Creutzfeldt-Jakob Disease

Creutzfeldt-Jakob Disease Part Of Spectrum Disease

Neurodegenerative disease is the fastest-growing cause of death in the world. It’s quietly become a public health disaster and it will get worse.

Alzheimer’s disease and Creutzfeldt-Jakob disease (CJD) are essentially the same disease at different points on the same spectrum. Misdiagnosis, suppression of diagnoses, misinformation and mismanagement are contributing to the epidemic. Alzheimer’s and CJD are part of a spectrum disease called transmissible spongiform encephalopathy (TSE). The operative word is “transmissible.”

A variety of factors can trigger neurodegenerative disease, including genetics, head trauma and neurotoxins. Alzheimer’s disease alone is taking the lives of 50-100 million people around the world now. The epidemics are more severe in some countries than others. As millions die, even more will be diagnosed. Millions more are suffering in silence with a misdiagnosis or no diagnosis. We’re facing a public health disaster. Misinformation and mismanagement are fanning the flames.

Alzheimer's disease caregivers

Despite millions of deaths, experts suggest that the prevalence of the disease will quadruple by 2050, if not sooner. Unfortunately, there is a growing stack of evidence that Alzheimer’s disease, Parkinson’s disease and other brain diseases are transmissible. They also are being misdiagnosed and undiagnosed at an alarming rate. Deadly, self-replicating proteins appear to be one of the common threads.

Prions

Prions (PREE-ons) are a deadly and unstoppable form of protein that migrates, mutates, multiplies and kills with unparalleled efficiency. Prions cause fatal neurodegenerative disease in humans and animals by converting the cellular version of prion protein into a toxic form that erodes the brain and body. Prion disease often is described as a wasting disease that causes a loss of body mass and brain mass.

Prion disease research

Dr. Stanley Prusiner, an American neuroscientist from the University of California at San Francisco, earned a Nobel Prize in 1997 for discovering and characterizing prions and prion disease. President Obama awarded Prusiner the National Medal of Science in 2010 to recognize the importance of his research.

Prion disease also is known as transmissible spongiform encephalopathy (TSE). The operative word is “transmissible.” Prusiner claims that all forms of TSE are caused by infectious prions.

TSE is a spectrum disease that varies in severity and symptoms. It depends on which region of the brain is impacted first and by what prion mutation. Few cases are identical in terms of symptoms and diagnoses. When the presenting symptom is memory loss, the diagnoses flow along the following chart.

prion disease spectrum

In humans, the prion spectrum includes Alzheimer’s disease, Parkinson’s disease and an extremely aggressive version known as Creutzfeldt-Jakob disease. The difference between these diseases is very slight and often indistinguishable to neurologists. For example, millions of people have the severe form of Alzheimer’s disease, which is known as Creutzfeldt-Jakob disease (CJD). CJD is the most severe form of prion disease in humans. CJD often kills within weeks or months of diagnosis (a definitive confirmation requires an autopsy).

According to neuroscientists Dr. Laura Manuelidis, at least 25 percent of Alzheimer’s diagnoses are actually CJD, which is further up the prion spectrum. CJD, without dispute, is extremely infectious to caregivers and loved ones, but it has not been declared a reportable disease across the U.S. and many other nations.

Millions of cases of deadly CJD are being misdiagnosed as Alzheimer’s disease. Millions of patients and caregivers are being misinformed, misguided and exposed to an aggressive prion disease.

“It is well known that CJD is transmissible via surgical or medical procedures involving prion-infected brain tissue. Our finding of infectious prions in skin is important since it not only raises concerns about the potential for disease transmission via common surgeries not involving the brain, but also suggests that skin biopsies and autopsies may enhance pre-mortem and post-mortem CJD diagnosis,” said Wenquan Zou, Associate Professor of Pathology and Neurology and Associate Director of the National Prion Disease Pathology Surveillance Center at Case Western Reserve School of Medicine. “The level of prion infectivity detected in CJD skin was surprisingly significant, but still much lower than that in CJD brains. Further investigation is necessary to determine whether extra precautions should be taken during non-neurosurgeries of CJD patients, especially when surgical instruments will be reused.”

Prion infectivity is highly concentrated in CJD patient brain tissue, but it’s also in all bodily fluids and tissue. Inter-personal CJD transmission has occurred after patients were exposed to surgical tools previously contaminated by CJD brain tissues. It’s happening due to many other pathways.

Meanwhile, chronic traumatic encephalopathy (CTE) is likely a form of transmissible spongiform encephalopathy—prion disease. In most of these cases, the head trauma was the change agent that caused prions to misfold and become toxic. Once the neurodegeneration of CTE begins, are these victims shedding infectious prions? Hopefully, prion researchers will fill in this important blank. Again, families and caregivers need to know if they are dealing with a TSE. If hospitals throw out surgical instruments used on such patients, others need to take similar precautions.

Concussion research

Unfortunately, Prusiner’s science is being ignored and we all are facing a public health disaster because of the negligence and reckless disregard for public health. Misinformed caregivers, family members, healthcare workers and others are caught in the crossfire of a deadly form of protein.

Neurologists prefer not to touch or even be in the same room as a patient with CJD. The CJD Foundation and other advocacy organizations also remain mum on the risk of transmission. The CDC remains silent. Is this cone of silence incompetence, negligence or criminal misconduct? Show me the evidence that you rely upon

Abnormal proteins also are associated with autism. In fact, it appears that age is the biggest difference between the neurodegenerative disease spectrum and autism spectrum disorders. Both spectrums share common environmental causes and pathologies. Plus, CJD is taking the lives of more and more young adults and teenagers.

Prion Disease

Prion disease is highly contagious, incurable and fatal. Despite all of the smoke and mirrors, prion disease is prion disease. It’s killing more and more mammals, including humans, every year. The hype about species barriers is ridiculous, reckless and irresponsible.

Prions are such a formidable threat that the U.S. government enacted the Bioterrorism Preparedness and Response Act of 2002, which included a provision to halt research on prions in all but two laboratories. It classified prions as select agents that pose an extreme risk to food, water and health systems. Unfortunately, the Center For Disease Control quietly took prions off the list about two years ago because the classification threatened to criminalize some multi-billion dollar industries and many industry practices.

Victims should be quarantined because prions are in the urine, feces, blood, saliva, mucus, skin and cell tissue of all victims–all human byproducts that are washed, dumped, or flushed down sinks and toilets. One can assume that the waste is extra infectious when it comes from funeral homes, nursing homes, hospitals, dental offices, veterinarians, slaughterhouses and some laboratories.

In humans, most diagnoses are a process of elimination. After eliminating all other possibilities, the medical guesswork begins:

  • If the patient has a memory disorder, it’s diagnosed as Alzheimer’s disease;
  • If they have a movement disorder, it’s diagnosed as Parkinson’s disease;
  • If the patient shows both symptoms, doctors flip a coin;
  • If the patient ever had a concussion, it’s now ruled as CTE;
  • If the person is incapacitated, it’s Creutzfeldt-Jakob disease (CJD) and very transmissible;
  • If the victim is in the deer family, it’s chronic wasting disease instead of prion disease;
  • If the victim is a beef or dairy cow, it’s called mad cow disease instead of prion disease;
  • In other mammals, it’s called different things, but prion disease has been found in dolphins, elephants, mink, cats and many other species. The suggestion of a reliable species barrier against thousands, if not millions of mutations is ludicrous.

Current diagnostic tools for CJD rely on brain tissue samples collected at either biopsy or autopsy, or through a spinal tap.

Prion disease causes memory loss, impaired coordination, and abnormal movements. It’s not known which patients with brain disease become infectious or when, but both CJD and Alzheimer’s patients are being mismanaged. Informed neurologists won’t touch patients with these symptoms because of the risk of transmission. They are making diagnoses from across the room. Why aren’t they talking about it?

“Creutzfeldt-Jakob disease behaves like Alzheimer’s disease on steroids,” said Dr. Jennifer Majersik, an associate professor of neurology at the University of Utah.

Creutzfeldt-Jakob disease often is confused with Alzheimer’s disease because they are very similar and both are always fatal. It seems as though CJD is caused by a more aggressive mutation of the deadly prion than Alzheimer’s disease. The only difference that the medical community discusses is the pace of disease progression.

Since prion disease is a spectrum disease, doctors can’t tell the difference between Alzheimer’s disease and CJD without a spinal tap and it’s still a guess. According to neuroscientists Dr. Laura Manuelidis, at least 25 percent of Alzheimer’s diagnoses are not Alzheimer’s disease. These misdiagnoses are actually CJD, which is further up the prion spectrum. CJD, without dispute, is extremely infectious to caregivers and loved ones. Millions of cases of deadly CJD are being misdiagnosed as Alzheimer’s disease. Millions of patients and caregivers are being misinformed, misguided and exposed to an aggressive disease. Misdiagnosis and misinformation regarding prion disease is a matter of life and death. The mismanagement doesn’t end here.

CJD transmissible disease

Prion Pathways

There are many sources and pathways for deadly prions. However, we can’t ignore the biggest pathways. The cruel irony of prion disease is that victims become part of the greater problem. Studies confirm that people and animals dying of prion disease contaminate the environment around them. Infectious prions are in the urine, feces, blood, skin, mucus and saliva of each victim. These infectious bodily fluids are contributing to the rapid spread of Alzheimer’s and other mutations of prion disease.

“There has been a resurgence of this sort of thinking, because 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 (prions).”

A study published in the journal Nature adds to the evidence about the transmissibility of Alzheimer’s disease between people. A second study by the same scientist in early 2016 supports the claim. Meanwhile, there is absolutely no evidence to the contrary. Even wildlife and sea mammals are contracting brain disease from people because of the dumping of infectious waste on farms, ranches and forests.

Spouses of those with Alzheimer’s disease are 600 percent more likely to contract the disease, which is further evidence that it is a transmissible disease. Caregivers, family members and others are in harm’s way.

Surgical instruments infected with prions, for example, are impossible to sterilize and hospitals throw them away. Meanwhile, caregivers and family members are not warned. CJD victims are not quarantined. They are sent home to die in many cases. Reckless.

Many factors are contributing to the epidemic. Prions are now the X factor. Industry and government are not accounting for prions 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.

 

biosolids land application and disease

Unfortunately, there are so many variations of prions, that even a spinal tap can only detect prion disease. It can’t distinguish between all of the various mutations because they aren’t even in the science books, yet. Dr. Prusiner just announced the discovery of a new form of prion. The first such characterization in 40 years. Just a few thousand more to go. Unfortunately, it’s time to focus on containment not characterization.

The only definitive diagnosis of prion disease requires an autopsy, which rarely happens with neurological disease due to concerns about deadly contamination and exposure. All doctors are guessing with each Alzheimer’s diagnosis. All they can do is attempt to gauge the severity of symptoms. This problem complicates the search for accurate statistics about the size and scope of the epidemic. The attempt to differentiate between CJD and Alzheimer’s represents a containment nightmare.

Kuru disease

The Fore tribe of New Guinea unknowingly became the first known “Guinea Pigs” for prion disease (villagers call CJD “kuru”). Rumors made it out that many tribal members were getting sick and experiencing horrifying deaths from kuru. Ultimately, pioneering pathologists from the U.S. and Australia spent time with the Fore to understand the disease.

The research began back in 1955. After years of study, scientists determined that some tribal members (mostly women and children) contracted the prion disease after eating the brains of relatives who died. At some point in time, someone died of the natural form of dementia and then the tribe unknowingly consumed that person’s brains in a ritual—including the deadly prions that killed the person. Many of those who ate these infected brains also withered away and died of this brain wasting disease. As those people died, the surviving villagers consumed the brains of the deceased and so on. The disease kept recycling and intensifying itself through cannibalism.

It’s impossible to know exactly how many years it took for the disease to start snowballing among the Fore. Once it reached a tipping point, it almost destroyed the tribe. Thanks to the efforts of some dedicated researchers, they eventually isolated the problem and helped instill some social change to curb contamination and the epidemic.

I haven’t heard of any modern updates, but once infected, the soil in the area and personal items are still infected. Therefore, further outbreaks are possible just because of environmental contamination—even decades after the initial outbreak.

Recent CJD Clusters

Elsewhere in the world, people have contracted the disease by eating meat from livestock and wildlife with prion disease. The UK had the most severe crisis back in the late 1980s and early 1990s. They killed almost 200,000 cattle in an attempt to eradicate mad cow disease.

land application sewage sludge and disease

Over the past 25 years, the world has seen several clusters of the disease in humans, which points toward areas of environmental contamination or contaminated food and water supplies. In Cleveland County North Carolina, for example, 17 patients died from CJD in just a few months in early 2013. In British Columbia, four people from the Fraser Valley were diagnosed with CJD in one day in 2013 (although one apparently died a few weeks earlier). Clusters of the disease have appeared before and they are still appearing thanks to sewage mismanagement and other forms of environmental contamination.

TSEs also mad cow disease and chronic wasting disease in deer. TSE has been found in mink, moose, mice, sheep, cats, elephants, dolphins and many other mammals. Sea mammals are extremely vulnerable, but they aren’t being tested. Sick mammals on land and at sea are a canary in a coal mine. Their sickness confirms an alarming epidemiological trend among humans. An environmental contagion is responsible for the spike among many mammals. The reckless management of infectious waste (sewage) is fueling the epidemic.

Anna’s Brave Battle Against Creutzfeldt-Jakob Disease

The following story is about a 62-year-old American woman from Washington state. She was diagnosed with CJD in the fall of 2012. She died just before Christmas in her husband’s arms at home near Spokane. As this testimonial describes, even the most deadly form of dementia, CJD, is misunderstood and mismanaged. The highly infectious patient was never quarantined and caregivers, including family, were not warned about deadly and highly contagious prions.

cjd victim
Anna Henderson was diagnosed with CJD in September of 2012. She passed away on Thanksgiving day. She cleaned the operating rooms at a hospital in Washington state. One of her co-workers died of the same disease within a few weeks of Anna’s death. Her dog died with similar symptoms 18 months later.

As Dwight, the surviving husband, explained, “I would like this story told because I believe this is a MUCH BIGGER problem than we know. None of Anna’s doctors ever mentioned anything about the prion risks to other people.” The following commentary reflects the opinions and perspectives of Mr. Dwight Henderson as he reflects on his wife’s final months and his noble battle to stand by her side at all costs.

Unfortunately, millions of others like them have been battling the mystery of dementia and prion disease in the dark. This is Dwight’s opening salvo to shed some light on the crisis for others to see. Read more

Prions are unstoppable. They kill everything in their path—man or beast. Prions are highly infectious and the bodies of victims become highly infectious. Blood, milk, meat, urine, saliva, feces and other bodily fluids of victims carry prions, which can cause Creutzfeldt-Jakob disease.

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