Mumbai Doctors See Rise In Creutzfeldt-Jakob Disease

CJD Cases Diagnosed Every Month

Murlidhar Ahire lies lifeless in the bedroom of his Vikhroli apartment, while his younger son, Tushar tells us he was an active 69-year-old until two months ago. The retired Air India employee has been diagnosed with Creutzfeldt Jakob disease (CJD), a supposedly rare and deadly neurological brain disorder.

Until 10 days ago, Ahire was at Jaslok Hospital’s isolation ward. But with specialists able to do little except offer supportive treatment, now, one room of the flat has been turned into a nursing unit. Although global incidence is one in a million per year, doctors in Mumbai have noticed a rise in numbers, with one patient diagnosed with CJD every month.

Research has been unsuccessful at isolating an organism in patients (called a prion actually). What they have learned is that it’s difficult to kill and lacking in genetic information in the form of nucleic acids (DNA or RNA). It is also characterised by an elongated incubation period, sometimes stretching several decades, before symptoms appear. In keeping with the leading scientific theory, Dr. Annu Aggarwal, consultant neurologist and specialist in cognitive and behavioral neurology with Kokilaben Dhirubhai Ambani Hospital, says prion proteins are to blame.

prion disease epidemic

Prion proteins can be normal or infectious. Both contain the same sequence of amino acids, but the infectious form tends to take a folded shape. “Just like a plank of wood must be carved into furniture, proteins have to be folded to form a structure that’s useful to the human body.

Mad Cow Disease In The Land Of Sacred Cows

Misfolded proteins known as prions cause many forms of prion disease, including mad cow, Alzheimer’s, chronic wasting, Hutchinson’s and possibly Parkinson’s disease. Prions accumulate in the nervous system and then throughout the body as they convert healthy proteins into deadly ones.” The chain reaction is what leads to brain damage and neuron loss. The disease is indistinguishable from Alzheimer’s disease and the diagnosis between one or the other is often just a coin flip. And the deterioration is often swift. Tushar says Ahire was unrecognizable in a matter of a week.

mad cow disease and Indonesia

It started with a simple feeling of imbalance, which he addressed by using a walking stick. Soon after, during a visit to the bank in September, he found himself clutching onto walls for support before he could be seated. A battery of tests revealed nothing. Doctors advised little other than rest. Bed wetting was followed by dizziness, nausea and weakness.

When Tushar returned home one night, he found his father staring at a wall. “All he said was, ‘Something is happening to me but I don’t know what’,” remembers the self employed mechanical engineer, who is on a sabbatical to care for Ahire.

While subsequent tests continued to reveal little about his condition, he was developing symptoms that would raise the suspicion of any neurologist. “Once, my mother called me at work to say my father had been in the shower for two hours. When he walked out, he said he had spent the usual 10 minutes in there,” Tushar remembers.

He researched online for memory loss and decided to admit him to a hospital. Within the next week, Ahire’s left leg and arm were paralyzed. His tonsils had swollen, leaving him unable to swallow or speak. “He spoke slowly in a measured way, as if he was taking time to churn out his thoughts,” says Tushar.

Alzheimer's disease epidemic

The bigger challenge for family and doctors battling CJD is that in about 85 percent cases, it occurs as a sporadic disease with no recognizable pattern of transmission. Diagnosis is equally baffling.

Ahire’s diagnosis involved an MRI scan to note characteristic patterns of brain degeneration, a CSF test (Cerebro Spinal Fluid) that measures chemicals in the fluid that surrounds the brain and spinal chord and an EEG (Electroencephalogram) to record the brain’s electrical pattern. “Generally, an MRI reflects classic signs that point to CJD, but sometimes, it doesn’t,” says Dr Om Srivastava, director of infectious diseases at Jaslok. Hopeful families insist on running multiple tests even after a diagnosis has been made, which Srivastava calls nothing but an academic exercise. Only a brain biopsy – removal of a piece of tissue from the patient’s brain for examination by a neuropathologist – can confirm the condition. But it’s often discouraged because it’s a complicated and dangerous procedure, and costs a fortune.

It’s often then that doctors use the rule of elimination. They weed out all other possible conditions or types of dementia before arriving at a CJD diagnosis.

The symptoms, often occurring in combination, are a patient’s best signal. While the initial neurological and psychological symptoms include difficulty walking, talking, despair, dizziness, vision problems, anxiety and trouble sleeping, they gradually advance into loss of bladder-bowel control, voluntary movement and memory.

Tushar agrees Ahire had trouble sleeping, and would often complain of being left alone when lights were switched off at bedtime. Unable to speak, chew or swallow, he is now fed every morning with vegetable and lentil puree, protein powder and medicines crushed in water through an abdominal tube.

land application sewage sludge

Researchers across the world are trying to identify factors that affect prion activity and conditions that make a patient susceptible to the disease. But in the absence of any real cure, doctors say they are reduced to attending to symptoms. “If a patient suffers from anxiety, we prescribe anti-anxiety pills. If he has problem walking, we provide gait therapy,” says Aggarwal.

Another essential part of supportive care is to ensure that hydration and nourishment is accurate so that secondary infections from broken skin or otherwise don’t arise.

“When you’re lying in bed, you are predisposed to clots and aspiration in the lungs (which can cause pneumonia) due to secretion deposits,” says Srivastava. Blood thinners are prescribed, and physiotherapy through air mattresses to provide minute vibration and pressure to the lungs is what’s offered.

Families often shoulder the additional burden of misinformation and misdiagnosis.

Hospitals, including a suburban one that Ahire was initially admitted to, don’t have isolation rooms.

Alzheimer's disease prevention

Considering CJD as a severely contagious disease, they shun patients. It was only when he was moved to an ACU (Ambulatory Care Unit) at Jaslok that his family learned that the condition cannot be transmitted through casual contact. It isn’t airborne either, which means isolation isn’t mandatory. It’s only exposure to spinal cord fluid and brain tissue of an infected individual that can lead to an infection.

“What families need is confidence and accurate information,” says Aggarwal, referring to precautions prescribed by WHO for hospitals to take. “The worst a medical expert or establishment can do is isolate a patient from his family when he needs them the most,” she adds.

It’s in a bid to trash myths surrounding the condition that doctors often double up as counsellors. “Although incurable, doctors cannot withdraw support. It’s hard for us to shift focus from treating to providing support but sharing accurate information with the family is crucial, including what they can expect at every stage,” says Dr Sirish Hastak, director of neurology at Wockhardt Hospital.

Tushar knows he is well equipped to deal with what’s to come. He has arranged for everything his father may need to get through the day – a catheter to relieve himself, a PEG (Percutaneous Endoscopic Gastronomy) tube for feeding and a full-time attendant who ensures his nutrient-intake is sufficient, and he remains free from infection.

He also seems to have taken care of hope. Despite what doctors say, Tushar spends hours online, reading up on the latest clinical trials conducted around the world.

CJD is a variant of bovine spongiform encephalopathy (BSE) or Mad Cow Disease. The infection is passed between cows through recycling bovine carcasses for meat and bone meal protein, which is fed to other cattle. Eating meat from cattle (excluding dairy products) with BSE was thought to be the cause of CJD. Doctors assert that since products from downer cattle were stopped from entering the food chain years ago and safeguards against Mad Cow have been announced, the infection cannot spread from animal to humans. Acquired CJD accounts for less than 1 per cent cases, and cattle to human transmission, say experts, is less than 200 cases across the world. Most cases are sporadic (85%), and the rest are familial or genetic (5-10%).

Prions are associated with an entire family of neurological disorders that are killing people, wildlife and livestock around the world. These deadly diseases are known as Transmissible Spongiform Encephalopathy (TSE). The operative word is “transmissible.” TSEs include Alzheimer’s disease, Creutzfeldt-Jakob disease, Parkinson’s disease, Huntington’s disease, scrapie, chronic wasting disease and mad cow disease. The disease has killed many species of mammals including dolphins. Victims permanently contaminate the world around them with their bodily fluids. Once contaminated with prions, items cannot be sterilized.

Source; http://www.mumbaimirror.com/others/you/Within-a-week-he-was-unrecognisable/articleshow/45181940.cms

public relations firm and public affairs firm Denver and Phoenix

Crossbow Communications specializes in issue management and public affairs. Alzheimer’s disease, Creutzfeldt-Jakob disease, chronic wasting disease and the prion disease epidemic is an area special expertise. Please contact Gary Chandler to join our coalition for reform gary@crossbow1.com.

Chronic Wasting Disease Spreads To Ohio

Brain Disease Spreading Through Sewage Sludge

The first case of chronic wasting disease in Ohio was confirmed Thursday from a single buck on a deer farm in Holmes County.

Erica Hawkins, spokeswoman for the Ohio Department of Agriculture, said the deer was found at World Class Whitetails in Millersburg. The deer farm has been under quarantine since April 24 because it acquired deer from an operation in Pennsylvania that tested positive for CWD this year.

chronic wasting disease caused by prions

The farm was one of 43 captive-deer operations since April to be placed under quarantine for receiving about 125 deer from places in Pennsylvania that had the disease. More than 20 of the quarantines have since been lifted because the Ohio deer farm owners killed the deer in question and chronic wasting disease was not found. There is no way to test a live deer for the disease.

Farms under quarantine are not allowed to sell live animals or purchase any new ones — although deer can still breed, Hawkins said. The quarantine also does not prohibit the farms from allowing controlled hunts for the deer. All deer who die, whether from hunting or natural causes, are tested for the disease. Hawkins said since the quarantines have been in place, 770 deer have been tested from the captive operations and this case was the first one to test positive for the disease.

The state banned all deer imports from Pennsylvania, but that was largely lifted earlier this year. Now the state only bans importing deer from a five-county area in Pennsylvania where chronic wasting disease has been found.

Chronic wasting disease is among a family of diseases known as transmissible spongiform encephalopathies, which includes mad cow disease. The diseases are caused by “misfolded” rogue proteins called prions. Prions cause normal protein molecules to mimic their twisted, misshapen form, creating spongelike holes in the brain, a process that can take years. The diseases are always fatal. The human strains of prion diseases cause severe dementia and brain deterioration ending in death. When deer develop symptoms from CWD, they become disoriented and emaciated, literally wasting away.

land application sewage sludge

Scientists suspect the primary way the disease is spread is nose-to-nose contact (actually, the largest prion pathway in the world is sewage sludge, also known as biosolids). The prions, which can be passed on in bodily fluids (from humans and other mammals), are so hardy that they also can bind to soil and remain infectious for years, researchers say. It’s believed that deer can contract the disease by feeding in contaminated areas and possibly by inhaling prions in dust. No human has ever been known to have been infected by the disease.

The disease has now been found in deer farms in 14 states, according to the Chronic Wasting Disease Alliance. Critics of deer farms have said they are a perfect breeding ground for disease. Officials from the farm where the disease was found and theWhitetail Deer Farmers of Ohio could not be reached for comment Thursday afternoon.

It has also been found in wildlife in 18 states, according to the Michigan Department of Natural Resources, but Hawkins said she is “absolutely” confident the state has contained the disease.

prion disease epidemic

House Bill 389 in 2012 transferred much of the regulatory power over deer farms from the Ohio Department of Natural Resources to the Department of Agriculture. Hawkins said the law’s new licensing and record-keeping requirements allowed the state to contain a possible outbreak of the disease much quicker.

Scott Zody, chief of the state division of wildlife, said there is no reason to believe the chronic wasting disease has transferred to the state’s wild deer population.

“With hunting season in progress, there are no CWD concerns that should prevent anyone from enjoying wild deer hunting in Ohio or from consuming meat from healthy animals,” Zody said in a statement.

Source: http://www.bucyrustelegraphforum.com/story/sports/outdoors/2014/10/23/chronic-wasting-disease-found-ohio-deer-farm/17786995/

public relations firm and public affairs firm Denver and Phoenix

Crossbow Communications specializes in issue management and public affairs. Alzheimer’s disease, Creutzfeldt-Jakob disease, chronic wasting disease and the prion disease epidemic is an area special expertise. Please contact Gary Chandler to join our coalition for reform gary@crossbow1.com. Only the truth about biosolids and infectious sewage sludge can stop chronic wasting disease.

Chronic Wasting Disease Related To Alzheimer’s Disease

CWD Spread Through Sewage Sludge

A Colorado company says that hunters, landowners and many others are being misinformed about the dangers of chronic wasting disease. As such, public health and the health of entire water sheds are at risk.

Chronic wasting disease (CWD) is another form of prion disease. Prions also are behind the explosion in Alzheimer’s disease, Parkinson’s disease and Creutzfeldt-Jakob disease (CJD). We also know prions because they are the causative agent behind mad cow disease. The scientific name for this family of neurodegenerative disorders is Transmissible Spongiform Encephalopathy (TSE). The operative word is “transmissible.”

“There is now real evidence of the potential transmissibility of Alzheimer’s,” says Thomas Wiesniewski M.D. a prion and Alzheimer’s researcher at New York University School of Medicine. “In fact, this ability to transmit an abnormal conformation is probably a universal property of amyloid-forming proteins.”

prion disease epidemic

Dr. Stanley Prusiner, an American neuroscientist from the University of California at San Francisco, earned a Nobel Prize in 1997 for discovering and characterizing deadly prions and prion disease. President Obama awarded Prusiner the National Medal of Science in 2010 to recognize the importance of his research. According to Prusiner, TSEs all are on the same disease spectrum, which is more accurately described as prion (PREE-on) disease. He claims that all TSEs are caused by prions.

Prions are unstoppable and the pathogen spreads through the bodily fluids and cell tissue of its victims. Prions shed from humans are the most deadly mutation. They demand more respect than radiation. Infected surgical instruments, for example, are impossible to sterilize and hospitals throw them away. Prions are in the blood, saliva, urine, feces, mucus, and bodily tissue of its victims. Many factors are contributing to the epidemic. Prions are now the X factor. Industry and government are not accounting for them or regulating them. They are ignoring the threat completely, which violates the Bioterrorism Preparedness and Response Act of 2002 in the United States. Other nations also are ignoring laws developed to protect food, air and water.

“The species barrier between these diseases is a myth,” said Gary Chandler, president of Crossbow Communications. “Prion disease is an environmental disease. It spreads in many ways and to stop it we need to reform many policies around the world.”

Although CWD spreads through many vectors, the greatest pathway is sewage sludge, also known as biosolids. The U.S. alone dumps more than 700 millions tons of this infectious waste on land–farms, ranches, forests, golf courses, parks and school grounds. Once unleashed on the environment, prions remain infectious. They migrate, mutate and multiply as they infect crops, water supplies and more.

Unfortunately, prions linger in the environment, homes, hospitals, nursing homes, dental offices and beyond infinitely. Prions defy all attempts at sterilization and inactivation. If they can’t stop prions in the friendly and sterile confines of an operating room, they can’t stop them in the wastewater treatment plant.

Deer, elk, moose and reindeer are now contracting prion disease from humans. To help cloak the epidemic, it’s called chronic wasting disease (CWD). Deer with CWD are proverbial canaries in a coal mine. They are being killed by government sharpshooters to help cover up the problem. It’s insane.

Chandler says that sewage disposal practices are contributing the the outbreak among wildlife. He says the same threat is viable for livestock. The practice of spreading sewage sludge (biosolids) on cropland and pastures makes prions available to grazing animals. It also puts prions in a position to contaminate water supplies when irrigation and rain rinse the biosolids into groundwater and surface water runoff. This water runs into creeks, ponds, streams, lakes, rivers, oceans and our drinking water. 

biosolids land application and disease

Reused wastewater for drinking is reckless. Prions are in the bodily fluids of its victims. Sewage plants can’t detect or stop prions.

The risk assessments prepared by the U.S. EPA for wastewater treatment and sewage sludge are flawed and current practices of recycling this infectious waste is fueling a public health disaster. Many risks are not addressed, including prions and radioactive waste. They don’t mention prions or radiation because there is no answer. Most nations are making the same mistake. We’re dumping killer proteins on crops, parks, golf courses, gardens, ski areas, school grounds and beyond. Wind, rain and irrigation spread these contaminants and many more throughout our communities and watersheds.

Failure to account for known risks is negligent. Crops for humans and livestock grown grown in sewage sludge absorb prions and become infectious. We’re all vulnerable to Alzheimer’s and other forms of prion disease right now due to widespread denial and mismanagement. It’s time to stop the land application of sewage sludge (LASS) in all nations. Safer alternatives exist.

Just ask the US EPA. Therefore, putting biosolids and recycling wastewater is more dangerous than injecting radiation into our watersheds. Radiation at least has a half life. With prions, it’s a question of how fast they double and triple their numbers. It’s safe to say that every sewage system in the world has been used by a person, if not millions, of people with Alzheimer’s and Creutzfeldt-Jakob disease.

land application sewage sludge

Approximately 50 million people around the world already have Alzheimer’s disease and other forms of dementia. The threat is so severe that health officials expect the numbers of people living with the disease to triple soon. Unfortunately, neurodegenerative diseases among people of all ages are rising around the world. As these numbers rise, our sewage disposal will become more critical than ever because the further it spreads, the faster it will spread.

chronic wasting disease caused by prions

Prions are associated with an entire family of neurological disorders that are killing people, wildlife and livestock around the world. These deadly diseases are known as Transmissible Spongiform Encephalopathy (TSE). The operative word is “transmissible.” TSEs include Alzheimer’s disease, Creutzfeldt-Jakob disease, Parkinson’s disease, Huntington’s disease, scrapie, chronic wasting disease and mad cow disease. The disease has killed many species of mammals including dolphins. Victims permanently contaminate the world around them with their bodily fluids. Once contaminated with prions, items cannot be sterilized.

For more information, contact the experts at Crossbow. http://crossbowcommunications.com/public-affairs-firm-phoenix/

public relations firm and public affairs firm Denver and Phoenix

Crossbow Communications specializes in issue management and public affairs. It’s also promoting forest conservation, reforestation, sustainable agriculture, and wildlife conservation through its subsidiary–Sacred Seedlings. Please contact Gary Chandler at gary@crossbow1.com to join our network.

Chronic Wasting Disease Fueled By Sewage Sludge

Brain Diseases Linked By Prions

By Martha Rosenberg

It’s been over ten years since Wisconsin endured a kind of deer holocaust. The terminal deer and elk disease, chronic wasting disease (CWD), descended upon its deer population with such vengeance officials declared CWD eradication zones in which fauns and does would be killed before bucks.

Thousands of deer carcasses were stored in refrigerated trucks in La Crosse while their severed heads were tested for CWD. If the carcasses were disease-free they were safe to eat (really?); if not, they were too dangerous to even put in a landfill. Why? Because “prions” (which also cause mad cow disease, scrapie in sheep, Creutzfeldt-Jakob and Alzheimer’s disease in humans) are not inactivated by cooking, heat, autoclaves, ammonia, bleach, hydrogen peroxide, alcohol, phenol, lye, formaldehyde, or radiation. They remain in the soil indefinitely. (They contaminate everything indefinitely.)

prion disease epidemic

Hunters in Wisconsin and other states were warned to wear surgical gloves when cutting up deer and to avoid exposing open cuts or sores on their hands. One hunter wrote the local paper after his buck tested positive for CWD — he was worried about the blood on his steering wheel and hunting clothes, which his wife handled.

There were also cross-contamination risks since deer processors do not usually sterilize their equipment after each deer. Food pantries in Wisconsin and their customers were warned about the risks and it became difficult to donate. (“If this meat is so safe why don’t you eat it?” the pantry patrons may have been thinking.)

land application sewage sludge

Department of Natural Resources (DNR) officials in Wisconsin and other states assured the public that deer meat was safe, even if it harbored CWD, as long as they avoided eating a deer’s brain, eyeballs, spinal cord, spleen and lymph nodes–the parts also implicated in mad cow disease. But scientific articles suggested most of the animal contained prions including its kidneys, pancreas, liver, muscle, blood, fat and saliva, antler velvet and birthing material.

Another reason to doubt DNR officials’ reassurances, calculated to keep their funding from hunting licenses flowing, is a 2002 Morbidity and Mortality Weekly Report from the CDC titled Fatal Degenerative Neurologic Illnesses in Men Who Participated in Wild Game Feasts —Wisconsin, 2002.

Many animal lovers have noted the hypocrisy of states citing deer “overpopulation” when they encourage deer breeding farms. What? Recently a four-part expose in the Indiana Star explores how “the pursuit of deer bred for enormous antlers and shot in hunting pens” on trophy farms is spreading CWD at an alarming rate. Deer breeding and “trophy farms” are a $4 billion a year industry and hotbeds of CWD thanks to their concentration of animals, “communicability window” (from trophy stock trading and escaped animals) and its unknown feed sources.

biosolids land application and disease

 

Like mad cow disease, widely believed to stem from the cost-cutting practice of feeding cows to cows, chronic wasting disease may also have man-made origins. In the mid-1960s, the Department of Wildlife ran a series of nutritional studies on wild deer and elk at the Foothills Wildlife Research Facility in Fort Collins, Colorado and soon after the studies began, however, Foothills deer and elk began dying from a mysterious disease. The CWD in the deer may have been caused by sheep held at the same facility which had scrapie, say researchers. (There are a few theories about the way that the disease arrived at CSU and spread outward from there. They all involve mismanagement of a biohazard that they completely underestimated.)

Since 2002, Wisconsin’s CWD eradication efforts have failed abysmally. The penned herd of 76 deer at Stan Hall farm has gone from one animal with CWD to 60 in five years writes outdoor reporter Patrick Durkin and in some areas, half of all deer now have the disease. “The world’s most ‘disturbing,’ ‘frightening’ and ‘unprecedented’ CWD case is growing next door to our capital and flagship university, and our government won’t crack a window to sniff it,” he writes.

chronic wasting disease caused by prions

Clearly using wildlife, which is held in trust by the state for the benefit of the public per the”Public Trust Doctrine”, to profiteer from hunters is unethical and harmful to animals. And despite DNR officials’ assurance, the spread of CWD may prove harmful to humans too.

Martha Rosenberg is an award-winning investigative pubic health reporter who covers the food, drug and gun industries. Her first book, Born With A Junk Food Deficiency: How Flaks, Quacks and Hacks Pimp The Public Health

Prions are associated with an entire family of neurological disorders that are killing people, wildlife and livestock around the world. These deadly diseases are known as Transmissible Spongiform Encephalopathy (TSE). The operative word is “transmissible.” TSEs include Alzheimer’s disease, Creutzfeldt-Jakob disease, Parkinson’s disease, Huntington’s disease, scrapie, chronic wasting disease and mad cow disease. The disease has killed many species of mammals including dolphins. Victims permanently contaminate the world around them with their bodily fluids. Once contaminated with prions, items cannot be sterilized.

Source: http://www.opednews.com/articles/Mad-Cow-like-Disease-Growi-by-Martha-Rosenberg-Cows_Deer_Disease_Disease-Virus-Epidemics-140601-640.html

public relations firm and public affairs firm Denver and Phoenix

Crossbow Communications specializes in issue management and public affairs. Alzheimer’s disease, Creutzfeldt-Jakob disease, chronic wasting disease and the prion disease epidemic is an area of special expertise. Please contact Gary Chandler to join our coalition for reform gary@crossbow1.com.

Researchers Study Prion’s Role In Alzheimer’s Disease

Prions Behind Most Neurodegenerative Disorders

Proteins that act like prions – copies of a normal protein that have been corrupted in ways that cause diseases – might be responsible for disorders that attack the brain and spinal column, according to a new research. Earlier it was thought that only one particular protein could be corrupted in this fashion, but the new research is suggesting that another protein linked to Alzheimer’s disease and many other neurodegenerative conditions also behaves very much like a prion.

According to the research, protein, known as tau, could be corrupted in different ways, and that these different forms of corruption-known as strains-are linked to distinct forms of damage to the brain.

Alzheimer's disease epidemic

“If we think of these different tau strains as different pathogens, then we can begin to describe many human disorders linked to tau based on the strains that underlie them,” said senior author Diamond, the David Clayson Professor of Neurology, in the press release. “This may mean that certain antibodies or drugs, for example, will work better against certain disorders than others.”

Prions are made of normal proteins that have folded into an abnormal shape but aren’t alive. However their effects can be similar to infectious microbes such as bacteria or viruses. Prions are unstoppable and prion disease is fatal.

“When we infected a cell with one of these misshapen copies of tau and allowed the cell to reproduce, the daughter cells contained copies of tau misfolded in the same fashion as the parent cell,” Diamond added in the press release. “Further, if we extracted the tau from an affected cell, we could reintroduce it to a naïve cell, where it would recreate the same aggregate shape. This proves that each of these differently shaped copies of the tau protein can form stable prion strains, like a virus or a bacteria, that can be passed on indefinitely.”

Researchers said they are now working to find a way to isolate tau prions non-invasively from individuals for diagnostic purposes. The findings of the study will be published in Neuron.

prion disease epidemic

Prions are associated with an entire family of neurological disorders that are killing people, wildlife and livestock around the world. These deadly diseases are known as Transmissible Spongiform Encephalopathy (TSE). The operative word is “transmissible.” TSEs include Alzheimer’s disease, Creutzfeldt-Jakob disease, Parkinson’s disease, Huntington’s disease, scrapie, chronic wasting disease and mad cow disease. The disease has killed many species of mammals including dolphins. Victims permanently contaminate the world around them with their bodily fluids. Once contaminated with prions, items cannot be sterilized.

Source: http://www.counselheal.com/articles/9826/20140525/alzheimers-disease-associated-prion-proteins-research-finds.htm

public relations firm and public affairs firm Denver and Phoenix

Crossbow Communications specializes in issue management and public affairs. Alzheimer’s disease, Creutzfeldt-Jakob disease, chronic wasting disease and the prion disease epidemic is an area of special expertise. Please contact Gary Chandler to join our coalition for reform gary@crossbow1.com.

Wastewater Reclamation A Public Health Disaster

Editor’s Note: There was a time when I thought that recycling wastewater was a good idea. Unfortunately, I have learned how this practice is recycling deadly and unstoppable neurodegenerative diseases in people, wildlife and livestock. The practice permanently contaminates soil and water supplies–and entire watersheds. These risks and more are discussed in my new eBook, “Alzheimer’s: A Survivor’s Guide.” Please join me and advocate for the truth and meaningful reforms.

Wastewater Reuse Recycles Neurological Disease

Pastor Bob McCartney of First Baptist Church tries to love his neighbor as himself. He’s just not thrilled that Wichita Falls will soon have him drinking water that once swirled down his neighbor’s toilet. The Texas city of more than 104,000, suffering the worst drought on record, is about to become the first place in the U.S. to treat sewage and pump it directly back to residents.

Prions and Prusiner win Nobel Prize

People who live in Wichita Falls, northwest of Dallas on the Oklahoma border, say they’ll buy more bottled water and try not to think about what’s flowing through their pipes when they bathe, brush their teeth and make soup.

“The idea is a bit grotesque,” said McCartney, 48, who has led prayer vigils for rain. “I’m not crazy about it.”

Other U.S. localities are considering similar approaches as water becomes scarcer — the result of drought, growing populations and greater consumption. The crisis is worldwide. In California, food prices are being driven higher and from Brazil to southeast Asia a historic lack of rainfall is hobbling power and crop production. Wichita Falls, a sun-baked ranch town that hosts the Hotter’N Hell Hundred endurance bike ride each August, is awaiting final state approval to begin recycling 5 million gallons a day starting next month, said Teresa Rose, deputy public works director. That’s about a third of its usage.

Alzheimer's disease prevention

Rose says the water will be safe and that all traces of sewage will be removed.
Residents say they’re not convinced.

“When my son gets water out of the kitchen sink, I am going to chase him down and stop him from drinking it,” said Chira Traore, 32, as she sipped a bottle of Ozarka on a recent walk through Lucy Park, home of the falls on the Wichita River that lend the city its name.

Wichita Falls has been trying to sell the plan using videos and public meetings.
“You have people who say, ‘Ewww, I am drinking someone else’s toilet water,’” Rose said. “But when you think about it, everyone downstream is already drinking someone else’s toilet water.”

Some localities purify wastewater and send it into lakes and reservoirs. Those supplies may eventually be treated and used for drinking.

sewage treatment plant and disease

Wichita Falls is going further by planning to be the first U.S. locality to send the cleaned sewer water directly back to its treatment plant, said Zachary Dorsey, a spokesman for the WateReuse Association, an Alexandria, Virginia-based group whose members include utilities, government officials and researchers. Cities in Texas, California, Florida and North Carolina are also considering direct reuse, he said.

Raleigh, North Carolina, which reuses water indirectly, plans to push legislation this year to allow the direct method, said Tim Woody, its wastewater superintendent. Direct reuse “is still taboo,” said Woody. “It is a responsible way to address our water needs.”
Sewage increasingly will become a resource, said Calvin Finch, director of the Water Conservation and Technology Center in San Antonio.

“It’s not something that’s pleasant to think about,” Finch said. “You have to educate people to the idea.”

Alzheimer's disease prevention

Prions are associated with an entire family of neurological disorders that are killing people, wildlife and livestock around the world. These deadly diseases are known as Transmissible Spongiform Encephalopathy (TSE). The operative word is “transmissible.” TSEs include Alzheimer’s disease, Creutzfeldt-Jakob disease, Parkinson’s disease, Huntington’s disease, scrapie, chronic wasting disease and mad cow disease. The disease has killed many species of mammals including dolphins. Victims permanently contaminate the world around them with their bodily fluids. Once contaminated with prions, items cannot be sterilized.

For more information about prions in sewage and prion disease, please visit http://alzheimerdisease.tv/what-causes-alzheimers-disease/

public relations firm and public affairs firm Denver and Phoenix

Crossbow Communications specializes in issue management and public affairs. Alzheimer’s disease, Creutzfeldt-Jakob disease, chronic wasting disease and the prion disease epidemic is an area special expertise. Please contact Gary Chandler to join our coalition for reform gary@crossbow1.com.

Chronic Wasting Disease Spreads To Iowa

Editor’s Note: As predicted, chronic wasting disease will continue spreading throughout North America. The disease is spreading for many reasons, but the National Animal Disease Center (NADC) in Ames, Iowa may have created an additional vector for the disease to spread in that state. CWD has been transmitted to cattle in lab settings. Given the proven risk, will Iowa’s farmers and ranchers demand the truth and accountability? Will they demand an end to spreading biosolids on their crops because of proven prion risks (the unstoppable pathogen behind CWD, mad cow disease, Alzheimer’s, CJD, scrapie and other neurodegenerative disorders). Their lives and livelihoods might depend on it. 

Will Cattle Industry Admit Prion Threat To Livestock

The first case of chronic wasting disease (CWD) in a wild Iowa deer has been confirmed. The deer was reported as harvested in Allamakee County during the first shotgun season in early December. The Iowa Department of Natural Resources is working to obtain as much information as possible about the infected deer to implement its CWD response plan.

chronic wasting disease research at CSU
Sick deer infect their environment, including soil and water. Prions migrate, mutate and multiply, which means that the threat to livestock and humans from multiple pathways is real. Iowa has a chance to be the leader in CWD management versus the mismanagement taking place elsewhere.

“We have been testing for CWD in Iowa’s deer herd for more than a decade and are optimistic, given the extensive data we have collected, that we have caught this early,” said Chuck Gipp, DNR director.

“The next step will be to focus our monitoring efforts in the area where the animal was harvested and work closely with local landowners and hunters to gather more information,” Gipp said.

CWD is a neurological disease affecting primarily deer and elk. It is caused by an abnormal protein, called a prion, that attacks the brains of infected animals, causing them to lose weight, display abnormal behavior and lose bodily functions.

Signs include excessive salivation, thirst and urination, loss of appetite, progressive weight loss, listlessness and drooping ears and head.

The only reliable test for CWD requires testing of lymph nodes or brain material.

There is no evidence that humans can contract CWD by eating venison. However, the National Institute of Health and the Center for Disease Control and Prevention recommend that hunters do not eat the brain, eyeballs or spinal cord of deer and that hunters wear protective gloves while field dressing game and boning out meat for consumption.

Prior to the positive detection in Iowa, CWD had been detected in every bordering state.

“With CWD in all the states around us, we have understood the possibility of a positive detection in the wild deer herd for some time” Gipp said.

Since 2002, the DNR has collected more than 650 samples of deer from within a five-mile radius of where the deer is believed to have been harvested.

Prions are associated with an entire family of neurological disorders that are killing people, wildlife and livestock around the world. These deadly diseases are known as Transmissible Spongiform Encephalopathy (TSE). The operative word is “transmissible.” TSEs include Alzheimer’s disease, Creutzfeldt-Jakob disease, Parkinson’s disease, Huntington’s disease, scrapie, chronic wasting disease and mad cow disease. The disease has killed many species of mammals including dolphins. Victims permanently contaminate the world around them with their bodily fluids. Once contaminated with prions, items cannot be sterilized.

Source: http://globegazette.com/features/cwd-detected-in-iowa-wild-deer-herd-for-first-time/article_c8484328-a1fa-5640-99bc-6d57aba24ad6.html

Creutzfeldt-Jakob Disease Spreads Contagion

Prion Contagion Unstoppable In Hospitals

Evidence continues to mount showing that many American hospitals are brimming with disease following a recent announcement by the Massachusetts Department of Public Health (MDPH). At least five patients who recently underwent spinal surgery at Cape Cod Hospital in Hyannis may have been exposed to Creutzfeldt-Jakob Disease (CJD), a brain infection similar to mad cow disease, according to the announcement, while eight others in New Hampshire and at least two others in another unidentified state may also have been exposed.

Surgical instruments shipped down from a hospital in New Hampshire were found to potentially be contaminated with prions, which are misfolded proteins that cause CJD and are not completely eliminated by standard sterilization protocols. The instruments had originally been used on a now-deceased patient at the Catholic Medical Center in Manchester, who has since been identified as having potentially been infected with prion disease (CJD), which can cause memory loss, cognitive disorders, behavioral changes and eventually dementia and death.

prion disease epidemic

“Our concern is with the health and well-being of the eight patients who may have been exposed to CJD,” said Dr. Joseph Pepe, CEO of Catholic Medical Center, in a recent joint statement with the New Hampshire Department of Health and Human Services about the affected patients at his hospital. “We will work closely with these families to help them in any way possible, even though the risk of infection is extremely low.”

CJD proteins known as prions are not completely eradicated by standard hospital sterilization procedures. As a result, the disease can spread indiscriminately and without warning from tool to tool, and from patient to patient, even after being rigorously cleaned.

CJD is said to affect less than 400 people annually in the U.S., according to the U.S. Centers for Disease Control and Prevention — the disease has no known cure, and is “rapidly progressive and always fatal,” according to health experts.

“The instruments included a metal reference frame and brace used in surgical navigation during the procedure,” stated Anne Roach, a spokeswoman for MDPH, about the potentially affected instruments, which were later used to conduct spinal surgeries on patients at other hospitals.

Alzheimer's disease epidemic

The Boston Globe reports that the potentially tainted equipment was borrowed by Cape Cod Hospital from Minneapolis-based manufacturer Medtronic, which also reportedly lent the equipment to another unidentified hospital previously. For nearly three months, the instruments were used to conduct special surgeries at Cape Cod Hospital, that is until Medtronic finally became aware of the CJD issue and notified the Hyannis-based hospital to stop using the equipment on August 28.

“[It] supposedly adheres well to many surfaces and is very difficult to remove,” Gail Horvath, an operating room nurse and patient safety analyst at the ECRI Institute, is quoted as saying to The Boston Globe about the threat of CJD at hospitals. “Bleaching for long periods of time can work, but that can damage sensitive instruments, and other effective chemicals can leave dangerous residue or otherwise harm the tools.”

 

Of particular concern is the fact that the implicated equipment had already went through a four-stage sanitation and sterilization process at Cape Cod Hospital prior to being used there, according to Dr. Donald Guadagnoli, the hospital’s chief medical officer. This process was meant to reduce CJD risk to the lowest possible level, and yet even this may not have been enough to render the equipment clean and safe.

“The reality is there are a lot of risks to undergoing surgery and most are substantially more significant than this,” says Dr. Guadagnoli. “The company and hospitals did everything they were supposed to do.”

Prions are associated with an entire family of neurological disorders that are killing people, wildlife and livestock around the world. These deadly diseases are known as Transmissible Spongiform Encephalopathy (TSE). The operative word is “transmissible.” TSEs include Alzheimer’s disease, Creutzfeldt-Jakob disease, Parkinson’s disease, Huntington’s disease, scrapie, chronic wasting disease and mad cow disease. The disease has killed many species of mammals including dolphins. Victims permanently contaminate the world around them with their bodily fluids. Once contaminated with prions, items cannot be sterilized.

Learn more about prion disease transmission: http://www.naturalnews.com/042058_mad_cow_disease_infected_hospital_patients_surgical_instruments.html#ixzz2f5PDiMGW

public relations firm and public affairs firm Denver and Phoenix

Crossbow Communications specializes in issue management and public affairs. Alzheimer’s disease, Creutzfeldt-Jakob disease, chronic wasting disease and the prion disease epidemic is an area of special expertise. Please contact Gary Chandler to join our coalition for reform gary@crossbow1.com.

Recycling Sewage Recycles Brain Disease

Sewage Sludge Spreading Deadly Diseases

I wish that I could support wastewater reclamation and the application of sewage sludge (biosolids). Unfortunately, I have reversed course on my position over the past decade and now I only see unacceptable risks. The reason for my reversal is a microscopic protein particle called a prion.

The problem is that prion diseases are on the rise around the world in people and animals. Since prions cause a deadly, incurable disease in people, wildlife and livestock, it seems to be prudent to question prion pathways and policies. Prion diseases kill everything in their path. There is no cure. They are always fatal. Since prions are unstoppable, they are a threat to food and water supplies around the world. Carelessly spreading prions via any pathway is reckless and criminal. Since the safety of biosolids cannot be proven, the practice must be stopped based on common sense.

We know these prion diseases (transmissible spongiform encephalopathies–TSEs) as:

mad cow disease and prions

Mad Cow (BSE) in cattle. Mad cow disease has emerged significantly around the globe over the past 30 years. Few countries have been immune.

Creutzfeldt-Jakob disease (CJD) and Alzheimer’s disease in humans. At least 10-20 percent of Alzheimer’s disease cases are actually Creutzfeldt-Jakob Disease. Since both are prion diseases, the difference is likely due to genetic and chemistry variations in the host or due to a prion mutation prior to exposure.

Chronic Wasting Disease (CWD) deer, elk and moose. Meanwhile, prion disease is on the rise among wildlife. Deer, elk, moose and other mammals have been dying from chronic wasting disease for more than 30 years, but the impacted regions continue to spread. The deadly disease has been found from Utah to Pennsylvania and from Canada south to Texas.

chronic wasting disease caused by prions

Scrapie in sheep. Farmers in Europe have reported sick and incurable sheep for about 300 years or more. Some speculate that this is one of the origins of the outbreak because they over-bred sheep for specific genetic traits and weakened the herds. Then some of the sick animals became feed for other livestock.

While the death rate for many major diseases, including heart disease and many forms of cancer, are declining, the death rate from Alzheimer’s disease and Creutzfeldt-Jakobs disease are on the rise among many populations (in some regions more than others). If Alzheimer’s and CJD were truly random diseases without environmental influence, the death rate from these diseases would be fairly consistent around the world. Unfortunately, that’s not the case. People who live in Washington State, for example, are lmost twice as likely to die from Alzheimer’s disease as people elsewhere in the nation. Women are almost twice as likely as men to die of Alzheimer’s disease. Why?

People and animals are exposed to prions in multiple ways. Many cattle got the disease from feed that was made from ground up cattle carcasses–a cheap source of protein and an elimination of disposal costs. Some animals have been infected by touching noses with infected animals or licking or ingesting material that sick animals touched. Since infected animals have the deadly prions in their blood, urine, feces, saliva and tissue, they basically contaminate their entire environment–even after death. Animals or carcasses that come along behind them are at risk of exposure and infection. Cattle also are exposed to prions in sewage sludge.

land application sewage sludge

The same risks are present for people. A person with prion disease will permanently infect cups, utensils, dental instruments and surgical instruments. In fact, most coroners refuse to conduct an autopsy on people who are suspected of having prion disease. Call your favorite coroner and ask.

Furthermore, people with prion disease also contaminate their toilets with their bodily fluids and excretions, which contaminate the sewage treatment plant. Just one person with a prion disease will contaminate every sewage system used–forever. Most cities have had more than one resident or visitor with prion disease, which means that prions are incubating and spreading within the pipes and the treatment plants of most sewage plants around the world. Additional prions arrive frequently thanks to the growing population of people with Alzheimer’s disease or CJD.

The prion problem grows thanks to sewage recycling efforts–prions are spread on golf courses, parks and crops as reclaimed water and as biosolid applications. Entire watersheds are at risk as rain, snow and irrigation can rinse the deadly prions into creeks, rivers, ponds, lakes, oceans and groundwater. Some states, such as Wisconsin, have applied biosolids in almost every county of the state. Wisconsin also has one of the worst epidemics of chronic wasting disease in the nation. Unfortunately, the sick deer contribute to the the contamination as they expose other animals, hunters, soil, and water.

The prion problem escalates when you realize that we are dumping millions of gallons of sewage into our rivers and oceans every day. I wonder how many dolphins and whales that beach themselves or just wash ashore are victims of prion disease?

Does it all sound too much like a sci-fi thriller? The plot thickens.

prion disease epidemic

Dr. Stanley Prusiner earned a Nobel Prize in 1997 for identifying, naming and studying deadly prions. President Obama awarded Prusiner the National Medal of Science in 2010 to recognize the growing significance of his discovery. (In June 2012, Prusiner confirmed that Alzheimer’s disease is a prion disease like CJD and mad cow.)

In fact, prions now are such a formidable threat that the United States government enacted the Bioterrorism Preparedness and Response Act of 2002 to halt research on infectious prions in the United States in all but two laboratories. Now, infectious prions are classified as select agents that require special security clearance for lab research. The intent is to keep prions and other dangerous biological materials away from terrorists who might use them to contaminate, food, water, blood, equipment, and entire facilities.

If prions must be tightly regulated in a laboratory environment today, the outdoor environment should be managed accordingly. If we can’t sterilize surgical equipment used on people who have prion disease, why are we kidding ourselves that we can neutralize prions in sewage? Dilution is not a solution to prion contamination. They don’t have a half-life like radiation. They multiply, which means even one will become many. They can’t be stopped.

biosolids land application and disease

Recycling water and waste is a good idea, except when it concentrates and recycles deadly diseases and pathogens that migrate, mutate and multiply. Prions are worse than radiation. That’s why the Department of Homeland Security has classified them as a “special agent” that must be controlled in only two labs in the entire country. Therefore, we should not make our lands and waters an outdoor chemistry experiment that can blow up in our face–and our children’s.

Any place that recycles sewage water and sewage sludge (biosolids) is spreading pathogens and misinformation–if not outright lies. These lands could someday be condemned as Superfund sites and our diminishing water supplies could be further lost to permanent contamination.

For more information on this topic please visit http://garychandler.com/pandoras-lunchbox-filled-with-prions/ you will see that the EPA and others are mismanaging these prion risks. I hope that you don’t make the same mistake. I would be happy to discuss this matter and consult with your agency to manage this issue.

Alzheimer's disease epidemic

Fact Sheet

Alzheimer’s disease patients shed infectious prions in their blood, saliva, mucus, urine
and feces. The infectious prions bind to the sewage sludge, including sludge biosolids compost, being applied on home gardens, US cropland, grazing fields and dairy pastures,
putting humans, family pets, wildlife and livestock at risk.

Other prion contaminated wastes discharged to sewers include rendering plants (which process remains of 2 million potentially BSE infected downer cows each year), slaughterhouses, embalmers and morticians, biocremation, taxidermists, butcher shops, veterinary and necropsy labs, hospitals, landfill leachates (where CWD infected and other carcasses are disposed), etc.

The US EPA lists prions as a contaminant of concern in sewage sludge and water
eight times. The EPA issued what it calls the “Sludge Rule,” which basically disclaims any responsibility for its premature and questionable risk assessments as it relates to all toxins and pathogens found in biosolids. It reserves the right to adjust the risk assessments as the test of time may disprove its pseudo-science.

Renown prion researcher, Dr. Joel Pedersen, University of Wisconsin, found that prions become 680 times more infective in certain soils. Dr. Pedersen’s research also proved sewage treatment does not inactivate prions.

sewage treatment plant and disease

“Our results suggest that if prions were to enter municipal waste water treatment systems, most of the agent would partition to activated sludge solids, survive mesophilic anaerobic digestion, and be present in treated biosolids. Land application of biosolids containing prions could represent a route for their unintentional introduction into the environment. Our results argue for excluding inputs of prions to municipal waste water treatment,” said Pedersen.

“Prions could end up in waste water treatment plants via slaughterhouse drains, hunted game cleaned in a sink, or humans with vCJD shedding prions in their urine or feces,” Pedersen says. “The disposal of sewage sludge was considered to represent the greatest risk of spreading (prion) infectivity to other premises.” It is well known that sewage sludge pathogens, pharmaceutical residue and chemical pollutants are taken up by plants and vegetables. 

The Canadian Food Inspection Agency recently warned that plants can uptake infectious prions: “. . . there is a potential risk to humans via direct ingestion of the compost or of compost particles adhered to skin or plant material (e.g. carrots). Another potential route of exposure is by ingestion of prions that have been taken up by plants.”

public relations firm and public affairs firm Denver and Phoenix

Gary Chandler is a public affairs, public relations and issue management strategist with Crossbow Communications, based in Denver and Phoenix. http://crossbowcommunications.com/alzheimers-disease-surging-due-to-misinformation-mismanagement/ Please contact Gary Chandler to join our coalition for reform gary@crossbow1.com.

Chronic Wasting Disease Being Mismanaged

Chronic Wasting Disease Unstoppable

Editor’s Note: As the following story illuminates, the global response to chronic wasting disease and other forms of prion disease demonstrate incompetence, negligence or criminal misconduct. I will debate anyone in the world and I will consult with anyone in the world. Mismanagement of this predatory disease is not acceptable and it could change life as we know it–if it hasn’t already. 

Initially the concern about Chronic Wasting disease (CWD) was focused on the health of the white tail deer population and the economic implications CWD could have on the deer hunting industry. New science has raised the possibility that we should be looking more closely at the potential effect CWD could have on human health, as well as hope that a new vaccine might stem the spread of CWD.

chronic wasting disease caused by prions

Chronic Wasting Disease has been a persistent issue among hunters and resource managers in all 25 states in which it now exists, including here in Wisconsin, since the first CWD positive elk was discovered in Colorado in 1967.

A positive doe found just west of Shell Lake in Washburn County in 2012 has rekindled discussion about CWD here in Wisconsin.

Initially the concern about CWD was focused on the health of the white tail deer population and the economic implications CWD could have on the deer hunting industry. New science has raised the possibility that we should be looking more closely at the potential effect CWD could have on human health, as well as hope that a new vaccine might stem the spread of CWD.

Wisconsin has more than 600,000 deer hunters who regularly harvest 300,000 to 400,000 deer annually, according to DNR Regional Wildlife Manager Mike Zeckmeister.

“Deer hunting … generates more than $500 million in retail sales and over $1 billion in total impact to the state’s economy,” he said. “A healthy whitetail deer population is critical to the state’s economy.”

land application sewage sludge

DNR CWD Wildlife Biologist Tim Marien said the state has spent roughly $43 million on the CWD program since the first whitetail tested positive in 2002. That money came from a combination of federal and state agencies including USDA and USFW. The most money was spent in 2003, $12.6 million, immediately after the initial detection of CWD in Wisconsin. The funding for surveillance, testing and research has decreased steadily since then to $600,000 in 2012.

If you do what a lot of folks do and just read the first couple of sentences of a paragraph and assume you’ve got the gist of what’s being said, this is what you’ll likely read in the first paragraph, of informational materials about the impact of CWD on human health, “there is no evidence that CWD poses a risk for humans,” followed shortly by, “The World Health Organization has reviewed available scientific information and concluded that currently there is no evidence that CWD can be transmitted to humans.” That’s not exactly what the science is saying.

prion disease epidemic

“I would not eat a deer that has not been tested (for CWD),” said retired veterinarian David Clausen, chairman of the Wisconsin Natural Resources Board.

Clausen said he is concerned that public access to information, including the latest science, isn’t what it should be to enable hunters and other consumers to make informed decisions about CWD. He’s also concerned government officials have been less than transparent in their decision-making process and that their priorities have been questionable when it came to preserving the resource and associated economy versus the health and well-being of citizens.

“We have a responsibility to be honest with the citizens of Wisconsin,” he said. “If we are doing nothing to influence the progression of CWD, we need to say that. We need to be more up front about the likely ramifications of our inaction. We cannot continue to maintain to the public that we can manage this disease by simply watching it.”

On March 20, 1996, British Health Secretary Stephen Dorrell rose before the House of Commons to inform colleagues that scientists had discovered a new variant of Creutzfeldt-Jakob disease (a fatal nervous system disease in humans), in 10 victims, and that they could not rule out a link to consumption of beef from cattle infected with bovine spongiform encephalopathy (BSE), also known as mad cow disease.

To date 217 cases of CJD have been diagnosed, mostly in the United Kingdom. The mad cow outbreak demonstrated that prion infections could cross species barriers between humans and cattle, increasing concern about the possible transmission to humans of a CWD another TSE.

Pursuant to the Federal Food, Drug, and Cosmetic Act, material from CWD positive deer and elk may not be used in any animal feed, and animal feed containing CWD positive material is considered adulterated and must be recalled from the market.

“Why isn’t the same degree of caution apparently necessary regarding human consumption of CWD positive deer?” Clausen asked.

In Wisconsin, the Department of Health Services has maintained surveillance for human prion diseases since 2002.

“Hospitals and physicians who suspect a case are legally mandated to report those to us,” said Jim Kazmierczak, state public health veterinarian.

It is DNR procedure to inform hunters who have had their deer tested for CWD, by phone, that their deer tested positive and that it is their recommendation, as well as that of the CDC and World Health Organization, that they do not eat that venison and instead dispose of it properly.

Beginning in 2004, if it was discovered that the hunter had already consumed venison from a contaminated deer for whatever reason, his or her name was referred to the DHS. A follow up call was made by DHS officials to conduct a brief interview to obtain the names of all of the people who had consumed venison from that particular contaminated deer (friends, family members etc.).

Those names are added to a registry of all hunters and other folks who have been identified as having eaten venison from a CWD positive deer. Today that registry consists of roughly 1,000 names.

“Based on the small numbers (consumers of venison) along with the long incubation period, we didn’t think it (the registry) would be a very valuable tool for at least 10 or 20 years after we initiated it,” said Kazmierczak.

Those names are regularly compared to the names of people who have been confirmed to have died from a human prion disease like CJD in the state of Wisconsin.

“The longer we don’t find any venison eaters on our list of cases, the more sure we can be. To date, we don’t have any incidences of human illnesses being related to CWD,” confirmed Kazmierczak.

Scientists have been experimenting in laboratories around the world to try and better understand how prion diseases work. Prions are tenacious abnormal proteins and impressively resilient responsible for spreading CWD. They are typically transmitted between whitetail deer in saliva, urine and feces. They can also be transmitted from soil.

A team of scientists at the UW-Madison, led by assistant professor of soil science Joel Pedersen, proved prions have an affinity for a particular type of clay, montmorillonite, found in many common types of soil. Prions bind to the soil and can remain as infectious as those transferred directly by animals for at least two years.

This is just the kind of science Clausen thinks people should know about.

“We should be asking what are the department’s (DNR) duties and responsibilities regarding increasing environmental contamination of soil and possibly plants with a disease agent that both CDC and WHO recommend people not consume,” he said.

As a result of the Mad Cow outbreak in the UK, scientists made a significant discovery. Prion infections were found in extra neural lymphoreticular tissues (tonsils, lymph nodes and spleen). Until that discovery, health officials had only been looking for evidence of prion infections in the central nervous system (CNS), brain and spinal column.

Recent research by French scientist Vincent Beringue using human and ovine transgenic mice, demonstrated that lymphoreticular tissue may be up to seven times less resistant to cross-species infection with prions than brain tissue. Beringue’s research also showed these mice lived normal life spans without showing any CNS symptoms but were none-the–less capable of transmitting disease when their splenic tissue was inoculated into other normal mice.

Because of this science and Clausen’s bringing it to the attention of the DHS, if a prion disease is suspected in Wisconsin, “both neural and extra neural tissues would now be sent to the CDC for examination,” said Kazmierczak.

CWD can cross the species barrier using a transmission mechanism called “serial passage.” Serial passage is occurring between deer naturally in nature.

“The longer CWD stays on the ground and the father it spreads,” said Clausen, “the more likely this type of thing is to happen.” In other words, the more adept CWD will become at crossing the species barrier.

At UW-Madison, the brains of hamsters were injected with CWD prions from infected deer and did not get sick. Hamsters appeared to be immune to CWD. Then the brains of ferrets were injected with CWD prions from infected deer and they got the disease and died. When infected brain tissue from the diseased ferrets was injected into the hamster’s brains, the hamsters got sick and died.

“So apparently CWD can make this end run around the species barrier,” said Kazmierczak. “It adds to our concern regarding the transmission and mutation of CWD when diseased deer carcasses are scavenged by other animals like coyotes, raccoons, and bears, even crows. We need to be guarded and cautious when we talk about CWD, we just don’t know.”

Dr. John Mapletoft works for Pan-Provincial Vaccine Enterprise Inc, (PREVENT). The Canadian-based organization connects and coordinates experts from public health, academic organizations, research institutes, the vaccine industry and the investment community to focus on the development of vaccine technologies.

PREVENT researchers have been working on a promising injectable vaccine to prevent the spread CWD among farmed cervids.

“Our goal is to prevent the disease in animals that don’t have it,” Mapletoft said.

Tami Ryan, a manager in the wildlife health section for the DNR, remembered being involved in several public forums back in 2010 when work on the vaccine was getting started.

“It did not evolve to fruition because at the time there were some pretty significant challenges to overcome with getting a vaccine developed in Canada into the U.S.,” she said. “About the same time, funding levels started to really decline (in Wisconsin) taking with it funds for research on CWD.”

A viable vaccine must meet three standards: it must demonstrate an immune response, it must be proven safe, and it must work. The CWD vaccine works by using a very short piece of protein that mimics a piece of the misfolded prion.

“We have demonstrated that our vaccine does induce an immune response in multiple species and that the immune response is specific for the misfolded prion protein,” said Mapletoft. “We also know it does not otherwise interact with or harm the brain.”

According to Ryan, one key question is if animals already infected with CWD tare vaccinated, can they still be carriers? Would they still be sharing prions and shedding prions into the environment?

Mapletoft said the current vaccine is “primarily seen to be a preventative (vs. cure) situation, so animals that already have the disease are not the target. That’s not to say it may not help them.”

The first vaccine tests began earlier this year on elk in Wyoming and another will be starting later this year in Saskatchewan.

One of the difficulties of testing a CWD vaccine is the long incubation period of the disease. Each of these tests will take a minimum of two years to determine if the vaccine is working, unlike a flu vaccine, which can be tested in a matter of days or weeks. Results from the Wyoming test aren’t expected until 2015.

“The vaccine has always been pursued with the idea that once the injectable vaccine is up and running and generating revenue, you would then have funds to proceed with a second generation oral vaccine which could be put out in the wild,” said Mapletoft.

“Frankly when the news came out recently about these trials in Wyoming, we were quite surprised,” said Ryan. “We’ll be paying attention.”

Prions are associated with an entire family of neurological disorders that are killing people, wildlife and livestock around the world. These deadly diseases are known as Transmissible Spongiform Encephalopathy (TSE). The operative word is “transmissible.” TSEs include Alzheimer’s disease, Creutzfeldt-Jakob disease, Parkinson’s disease, Huntington’s disease, scrapie, chronic wasting disease and mad cow disease. The disease has killed many species of mammals including dolphins. Victims permanently contaminate the world around them with their bodily fluids. Once contaminated with prions, items cannot be sterilized. 

source: http://www.rivertowns.net/event/article/id/38779/publisher_ID/13/

public relations firm and public affairs firm Denver and Phoenix

Crossbow Communications specializes in issue management and public affairs. Alzheimer’s disease, Creutzfeldt-Jakob disease, chronic wasting disease and the prion disease epidemic is an area special expertise. Please contact Gary Chandler to join our coalition for reform gary@crossbow1.com.

Transmissibility Of Prion Disease In Soil

Sewage Sludge Expands Prion Pathways

Soil may serve as an environmental reservoir for prion infectivity and contribute to the horizontal transmission of prion diseases (transmissible spongiform encephalopathies [TSEs]) of sheep, deer, and elk. TSE infectivity can persist in soil for years, and we previously demonstrated that the disease-associated form of the prion protein binds to soil particles and prions adsorbed to the common soil mineral montmorillonite (Mte) retain infectivity following intracerebral inoculation. Here, we assess the oral infectivity of Mte- and soil-bound prions.

land application sewage sludge

We establish that prions bound to Mte are orally bioavailable, and that, unexpectedly, binding to Mte significantly enhances disease penetrance and reduces the incubation period relative to unbound agent. Cox proportional hazards modeling revealed that across the doses of TSE agent tested, Mte increased the effective infectious titer by a factor of 680 relative to unbound agent. Oral exposure to Mte-associated prions led to TSE development in experimental animals even at doses too low to produce clinical symptoms in the absence of the mineral.

We tested the oral infectivity of prions bound to three whole soils differing in texture, mineralogy, and organic carbon content and found soil-bound prions to be orally infectious. Two of the three soils increased oral transmission of disease, and the infectivity of agent bound to the third organic carbon-rich soil was equivalent to that of unbound agent. Enhanced transmissibility of soil-bound prions may explain the environmental spread of some TSEs despite the presumably low levels shed into the environment. Association of prions with inorganic microparticles represents a novel means by which their oral transmission is enhanced relative to unbound agent.

chronic wasting disease caused by prions

Transmissible spongiform encephalopathies (TSEs) are a group of incurable neurological diseases likely caused by a misfolded form of the prion protein. TSEs include scrapie in sheep, bovine spongiform encephalopathy (“mad cow” disease) in cattle, chronic wasting disease in deer and elk, and Creutzfeldt-Jakob disease in humans. Scrapie and chronic wasting disease are unique among TSEs because they can be transmitted between animals, and the disease agents appear to persist in environments previously inhabited by infected animals.

Soil has been hypothesized to act as a reservoir of infectivity and to bind the infectious agent. In the current study, we orally dosed experimental animals with a common clay mineral, montmorillonite, or whole soils laden with infectious prions, and compared the transmissibility to unbound agent. We found that prions bound to montmorillonite and whole soils remained orally infectious, and, in most cases, increased the oral transmission of disease compared to the unbound agent. The results presented in this study suggest that soil may contribute to environmental spread of TSEs by increasing the transmissibility of small amounts of infectious agent in the environment.

biosolids land application LASS

Citation: Johnson CJ, Pedersen JA, Chappell RJ, McKenzie D, Aiken JM (2007) Oral Transmissibility of Prion Disease Is Enhanced by Binding to Soil Particles. PLoS Pathog 3(7): e93. doi:10.1371/journal.ppat.0030093

Introduction

Bovine spongiform encephalopathy, human Creutzfeldt-Jakob disease and kuru, sheep scrapie, and chronic wasting disease of deer, elk, and moose belong to the class of fatal, infectious neurodegenerative diseases known as transmissible spongiform encephalopathies (TSEs) or prion diseases. The precise nature of the etiological agent of these diseases remains controversial, but most evidence points to a misfolded isoform of the prion protein (PrPTSE) as the major, if not sole, component of the pathogen.

Sheep scrapie and cervid (deer, elk, and moose) chronic wasting disease are distinct among TSEs because epizootics can be maintained by horizontal transmission from infected to naïve animals, and transmission is mediated, at least in part, by an environmental reservoir of infectivity. The presence of an environmental TSE reservoir impacts several epidemiological factors including contact rate (the frequency animals come in contact with the disease agent), duration of exposure (time period over which animals come in contact with the pathogen), and the efficiency of transmission (the probability that an exposed individual contracts the disease).

The oral route of exposure appears responsible for environmental transmission of chronic wasting disease and scrapie; the propagation of bovine spongiform encephalopathy epizootics (feeding TSE-infected meat and bonemeal to cattle); the appearance of variant Creutzfeldt-Jacob disease in humans and feline spongiform encephalopathy in cats (presumably by consumption of bovine spongiform encephalopathy–infected beef); the spread of kuru among the Fore of Papua New Guinea (ritualistic endocannibalism); and outbreaks of transmissible mink encephalopathy (TME) in farm-reared mink. Following consumption, TSE agent is sampled by gut-associated lymphoid tissue, such as Peyer’s patches or isolated lymphoid follicles, and accumulates in lymphatic tissues before entering the central nervous system via the enteric nervous system. While ingestion is a biologically relevant TSE exposure route, oral dosing is a factor of ~105 less efficient than intracerebral inoculation in inducing disease in rodent models. The amounts of TSE agent shed into the environment are presumably small. The assumed low levels of TSE agent in the environment and the inefficiency of oral transmission have led to uncertainty about the contribution of environmental reservoirs of infectivity to prion disease transmission.

prion disease epidemic

We and others have hypothesized that soil may serve as a reservoir of TSE infectivity. Deliberate and incidental ingestion of soil by ruminants can amount to hundreds of grams daily. Prions enter soil environments via decomposition of infected carcasses, alimentary shedding, deliberate burial of diseased carcasses/material and possibly, urinary excretion. TSE agent persists for years when buried in soil. The disease-associated prion protein sorbs to soil particles, and the interaction of PrPTSE with the common aluminosilicate clay mineral montmorillonite (Mte) is remarkably avid. Despite this strong binding, PrPTSE–Mte complexes are infectious when inoculated into brains of recipient animals.

For TSEs to be transmitted via ingestion of prion-contaminated soil, prions bound to soil components must remain infectious by the oral route of exposure. We therefore investigated the oral infectivity of Mte- and soil-bound prions. We examined the effects of prion source (viz. infected brain homogenate [BH] and purified PrPTSE) and dose on disease penetrance (proportion of animals eventually exhibiting clinical TSE symptoms) and incubation period (time to onset of clinical symptoms) in experiments with Mte. We investigated the oral infectivity of soil particle–bound prions to Syrian hamsters using four dosing regimes: (1) infected BH mixed with Mte (BH–Mte mixtures), (2) isolated complexes of purified PrPTSE bound to Mte (PrPTSE–Mte complexes), (3) purified PrPTSE mixed with Mte (PrPTSE–Mte mixtures), and (4) PrPTSE mixed with each of three whole soils (PrPTSE–soil mixtures). The rationale for each dosing regime is described below. Survival analysis was used to assess risk of clinical disease manifestation and quantify differences in effective titer. Application of survival analysis to oral bioassays of TSE transmissibility is discussed in Figure S1 and Text S1.

biosolids land application and disease

Oral Infectivity of BH–Mte Mixtures

To examine the effect of Mte on the oral transmissibility of prions in BH, we incubated infected BH with clay particles for 2 h to allow sorption of the agent; controls lacking Mte were treated identically [22]. Three doses of 10% BH (30, 3, and 0.3 μL) were assayed. Diminished gastrointestinal bioavailability was expected to be evidenced by significant lengthening of incubation period, reduced disease penetrance, or both. Binding of either 30 or 3 μL of brain material to Mte yielded disease penetrance and incubation periods similar to BH alone (Figure 1A and 1B), a finding consistent with our previous report that a substantial fraction of PrPTSE in clarified BH binds to Mte and that Mte-bound prions remain infectious [22].

No Loss of Oral TSE Transmissibility Following Sorption of Prions from Infected BH to Mte (BH–Mte Mixtures)

The oral transmissibility of prions in 30 (A) and 3 (B) μL was not diminished by dosing with Mte. Indicates non-TSE intercurrent death. Animals dosed with Mte alone remained healthy throughout the course of the experiment (unpublished data).

Surprisingly, at the lowest BH dose (0.3 μL, Figure 2), sorption of TSE agent to Mte enhanced transmission, increasing disease penetrance and shortening incubation period. Adjusted for the amount of BH administered and combined across doses, Mte significantly enhanced oral transmissibility (p < 0.0001). Survival analysis indicated the risk of clinical disease manifestation relative to Mte-free controls was 3.03 (95% confidence interval [CI]: 1.68, 5.45), signifying an increase in the effective titer of TSE agent. While the influence of Mte was significant when tested across all BH doses, the effect was most readily observed at 0.3 μL. The dose-dependent difference in the influence of Mte on transmissibility may be attributable to competition between macromolecules in BH (e.g., lipids, other proteins, nucleic acids) with PrPTSE for sorption sites on the clay surface. Such competition was evidenced by detection of unbound PrPTSE and other proteins in incubations of Mte with 30 and 3 μL BH (unpublished data).

Ingestion of Mte mixed with a lower dose of TSE-infected BH (0.3 μL) markedly shortens incubation period and increases disease penetrance relative to an equal amount of unbound BH. * indicates non-TSE intercurrent death. Animals dosed with Mte alone remained healthy throughout the course of the experiment (unpublished data).

Oral Infectivity of PrPTSE–Mte Complexes

To examine the influence of Mte on oral transmissibility without the interference of other macromolecules from brain homogenate, we purified PrPTSE and inoculated hamsters using two different dosing regimes. The first dosing regime (PrPTSE–Mte complexes) was designed to directly assay the infectivity of PrPTSE sorbed to Mte surfaces (i.e., the amount of unbound PrPTSE was minimized in treatments containing Mte). Purified PrPTSE was clarified to remove large aggregates, and after 2-h incubation with Mte, PrPTSE–Mte complexes were separated from unbound protein by centrifugation through a sucrose cushion [22]. Hamsters were orally challenged with the isolated PrPTSE–Mte complexes [22] or an amount of unbound clarified PrPTSE (200 or 20 ng) equivalent to that introduced into the clay suspension (Table 1). Immunoblot analysis of the inocula (Figure S2A) demonstrated that the amount of PrP in the unbound samples was not less than that in PrPTSE–Mte complexes.

Prions Adsorbed to Mte Clay Are Infectious Perorally

Sorption of PrPTSE to Mte dramatically enhanced prion disease transmission (Table 1). Approximately 38% of animals receiving 200 ng of unbound clarified PrPTSE exhibited clinical symptoms with an incubation period for infected animals of 203 ± 33 (mean ± standard deviation) days post inoculation (dpi). In contrast, all animals orally dosed with an equivalent amount of Mte-bound PrPTSE manifested disease symptoms (incubation period = 195 ± 37 dpi), an enhancement of transmission comparable to that observed for the lowest BH dose (Figure 2). Animals inoculated with Mte alone or 10-fold less unbound clarified PrPTSE (20 ng) remained asymptomatic throughout the course of the experiment (>365 dpi), whereas 20 ng of clarified PrPTSE adsorbed to Mte produced TSE infection in 17% of animals. These data establish not only that the Mte-bound prions remain infectious via the oral route of exposure, but that agent binding to Mte increases disease penetrance, enhancing the efficiency of oral transmission.

Oral Infectivity of PrPTSE–Mte Mixtures

The second oral dosing regime using purified PrPTSE (PrPTSE–Mte mixtures) was designed to ensure that treatments with and without Mte contained equivalent PrPTSE doses. These experiments differed from those above in two important aspects. First, PrPTSE–Mte complexes were not separated from suspension prior to inoculation so that comparable amounts of infectious agent were administered to both treatment groups. In the first dosing regime, some PrPTSE may have been lost during sedimentation of PrPTSE–Mte complexes (Figure S2A). Second, the purified prion preparation was not clarified and therefore contained a range of PrPTSE aggregate sizes. The sizes of PrPTSE aggregates attached to Mte particles were expected to be more heterogeneous than those in the first dosing regime.

Compared to Mte-free controls, administration of purified PrPTSE mixed with Mte increased disease penetrance at all doses and shorted incubation times in the 1-μg PrPTSE treatment (Figure 3A). At the two lower doses (0.1 and 0.01 μg PrPTSE), binding of the agent to Mte dramatically increased disease penetrance (31%) at PrPTSE doses failing to yield clinical infection in 31 of 32 animals in the absence of the clay mineral (Figure 3B and 3C). Comparison of the survival curves in Figure 3A and 3C indicates that the 0.01-μg PrPTSE–Mte mixture was at least as infectious as 1-μg PrPTSE Mte-free samples, suggesting that sorption of purified PrPTSE to Mte enhanced transmission by a factor of ≥100.

Figure 3. Concurrent Peroral Administration of Mte and PrPTSE Dramatically Increases Disease Penetrance at Agent Doses That Typically Fail to Produce Clinical Symptoms (PrPTSE–Mte Mixture)

Mte increases disease penetrance and shortens incubation periods associated with ingestion of 1 μg of purified PrPTSE. Concurrent peroral dosage of lower, typically subclinical doses of purified PrPTSE (0.1 or 0.01 μg, [B and C]) with Mte increases disease incidence. Animals dosed with Mte alone remained healthy throughout the course of the experiment (unpublished data).

To quantify the contributions to changes in relative risk of prion dose and agent sorption to Mte, we constructed a multivariate Cox proportional hazards model with two covariates: log10 PrPTSE dose and Mte presence (Table 2). Each log10 increase in PrPTSE dose multiplies the relative risk by a factor of ~2 (i.e., a 10-fold increase in dose approximately doubles the risk of infection). Notably, sorption of purified PrPTSE to Mte multiplies the relative risk by a factor of ~8. These values allowed computation of a multiplicative equivalence factor between PrPTSE dose and Mte presence in the inoculum. Expressed in terms of PrPTSE dose, addition of Mte to the inoculum is equivalent to multiplying the PrPTSE dose by a factor of 680 (95% CI 16, ∞); that is, inclusion of Mte increases the effective titer of a given PrPTSE dose by 680-fold. Estimates of effective titer span a wide range (95% CI 16, ∞), and the present data do not allow us to place an upper bound on the increased risk associated with the presence of Mte in a sample. At a minimum, effective titer increased by 1.2 orders of magnitude, but the effect could be substantially larger. The best estimate of the Cox analysis represents a 2.8 order-of-magnitude increase in effective titer.

Estimated Hazard Ratios due to Prion Dose and Mte Addition

Strain PropertiesOral administration of Mte-bound PrPTSE did not appear to alter strain properties. Following limited proteinase K (PK) digestion, many PrPTSE strains can be discriminated by the size and glycoform pattern of PK-resistant core of PrPTSE (PrP-res) [3336]. Strain differences are also manifested in specific clinical symptoms. At the conclusion of the oral transmission experiments described above, the brains of clinically infected animals were assayed for PrP-res by immunoblotting (Figure S3). Differences in the molecular mass and glycoform distribution of PrP-res were not apparent between the treatment groups. Furthermore, clinical presentation of disease (symptoms or length of clinically positive period) did not differ between treatments.

The experiments described above were conducted using the Hyper (HY) strain of hamster-adapted TME agent (PrPHY). To further examine the strain stability of Mte-bound PrPTSE, we employed the Drowsy (DY) strain of hamster-passaged TME agent (PrPDY) to investigate the molecular mass of PrP desorbed from Mte and the effect of this clay mineral on oral transmissibility [35,36]. We previously reported the N-terminal cleavage of PrPHY extracted from Mte yielding a product similar in size to PK-digested PrPHY [22]. PK digestion of PrPHY and PrPDY results in products of characteristically different molecular masses [35,36]: the length of the PrPHY digestion product exceeds that of PrPDY by at least ten amino acids [35,36]. We found that extraction of bound PrPDY from Mte resulted in a product similar in molecular mass to PrPDY cleaved by PK (Figure 4). These data are consistent with the idea that strain properties are preserved when PrPTSE binds to Mte. DY agent is not orally transmissible [37], and we find that sorption of DY to Mte does not facilitate oral transmission (Text S1).

BH from hamsters clinically affected with either HY or DY agents were incubated with Mte to allow binding. Desorbed proteins were analyzed by SDS-PAGE and immunoblotting. Cleavage patterns of PrPHY and PrPDY extracted from Mte parallel PK cleavage patterns for the respective proteins: cleaved PrPDY migrates further (corresponding to a 1- to 2-kDa molecular mass difference) than cleaved PrPHY. Immunoblot used the PrP-specific antibody 3F4.

Natural soils are composed of a complex mixture of inorganic and organic components of various particle sizes. Smectitic clays such as Mte are important constituents of many natural soils and contribute significantly to their surface reactivity [38]. In natural soils, metal oxide and organic matter often coat smectite surfaces and may alter their propensity to bind PrPTSE. Furthermore, additional sorbent phases may be important in the binding of TSE agents to whole soils. We previously demonstrated that PrPTSE binds to whole soils of varying texture, mineralogy, and organic carbon content [22]. To examine the impact of agent binding to whole soil on oral TSE transmission, we incubated 1 μg of purified PrPTSE with each of three whole soil samples (Elliot, Dodge, and Bluestem soils) to allow sorption, and then orally dosed hamsters with the PrPTSE–soil mixtures. Soil-bound TSE agent remained infectious perorally, and two of the soils significantly enhanced oral disease transmission (Figure 5). Hazard ratios between Elliot (4.76 [95% CI: 1.38–16.4], p = 0.019) and Bluestem (6.04 [95% CI: 1.59–22.9], p = 0.013) soils and unbound PrPTSE indicate a significant increase in transmissibility, but no difference for the Dodge soil (1.66 [95% CI: 0.52–1.66], p = 0.578). The hazard ratios for the Elliot and Bluestem soils did not differ from one another (0.79 [95% CI: 0.19–3.25], p = 0.543) indicating statistical equivalence in transmissibility. The limited numbers of animals in the treatment groups precluded derivation of a multiplicative equivalence factor to equate the presence of Elliot or Bluestem soil with dose of infectious agent; however, substantially more animals in the Elliot and Bluestem treatment groups (14 of 16 animals, 87.5% penetrance) displayed clinical symptoms compared to the unbound PrPTSE treatment group (two of eight animals, 25% penetrance).

Prions Bound to Whole Soils Remain Orally Infectious and Some Soils Increase Transmission

Three soils (Dodge, Elliot, and Bluestem) were incubated in the presence of purified PrPTSE. The samples were orally dosed into hamsters and found to remain orally infectious. Agent association with Elliot and Bluestem soils increases disease incidence, whereas Dodge soil does not influence disease transmission. Animals dosed with soil alone remained healthy throughout the course of the experiment (unpublished data).

These experiments address the critical question of whether soil particle–bound prions are infectious by an environmentally relevant exposure route, namely, oral ingestion. Oral infectivity of soil particle–bound prions is a conditio sine qua non for soil to serve as an environmental reservoir for TSE agent. The maintenance of infectivity and enhanced transmissibility when TSE agent is bound to the common soil mineral Mte is remarkable given the avidity of the PrPTSE–Mte interaction [22]. One might expect the avid interaction of PrPTSE with Mte to result in the mineral serving as a sink, rather than a reservoir, for TSE infectivity. Our results demonstrate this may not be the case. Furthermore, sorption of prions to complex whole soils did not diminish bioavailability, and in two of three cases promoted disease transmission by the oral route of exposure. While extrapolation of these results to environmental conditions must be made with care, prion sorption to soil particles clearly has the potential to increase disease transmission via the oral route and contribute to the maintenance of TSE epizootics.

Two of three tested soils potentiated oral prion disease transmission. The reason for increased oral transmissibility associated with some, but not all, of the soils remains to be elucidated. One possibility is that components responsible for enhancing oral transmissibility were present at higher levels in the Elliot and Bluestem soils than in the Dodge soil. The major difference between the Dodge soil and the other two soils was the extremely high natural organic matter content of the former (34%, [22]). The Dodge and Elliot soils contained similar levels of mixed-layer illite/smectite, although the contribution of smectite layers was higher in the Dodge soil (14%–16%, [22]). The organic matter present in the Dodge soil may have obstructed access of PrPTSE to sorption sites on smectite (or other mineral) surfaces.

The mechanism by which Mte or other soil components enhances the oral transmissibility of particle-bound prions remains to be clarified. Aluminosilicate minerals such as Mte do not provoke inflammation of the intestinal lining [39]. Although such an effect is conceivable for whole soils, soil ingestion is common in ruminants and other mammals [25]. Prion binding to Mte or other soil components may partially protect PrPTSE from denaturation or proteolysis in the digestive tract [22,40] allowing more disease agent to be taken up from the gut than would otherwise be the case. Adsorption of PrPTSE to soil or soil minerals may alter the aggregation state of the protein, shifting the size distribution toward more infectious prion protein particles, thereby increasing the specific titer (i.e., infectious units per mass of protein) [41]. In the intestine, PrPTSE complexed with soil particles may be more readily sampled, endocytosed (e.g., at Peyer’s patches), or persorbed than unbound prions. Aluminosilicate (as well as titanium dioxide, starch, and silica) microparticles, similar in size to the Mte used in our experiments, readily undergo endocytotic and persorptive uptake in the small intestine [4244]. Enhanced translocation of the infectious agent from the gut lumen into the body may be responsible for the observed increase in transmission efficiency.

Survival analysis indicated that when bound to Mte, prions from both BH and purified PrPTSE preparations were more orally infectious than unbound agent. Mte addition influenced the effective titer of infected BH to a lesser extent than purified PrPTSE. Several nonmutually exclusive factors may explain this result: (1) other macromolecules present in BH (e.g., lipids, nucleic acids, other proteins) compete with PrPTSE for Mte binding sites; (2) prion protein is more aggregated in the purified PrPTSE preparation than in BH [45], and sorption to Mte reduces PrPTSE aggregate size, increasing specific titer [41]; and (3) sorption of macromolecules present in BH to Mte influences mineral particle uptake in the gut by altering surface charge or size, whereas the approximately 1,000-fold lower total protein concentration in purified PrPTSE preparations did not produce this effect.

We previously showed that other inorganic microparticles (kaolinite and silicon dioxide) also bind PrPTSE [22]. All three types of microparticles are widely used food additives and are typically listed as bentonite (Mte), kaolin (kaolinite), and silica (silicon dioxide). Microparticles are increasingly included in Western diets. Dietary microparticles are typically inert and considered safe for consumption by themselves, do not cause inflammatory responses or other pathologies, even with chronic consumption, and are often sampled in the gut and transferred from the intestinal lumen to lymphoid tissue [39,46,47]. Our data suggest that the binding of PrPTSE to dietary microparticles has the potential to enhance oral prion disease transmission and warrants further investigation.

In conclusion, our results provide compelling support for the hypothesis that soil serves as a biologically relevant reservoir of TSE infectivity. Our data are intriguing in light of reports that naïve animals can contract TSEs following exposure to presumably low doses of agent in the environment [5,79]. We find that Mte enhances the likelihood of TSE manifestation in cases that would otherwise remain subclinical (Figure 3B and 3C), and that prions bound to soil are orally infectious (Figure 5). Our results demonstrate that adsorption of TSE agent to inorganic microparticles and certain soils alter transmission efficiency via the oral route of exposure.

TSE agent source.

Syrian hamsters (cared for according to all institutional protocols) were experimentally infected with the HY or DY strain of hamster-adapted TME agent [48]. Brain homogenate, 10% w/v, was prepared in 10 mM NaCl. PrPTSE was purified to a P4 pellet from brains of hamsters infected with the HY strain using a modification of the procedure described by Bolton et al. [49,50]. The P4 pellet prepared from four brains was resuspended in 1 mL of 10 mM Tris (pH 7.4) with 130 mM NaCl. In the subset of experiments using PrPTSE–Mte complexes, larger prion aggregates were removed from the preparation by collecting supernatants from two sequential 5-min centrifugations at 800 g (clarification). Protein concentrations were determined using the Bio-Rad (http://www.bio-rad.com) DC protein assay as directed by the manufacturer’s instructions.

Preparation of inocula and oral dosing.Four types of Mte- or soil-containing inocula were prepared: BH–Mte mixtures, PrPTSE–Mte mixtures, PrPTSE–soil mixtures, and PrPTSE–Mte complexes (see below). To prepare mixtures of BH or PrPTSE with Mte, the indicated amount of 10% brain homogenate (Figures 1 and 2) or PrPTSE (Figure 3) was added to 500 μL of 10 mM NaCl in the presence or absence of 500 μg of Na+-saturated Mte (particle hydrodynamic diameter = 0.5–2 μm) (prepared per [51]). Mixtures of PrPTSE and whole soils (Figure 5) were prepared by adding 1 μg of PrPTSE to 500 μL of 5 mM CaCl2 in the presence or absence of 1 mg of each soil type. Samples were rotated at ambient temperature for 2 h, like samples were pooled, and the equivalent of 500 μg of Mte or 1 mg of whole soil was orally inoculated into each hamster. We previously showed that absorption of purified PrPTSE to Mte was complete within 2 h [22].

Isolated PrPTSE–Mte complexes were prepared as previously described [22]. Briefly, the indicated amount of clarified PrPTSE (200 or 20 ng, Table 1) was added to 500 μg of Mte in 10 mM NaCl (500 μL final volume) per sample. Mixtures were rotated at ambient temperature for 2 h. Each PrPTSE–Mte suspension was placed over a 750-mM sucrose cushion prepared in 10 mM NaCl and centrifuged at 800 g for 7 min to sediment mineral particles and adsorbed PrPTSE. PrPTSE–Mte complexes were resuspended in 500 μL of 10 mM NaCl and pooled. The equivalent of 500 μg of Mte was orally inoculated into each hamster. To control for potential sedimentation of unbound PrPTSE, “mock” samples lacking Mte were processed identically, and any sedimented material was inoculated into hamsters. As a positive control, unbound PrPTSE (200 or 20 ng) was orally administered to hamsters. All oral inoculations were via pipette and voluntary consumption. Following oral dosing, hamsters were observed twice weekly for the onset of clinical symptoms [48] for at least 300 d, a period of time found sufficient to observe most or all clinical cases.

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Crossbow Communications specializes in issue management and public affairs. Alzheimer’s disease, Creutzfeldt-Jakob disease, chronic wasting disease and the prion disease epidemic is an area of special expertise. Please contact Gary Chandler to join our coalition for reform gary@crossbow1.com.

CJD Kills North Carolina Boy

Creutzfeldt-Jakob Disease Taking Younger Victims

By Andrew Kenney

It wasn’t bacteria or a virus that plagued young Michael Mendy’s body and mind. He did not inherit his symptoms from his mother or father. Nothing toxic was in his blood. An autopsy reveals that he died of prion disease. And while he was sick, Michael’s parents had no explanation.

“I had to figure it out. I had to find an answer. I had to find a doctor that could help him,” said Michael’s mother, Kathleen Mendy, who lives in western Cary. “You don’t think you’re going to come across something that nobody’s ever seen.”

Michael Mendy dies of CJD

But they had. Dozens of specialists and three years of suffering brought no diagnosis. Michael died a year ago, at age 16. And only then did the explanation and the terrible significance of his case emerge.

Doctors estimate that fewer than one in 100 million young people will share Michael’s journey. The sports nut and East Cary Middle School student was killed by a disease that mostly afflicts the elderly. Michael was a heart-wrenching outlier, apparently among the youngest ever to suffer a spontaneous ravaging of the brain.

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Kathleen Mendy thinks it started on Michael’s 13th birthday in 2009. On that January day, another boy knocked Michael’s head to the court during a basketball game. He was playing again a minute later, his mother watching with a tinge of worry.

The symptoms of a years-long illness crept in that weekend, during a mother-son Super Bowl trip to Florida. That’s where she first saw Michael’s confusion, his unsure movements and his inexplicable crying.

It seemed at first like the troubling wake of the teen’s second concussion in three months, but that theory would erode and change. Across the next three years, in a nightmare that kept unfolding, the brawny teenager would drop almost half his body weight, spend months in the hospital, lose his speech and lay debilitated by simple infections.

The printed record of Michael’s hospital visits and test appointments is three and a half pages long. It documents an increasingly desperate search, listing 140 days in the hospital, 91 visits with doctors and 426 therapy appointments from 2009 to 2012.

“I always thought he would get better,” Kathleen Mendy said. “I used to always tell him, ‘Michael, one day you’re just going to run out of your bedroom, and you’re going to come running downstairs, and you’re going to be all better.’ ”

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While his friends went on to high school, Michael was confined to a wheelchair and fed through a tube. His care grew so intense that his mother brought on a full-time medical aide. His father, divorced from his mother, drove in from Florida each time he entered the hospital, and Kathleen Mendy’s family often visited from New York. Each treatment was more esoteric than the last. By the end of 2011, the Mendys had seen more than 30 doctors, medical specialists, faith healers and alternative practitioners.

“I tried chiropractors, reflexology, myofacial therapy,” Kathleen Mendy said. “I tried everything.”

The realization came to Kathleen Mendy on the last night of January 2012, the 22nd day that Michael spent in a UNC hospital bed. He’d been kept alive in an intensive-care unit by a breathing machine while an infection took hold of his lungs. The mysterious disorder had left his body unable to respond. The memory shakes Kathleen Mendy to tears. The scene sticks in her mind.

“Not until the night before he died, is when, honestly, it hit me,” she recalled.

The doctors laid a choice before his parents that night. Michael could go home with a tracheotomy and a ventilator, but they believed he’d live just a few months longer. Or doctors could remove him from life support. Michael’s parents didn’t want him to suffer anymore. He died on Feb. 1, 2012.

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Only months later would Michael’s family learn the reason for his degradation and death. An autopsy showed that the teenager died of sporadic fatal insomnia, a subtype of Creutzfeldt-Jakob Disease.

“I’m so glad I didn’t know what it was” before Michael died, Kathleen Mendy said. “Because then I wouldn’t have had hope.”

“This disease is a descent into hell,” said Florence Kranitz, president of The Creutzfeldt-Jakob Disease Foundation in New York City.

She saw her own husband die in 2001 of an ailment similar to Michael’s. Since then she has heard the stories of many of the 300 CJD victims her organization identifies each year, including cases of fatal insomnia.

“We get this phone call, and tragically it’s the same phone call over and over again,” Kranitz said. “They’ve never heard of this disease.”

The story she heard from Kathleen Mendy fit the profile, with one beguiling exception. Almost everyone afflicted by CJD subtypes are older than 45, except those who contract a variant of the disease genetically or through contaminated beef, which Michael had not.

biosolids land application and disease

Michael’s case quickly drew the attention of national experts, including Pierluigi Gambetti, director of the National Prion Disease Pathology Surveillance Center in Ohio. Gambetti, a pioneering researcher, examined Michael’s brain and in April identified his disease as sporadic fatal insomnia.

He’d later take hours to talk with Kathleen Mendy about her son’s death. Sporadic fatal insomnia and CJD, he explained, are part of the still-mysterious field of neurodegenerative diseases, including Alzheimer’s and Parkinson’s.

Some of these ravages of the brain, such as so-called mad cow disease (another form of prion disease), begin with an infection of prions, or pathogenic proteins, from the outside. CJD in the young also can be caused by prions, often transmitted during surgery.

Prions and Prusiner win Nobel Prize

Like a virus, a prion can essentially “breed.” The virus hijacks human cells, and the prion reshapes other proteins into its own mutated form. And when a prion or virus propagates enough, it can destroy its host.

But there’s a crucial difference: The prion also can come from within. Gambetti believes that Michael’s disease began when the boy’s brain misfolded a protein, creating a prion instead. The defect may then have multiplied out of control and ruined the delicate balance of the body.

It’s not uncommon for the body to make mistakes. Neurons and other cells normally catch and eliminate prions before they replicate. These defensive systems may grow weaker with age; some people may also inherit weaker defenses.

But Michael was a teenager, with no apparent family history of neurodegenerative diseases. Gambetti put the odds of such a case at one in 100 million in the general population; another doctor said it was one in 600 million. In fact, Michael may be among the youngest ever to be affected by a neurodegenerative disease without an inherited or outside cause.

“We just haven’t seen this disease affect someone this young,” said John Trojanowski, a professor of geriatric medicine at the University of Pennsylvania.

Gambetti, who played a key role in the discovery of fatal insomnia, theorizes that Michael’s illness was random, despite the odds. It may be that, by chance or some unknown factor, Michael’s brain perfectly bred its own pathogen.

“The bodies of all animals are a marvel of things, in positive and negative,” Gambetti said. “They can do things striking for the good, but also for the bad.”

Nearly a year after Michael’s death, Kathleen Mendy finds love and support from family, friends and Compassionate Friends, a local group. But the extreme rarity of Michael’s case is isolating.

When she attended a CJD conference last summer with her twin sister, they met the families of people who had mostly died in middle and old age.

Some nights she goes up to her only child’s room. It’s lined with dozens of sports team caps and trophies. Athletes’ names are still painted on the blades of his ceiling fan, and the UNC comforter is still on his bed. All that’s new is the shrine on the desk, where Michael’s photo stands near a glazed statue of praying hands.

“Sometimes I think I’m OK, and other times it’s like it just happened last night. It’s like a rollercoaster,” Kathleen Mendy said.

She may have as many logical answers now as she’ll ever get – a medical, if not a spiritual description of why Michael died.

She still doesn’t know what it was that made her son vulnerable. She believes Michael’s concussions triggered his illness, but his doctors haven’t confirmed the idea.

“I’m a little bit resolved that I’ll never hear the answer,” she said. “It would be nice to know, but if I don’t know it, it’s not what matters now.”

She finds hope instead in the idea that she could help others; she’s thinking of writing a book and becoming a public advocate for those who suffer with CJD.

Meanwhile, as Michael’s birthday and the first anniversary of his death approach, Gambetti and one of Michael’s former doctors are preparing to present his case to their respective medical communities. As painful as the case is, “its rarity may contribute to expand the knowledge on this terrible disease,” according to Gambetti.

He hopes his research will one day allow much earlier diagnosis and treatment of fatal insomnia. Such a breakthrough could key medical progress across the spectrum of prion-related diseases, which are fatal in practically all cases.

Gambetti’s research into Michael’s case will soon yield a more immediate result too: He’ll be able to tell caregivers that sporadic fatal insomnia can strike not just the mature, but perhaps people who are just beginning their lives.

And with Michael’s story spreading, the next stricken family may at least know the harrowing path ahead.

Original Post At: http://www.newsobserver.com/2013/01/19/2619247/at-last-answers-to-a-mothers-grief.html

public relations firm and public affairs firm Denver and Phoenix

Crossbow Communications specializes in issue management and public affairs. Alzheimer’s disease, Creutzfeldt-Jakob disease, chronic wasting disease and the prion disease epidemic is an area of special expertise. Please contact Gary Chandler to join our coalition for reform gary@crossbow1.com.