Tracie McEwen's Story
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How unusual is CJD onset at age 29?

What happened to Doug McEwen?

8 Jan 99 by Tracie McEwen <email deleted by request>
This story is offered to our readership with the personal blessing and best wishes of Tracie MeEwen of Salt Lake City, Utah

"I have been added to the list by Melvin Steiger of SLC, Utah, at my request [whose wife, Ellie, died of CJD in early 1998 -- webmaster]. He has been a great help to me these past few weeks.

My name is Tracie McEwen and I am 28 yrs. old, my husband is Doug and he is 30 yrs. old. We have two little girls, Sharon who is 8 yrs. old and Rilee who is 3 yrs. old. We live about 30 miles north of SLC.

My husband became having problems that I noticed in early summer. The first thing I remember was he forgot how to spell my name. Then I just noticed things like if I called him at work and asked him to bring home milk, he would forget the milk or that I had even called. He was having problems getting all of his paperwork done, so I was doing his monthly expense report.

I didn't realize until about a month later that he was actually having a hard time doing basic math. By the end of July, he was really having trouble. He was in Idaho on business, and was very late calling home one night, when he did he told me he couldn't remember our phone number.

I asked why he hadn't called directory assistance, he told me he couldn't remember how to spell his last name.

I told him to come home right away. By the middle of August he could hardly perform his job tasks. He blamed it on the stress from being out of town on business so much. He felt that if he got another job, things would be better. So I typed up a letter of resignation for him (because he couldn't remember how to use the computer).

Well, he found he couldn't fill out a job application by himself. Near the end of the month, we sold the mobile home we had lived in while we were finishing college. We had just had a house built and were suppose to be moving in, but now he had no job, and I hadn't found a contract to teach yet.

When some good friends of ours came to help us move, and he didn't know who they were I told him that he was going to the doctor. When he resigned, we lost all of our insurance, but the house builder was very nice and gave us back all of the money we had put down on the new home. We used that money to go to the doctor.

The first was a family practioner, who did the standard blood tests and then when they all came back negative referred us to a psychologist. He felt Doug was either depressed or suffering from a conversion disorder (meaning life had become to stressful for Doug and all of his problems were in his head, or at least mental so that he could get out of working).

I knew this was wrong, Doug was a hard worker, always had been. The psychologist gave Doug a MMPI after she met with him. Her feelings were that this was a neurological problem and recommended a neurologist. The night before I took Doug to the neurologist was the first time I ever heard of CJD. I saw Mel Steiger on the TV talking about his wife Ellie.

When we got to the neurologist the next day, I still remember him saying "I know what you are worrying about, but he is too young and this disease is too rare." So began a long period of testing, over 100 lab tests, 4 MRI/MRA's, EEG (that was fine), CAT scan, spinal tap (that showed elevated protein, but tested negative for CJD at NIH [by 14-3-3 test]), a cerebral angiogram, and finally a SPECT [single photon emission computed topography] of the brain, which showed an area of low and no blood flow on the left side of his brain.

With no other alternatives, we had a brain biopsy performed and a the second spinal tap. He had the biopsy on November 24, and on November 25 I was told he had TSE which of course was indicative of CJD. I argued that he had had the EEG and spinal tap which had been fine. They assured me there was no mistaking what they had seen.

Two weeks later, I was told this CSF sample also tested for the CJD. I was told that he had six to eight weeks left to live, at best. Despite doctors protests, I checked him out the day I found out, straight out of Neuro-Critical Care. My feelings were if there was nothing they could do for him, then he might as well be home with those who love him.

I quickly arranged for home hospice. That is working out nicely. He is doing better than expected, but I told a friend yesterday that he is not the man I married and loved, he is the shell of that man. I have let go of my husband the best that I can, I am dedicated to caring for him at home until this is over. My work has been so good, I am now on catastrophic leave.

Our youngest really seems unaffected, she just turned three last month and is very happy-go-lucky. Our eight-year old is heartbroken, her daddy goes through periods where he will not speak to any of us, she will climb up on his lap and say I love you daddy, and he will not even look at her. These two just this last June were the best of buddies, they were together all of the time.

I made the very painful decision to have her go stay with some close personal friends the other day until this is over. He gets easily agitated and the other night he thought she said something she did not and tried to chase her, arms flailing, yelling. It was horrific. She was devasted.

Shortly after she climbed on his lap, to apologize for making him mad (which I tried to explain wasn't her fault), she begged him four or five times to talk to her, but he wouldn't even look at her, and then he pushed her off his lap.

I knew then that she can't stay, she can't have these as her last memories of her daddy, her best friend. This is the worst thing I have ever seen, I wouldn't wish it on my worst enemy.

Last night, he had his first seizure in bed that lasted for about three minutes, maybe, but when it was over he looked right at me and said he loved me ( I have not heard those words from him in over two months), while he tried to get his breathing back to normal. I feel that my time with him is soon to end.

This is who we are right now, but this is not who we were six months ago. Then we were two recent college graduates, with two little girls, getting ready to move into a new home, and begin a whole new chapter of our life. Now we are finishing the book of our life together, and I want to know why, how, and when this disease will get enough attention, so that someone somewhere is allowed to find answers and solutions to this life stealer.

Tracie McEwen

[Tracie writes on 8 Jan 98, "I am feeling very overwhelmed with everything right nowand I am going to unsubscribe to the CJD Voice until my husband passes away. I am very appreciative for the wonderful kindness shown to me,but am having a very hard time emotionally. You have my blessing to reprintmy story, and for the work that you do, thanks again for your support."]

Brain disease a slow goodbye: His illness at center of global debate

By Elaine Jarvik Deseret News special writer 17 Jan 99
Doug McEwen's decline began in the summer of 1998 as something so benign you would have sworn it was just a touch of forgetfulness. Now, six months later, McEwen and his wife, Tracie, find themselves in the center of a national debate about "mad cow" disease and the government's role in preventing it.

Tracie McEwen also finds herself facing tough decisions, as dueling scientists lobby to be the recipient of her husband's brain once he dies. In early January, Tracie McEwen joined several activist groups including the Center for Food Safety and the Center for Media and Democracy as a petitioner in two legal actions demanding changes in the way the government monitors and prevents mad cow disease and related disorders, including a human version known as CJD, short for Creutzfeldt-Jakob disease.

"Terminally ill Utah hunter, age 30, could be the first victim of US 'mad deer' disease," read the headline of a press release issued last week from the Center for Food Safety in Washington, D.C.

November 1998 family photo

Like everything surrounding mad cow disease and its medical cousins, the case of Doug McEwen reveals how confusing, controversial and provocative the subject is. Can CJD be caused by the food we eat? Does that food include deer meat? Are cases like this the beginning of a world epidemic, or are food activists using scare tactics?

The issues are further confounded by a string of initials and disease names: CJD, CWD, BSE, TSE, mad cow, mad deer. What they have in common is a set of similar and terrifying neurological symptoms, brain tissue that looks like sponge (hence the term "spongiform encephalopathy," the medical term for the diseases), and a disease course that ends, always in death.

The disease can incubate for decades before symptoms appear. Diagnosis is difficult, and reporting of the disease has been lax, so it's not even clear how many cases of CJD there are in the United States. Perhaps most frightening, the disease agent -- a deviant form of protein called a prion -- can't be killed by radiation, freezing or sterilization.

In England, a "new variant" form of CJD has been linked to bovine spongiform encephalopathy, also known as mad cow disease. So far 40 people have been diagnosed with BSE, which many scientists believe came from eating British beef. But this is a point of contention, too. Stanley Prusiner, who received the 1997 Nobel Prize in medicine for his work on prions, is among those who isn't totally convinced.

The link between human disease and "mad deer" disease that is, between CJD and CWD (chronic wasting disease) is even more unclear. Six percent of deer in northeastern Colorado and southeastern Wyoming have been found to have chronic wasting disease. If it turns out that Doug McEwen has a form of CJD similar to this "mad deer" disease, his would be the first confirmed case. "If there's going to be a canary in the coal mine," says one researcher, "it will be McEwen." But it may take years to find out.

The beginning

Doug McEwen was 29 when he developed his first vague symptoms of CJD. Tracie would call Doug at work and tell him to bring home some milk and he'd forget to do it and then say he didn't even remember her call. That was in early July last year. Then, in late July, Doug went to Idaho on a business trip. As usual, Doug would call home every night to tell Tracie and their two daughters good night. One night, though, Tracie didn't get a call till 11.

"I couldn't remember our phone number," Doug told her.
"Why didn't you call directory assistance?" she asked.
"I can't remember how to spell my last name," he said.

By August, when he decided to quit his job, he couldn't write the letter of resignation because he couldn't remember how to turn on the computer. At first his doctor suspected depression or stress, and Doug was sent to a psychiatrist. In mid-September, on the eve of Doug's first appointment with a neurologist, the McEwens happened to see Kearns resident Mel Steiger being interviewed on TV about his wife, Ellie, who died of CJD last March. Ellie's symptoms -- the memory loss that proceeded eventually to Ellie's inability to recognize her own family -- sounded distressingly familiar.

"When we got to the neurologist the next day, I still remember him saying 'I know what you are worrying about, but he is too young and this disease is too rare,' " Tracie recalls in a letter posted on CJD Voice, a Web site for family members of people with the disease. CJD generally shows up in people older than 50.

When testing of McEwen pointed to the possibility that it was CJD, doctors decided to send tissue of McEwen's brain to researchers in Ohio. The biopsy confirmed the CJD diagnosis. Currently, scientists at the National Institutes of Health are testing Doug's DNA to determine if he has a genetic flaw that caused the disease. Ten percent of CJD cases are believed to be inherited. If Doug falls into this category, his disease will be an open-and-shut case. But if he didn't inherit the mutated gene, the case gets murkier. Like other CJD cases, Doug's makes clear how much we still don't know about the disease.

About 1 percent of CJD cases are believed to be "iatrogenic," that is resulting from medical procedures, including corneal transplants, contaminated brain electrodes and growth hormones. Doug has had none of these. (A couple of CJD cases have raised questions about dental procedures, but there has been no evidence to date that can prove a link.)

CJD cases that aren't genetic and aren't iatrogenic are labeled "sporadic." But, although the name implies that the disease appears randomly the estimate is that one in a million people for some reason develop mutations in their brain cells it may be that we just don't yet understand what causes sporadic CJD, says an Oregon researcher.

Eating infected meat could be one of those causes. In Doug's case, all that's known for sure right now is that he is not suffering from "new variant CJD," the English version that may be connected to English beef. But what about deer meat?

Hunting connection?

According to Tracie McEwen, Doug has been eating deer, elk and antelope since he was a little boy; as a couple, they ate game two or three times a month in the fall and winter. As a hunter, he also handled deer carcasses. Tracie's gut feeling is that Doug did not get CJD from game, especially since she doesn't think he or his brother ever hunted in northeastern Colorado or southeastern Wyoming.

But other people think Doug could end up being the first link between CWD and CJD. Epidemiological studies have identified several "risk factors" for CJD -- that is, some things that many of the cases had in common -- and one of those was a diet of "wild game." It doesn't prove causation, but it is a correlation, said one researcher.

The Utah Department of Health is investigating Doug McEwen's illness, as well as the seven other cases of CJD in Utah in 1998. Meanwhile, the official position is that "there is nothing right now to link his illness to eating wild meat" or handling wild meat, says epidemiologist Gerrie Dowdle, manager of the department's communicable disease control program. To suggest that Doug's diet may have caused his disease is "purely assumption," says Dowdle. "We haven't heard of any cases of humans getting it from elk or deer. That's not to say it's not possible," she adds.

Can CWD be spread to humans? Scientists at the Rocky Mountain Labs in Hamilton, Mont., are conducting tests that may provide some part of the answer, according to researcher Byron Caughey. Scientists don't even know how deer get CWD. Did it first appear as a sporadic mutation and then get spread to other deer? Did deer get it from sheep, who can have a similar disease known as scrapie?

At the Center for Food Safety, Ronnie Cummings's theory is that the disease was spread to deer at central deer-feeding stations, through deer feed that includes rendered animal protein that is, ground up meat that might have come from other diseased animals. (Feeding animals to animals is believed by many to be the cause of the spread of other forms of the disease, including the bovine version. The Center for Food Safety's petition filed Jan. 7 called on the FDA to "close serious loopholes in U.S. animal-feed regulations which currently allow types of cannibalistic feeding practices known to cause and spread 'mad cow' type disease." These include "feeding pigs to pigs, pigs to cattle and cattle blood products to calves.") [See American Proteins site that discusses calf nutrition products that contain bovine blood products. -- webmaster]

If a deer has CWD, would a hunter be able to tell? "We don't know if animals can be infectious without showing symptoms," says Beth Williams, professor of veterinary science at the University of Wyoming and a lead researcher in CWD. "But we can detect the abnormal protein in the brain of animals before they get sick." Are hunters shooting deer whose initial symptoms might only include a clumsiness thatmakes shooting them easier?

According to Utah state veterinarian Michael Marshall, animal health officials in Utah are "monitoring carefully" for chronic wasting disease at privately owned elk farms, "and have found no occurrence whatsoever." In addition, some 150 Utah deer brains have been collected and sent to the state veterinary diagnostic laboratory at Utah State University. So far all 80 tested have proved negative, he says. [This raises two questions: lack of experience with the pathology of this disease compared to Laramie and in-state economic conflicts of interest. -- webmaster.]

More questions than answers

Doug McEwen's brain may provide some answers about CJD and perhaps even its connection to "mad deer" disease. And that means that several different laboratories will be vying to get their gloved hands on his brain once he dies."We want to make sure reliable people get it," says Cummings of the Center for Food Safety. "We don't trust Prusiner and (Paul) Brown (of the National Institutes of Health). We think they're capable of repressing results." But it could take a couple of years for the brain tissue to yield answers. First researchers have to determine what the strain of CWD would look like once it was transmitted to humans.

At this point, Tracie McEwen isn't sure she wants to give her husband's brain away. An autopsy would not only mean no open casket, it would also mean Doug might not be embalmed.

This past weekend, 220 members of the Utah Funeral Directors Association met in St. George; high on their list of agenda items was what to do about CJD. The association has issued no guidelines, says UFDA president Kurt Soffe, but is trying to educate its members about the dangers of the disease.

One study that strikes Soffe as significant: Prions placed on an inanimate object were found, three years later, to still be infectious. The inanimate objects mortuary workers would be concerned with, of course, include embalming tables and instruments. Cleaning and sterilizing equipment can reduce the number of prions but don't seem to totally eliminate them. It is not known how much infectious material is needed to cause disease. In addition, it turns out, the formaldehyde used by embalmers makes prions even more durable, hardening the encasement around the protein.

What Soffe and other morticians also worry about are the cases of undiagnosed CJD. The disease is difficult to diagnose without a brain biopsy or autopsy and CJD has symptoms nearly identical to Alzheimer's. There is evidence that some cases of Alzheimer's have been misdiagnosed CJD cases. Often cited is a Yale study that found that six of 46 people who reportedly died from Alzheimer's actually had CJD.

A new study of CJD just reported last week in the British journal "Lancet" raises even more troubling questions. Researchers in Great Britain reported that CJD prions could be found in tonsil and appendix tissue.

"Although the concentration of infectious prions is expected to be lower in these tissues than in the brain, the opportunity for iatrogenic transmission from appendicectomies or tonsillectomies should not be overlooked. . . . Perhaps the adoption of universal precautions, based on the assumption that the material is infectious until proven otherwise, would be the most prudent course."

There are still more questions than answers. In the meantime, Tracie McEwen gets calls from well-meaning strangers who insist that they know just the right herb/magnet/tonic to make Doug well. One woman even wanted to send breast milk.

Doug McEwen continues to get worse, almost daily. He has to work hard to remember how to use the stairs. His speech has become garbled; he has begun having seizures and has become incontinent. Most disturbing, he will often withdraw from his family, refusing to even look at 8-year-old Sharon when she climbs on his lap.

"This is the worst thing I have ever seen," says Tracie McEwen. "I wouldn't wish it on my worst enemy."

X-From_: Tue Jan 19 22:44:12 1999 From: "Hansen, Michael" Subject: USA Today story on CJD Date: Wed, 20 Jan 1999 01:39:55 -0500 MIME-Version: 1.0

USA Today January 19, 1999

Rare Illness taking a swift toll on young family

By Anita Manning special for USA TODAY 19 Jan 99
A young man in Kaysville, Utah, is dying of a disease so rare but with such international notoriety that it has turned what would normally be a private tragedy into a rallying point for public health activists. Creutzfeldt-Jakob Disease or CJD, is thought to strike about one in a million people around the world, most of them over the age of 50.

But Doug McEwen is only 30.

At his age and younger, only five cases of CJD per billion people are reported each year worldwide, says Lawrence Schonberger of the Centers for Disease Control and Prevention. "It's rare but it's not unheard of,'' he says. But with the unnerving news that surfaced in the past two years about a variant of CJD, "you can understand the concern,' Schonberger says.'

"New variant'' CJD is the fatal illness that has struck at least 33 young people in the United Kingdom and has been linked to "mad cow'' disease, the latter thought to have emerged because of certain animal feeding practices. People were infected by eating meats, leading Great Britain to slaughter thousands of cattle. There has never been a connection made between feeding practices and classic CJD, which even looks different under a microscope than its new variant, Schonberger says.

In McEwen's case, "there was concern initially about it being new variant because of his age.'' A brain biopsy performed Nov. 24 confirmed that McEwen has CJD - and ruled out the new variant. But some people are unconvinced, and many still-unanswered questions are being investigated by state and federal health officials: How did McEwen get this disease? Does the fact that he was a frequent plasma donor suggest a risk to the blood supply?

Could McEwen, a hunter, have contracted this illness by contact with wild deer and elk, which sometimes carry a disease similar to mad cow disease? Could he have come in contact with infected beef or some other food during a year in rural parts of Canada more than a decade ago, when he was serving as a missionary with the Mormon Church?

Earlier this month, a group of consumer activists and families of CJD victims, including the McEwens, petitioned the CDC and the Food and Drug Administration to enact stricter measures to protect the public from "transmissible spongiform encephalopathies'' (TSEs), the family of diseases that includes CJD and similar diseases of animals. The petitioners think there's a much bigger threat to U.S. consumers posed by animal feeding practices and imcomplete reporting than government agencies acknowledge.

So the activists asked the FDA to restrict the feeding of animals to other animals -- a practice associated with the emergence of mad cow disease in England -- and to ban any TSE-infected material from animal feed, cosmetics, fertilizer or other products. They also want the FDA to require meat producers and renderers to keep records for ten years. The coalition called on the CDC to initiate a national program to test, study and require reporting of all human cases of TSE.

Michael Hansen, a scientist with Consumer's Union who has criticized U.S. policies permitting animal-to-animal feeding, says "There's some suggestive evidence that some of the sporadic CJD might have a connection with animal products.'' He notes that McEwen hunted and ate game from areas where animals have been known to be infected with a TSE called chronic wasting disease. He suspects that in some cases, at least, "there a genetic predisposition triggered by something in the environment.''

McEwen's wife, Tracie, 28, who is caring for him at home with the help of friends and hospice, can only speculate. She doubts he acquired the disease from eating deer or elk, because the whole family ate that food. But she wonders about that trip to Canada. "LDS (Latter-Day Saints, or Mormon) missionaries go out and they eat what's served to them. I wondered if he did ingest something, animal brain or something he didn't know,'' she says.

But aside from the immediate crisis, what concerns her most is the idea that her husband might have passed the infection unwittingly to someone else through his donations of plasma. "He donated well after his symptoms started,'' she says. "He was at the point where he was forgetting phone numbers and names, but if I'd thought he had a fatal disease, he wouldn't have donated.''

Health officials have said there is no evidence that CJD can be acquired through blood transfusion, but the FDA has quarantined all the blood products containing McEwen's plasma donation since last January. Plasma donated before January is thought to have been already used. "They're tried to assure me it can't be transmitted that way,'' says Tracie McEwen, but she's skeptical: "I don't think they know.''

So much is unknown about CJD, first described in the 1920s by two German neurologists. Symptoms include personality changes, problems with memory and thinking, and loss of muscular coordination. Death usually comes within a year of the start of symptoms. A growing number of people, including family members of CJD victims, believe the disease is underreported, often going misdiagnosed as Alzheimer's disease or dementia.

"Dementia is a symptom, not a disease,'' says Mel Steiger of Salt Lake City, whose wife Ellie died of CJD on March, 19, 1998, at the age of 57. "I'm of the opinion that a fairly high percentage of Alzheimer's victims are really sick with CJD.'' At least one small study supports that. In 1989, Yale researchers reported that of 46 cases diagnosed as Alzheimer's disease, six were proved at autopsy to be CJD.

In Utah last year, seven people in a population of 2 million were diagnosed with CJD, including McEwen. State epidemiologist Craig Nichols says that's an anomaly. "It's not unusual to have clusters, just statistical abnormalities,'' he says. "The rate over time is consistent with what you see in the U.S. and Britain.''

Nichols cautions against drawing alarming conclusions based on incomplete data. "What people have to understand is this is the infancy of investigation. You need to go back to the early 1980s when we were doing HIV investigation and look at the controversy and hypotheses at that time,'' he says. "We will ask about consumption of British beef and wild game without having any evidence that those are linked to the patient or any other patient.''

"But people who don't understand how epidemiology operates jump to conclusions and think that the questions have significance. We do not know how (McEwen) became infected. We have recommended genetic testing, because familial links are known to exist. That has not been ruled out. As to any other source, it's completely unknown.''

Six months ago, Doug McEwen was a big, strapping guy, vital and healthy. He was advancing as a territory manager for a grocery brokerage and looking forward to moving into a new home with his wife, who teaches 9th grade geography, and their two little girls, Sharon, 8, and Rilee, 3.

"This is when life is supposed to get good,'' says Tracie.

It was not to be. His illness started in early summer, when he forgot how to spell his wife's name. In an account of the chronology of events, which she allowed to be posted on an Internet Web site last week (, Tracie McEwen writes that he quickly grew more disoriented.

"By the end of July, he was really having trouble. He was in Idaho on business and was very late calling home one night.'' When he did call, he said he'd been unable to remember their phone number - and unable to call directory assistance because he could not remember how to spell his last name. Because his work performance was suffering, Doug quit his job on Aug. 10.

"He thought it was the stress of travel,'' she says in an interview. "He didn't want to not do a good job.'' But when he went to look for new employment, he could not fill out applications. That's when he sought medical help and began a staggering round of tests. "I was told that he had six to eight weeks left to live, at best,'' she says. "He's still doing pretty well,'' despite bad days.

"He told me he didn't want to continue to live like this,'' she says. "This is what's troubling - he knows he's dying. He knows when he wants to say something and can't. He's aware and it's an extremely horrible situation for him. My husband is trapped and dying in his body and there's nothing he can do about it. He knows.''

Side Box: Creutzfeldt-Jacob disease A distant cousin of the illness know as "mad cow" disease in England, Creutzfeldt-Jacob disease (CJD) is one of a family of killers that infect the brain, causing characteristic holes that resemble sponges under a microscope. They're called "transmissible spongiform encephalopathies," or TSEs. The National Institute of Neurological Disorders and Stroke says there are three major categories:

* Sporadic. The most common type, accounting for at least 85% of cases, it affects people who have no known risk factor.

* Hereditary. About 5% to 10% of CJD cases in the USA occur in people who have a family history of the disease and/or test positive for a genetic mutation associated with the disease.

* Acquired. Fewer than 1% of cases are caused by exposure to brain or nervous system tissue, usually with the disease. The appearance of a new variant (nvCJD) in younger-than-average people in Europe has been linked to consumption of beef contaminated with bovine spongiform encephalopathy, or BSE, commonly called mad cow disease. It is one of several TSEs that affect animals.


Wednesday, January 20, 1999, 7:00PM
CJD (Crutzfeld Jacob's Disease) 
It Is Here! You saw it in our Papers and TV News
NV CJD: New Variant or presumed biological cousin to Mad Cow Disease. 
Is it killing our Utah loved ones? 
Are we also at risk?
Wednesday, January 20, 1999, 7:00PM
Old Layton Post Office, 109 West Gentile, Layton Utah
(Just west of Bank on corner of Gentile & Main Street in Layton.  
Plenty of free parking. 
If you can bring a folding chair or two it may be helpful if needed.)
No cost,  but some commercially published books on the subject will be available if you desire.

Also called "fast" Alzheimer's Disease
Both Mad Cow Disease and CJD are fatal in humans or animals
6 Utah victims in 1998 -- 5 have died*
All 6 victims lived in Northern Utah (West Valley City northwards)
Disease starts in spleen, progresses to brain and spinal column
Victims die within one year
No known cure
*Local friends of the Layton Utah family of 6th yet living victim have arranged this open forum, and are petitioning for proper education and reporting on this disease. Family, Medical workers and Morticians are at potential risk; you need to know!
Special Presentation/Speakers:
Melvin Steiger, Husband of West Valley victim (Last Year)
Dr Douglas Lyons, Utah Center for Disease Control / Unv of Utah

Open forum:
Is it contagious?
What can we do??
What are we not being told???

Question and answer fielded

For Information call (801) 543-1876 
(or if no answer 298-9095 - leave message)

New CJD fears after test finds disease in tonsils

Thu, Jan 14, 1999 By John von Radowitz, Medical Correspondent, PA News
16 Jan 99 Lancet has free full text after registration
Scientists are to test thousands of people for CJD, the human form of mad cow disease, after discovering evidence in people's tonsils, it emerged today. Previously the disease could only be confirmed after victims had died. The find means it may be possible in the next three years to establish if a CJD time bomb is ticking within Britain's population. But it also raises renewed concerns about the risk of infection from surgical equipment in hospitals, the expert behind the discovery said.

Scientists plan to screen thousands of people using a new test. A significant positive result would provide early warning of a major epidemic to come and allow time for action aimed at averting the disaster. Professor John Collinge, from St Mary's Hospital, London, said today: "If we were to screen several thousand tonsils and found that several were positive that would be a real cause for concern."

The concern about infection raised by the new findings centres on rogue prion proteins that spread the disease and cannot be cleaned from surgical instruments no matter how thoroughly they are sterilised. In future therefore it may be necessary to introduce disposable instruments for certain procedures. A special committee of experts advising the Government is already looking at this issue.

Animals studies have shown that spongiform encephalopathy diseases, which include different forms of CJD, BSE in cattle and the sheep infection scrapie, tend to reside in the lymph system before attacking the brain. Tonsils are linked to the lymph system. The new research by Professor Collinge's team showed this also appeared to be the way new variant CJD behaved in humans. But only the new variant form of CJD, which is effectively mad cow disease transferred to humans through infected beef, was detected in tonsils. The "classical" form of CJD, which appears for no known reason in one person in a million, was not seen in the tissue samples.

The scientists tested tonsil, lymph node and spleen samples taken from people who had died from various types of CJD and neurological diseases. Encouraged by the results, they then tested tonsil samples from 20 patients suspected of suffering from CJD. Nine of the 20 were found to be infected with the new variant disease, of whom four have since died. Of the nine, only three have been added to the official statistics. These currently show that 33 confirmed cases of new variant CJD have occurred in the UK since the disease was identified in 1996. [The revised total for 18 Jan 99 is 40 cases UK + 1 case France. -- webmaster]

The results mean that scientists now know they can diagnose new variant CJD by analysing a surgically removed piece of tonsil tissue. Until now scientists have had to wait until the death of a patient to find out definitively if there is CJD infection.

No-one knows at present what the fall-out might be from people eating BSE-infected beef that was routinely used in burgers and other meat products before a ban on suspect offals was introduced in 1989. New variant CJD has a long incubation period which may run into decades, in which case the trickle of cases seen so far may be just the start of a flood. Alternatively if the incubation period is shorter the problem may not get much worse than it is now.

Professor Collinge, whose research was published today in the Lancet medical journal, said the new test "offers the possibility that we could screen tonsils from the population to determine what proportion were carrying this rogue form of the prion.

"That would give us some idea of the prevalence of new variant CJD, which is unknown - whether it's going to be a relatively small problem or whether many thousands are incubating the disease. It may give some advance warning of a major epidemic."

With this in mind a screening programme is being planned which will test tonsils removed in routine operations around the country. The study, funded by the Medical Research Council and the Government, is expected to start in about six months.

Professor Collinge admitted he was concerned about the possibility of new variant CJD being transmitted via infected surgical instruments. This had always been a theoretical risk. But the conclusive evidence of tonsil infection meant it was now a real possibility. But throwing away expensive surgical instruments had to be ruled out because of the cost, said Professor Collinge. He added: "There is no means of sterilising surgical instruments adequately for prions. "My own view of this is that we may need to consider using more disposable instruments for certain procedures."

Experts from the Joint Advisory Committee on Dangerous Pathogens and the Spongiform Encephalopathy Advisory Committee were now considering the surgical implications and will be advising the Government about what to do. Acting on expert advice the Government has already introduced leucodepletion - removal of white blood cells - for donated blood and banned the use of British-produced plasma in blood products.

At present there is no cure for new variant CJD, but research has shown that a drug used in America to treat cystitis may block its spread. [There are only very preliminary studies on pentosan -- webmaster.] Professor Liam Donaldson, the Government's Chief Medical Officer, said the use of tonsil tissue to diagnose new variant CJD was important but needed further evaluation. "The significance of the finding for patients who do not have symptoms of nvCJD will require further research," he said.

He added that the experts looking at the issue of surgical instruments had been asked to consider Prof Collinge's findings and provide advice about clinical policy.

"Current policy based on advice from the Advisory Committee on Dangerous Pathogens published in April 1998 is that when any patient with symptoms of nvCJD, or suspected of having nvCJD, undergoes a surgical operation the instruments must be removed so that they cannot be used again."

New CJD Fears After Test Finds Disease In Tonsils

From the Press Association
Guardian ... Friday 15 January 1999
Scientists have discovered evidence of CJD, the human form of mad cow disease, in people's tonsils. The find means it may be possible in the next three years to establish if a CJD time bomb is ticking within Britain's population. But it also raises renewed concerns about the risk of infection from surgical equipment in hospitals, the expert behind the discovery said.

Scientists plan to screen thousands of people using the new test. A significant positive result would provide early warning of a major epidemic to come and allow time for action aimed at averting the disaster. Professor John Collinge, from St Mary's Hospital, London, said: "If we were to screen several thousand tonsils and found that several were positive, that would be a real cause for concern."

The concern about infection raised by the new findings centres on rogue prion proteins that spread the disease and cannot be cleaned from surgical instruments no matter how thoroughly they are sterilised. In future therefore it may be necessary to introduce disposable instruments for certain procedures. A special committee of experts advising the Government is already looking at this issue.

Animal studies have shown that spongiform encephalopathy diseases, which include different forms of CJD, BSE in cattle and the sheep infection scrapie, tend to reside in the lymph system before attacking the brain. Tonsils are linked to the lymph system.

The new research by Professor Collinge's team showed this also appeared to be the way new variant CJD behaved in humans. But only the new variant form of CJD, which is effectively mad cow disease transferred to humans through infected beef, was detected in tonsils. The "classical" form of CJD, which appears for no known reason in one person in a million, was not seen in the tissue samples

CJD can be transmitted by surgery

By Steve Connor, Science Editor
Independent ... Thursday 14 January 1999
Scientists have found new evidence to suggest that the human version of "mad cow" disease might be transmitted during surgery via contaminated surgical instruments .

A test for the new variant form of Creutzfeldt-Jakob disease (nvCJD) shows the infectious agent responsible for the brain disorder is present in tonsils and certain other tissues handled in thousands of routine operations. Experts fear the nvCJD agent could survive the sterilisation procedures for surgical instruments and bepassed from person to person during hospital treatment.

Government experts are drawing up a set of new guidelines on the use of disposable scalpels, forceps and other surgical instruments to limit the risks to patients. An anonymous mass screening programme is also planned for later this year to detect people incubating nvCJD .

Thousands of tonsils are to be tested at random over the next few years to try to determine how many in the general population are infected with the human form of bovine spongiform encephalopathy (BSE). At present there are 35 confirmed cases of nvCJD, which scientists believe developed as a result of people eating beef contaminated with BSE, but there are fears that this might be the first signs of an epidemic . Attempts to estimate the future course of the disease have been hampered by a lack of knowledge and a suitable test for early diagnosis.

Professor John Collinge, a consultant neurologist at the Imperial College School of Medicine at St Mary's hospital in London, said yesterday he has developed a tonsil test for nvCJD which will be used in the mass screening programme to diagnose the infectious agent, believed to be a rogue "prion" protein. Previously, doctors could only reliably confirm nvCJD with an examination of the brain after death. "[The prion] is present in every tissue of every case of nvCJD [we studied] and it's present at quite considerable levels," Professor Collinge said. "This has implications for the risk of the infection passing from one person to anothers. We don't know yet the level of infectiousness in those tissues."

This raised the prospect of using disposable instruments wherever possible because "there is no means of sterilising surgical instruments adequately against prions ".

Research funded by the Medical Research Council and the Wellcome Trust, published in The Lancet, shows that although the tonsil test could detect the infectious prion protein in nvCJD cases, this was not the case for "classical" CJD. Professor Collinge said this shows how the rogue prion protein behaves quite differently to CJD.

Professor Liam Donaldson, the Chief Medical Officer, said last night: "Current policy based on advice from the Advisory Committee on Dangerous Pathogens published in April 1998 is that when any patient with symptoms of nvCJD, or suspected of having nvCJD, undergoes surgical operation, the instruments must be removed so that they cannot be used again."

Further details from Lancet tonsil article

17 Jan 99 highlights of Lancet article
The Lancet article says all 9 were met/met at codon 129 of the prion gene. 5 are still alive. Ages at onset were 18, 21, 21, 22, 24, 28, 29, 34, 35 which average to 25.8. Three cases were in the DoH statistics at the time the article was written [34 cases] and 1 has been added since making for 5 new cases. 9 out of 20 patients with suspected prion disease had nvCJD; the 20 could potentially be 20 referals to the CJD Unit or alternately 20 referals that had passed further screening [but see below].

The 4 who died had clinical duration averaging 15 months, whereas the 5 further cases are averaging 14 months, suggesting that most of the 5 new cases would have appeared at DoH in the next few months using the previous surveillance method.

Two patients, with no family history of neurodegenerative disease on referral for biopsy sampling, were confirmed as E200K M129M and A117V M129V.

A first case was diagnosed as sporadic CJD was confirmed on neuropathological examination, and the second was diagnosed as sporadic CJD with a typical electro-encephalogram, but necropsy was not done.

Of the seven living patients, five have made a part or complete recovery, inconsistent with a diagnosis of prion disease.

A M129V patient has been affected for over 33 months and is thought to have dementia with Lewy bodies.

The second patient, V129V, who presented with a most unusual clinical picture of very young onset progressive aphasia, has continued to deteriorate--no diagnosis having been reached. [This case is potentially the first case of nvCJD in V129V]

They "were unable to detect PrPSc in lymphoreticular tissues from patients with iatrogenic CJD. It is possible that the oral route of exposure, presumed to be the route of BSE infection of patients with variant CJD, results in a more pronounced lymphoreticular phase. In this regard, it will be of interest to study lymphoreticular tissues from patients with kuru."

"These findings suggest that human tonsil biopsy samples may allow presymptomatic diagnosis of variant CJD. Indeed, it is possible, assuming sufficiently sensitive tests were available, that PrPSc will be detectable in human tonsil within months of exposure to BSE.... It is also possible that the current patients affected by variant CJD will prove to be atypical in the degree of their lymphoreticular involvement. There is, at present, no epidemiological evidence to suggest an unusual dietary or occupational exposure to BSE amongst the patients currently recognised as having variant CJD, and therefore it is possible that they represent a particularly susceptible subgroup with unusual lymphoreticular sensitivity to BSE prions resulting in short incubation periods. "

Lancet Volume 353, Number 9148  16 January 1999
A F Hill, R J Butterworth, S Joiner, G Jackson, M N Rossor, D J Thomas, A Frosh, N Tolley, J E Bell, M
Spencer, A King, S Al-Sarraj, J W Ironside, P L Lantos, J Collinge


Lymphoreticular tissues (68 tonsils, 64 spleens, and 40 lymph nodes) were obtained at necropsy from patients affected by prion disease and from neurological and normal controls. Tonsil biopsy sampling was done on 20 patients with suspected prion disease. Tissues were analysed by western blot to detect and type PrPSc, by PrP immunohistochemistry, or both.


: All lymphoreticular tissues obtained at necropsy from patients with neuropathologically confirmed variant CJD, but not from patients with other prion diseases or controls, were positive for PrPSc. In addition, PrPSc typing revealed a consistent pattern (designated type 4t) different from that seen in variant CJD brain (type 4) or in brain from other CJD subtypes (types 1-3). Tonsil biopsy tissue was positive in all eight patients with an adequate biopsy sample and whose subsequent course has confirmed, or is highly consistent with, a diagnosis of variant CJD and negative in all patients subsequently confirmed to have other diagnoses.

US blood safety science-based?

18 Jan 99 webmaster opinion
If you were wondering, like me, why this sudden burst of US journal articles asserting blood from a donor with CJD is safe, which amount to equating absence of evidence as evidence of absence, the answer is simple, in my opinion. There have been huge CJD losses for some of the blood industry stakeholders including the American Red Cross.

It was decided in the spring of 1998 to reverse the policy of the last five years 180 degrees (which relied on exactly the same meagre data but followed the precautionary principle) and put the CJD blood back on the shelves without a warning label or obtaining informed consent from recipients. But this required a few months delay until articles commissioned to back up this policy decision could be placed in reputable journals. For added credibility, nvCJD donors were excluded (affordable because none were expected).

Just when all the ducks were in a row, the Utah case most inconveniently broke. Some of the players lost their nerve, others were rattled. The enormity of the risk being advised briefly penetrated their consciousness. A drug company affected by the Utah donor broke ranks with the CDC and issued a _withdrawal_ of antihemophilic Factor (Human) and Factor IX Complex on 15 Dec 98. (I have the fax. It says 'withdrawal' in several places. The word 'hold' does not appear.)

But they finally got the ducks back in a row. A press story went around on 14 Jan 98 saying there had been no withdrawal of Utah blood. Instead it had been a hold. Health Canada buckled on 24 Dec 98 and reversed a 5 year old policy without significant new data. Blood products containing McEwen-donated plasma are again being offered for sale by injection, this time with full knowledge and blessing of the regulatory agencies.

But, in my opinion, without adequate labelling of product, without informed consent from the recipients, without a monitoring program. And, because of the spate and spin of articles they had the foresight to create, perhaps without liability for participants should there be a replay of hepatitis C and AIDS ("we used the best available science at the time").

The problem with null results in testing blood is the sensitivity of the test. Can it detect the minimum lethal infectious unit that suffices to cause CJD over a 70 year life span? In reality, no one has the slightest idea of how small a dose might be infectious over such a time period. What good does it do not to detect a nanogram of rogue prion if a femtogram is infectious?

While there are many genuine medical emergencies and ongoing problems that would surely cause present harm if the blood supply was inadequate, there are also a great many elective medications and surgeries and ones that could be put off. In my opinion, this latter class of recipient is unwittingly participating in a potentially lethal large-scale experiment.

Better to label and obtain informed consent before injecting products known to contain material derived from any and all CJD donors. People then know that there is a non-zero risk, choose to accept it (or not), and this risk factor can be put in their medical record. Should they later be corneal transplant donors etc., it is on the record. -- webmaster

Blood: An Epic History of Medicine and Commerce

by Douglas P. Starr Hardcover - 448 pages 1 Ed edition (September 1998) Knopf; ISBN: 067941875X ; $19.25

pg 350.

"No organization suffered under the new economic order [AIDS testing increased costs by $25-35 per unit] as much as the American Red Cross. After decades of wealth and glory, the agency crashed, both in its balance sheet and in public opinion. Having failed to live up to its 1988 agreement with the FDA, the agency was slapped with a court-ordered decree, under which it must make a series of improvements under a rigigly enforced scedule. The Red Crosss had hired a new and famous president -- former Labor Secretary Elizabeth Dole - to reverse its sagging fortunes. Shortly after taking office, she announced a "transformation" campaign to cut personnel, dencentlize laboratorees, and revamp the computer system, a seven-year program that cost $287 million.

And at the same time that the agency's finances were failing, it faced an unexpected health crisis. In 1994, a longtime Red Cross donor died of a rare malady known as CJD. This poorly understood affliction, also known as mad-cow disease, can linger for decades in the victim before creating spongelike holes in the brain, ultimately leading to dementia and death. (A veritable panic erupted in Europe when British beef was named as a possible disease vector.) No transufsion-related cases of CJD had ever been documented, but in the lingering wake of the AIDS epidemic, the Red Cross quarantined all the plasma pools to which the man had contributed. When, after months of deliberation, the FDA ordered the material's destruction, it cost the Red cross several million dollars. Subsequent withdrawals have cost the agency a total of $130 million.

Those losses caused a hemorrhage in the agency's biomedical services division, which lost $113 million in 1995 alone....." [pg 354] In America, dozens of recalls and withdrawals have occurred, resulting in industry-wide losses of hundreds of millions of dollars. The massive withholding of suspected CJD-tainted material contributed to national shortages of immune globulin, vital to tens of thousands of people. The shortage caused a problem of such magnitude that Congressman Christopher Shays held hearings in the spring of 1998 to determine if the industry was hoarding the material or shipping too much overseas. Multiple causes actually contibuted -- inceasing demand, factory shutdowns while companies retooled to meet necessary health standards, foreign market demands, and the need to to avoid even the theoretical risk of spreading disease. In short, the crisis arose from the complexities of marketing blood products in a post-AIDS society."

FDA rules allowed fatal blood trials without consent, officials say

January 17, 1999  Associated Press 
CHICAGO - A company conducted an ill-fated blood substitute trial without the informed consent of patients in the study - some of whom died, federal officials say. Baxter International Inc. was able to test the substitute known as HemAssist without consent because of a 1996 change in federal FDA regulations. The changes broke a 50-year standard to get consent for nearly all experiments on humans. They were designed to help research in emergency medicine that could not happen if doctors took the time to get consent.

But the problems with the HemAssist trial are prompting some medical ethicists to question the rule change. "People get involved in something to their detriment without any knowledge of it," George Annas, a professor of health law at the Boston University School of Public Health, told the Chicago Tribune. "We use people. What's the justification for that?" No other company has conducted a no-consent experiment under the rule, FDA officials said.

Baxter officials halted their clinical trial of HemAssist last spring after reviewing data on the first 100 trauma patients enrolled in the nationwide study. Of the 52 critically ill patients given the substitute, 24 died, representing a 46.2 percent mortality rate. The Deerfield, Ill.-based company had projected 42.6 percent mortality for critically ill patients seeking emergency treatment. There has been an intense push to find a blood substitute because artificial blood could ease the effects of whole-blood shortages. Researchers say it lasts longer than conventional blood, eliminates the time-consuming need to match blood types and wipes out the risk of contamination with such viruses as HIV and hepatitis.

The principle of informed consent was first articulated following the revelation that Nazi doctors performed hideous experiments during World War II. It became even more important because of U.S. -backed syphilis experiments on blacks in the South that went on for decades after a cure for the disease were revealed.

The 1996 regulations require a level of community notification that is not used in most scientific studies, including community meetings, news releases and post-study follow-up. Some say such notification cannot replace direct consent from patients or their relatives.

"Public notification means nothing," said Dr. Arthur Caplan, director of the Center for Bioethics at the University of Pennsylvania. "I know people are enamored of it, but it means nothing." No lawsuits have arisen from the blood substitute trial, Baxter officials said. "The regulations worked in this instance," company spokeswoman Mary Thomas said Sunday. "We voluntarily stopped the trial early on at the first sign of unexpected results to insure patient safety."

Canada to sell McEwen blood

THE LANCET, Volume 353, Number 9147 9 January 1999 Wayne Kondro
[This is a terribly cynical press release -- full of inaccuracies and misinformation from start to finish. Canada has learned nothing from its AIDS and hepatitis fiascos. --webmaster]

Saying that there is no scientific evidence that "classic" Creutzfeldt-Jakob disease is bloodborne, the Canadian government has lifted a 4-year policy obligating the quarantine of blood products made from plasma donated by people with CJD.

After ordering a mid-December quarantine of thousands of vials of fractionated blood products made from plasma donated by a Utah man who was thought to have had variant CJD, Health Canada's Bureau of Biologics late last month freed Canada's blood agencies to use albumin, immunoglobulins, and factor VIII made from the man's plasma, after it was determined the 29-year-old had had classic CJD. [This had not been determined as of 20 Jan 99 --webmaster] He had donated blood over 100 times in the past 2 years, including some after he had begun to show signs of CJD.

"In this case, we are absolutely sure that this is a case of classical CJD [work has not even started on ruling out familial CJD and cwdCJD remains very much a possibility], and the expert advice that we have received in the past several months is that there is no evidence whatsoever that there has been any transmission or is any transmission of classical CJD through blood or blood products", Bureau of Biologics director Keith Bailey told reporters. "Our assurance would be that to the best of our knowledge, there isn't a problem with these products", Bailey said. [There is little data one way or the other on transmission by blood -- webmaster.]

While acknowledging that there is still a theoretical possibility that classic CJD could be bloodborne, Health Canada officials said the preponderance of the evidence suggests it is not, so there is little rationale for maintaining the bureau's quarantine policy, which was introduced in 1995. [So why was the ban installed in the first place? Why was McEwen's blood quarantined back in December? What new studies on blood studies have been published ?-- webmaster]

The new policy mirrors that of the USA and only obligates the withdrawal of plasma from donors with variant CJD.

How unusual is CJD onset at age 29?

CDC data on age distribution of CJD

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