004 United Press International by STEVE MITCHELL

A New York man who died from a rare brain disorder similar to mad cow disease in May underwent an eye procedure prior to his death that raises concerns about the possibility of transmitting the fatal disease to others, United Press International has learned. The development comes on the heels of the announcement Thursday by U.S. Department of Agriculture officials of a possible second case of mad cow disease in U.S. herds.

Richard Da Silva, 58, of Orange County, N.Y., died from Creutzfeldt Jakob disease, an incurable brain-wasting illness that strikes about one person per million.

Richard's wife Ann Marie Da Silva told UPI he underwent a check for the eye disease glaucoma in 2003, approximately a year before his death. The procedure involves the use of a tonometer, which contacts the cornea -- an eye tissue that can contain prions, the infectious agent thought to cause CJD.

Ann Marie's concern is that others who had the tonometer used on them could have gotten infected.

A 2003 study by British researchers suggests her concerns may be justified. A team led by J.W. Ironside from the National Creutzfeldt-Jakob Disease Surveillance Unit at the University of Edinburgh examined tonometer heads and found they can retain cornea tissue that could infect other people -- even after cleaning and decontaminating the instrument.

"Retained corneal epithelial cells, following the standard decontamination routine of tonometer prisms, may represent potential prion infectivity," the researchers wrote in the British Journal of Ophthalmology last year. "Once the infectious agent is on the cornea, it could theoretically infect the brain."

Prions, misfolded proteins thought to be the cause of mad cow, CJD and similar diseases, are notoriously difficult to destroy and are capable of withstanding most sterilization procedures.

Laura Manuelidis, an expert on these diseases and section chief of surgery in the neuropathology department at Yale University, agreed with the British researchers that tonometers represent a potential risk of passing CJD to other people.

Manuelidis told UPI she has been voicing her concern about the risks of corneas since 1977 when her own study, published in the New England Journal of Medicine, showed the eye tissue, if infected, could transmit CJD.

At the time the procedure was done on Richard Da Silva, about a year before he died, she said it was "absolutely" possible he was infectious.

The CJD Incidents Panel, a body of experts set up by the U.K. Department of Health, noted in a 2001 report that procedures involving the cornea are considered medium risk for transmitting CJD. The first two patients who have a contaminated eye instrument used on them have the highest risk of contracting the disease, the panel said.

In 1999, the U.K. Department of Health banned opticians from reusing equipment that came in contact with patients' eyes out of concern it could result in the transmission of variant CJD, the form of the disease humans can contract from consuming infected beef products.

Richard Da Silva was associated with a cluster of five other cases of CJD in southern New York that raised concerns about vCJD.

None of the cases have been determined to stem from mad cow disease, but concerns about the cattle illness in the United States could increase in light of the USDA announcement Thursday that a cow tested positive on initial tests for the disease. If confirmed, this would be the second U.S. case of the illness; the first was detected in a Washington cow last December. The USDA said the suspect animal disclosed Thursday did not enter the food chain. The USDA did not release further details about the cow, but said results from further lab tests to confirm the initial tests were expected within seven days.

Ann Marie Da Silva said she informed the New York Health Department and later the eye doctor who performed the procedure about her husband's illness and her concerns about the risk of transmitting CJD via the tonometer.

The optometrist -- whom she declined to name because she did not want to jeopardize his career -- "didn't even know what this disease was," she said.

"He said the health department never called him and I called them (the health department) back and they didn't seem concerned about it," she added. "I just kept getting angrier and angrier when I felt I was being dismissed."

She said the state health department "seems to have an attitude of don't ask, don't tell" about CJD.

"There's a stigma attached to it," she said. "Is it because they're so afraid the public will panic? I don't know, but I don't think that the answer is to push things under the rug."

New York State Department of Health spokeswoman Claire Pospisil told UPI she would look into whether the agency was concerned about the possibility of transmitting CJD via tonometers, but she had not called back prior to story publication.

Disposable tonometers are readily available and could avoid the risk of transmitting the disease, Ironside and colleagues noted in their study. Ann Marie Da Silva said she asked the optometrist whether he used disposable tonometers and "he said 'No, it's a reusable one.'"

Ironside's team also noted other ophthalmic instruments come into contact with the cornea and could represent a source of infection as they are either difficult to decontaminate or cannot withstand the harsh procedures necessary to inactivate prions. These include corneal burrs, diagnostic and therapeutic contact lenses and other coated lenses.

Terry Singletary, whose mother died from a type of CJD called Heidenhain Variant, told UPI health officials were not doing enough to prevent people from being infected by contaminated medical equipment.

"They've got to start taking this disease seriously and they simply aren't doing it," said Singletary, who is a member of CJD Watch and CJD Voice -- advocacy groups for CJD patients and their families.

U.S. Centers for Disease Control and Prevention spokeswoman Christine Pearson did not return a phone call from UPI seeking comment. The agency's Web site states the eye is one of three tissues, along with the brain and spinal cord, that are considered to have "high infectivity."

The Web site said more than 250 people worldwide have contracted CJD through contaminated surgical instruments and tissue transplants. This includes as many as four who were infected by corneal grafts. The agency noted no such cases have been reported since 1976, when sterilization procedures were instituted in healthcare facilities.

Ironside and colleagues noted in their study, however, many disinfection procedures used on optical instruments, such as tonometers, fail. They wrote their finding of cornea tissue on tonometers indicates that "no current cleaning and disinfection strategy is fully effective."

Singletary said CDC's assertion that no CJD cases from infected equipment or tissues have been detected since 1976 is misleading.

"They have absolutely no idea" whether any cases have occurred in this manner, he said, because CJD cases often aren't investigated and the agency has not required physicians nationwide report all casesof CJD.

"There's no national surveillance unit for CJD in the United States; people are dying who aren't autopsied, the CDC has no way of knowing" whether people have been infected via infected equipment or tissues, he said.

Ann Marie Da Silva said she has contacted several members of her state's congressional delegation about her concerns, including Rep. Sue Kelly, R-N.Y., and Sen. Charles Schumer, D-N.Y.

"Basically, what I want is to be a positive force in this, but I also want more of a dialogue going on with the public and the health department," she said.



http://www.upi.com/NewsTrack/Science/2004/11/18/eye_procedure_raises_cjd_concerns/2974/




Cadaver corneal transplants -- without family permission Houston, Texas channel 11 news 28 Nov 99 Reported by Terry S. Singeltary Sr.son of CJD victim



http://www.mad-cow.org/dec99_news.html#bbb




Wednesday, July 8, 2009

Transgenic mice expressing porcine prion protein resistant to classical scrapie but susceptible to sheep bovine spongiform encephalopathy and atypical scrapie. Emerg Infect Dis. 2009 Aug; [Epub ahead of print]



http://nor-98.blogspot.com/2009/07/transgenic-mice-expressing-porcine.html



Wednesday, July 1, 2009

Nor98 scrapie identified in the United States J Vet Diagn Invest 21:454-463 (2009)



http://nor-98.blogspot.com/2009/07/nor98-scrapie-identified-in-united.html




Monday, July 06, 2009

Prion infectivity in fat of deer with Chronic Wasting Disease



http://chronic-wasting-disease.blogspot.com/2009/07/prion-infectivity-in-fat-of-deer-with.html




Saturday, June 13, 2009

Monitoring the occurrence of emerging forms of Creutzfeldt-Jakob disease in the United States 2003 revisited 2009



http://cjdusa.blogspot.com/2009/06/monitoring-occurrence-of-emerging-forms.html





Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (TRANSCRIPT)



http://www.fda.gov/downloads/AdvisoryCommittees/CommitteesMeetingMaterials/BloodVaccinesandOtherBiologics/TransmissibleSpongiformEncephalopathiesAdvisoryCommittee/UCM171810.pdf



Meeting of the Transmissible Spongiform Encephalopathies Committee On June 12, 2009 (Singeltary submission)



http://tseac.blogspot.com/2009/05/meeting-of-transmissible-spongiform.html





TSS