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Pennsylvania Hampden Twp. man dies of mysterious disease sometimes associated with mad cow and chronic wasting

Posted Jan 07 2014 1:01pm
Hampden Twp. man dies of mysterious disease sometimes associated with mad cow and chronic wasting

 

By David Wenner | dwenner@pennlive.com Email the author | Follow on Twitter on January 07, 2014 at 9:00 AM, updated January 07, 2014 at 9:08 AM

 

 

As recently as July, Weston Ruhrig seemed perfectly sound of body and mind.

 

The 66-year-old was retired from a government career, but busy as an auctioneer, substitute teacher and antique dealer.

 

Today, Ruhrig’s family would be hard pressed to pinpoint the most heart-breaking moment preceding his death on Dec. 19.

 

It could have been when his stepson noticed the formerly affectionate grandfather now seemed annoyed by his grandchildren.

 

Or when his wife, perplexed he didn’t pick up a ringing telephone, found him holding a pad on which he had written “Hello.”

 

Or when he looked up from Thanksgiving dinner, in his own home, and asked “Where are we?”

 

Ruhrig, it turned out, had a rare and terrifying illness: Creutzfeldt-Jakob Disease. No one knew until after he died. Creutzfeldt-Jakob Disease strikes about one in one million people annually. It’s known mostly because of association with the “mad cow disease” which occasionally appears in beef cattle.

 

But Ruhrig died of a version which occurs only in humans and has no connection to the kind that occurs in animals. His family has no idea how he came down with Creutzfeldt-Jakob, which attacks the nervous system and causes personality change and dementia. Nor do scientists have a complete understanding of what causes it and how people get it.

 

This much is known: It’s always fatal and usually kills within a year.

 

Donna VandeMortel talks about her husband

 

Weston Ruhrig was bright and vibrant before being stricken with Creutzfeldt-Jakob Disease.

 

Weston Ruhrig served in the U.S. Army from 1965 to 1968. He spent 25 years as a public health advisor for the U.S. Centers for Disease Control and Prevention. He then joined the Pennsylvania Department of Health, serving as a division director before retiring in 2011. He was married for 35 years to Donna VandeMortel, the former executive director of the victim services division of the Cumberland County District Attorney’s Office.

 

Following his retirement from the government, Ruhrig became a substitute teacher in East Pennsboro and Mechanicsburg, and continued working as an auctioneer for several local companies.

 

According to his family, he was a man of broad interests and deep knowledge. He loved baseball and old blues music. He was an avid watcher of the Sunday morning political shows and a fan of Jon Stewart and Stephen Colbert. He and his wife operated a booth at the Antique Marketplace of Lemoyne.

 

“He was sharp as a tack,” said a stepson, Tim Lipari of Duncannon.

 

The 6-2, 210-pounder was up by 7 a.m. daily, avoided red meat and other foods he considered unhealthy and seemed always on the move. In June, he conducted a charity auction for United Cerebral Palsy of Central Pennsylvania, just as he had since 1987. He seemed normal.

 

But his family began noticing odd behavior. Ruhrig became withdrawn. He continually locked doors, sometimes locking out his wife after she had gone to the yard or garage during daylight. Ruhrig was known for harping on people to turn off lights to save electricity. Now he switched on lights for no reason and left the room.

 

By September, his family had persuaded him to see his family doctor. The doctor found no medical problems but referred him to a neurologist. Ruhrig felt nothing was wrong. In November, the neurologist gave Ruhrig cognitive tests. Ruhrig named the president and recalled facts including his wife’s birth date. But he couldn’t correctly state her age or calculate it. Still, he joked during the visit.

 

But at an appointment with a neuropsychologist only about a week later, Ruhrig couldn’t answer most questions. Agitated, he repeatedly stated and spelled his last name. He said he was being followed and someone might be hiding behind the door.

 

 

Donna VandeMortel describes her husband's rapid decline

 

In less than four months, Wesley Ruhrig went from healthy to dead of a rare and frightening disease. Blood work, a brain scan and an MRI had detected nothing unusual. The doctors were leaning toward a rapidly progressing case of dementia, possibly resulting from Alzheimer’s disease.

 

VandeMortel, however, spoke to people familiar with Alzheimer’s and became convinced his symptoms didn’t exactly match those of Alzheimer’s. All the while, everyone was astounded by the speed of Ruhrig’s decline.

 

He stopped preparing food for himself and ate only when VandeMortel put food in front of him. He could still give himself a shower, but his wife had to instruct him to remove each piece of clothing. He lost more than 20 pounds. One day he asked his stepson to explain how to use the telephone. In late October, with his beloved St. Louis Cardinals in the World Series, he barely showed interest. VandeMortel could no longer converse with her husband.

 

Creutzfeldt-Jakob Disease strikes about 200 people annually in the United States. It’s one of a family of human and animal diseases called transmissible spongiform encephalopathies, according to the National Institutes of Health. “Spongiform” refers to how the disease attacks brain tissue and fills it with holes, making it resemble a sponge when viewed under a microscope. Other versions of the disease are commonly referred as “mad cow disease” in cattle and “chronic wasting disease” in deer.

 

It’s believed humans can get a variant of Creutzfeldt-Jakob Disease by eating or touching the brain or spinal tissue of an animal infected with mad cow. That has resulted in several public health scares since the 1980s. However, the CDC says no one in the United States has ever been stricken with the variant.

 

A spinal tap taken during the effort to diagnose Ruhrig confirmed Creutzfeldt-Jakob, which is listed as his cause of death. Cumberland County Coroner Charles Hall, who filled out the death certificate, said the spinal tap results showed a high level of a protein associated with Creutzfeldt-Jakob. The results arrived after Ruhrig died.

 

Doctors know little about how humans get the disease. According to the NIH, it usually strikes people 60 or older. In about 85 percent of cases, it strikes people with no known risk factor. About 5 to 10 percent of cases involve a family history of the disease and a genetic mutation. A small number of people have gotten it through exposure to infected brain or nervous system tissue, usually from a medical procedure.

 

Eventually VandeMortel couldn’t run errands without having someone look after Ruhrig. She was looking into a full-time aide and adult day care. All the while, Ruhrig seemed unaware anything was wrong with him. Sometimes he seemed to improve briefly.

 

On the evening of Dec. 19, he laughed frequently and seemed unusually happy while watching television as his wife sat nearby. Eventually he rose and walked to the bathroom. In a little while VandeMortel heard a crash. She found him face down on the floor, bleeding from his mouth and where his eyeglasses had gouged the bridge of his nose.

 

He tried to get up as VandeMortel urged him to remain on the floor to wait for an ambulance. She ended up helping him to a seat. Ruhrig made no sound after falling and said nothing during the ordeal. His sat with his eyes open as his wife spoke to 911.

 

Before the ambulance arrived she noticed he wasn’t breathing. The medical crew tried for 25 minutes but couldn’t revive him.

 

According to VandeMortel, Ruhrig’s final months were especially frustrating for her because of delays in getting appointments with a neurologist, which are in short supply.

 

 

 

 She said one doctor told her she had never seen someone decline so quickly. That’s one of the reasons VandeMortel suspected his illness was different than the dementia suggested by doctors and wanted to see additional specialists. She said the spinal tap that confirmed Creutzfeldt-Jakob resulted from her insistence. As a result, she wonders if the disease is more common than experts believe, with some deaths attributed to conditions such as Alzheimer’s.

 

Moreover, she recently learned that a Cumberland County acquaintance whom she had told about Ruhrig’s symptoms had lost his father to Creutzfeldt-Jakob. The acquaintance felt the symptoms matched his father’s. But he said nothing at the time, because he felt the odds of having it were so low, and the outlook so bleak.

 

Now, VandeMortel hopes to raise awareness by telling her husband’s story. Maybe it will help someone understand what is happening to their loved one. “If it causes something to click for someone else, it will be worth it,” she said.




see comments ;


 
 
His family has thought about those things, but is aware of nothing obvious. Weston, as a matter of fact, was a frequent donor of blood, and received certificates acknowledging the many gallons he gave. So with his background in public health, you have to think he well understood the donation form question pertaining to CJD and would have ruled himself out. His family also has given though to his background in public health. Early in his career, he did work that required him to draw blood samples, and he also was involved in tests for sexually transmitted diseases. But as far as they know, he did nothing that might expose him to CJD. They also pointed out that he didn't hunt and rarely ate red meat.
 

 






OUR sincere condolences go out to the family and friends of Mr. Ruhrg.

 

PLEASE be aware of new science just out by the CDC emerging infectious disease journal on CWD and it’s potential link to sporadic CJD...URGENT UPDATE AS FOLLOWS ;

 

please see ;



Volume 20, Number 1—January 2014

 

Dispatch

 

Detection of Infectivity in Blood of Persons with Variant and Sporadic Creutzfeldt-Jakob Disease

 
snip...



 

>>>They found infectivity in the red and white blood cells and plasma of a variant CJD patient and in the plasma of two of four sporadic CJD patients tested. These findings indicate the need to continue assessing the possible risk for CJD transmission via transfusion of blood products.<<<
 

>>>In tgBov inoculated with vCJD and tgHu inoculated with sCJD, the PrPres banding patterns observed by Western blot in animals challenged with brain homogenate and blood components were identical (Figure, panels C, D). These results support the contention that the TSE agent propagated in tgBov mice and tgHu were vCJD and sCJD agents, respectively.<<<
 
 




Wednesday, December 11, 2013

Detection of Infectivity in Blood of Persons with Variant and Sporadic Creutzfeldt-Jakob Disease

http://creutzfeldt-jakob-disease.blogspot.com/2013/12/detection-of-infectivity-in-blood-of.html
 


Monday, December 02, 2013

A parliamentary inquiry has been launched today into the safety of blood, tissue and organ screening following fears that vCJD – the human form of ‘mad cow’ disease – may be being spread by medical procedures

http://creutzfeldt-jakob-disease.blogspot.com/2013/12/a-parliamentary-inquiry-has-been.html

 
 

*** PRICE OF CWD TSE PRION POKER GOES UP 2014 ***

 

Transmissible Spongiform Encephalopathy TSE PRION update January 2, 2014

 

 

*** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.

 

 

*** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.

 

 

Wednesday, January 01, 2014

 

Molecular Barriers to Zoonotic Transmission of Prions

 

 

*** chronic wasting disease, there was no absolute barrier to conversion of the human prion protein.

 

 

*** Furthermore, the form of human PrPres produced in this in vitro assay when seeded with CWD, resembles that found in the most common human prion disease, namely sCJD of the MM1 subtype.

 

 

 


 

 

 


 

 

 

*** The potential impact of prion diseases on human health was greatly magnified by the recognition that interspecies transfer of BSE to humans by beef ingestion resulted in vCJD. While changes in animal feed constituents and slaughter practices appear to have curtailed vCJD, there is concern that CWD of free-ranging deer and elk in the U.S. might also cross the species barrier. Thus, consuming venison could be a source of human prion disease. Whether BSE and CWD represent interspecies scrapie transfer or are newly arisen prion diseases is unknown. Therefore, the possibility of transmission of prion disease through other food animals cannot be ruled out. There is evidence that vCJD can be transmitted through blood transfusion. There is likely a pool of unknown size of asymptomatic individuals infected with vCJD, and there may be asymptomatic individuals infected with the CWD equivalent. These circumstances represent a potential threat to blood, blood products, and plasma supplies.

 


 

 

 

 

Thursday, January 2, 2014

 

*** CWD TSE Prion in cervids to hTGmice, Heidenhain Variant Creutzfeldt-Jacob Disease MM1 genotype, and iatrogenic CJD ?? ***

 


 

 

 

Wednesday, January 01, 2014

 

APHIS-2006-0118-0100 Chronic Wasting Disease Herd Certification Program and Interstate Movement of Farmed or Captive Deer, Elk, and Moose

 


 

 

Friday, November 22, 2013

 

Wasting disease is threat to the entire UK deer population CWD TSE Prion disease Singeltary submission to Scottish Parliament

 


 

 

Thursday, October 10, 2013

 

*** CJD REPORT 1994 increased risk for consumption of veal and venison and lamb

 


 

 

 

Thursday, January 02, 2014

 

Tests Confirm CWD Case in Pennsylvania Release #001-14

 


 

 

 

Wednesday, September 04, 2013

 

*** cwd - cervid captive livestock escapes, loose and on the run in the wild

 


 

 

Sunday, December 15, 2013

 

FDA PART 589 -- SUBSTANCES PROHIBITED FROM USE IN ANIMAL FOOD OR FEED VIOLATIONS OFFICIAL ACTION INDICATED OIA UPDATE DECEMBER 2013 UPDATE

 


 

 

Saturday, December 21, 2013

 

Complementary studies detecting classical bovine spongiform encephalopathy infectivity in jejunum, ileum and ileocaecal junction in incubating cattle

 


 

 

Friday, August 16, 2013

 

*** Creutzfeldt-Jakob disease (CJD) biannual update August 2013 U.K. and Contaminated blood products induce a highly atypical prion disease devoid of PrPres in primates

 


 

 

Sunday, August 11, 2013

 

Creutzfeldt-Jakob Disease CJD cases rising North America updated report August 2013

 

*** Creutzfeldt-Jakob Disease CJD cases rising North America with Canada seeing an extreme increase of 48% between 2008 and 2010

 

 


 

 

Sunday, October 13, 2013

 

*** CJD TSE Prion Disease Cases in Texas by Year, 2003-2012

 


 

 

Saturday, November 16, 2013

 

Management of neurosurgical instruments and patients exposed to creutzfeldt-jakob disease 2013 December

 

Infect Control Hosp Epidemiol.

 


 

 

Monday, December 02, 2013

 

*** A parliamentary inquiry has been launched today into the safety of blood, tissue and organ screening following fears that vCJD – the human form of ‘mad cow’ disease – may be being spread by medical procedures

 


 

 

Wednesday, December 11, 2013

 

*** Detection of Infectivity in Blood of Persons with Variant and Sporadic Creutzfeldt-Jakob Disease

 


 

 

 

layperson,

 

 kindest regards, terry

 

 Terry S. Singeltary Sr.

 

 Bacliff, Texas USA 77518

 


 

 
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