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Contributor
Posts: 36
Registered: ‎04-07-2015

When my dad was diagnosed that's when I found out there are 3 forms of CJD. First there is variant CJD. That is the one that caused the beef eating scare in the UK. Then there is sporadic CJD which is what killed my dad. That just happens spontaneously in a person, no known cause and the most common form of CJD. Lastly there is heredity CJD. The worst one of all, I'm my opinion. You can carry the gene for 50 years and not know it. If

You have the gene you have a 50% chance of contracting it. CJD is rare but it is often missed diagnosed as advanced Alzheimer's or Parkinson's. CJD  has no treatments and there is no cure. It is always fatal. A person usually passes within 1 to 3 months after

Symptoms appear. 

It is a heartbreaking way to see your loved one die. So much suffering. To see my dad's beautiful blue eyes turn completely white due to blindness 

Was just so sad.

Trusted Contributor
Posts: 1,923
Registered: ‎03-10-2010

Thank you for posting on this important topic.  Sometimes we get distracted by so many things happening in our lives that we forget about things we learned previously.

 

What is most disturbing about the posts on this topic is that I cannot imagine there are millions of QVC forum participants, but there are a few among us who know someone who has been diagnosed and who will die from CJD.  It is 100% fatal.  

 

Years ago, I read a book by Howard F. Lyman called Mad Cowboy, Plain Truth From the Cattle Rancher Who Won't Eat Meat.

 

It is my belief from what I read years ago that eating red meat from animals that are forced to be "cannibals" (by the meat industry that feeds their food animals ground up dead animals that may be ill with prion disease), is the cause of human CJD. 

 

If people consume prions from improperly fed animals (essentially animal cannibals), they are at risk of CJD, too.  Only the meat industry denies this.  If you eat meat, it is particularly important to eat grass fed beef.  England and France had a huge problem with CJD in the late 1980's.  That this is happening in the USA, even now, indicates there is a problem with the feeding of cattle raised for food in the USA as well.  That is sad and very disturbing.

 

People who are methionine homozygous at codon 129 in their genetics are the most susceptible to developing CJD in their lifetimes.  Recently, those with valine instead of methionine were also found to be susceptible to prion disease, and now it is suspected that many cases of CJD may have been missed as a result.  Alzheimer's and Parkinsons Disease have many similar features.

 

https://jamanetwork.com/journals/jamaneurology/fullarticle/791776

 

Thank you for confirming that diagnoses of CJD are still happening in the USA.  This fact should be headline news, yet it is not.

 

"the significance of codon 129

The gene for the prion protein has been identified and examination of it reveals a common polymorphism at codon 129 for either methionine or valine. This has relevance to both iatrogenic and sporadic forms of prion disease.

In the caucasian population approximately 38% are homozygous for methionine alleles, 51% are heterozygous and 11% are homozygous for valine.

  • in all cases of CJD, including sporadic CJD, there is an overrepresentation of homozygosity at the polymorphic codon 129 of the prion protein gene
    • in sporadic CJD
      • approximately 85% of the patients are homozygous at codon 129, compared to about 50% in the general population in Europe, among whom two thirds are genotype methionine homozygous (Met/Met), indicating that homozygotes have a higher risk of developing CJD compared to heterozygotes
    • in iatrogenic CJD
      • in CJD caused by cadaveric pituitary human growth hormone the overrepresentation is due to the val/val genotype (31% compared to approximately 11% of the normal population) and not to the methionine homozygosity (Met/Met) seen in most other cases of CJD
      • codon 129 methionine homozygosity has been found in 74% of patients with CJD caused by dura mater grafts"

Reference:

  1. Pedersen NS, Smith E. Prion diseases: epidemiology in man. APMIS. 2002 Jan;110(1):14-22.

 

Even blood transfusions from infected people, and the use of contaminated surgical and dental instruments and the use of growth hormone from infected people can give rise to CJD and variant CJD infections in others.  

 

Sorry for the length of this post. Very sorry for your friends and loved ones who contracted this horrific disease.

 

 

 

 

Respected Contributor
Posts: 4,807
Registered: ‎03-10-2010

My mother-in-law died from Sporadic CJD in 1973.  She deteriorated quickly and was diagnosed by a brain biopsy.  Unfortunately she never woke up from the anesthesia and stayed in a coma for almost a year.  She was only 49 when she became ill and 50 when she died.  Never lived to see her grandchildren or even her daughter get married. DH & I had been married only a year.

 

More than a decade later someone won a Nobel Prize for identifying and describing prions.  I never, ever imagined I see a discussion about CJD on the QVC boards because over the years I've never run into anyone who knew what I was talking about if I mentioned it.  It's a terrifying disease.

Contributor
Posts: 36
Registered: ‎04-07-2015

I became obsessed with reading and watching you tube vides about CJD. I just wanted to

Learn everything I possibly could. No one in my family talks about it at all. I don't know

Why I feel the need to.

Honored Contributor
Posts: 8,913
Registered: ‎05-23-2010

@software wrote:

Creutzfeldt-Jakob-Disease-Fact-Sheet

 

I got a call from a friend yesterday, she told me a mutual friend of ours was in the hospital, diagnosed with sporatic CJD.    I had heard about it before just as general information but dfidn't know how serious a disease it is.

 

He will be released from the hospital this week to hospice.

I saw him about 2 months ago, he was vital, working.   Lots of energy, he always has something to say, big personality.   He's 65 and in otherwise good health.

 

She told me his symptoms are the same as in the attached article but she didn't know exactly how he was diagnosed.   


CJD, kuru, mad cow disease and other diseases caused by prions has been a special interest ipif mine for over 35 years. The disease is as serious as possible. It’s very rare. There are five forms of CJD. The brain degenerates. There is no cure. I will provide you with two links about this rare disease.

 

 https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Creutzfeldt-Jakob-Diseas...

Honored Contributor
Posts: 8,913
Registered: ‎05-23-2010

Here’s my other link. I’m very sorry for the patient and your friend.

 

 

https://www.cdc.gov/prions/cjd/index.html

Respected Contributor
Posts: 4,807
Registered: ‎03-10-2010

@Mindy D wrote:

@software wrote:

Creutzfeldt-Jakob-Disease-Fact-Sheet

 

I got a call from a friend yesterday, she told me a mutual friend of ours was in the hospital, diagnosed with sporatic CJD.    I had heard about it before just as general information but dfidn't know how serious a disease it is.

 

He will be released from the hospital this week to hospice.

I saw him about 2 months ago, he was vital, working.   Lots of energy, he always has something to say, big personality.   He's 65 and in otherwise good health.

 

She told me his symptoms are the same as in the attached article but she didn't know exactly how he was diagnosed.   


CJD, kuru, mad cow disease and other diseases caused by prions has been a special interest ipif mine for over 35 years. The disease is as serious as possible. It’s very rare. There are five forms of CJD. The brain degenerates. There is no cure. I will provide you with two links about this rare disease.

 

 https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Creutzfeldt-Jakob-Diseas...


@Mindy DDo you mind my asking how you became interested in this group of extremely rare diseases?  Usually we only become aware of it because someone close to us is afflicted.  I wish more medical researchers were interested!

Honored Contributor
Posts: 25,366
Registered: ‎05-22-2016

Re: Sporatic CJD

[ Edited ]

I studied this disease when I went to grad school. It was a subject they discussed in the pathology department. I got to see cow and deer brains that were extracted from the dead animals. The brains were full of holes and they were noticeably smaller than the normal brains. 

Spongiform encephalopathy...looks like a sponge.

Honored Contributor
Posts: 8,913
Registered: ‎05-23-2010

@shoesnbags wrote:

@Mindy D wrote:

@software wrote:

Creutzfeldt-Jakob-Disease-Fact-Sheet

 

I got a call from a friend yesterday, she told me a mutual friend of ours was in the hospital, diagnosed with sporatic CJD.    I had heard about it before just as general information but dfidn't know how serious a disease it is.

 

He will be released from the hospital this week to hospice.

I saw him about 2 months ago, he was vital, working.   Lots of energy, he always has something to say, big personality.   He's 65 and in otherwise good health.

 

She told me his symptoms are the same as in the attached article but she didn't know exactly how he was diagnosed.   


CJD, kuru, mad cow disease and other diseases caused by prions has been a special interest ipif mine for over 35 years. The disease is as serious as possible. It’s very rare. There are five forms of CJD. The brain degenerates. There is no cure. I will provide you with two links about this rare disease.

 

 https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Creutzfeldt-Jakob-Diseas...


@Mindy DDo you mind my asking how you became interested in this group of extremely rare diseases?  Usually we only become aware of it because someone close to us is afflicted.  I wish more medical researchers were interested!


I have always had an interest in science since I was a small child. I am a retired high school science teacher certified in many science subjects. One college major I had was anthropology. I was fascinated by a tribe, the Fore, in Papua New Guinea  which incorporated canibalism in their funeral practices. Mny if the tribe members had a strange disease called Kuru. (Kuru is a rare disease that is caused by an infectious protein that is concentrated in the brain and nervous tissue of an infected family member.) Tribal members, especially the women, would eat tiny parts of their relatives. I never gave up my interest in anthropology and kuru. Years later, mad cow disease (bovine spongiform encephalopathy) was in the news. The symptoms were similar to kuru and I knew there had to be a connection. The puzzle had me hooked. At that time there was no no known cause but it was known that eating beef that was cooked did not stop the transmission of mad cow. When mad cow disease showed up in the UK, British beef was no longer imported into the U.S. The disease had a very long incubation period. It can show up years after exposure. So does kuru. In fact, one Fore tribesman had a fifty year incubation period before the onset of symptoms. Mad cow disease (technically bovine spongiform encephalopathy) closely resembled CKJD, which is now considered the human form of the disease. There is also a related disease in sheep called scrapie. It was proposed that MC, CJKD and kuru were not transferred by a virus or bacteria. The mode of transmission was thought to be a prion. There was no understanding of what a prion was at that time. Now it is known that prions are misfolded proteins that are infectious agents.  Currently, a related disease is spreading among deer worldwide. Wild deer  (cervids) and farmed deer are being closely monitored by the CDC here in the U.S. This emerging prion disease is named Chronic Wasting Disease, aka, CWD, or technically, transmissible spongiform encephalopathy. The disease has been shown to be transmissible via ingestion of the meat to macaques monkeys under laboratory conditions. So far, no transmission to humans has been found. 

 

 

 

 

startereading all the medical school textbooks of a lady I babysat for when I was twelve. From there I developed an interest in medicine. I’ve never stopped. My hobby is reading medical and science studies. I’m obsessed. 

 

 

Respected Contributor
Posts: 4,807
Registered: ‎03-10-2010

@Mindy D

Thanks for your reply. I think it's fascinating that you've maintained this interest all of these years. While nothing in your response was new to me, I had a personal reason to acquire knowledge about CJD.  Having a close family member die of a horrible disease that there was very little knowledge about was a terrible experience for the entire family and I wanted to know as much as I could about it.  I hope there are researchers out there who are driven by their own interest in CJD and and whose work will find a treatment. Again, thanks for your extensive answer!