CJD is a disease caused by a misfolding protein, called a prion. (PREE-on) Prion diseases are a group of rare and fatal brain diseases which occur in both humans and animals. In humans, it is known as CJD. Cows get BSE, which stands for bovine spongiform encephalopathy. Deer and elk contract CWD, or chronic wasting disease. There is no cure or clinical diagnosis for CJD. There is no cure for any of the diseases in the prion family.

Thursday, April 27, 2006

It’s National Prime Rib Day

My local Fox station says today is “Prime Rib Day,” and who decides this, I have no idea. Perhaps Rick Berman and his deep pockets are behind it, or perhaps the murderous ConAgra is behind it. ConAgra let consumers eat E. coli-tainted beef for two days while more tests were run on the suspect beef. People died because of it and others suffered permanent kidney and liver damage. Possibly some other large beef company is behind it. But at any rate, today has been declared “National Prime Rib Day.” I assume you’re supposed to celebrate it by blindly going to a supermarket to buy a cut of prime rib for dinner.

Less than one percent of beef in America is tested for BSE, therefore, you have no idea what you are eating. You have no idea if that prime rib you are about to eat was tested for BSE or not. You don’t know what you are eating when you eat beef.

Don’t ever blindly go to any store to purchase beef. Don’t just buy any cut you see. Go to a store that sells “natural,” grass-fed or organic beef if you must eat prime rib today. The problem with prime rib is that the meat is on a bone – the rib. Prions in meat hang around in nervous tissue and that means around bones. Always avoid cuts of meat exposed to bones and nervous tissue. Always! Unless you know that cow you are about to eat was raised on a small farm you can trust and fed grass it’s entire life. Organic is really best, if you must eat meat.

Personally, I will never eat meat again. Many CJD families are this way simply because we won’t give money to the beef industry so that it can keep killing its consumers. The beef industry and tobacco industry are the same in my book. Both still hock deadly products despite research proving their products are deadly. At least the tobacco industry admits to it now.

Monday, April 24, 2006

This Site Makes No Money

There will be no Google AdSense, banners, mall links etc. on this blog because I am doing this blog as a public service to other people like me who have lost a loved one to CJD. I am here to simply network and exchange information with anyone interested in prion diseases. That includes CJD families, doctors, nurses, funeral homes, cattle ranchers, politicians, the news media, educators, and researchers.

You can reach me at heatlarson@yahoo.com with questions, complaints, comments and suggestions.

The other plan I have for this site is to post all original content. You may repost it with my permission. It is perfectly acceptable for you to link to my site as well. Check back frequently for updates!

Thanks,

Heather Larson
Phoenix, Arizona

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What Terry and I Talked About

Terry Schwan and I talked about so many things last week when we met face to face, so I’m going to be blogging about each topic individually. The thing Terry said to me that stood out the most was about the U.S. blood supply.

But first, some background on Terry and I. I lost a parent, my mother at the age of 56; Terry lost a son Jeff at the age of 26. My mother’s case was familial, while Jeff’s autopsy showed his was sporadic. Technically, a 26-year-old isn’t supposed to get sporadic CJD. When someone this young dies of CJD, it is usually the variant form, or the form caused by BSE in meat. Terry thinks Jeff could have contracted an American strain of BSE...how else could you explain how someone so young died of CJD? And there are many who have died in thier 20’s, 30’s and 40’s of this disease that no one is really safe. This disease defies all odds and acts unpredictably. With so many clusters of the disease and so many young people dying of it, I hope I can tell as many of their stories right here, at CJDTalk.

If you want to read more about Jeff Schwan, check out this story I wrote for the Paradise Valley Community College newspaper, the Puma Press:

http://www.pvc.maricopa.edu/puma/may05/cjd_schwan.html

Now, onto the blood supply issue. Terry and her husband Steve won’t be attending the family conference this year in DC as I will be, but they have attended in the past and say it keeps getting better every year. Since I plan to go this year and since it will be my first time attending the conference and going to Congress, Terry gave me some advice.

The great thing about going to the CJD Family Conference is that you get to talk to the doctors and ask questions. Everyone gets to share ideas and insight, and you get a chance to meet Dr. Gambetti from the NPDPSC. (He did my mom’s autopsy). Then you get to go to Congress so you can have a shot at making your state’s representatives care about CJD issues in America.

Terry told me there is one certain way to get the attention of doctors and politicians alike – tell them how much this disease is affecting the blood supply. That’s when their ears perk up, so this is what I will talk about. Terry said to imagine how many people are no longer allowed to donate blood in the U.S. because they lost a family member to CJD. I am not sure what the estimate would be, but it would be huge. Everyone who has lost a family member to CJD is forbidden to donate blood. I can’t donate blood, organs or tissue. Ever. Period. And I don’t even know if I carry the mutation. I don’t even know if I’d be allowed to donate blood if it were proven I didn’t carry the mutation. I just know my mom died of fCJD, confirmed by Dr. Gambetti’s autopsy at the NPDPSC.

So that will be the angle for CJD families to take, seeing as it has worked for Terry. I will be lobbying for research for a cure in humans and animals. (I know, why not shoot a little higher, right?) My state, Arizona, doesn’t have a lot of problems to contend with. CJD is a reportable disease in this state, we are overrun with hospices and care centers since our population is so grey, and we have some of the best hospitals. (Barrow, Mayo). I had no trouble arranging my mother’s cremation since I live in a big city. So my choice is to lobby for biotech and other research into CJD. I may talk to a friend about lobbying for CWD-related issues too, but then again, that may be for another time.

Tuesday, April 18, 2006

I'm very excited!

Tomorrow night, two CJD families will be getting together and meeting for the first time here in Scottsdale, Arizona. That would be my family and that of Terry Schwan. I lost a parent, Terry lost a child. I can't wait to swap stories. I also can't wait for my dad to see - live and in person - other people like us. He hasn't delved into meeting others and learning about CJD as much as I have. I'd say he's vaguely interested in the details at best. I guess that is how I take after my mother; I have to know everything about the disease. I need to know everything there is to know about what killed my mother. She was like that. She always wanted to know the facts about everything so she could tell everyone else about it. I bet she would have made a great journalist!

Monday, April 17, 2006

My license plate


This is how I drive around Arizona, with my CURE CJD plates. I have to get the message across somewhow, and in every way I can possibly think of. If you have a CJD plate, llicense plate frame, t-shirt or bumper sticker, please take a photo and send it to me! I'll post a photo of how you are taking the CURE CJD message to the streets and will also tell you story. You can e-mail it to heatlarson@yahoo.com.

Monday, April 10, 2006

Share your ideas

Please let me know if there is something that you want to know more about, something you'd like me to talk about or if you have any questions about CJD. I'm working on an upcoming CJD Talk podcast, not sure when it will be out, but it will be soon!

Sunday, April 09, 2006

About Me:

I lost my mother to fCJD on November 10, 2004. It was the day after her 56th birthday. So no, this is not a "disease just for old people." People of all ages die of CJD every day, all over the world. Since my mother's death in late 2004, I have met many other CJD families and have learned a lot about the disease. I made the personal choice to become vegan. I volunteer for the CJD Foundation.

What is CJD?

CJD is a disease caused by a misfolding protein, called a prion. (PREE-on) Prion diseases are a group of rare and fatal brain diseases which occur in both humans and animals. In humans, it is known as CJD.

Cows get BSE, which stands for bovine spongiform encephalopathy. Deer and elk contract CWD, or chronic wasting disease. There is no cure or clinical diagnosis for CJD. There is no cure for any of the diseases in the prion family.

According the CJD Foundation, “The sporadic form accounts for approximately 85% of the cases, the familial form approximately 15%.” The Foundation also says, “Most of the cases are "classical" or "sporadic" CJD (sCJD), occurring for no, as yet, known reason.” When you hear people talking about vCJD, or variant CJD, they are talking about the form that affects typically younger patients and is, in fact, caused by eating beef tainted with BSE.

There is no treatment for CJD. Once it is determined that is what a patient has, doctors can only make every effort to keep the patient comfortable until death, which can come in a matter of days, weeks or months. The length of the disease depends on the mutation.

All prion disease are considered spongiform encephalopathies. Encephalopathy means “brain disease,” and “spongiform” means sponge-like. In other words, the brain becomes filled with holes and looks like a sponge under the microscope.

As the brain is being eaten by prions, the patient will shake, experience mycolonus, hallucinate, become incontinent, lose the ability to walk, lose the ability to speak, experience aphasia, and other symptoms that are very difficult for a family to watch.

My mother died one month and two days after being taken to the emergency room for what we thought were the symptoms of Parkinson’s disease or multiple sclerosis, maybe early-onset Alzheimer’s Disease. Never did we think she had a fatal disease that would kill her in weeks.

Very Inflammatory Article!

Copy and paste this link into your browser for an especially inflammatory article on the NPDPSC:

http://www.sciencedaily.com/upi/index.php?feed=Science&article=UPI-1-20051021-21481300-bc-us-cjdcases.xml

I have a link to the NPDPSC to the right, by the way. I always wondered how that Patrick Hicks case was going to work out. Last I remembered hearing, the family was having trouble coordinating the lab work in France. If you read the article, well, it sounds like the trouble never ended!

The article is interesting and just another shining example of how our national surveillance system seems set up to fail most of the time. With genetic cases like my mom's, it's easy. The Center knows what it is getting in the mail when it knows it is getting tissue from a 56-year-old woman of Polish descent who already lost two relatives on her paternal side to CJD.

I'd sure hate to have to deal with a "sporadic" diagnosis in my family. I watch these sporadic families go through a lot of hell trying to get answers about how their loved ones died. I guess I have it easier since there is even actual research out there on E200K, 129M.

Tuesday, April 04, 2006

When it isn't CJD

Part of what I hope to accomplish with this blog is networking. If someone going through the uncertainty of losing a loved one to CJD finds this blog and gets to have some questions answered, I've done something right.

CJD is extremely difficult to diagnose, and no clinical diagnosis exists. The only sure way to find out if a patient has CJD is to perform an autopsy after death. This takes forever and is done by the NPDPSC, or National Prion Disease Pathology Surveillance Center in Cleveland, Ohio at Case Western Reserve University.

But sometimes, a patient shows every possible symptom of CJD -- only it isn't CJD. Over a year ago, I met a local women whose mother in law was dying of something I was pretty sure was CJD. The woman had symptoms like myoclonus, balance problems and I can't remember what else. Whatever she had progressed fast, leading everyone to believe she had CJD. An autopsty after death showed it wasn't CJD. It was Parkinson's or something like that. At any rate, I lost touch with this woman who lost her mother in law. I don't blame her. The world of CJD is so dark, I don't blame people for running away from it. The eerie thing was that I talked to this woman over the phone and walked her through the dying process of CJD as it happened to my mother and her mother in law died exactly the same way! Step by step, she died like my mom did -- about 48 hours after the feeding tube came out.

This year though, we witnessed a sort of miracle with one family. I guess a miracle would be finding the cure, but I'll call this a miracle nonetheless. A man in South Carolina joined our support group, CJD Voice, looking for answers. Everyone in the group, self included, seemed to think the man's father in law had CJD. He had every symptom in the book. But his family kept digging for answers and battling with doctors. (I have been there!) Recently, the man's suffering was found to be caused by psychological problems. He was given meds, anti-depressants in strong doses, if I remember correclty. He's fine now. It wasn't CJD. I still talk to this man in North Carolina about his father in law.

When my mom was dying, I read somewhere that there are about 600 rare neurological conditions. It never ceases to amaze me what a mystery the human brain is.

Sunday, April 02, 2006

My new friend

On Saturday, April Fool's day, my boyfriend and I went to eat at an awesome restaurant in Scottsdale, called Leo. It's on Scottsdale Rd., just south of Old Scottsdale...can't remember which street, sorry. At any rate, we'd both been meaning to try it for ages. So we go in and get this waiter, Paul. I am shocked and impressed when this guy starts talking about mad cow disease! He starts rattling off information about testing cows for BSE. He asks my boyfriend how many cows get tested in Japan, and of course he doesn't know. So he asks us how many cows in the U.S. are tested? One in 90, he says. I am so surprised a regular person (as in someone who has never seen CJD) actually knows anything about BSE, much less cares. And he complained about how it is illegal for the government to tell you where the cow came from. You never get that tidbit of information when a cow tests positive for BSE. Ever notice that? It always manages to leak out somehow anyway. Rumors like that are hard to keep quiet!

Center for Consumer Freedom Is Lying

Now the AdSense has placed an ad for the Center for Consumer Freedom on my page. This website is about the worst meat p.r. out there! I am having a little too much fun with the Google AdSense today, as you can tell. I'll turn off the content matching tomorrow with some help from my guru, but I can't figure it out today. Until then, I guess here's another opportunity for me to educate. The Center for Consumer Freedom is all public relations. Don't believe a word of it!

Here's a link to the REAL center for consumer freedom, the Center for Media and Democracy:

http://www.prwatch.org/taxonomy/term/107/9

Do beef companies really pay for advertising to promote their products regardless of what they will do for your health? Are you kidding me? Of course they do!

Actually, Mad Cow USA is one of the best books out there. Sheldon Rampton and John Stauber really did a lot of work to get you the truth.

Irony

I guess you can take it as an April Fool's joke, but yes, I am aware that my Google AdSense has placed an ad for steaks on my page. Even I can appreciate the irony in that and have a laugh. Just make sure you're going to enjoy safe steak, like safe sex. Know where your meat came from, where it has been, what it was fed in its lifetime...etc. Make sure your steak is at least grass-fed if not organic! Hey, as long as I'm going to advertise steaks, I might as well do it right and give the disclaimer! Beef: eat at your own risk! By the way, I am a vegan. That's how much steak I eat--NONE!

The E200K, 129M Club

One of my goals with this blog is to find and network with other E200K, 129M families. I found one man in Chile in the same age range as me, who lost a parent in the same age range as my mom. It's a small world when you're E200K! There's a big language barrier there, unfortunately, as my Spanish isn't great and his English isn't either. Right now, I'd like to gather as much information on the E200K mutation as I can possibly find.

I don't wish to be tested to see if I carry the mutation myself for a variety of reasons. I would only want the answer if it wasn't a death sentence. I've got a 50/50 shot. I guess those aren't terrible odds!