British Columbia - Creutzfeldt-Jakob disease being probed in Fraser Valley - one person has classical CJD and two others are very likely to have it

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Creutzfeldt-Jakob disease being probed in Fraser Valley

The disease is often linked to bovine spongiform encephalopathy, better known as mad cow disease

By Zoe McKnight, Vancouver Sun June 14, 2013 5:08 PM

The Fraser Health authority has confirmed they are looking into several suspected cases of Creutzfeldt-Jakob disease.

Spokeswoman Naseem Nuraney said few details were yet available but the condition is ?a very rare disease ? statistically unusual, a cluster like this.?

More...

http://www.vancouversun.com/news/metro/Creutzfeldt+Jakob+disease+being+probed+Fraser+Valley/8528855/story.html

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Possible mad cow cases in Fraser Valley

Vancouver/CKNW (AM980)
Marcella Bernardo | Email news tips to Marcella
6/14/2013

...
"Fraser Health Authority are looking into four possible cases of Creutzfeldt-Jakob syndrome --one of whom is in a deceased patient and three of whom are in hospital."

Doctor Perry Kendall says on average, Canada records 30 cases every year.

...These cases have not yet been confirmed. They're suspect."

Kendall also says there's no chance these people became sick after eating tainted beef.
...

Full text:

http://www.cknw.com/news/vancouver/story.aspx/story.aspx?ID=1985169

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Re: British Columbia - Creutzfeldt-Jakob disease being probed in Fraser Valley

1 dead, 3 ill in possible Creutzfeldt-Jakob cases in B.C.

Health officials in Fraser Valley say there is no risk from eating beef from the region

CBC News
Posted: Jun 14, 2013 5:42 PM MT
Last Updated: Jun 14, 2013 6:35 PM MT
...
B.C.'s Provincial Health Officer Dr. Perry Kendall said the cases were diagnosed by neurologists on Friday, but none have been confirmed because complete diagnosis can only be made on death.

"Every indication is that if it is Creutzfeldt-Jacob disease it would be the classic sporadic kind for which there is really no cause known," said Kendall

He said B.C. gets between five and six cases reported a year, but what makes this situation unusual is to have so many cases reported at once.
...
The Canadian Food Inspection Agency runs a surveillance program which Kendall says has ensured that no suspect beef has made its way into the food supply.

"This is not going to be a food-borne cluster of cases."
...

http://www.cbc.ca/news/canada/edmonton/story/2013/06/14/bc-creutzfeldt-jakob-disease-fraser-valley.html

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Re: British Columbia - Creutzfeldt-Jakob disease being probed in Fraser Valley - 1 dead, 3 ill

While this could just be a coincidence, the possibility of an iatrogenic cluster; that is one caused by tissue donation from a sporadic CJD case, must be investigated and discarded.

I agree that the chance that this is due to variant CJD ("human mad cow") is essentially zero.

The only way for sure to know is to examine the prions in the brain after a patient dies. I hope that is being done for the deceased patient.

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Re: British Columbia - Creutzfeldt-Jakob disease being probed in Fraser Valley - one person has CJD and two others are very likely to have it

Fraser Health Statement on Creutzfeldt-Jakob Disease

June 14, 2013
<!--Fraser Health Statement on Creutzfeldt-Jakob Disease
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Dr. Paul Van Buynder, Chief Medical Health Officer has released the following statement to clarify reports about suspect cases of ?human mad cow disease? in the Lower Mainland:

?I am concerned to see reports this evening in social and traditional media related to our investigation into a small number of possible cases of Creutzfeldt-Jakob disease (CJD). Media have reported that these cases are connected to Bovine Spongiform Encephalopathy ? more commonly known as mad cow disease.

Fraser Health has investigated six possible cases of CJD over the past year. After a case review today we can say that one person has CJD and two others are very likely to have it. The other three cases are very unlikely to have CJD.

I want to be clear there is absolutely no evidence that these three confirmed or probable cases are linked to food consumption.

CJD is a neurological disorder reported an average of about 30 times a year in Canada. There are a handful of cases reported in B.C. each year and we expect around 2 cases in the Fraser region. While three in this time frame is one or two more than I expect, it is within statistical likelihood.

There is no outbreak and I want to reassure residents of the lower mainland that there is no risk to the public or to residents in our facilities.

These are classical cases of CJD. Tests conducted on these patients have ruled out variant CJD associated with disease in cattle. There has never been a home grown case of variant CJD in Canada so this is not surprising.
We have a strong public health surveillance system in B.C. and a review of the cases has not revealed any links between them which would suggest a common source of any kind. The patients investigated come from a number of different cities and settings.

Our sympathies are with the families of these cases of severe progressive disease."

Dr. Paul Van Buynder
Learn more about Creutzfeldt-Jakob Disease.

http://www.fraserhealth.ca/about_us/media_centre/news_releases/2013-news-releases/statement-on-creutzfeldt-jakob-disease

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Re: British Columbia - Creutzfeldt-Jakob disease being probed in Fraser Valley - one person has classical CJD and two others are very likely to have it

Published Date: 2013-06-16 11:36:29
Subject: PRO/EDR> Creutzfeldt-Jakob disease - Canada: (BC)
Archive Number: 20130616.1776151

CREUTZFELDT-JAKOB DISEASE - CANADA: (BRITISH COLUMBIA)
************************************************** ****
A ProMED-mail post

http://www.promedmail.org

ProMED-mail is a program of the
International Society for Infectious Diseases

http://www.isid.org

Date: Sun 16 Jun 2013
Source: The HuffPost, British Columbia [edited]

http://www.huffingtonpost.ca/2013/06/14/creutzfeldt-jakob-outbreak-fraser-valley_n_3444680.html

One case of a rare, degenerative brain disease has been confirmed in British Columbia [BC]'s Fraser Valley, and a health official says 2 other cases are now suspected. However Paul Van Buynder, chief medical officer for the Fraser Health authority, said late on Friday [14 Jun 2013] that even if the 2 suspected cases end up being Creutzfeldt-Jakob disease cases, it's not "drastically unusual".

The confirmed case of Creutzfeldt-Jakob disease, or CJD, was a resident in a long term care home who died a year ago, Van Buynder said. He said the 2 other patients suspected of having Creutzfeldt-Jakob disease are currently in hospital. None of the patients are related, and they all resided in different towns, Van Buynder added. The findings stem from a review officials conducted after they initially believed there were 6 CJD cases in the Fraser Valley, east of Vancouver, over the past year.

Buynder said it's "highly unlikely" that the remaining 3 cases will be confirmed as CJD. Cases can only be confirmed after a sample of brain tissue, taken from the patient post-mortem, is tested at a national laboratory, he said. Before that happens, doctors make an initial diagnosis based on MRI images, blood tested for specific proteins, and an electroencephalogram, a test that measures the brain's activity. Only 30 to 50 cases are reported annually in Canada, and 5 of those cases [would be expected] to occur in BC, he said. "I think the public really need to know that this is probably one or 2 more than I expect, but it's not drastically unusual," said Van Buynder.

"It's not mad cow disease. It has nothing to do with the food chain. Neither the public nor anybody in our hospitals should be worried that they're about to get this nasty disease." Provincial health officer Dr Perry Kendall said CJD causes the rapid onset of dementia leading to a coma, usually within a 6 month time frame, because agents known as prions destroy the brain. One strain has been linked with mad cow disease, but Van Buynder said more than 90 per cent of the cases are sporadic, and health officials don't know their cause, and there is no treatment and no cure. "It's a tragic disease that is rapidly progressing," he said. "It's a degenerative disease of the brain, and usually within 6 months, the afflicted pass away. It's a tragic disease for families, and we just really don't understand it."

[byline: Keven Drews]

--
communicated by:
ProMED-mail rapporteur Kunihiko Iizuka

[According to the Fraser Valley Regional [FVR] District Administration, the population of the district in 2011 was estimated to be 277 593 (http://www.fvrd.bc.ca/AboutUs/Pages/...tatistics.aspx). Therefore, the occurrence of more than a single case of CJD would be unusual, and 3 cases exceptional, since the incidence of CJD in the population is generally accepted to be about one in one million. The single confirmed case and the 2 suspected cases are clearly not cases of variant CJD, since there is no evidence that the food chain is involved. It would be relevant also to investigate whether blood transfusion is involved in any of these cases. Confirmation of the diagnosis in the 2 suspected cases is awaited. - Mod.CP