More CJD legal news

This is a nice example of things going right. CJD needs to be reportable in every state so that we can accurately track the disease. Since CJD isn’t yet reportable in all 50 states here in America, we cannot accurately tell anyone how many people are really dying of this disease. As the article also points out, sometimes doctors aren’t well trained and miss it. They misdiagnose CJD as something else. I have noticed this is especially true in smaller cities – not so here in Phoenix where we are lucky enough to have Barrow Neurological Institute.

The link again: WTHR, Indiana

And a link to Indiana Representative David Orentlicher: Send him a note to say thanks and give encouragement.

Here's something you don't see everyday

Aside from the snow in Malibu and the rain in Phoenix, a widow in the UK is suing the government because her husband died of CJD after undergoing a government-sponsored treatment with growth hormone. Children with growth hormone deficiency were targeted for treatment in the national program between 1979 and 1985 with growth hormone extracted from corpses.

You don't see this type of thing every day; seldom is a family able to find a reason to sue their government in a case of CJD. It's pretty rare when you're able to pinpoint how one acquired the disease and even more rare that it would be the government's fault. I don't know about you, but I'd rather be four feet tall and live a long life than be "normal" and die of CJD. The question is, should the UK government have known better back in the late 70s and early 80s?

Read the article in Sheffield Today.

The realities of vCJD infection via blood transfusion

This was just posted in the comments section of my last post, but it's good enough to be center stage in its own post. A new, and fourth, case of vCJD via blood transfusion has been diagnosed in the UK.

Here is the link to the artcle on the Health Protection Agency website.

Makes you wonder a lot of things! It also makes you feel horrible for those other 23 people who received blood transfusions infected with vCJD. Will they get sick? When? Are these first four patients to contract vCJD from blood transfusions M/M? Are they the first wave of what will be many more?

Could two more waves of vCJD be coming?

This article in The Guardian talks about how there could be two more possible waves of vCJD to come in the future. It’s a good article, pretty long, but worth reading because it’s filled with stats and facts.

I’ve been wondering about titer lately, as in how much of the infectious agent can make you sick. If I eat one infected steak, is that enough? Or must I eat infected steak a few times a week every week for years in order to get infected with BSE and thus come down with vCJD? Nobody really knows that answer to that as far as I can tell.

(My example is purely hypothetical, as I don’t eat steak and my family carries the E200K mutation. It doesn’t matter what I eat at this point, it just matters whether I carry the marker – again, as far as we know to date. I wish there were research done specifically on this).

If I get one pint of blood in a transfusion that is infected with vCJD, is that enough to infect me? Apparently, yes, according to this article. And that is just the point of this article. Who cares about steak and cattle anymore when an indeterminable amount of human beings are carrying the infectious agent and unknowingly giving blood?

It is a huge problem.

That brings me to my next point. There are two possible answers to the problem of the second wave:

1. Come up with a blood test for vCJD.
2. Cure CJD!

The blood test for vCJD would be a great advancement for blood donation. But I feel the only real answer is to just cure the damn disease. End of story. A cure for CJD would end all these issues. It would also save my life (if I carry the marker) and the lives of others like my relatives who carry the E200K mutation or any other mutation. This disease isn’t just about cattle; some of us don’t need beef to get sick. We’re the ones who will need the cure someday regardless of whatever political policy changes do or don’t get made.

Read the article here. One more item of food for thought, taken from the article:

"The fear of some researchers now is that, 20 years on, interest in answering the crucial remaining questions will wane. But leaving them unanswered would be a desperate mistake, says Professor Bird. "If that happens, we won't have all the information, and that's extremely dangerous.""

Familial CJD Support Group tonight, 8-9:30 pm EDT

Please join Deana Simpson TONIGHT for the Familial CJD online support group.

Deana will guide the discussion as well as answer your questions.

As you will see when you go to the website, the CJD Foundation has a new look. To access the Support Group click on Family Support and then in the 2nd line there is a link to the Chat Room. When the next page comes up, give it a few seconds for the actual chat room to come on the screen.

You will enter the room with a Guest number. To change that to your name, just click in the little box that has Guest in it and another small window will open asking for your screen nickname.

The group runs from 8:00 p.m. to 9:30 p.m. EDT.

Grand rounds today at Barrow Neurological Institute

Today went really well. I estimate there were 50-60 physicians in attendance, my dad thought it was more like 80. I definitely learned a couple things! Dr. Sejvar was wonderful. He was very nice and so well-informed that it was good to hear him speak.

He let me have a few minutes at the end to talk about the CJD Foundation, so I explained what the Foundation offers and how the folks there can help you with the dreaded autopsy – all for free.

The importance of autopsy
The key thing is you don’t have to pay for an autopsy in a suspected case of CJD. The Foundation will hook you up with the NPDPSC and they’ll take care of it for you. Not every family knows this and not every doctor does either. It’s important for the surveillance of the disease in our country.

But in my case, it gave me the genetic answer I needed so badly. So I’m very grateful we were able to get an autopsy done on my mom. It was tough to get done because she was dying so fast and we were having a hard time finding someone who would do the autopsy. The NPDPSC finally found someone in New Mexico to do it and my mom’s body made a posthumous trek there and back! But the information from the autopsy was priceless because, as far as I know, I am the first person in the family to obtain it.

What I learned today
Two things in Dr. Sejvar’s presentation really made sense. I’m sure I’ve griped here on the blog before about a lack of a clinical diagnosis with CJD. It makes you waste precious final days with your loved one as doctors try to diagnosis the problem with a million tests. If only there were a diagnostic test that could give the answer sooner so that families wouldn’t have to go through so much run-around in hospitals!

One of the tests my mom endured a couple times was the EEG. It didn’t show what we were looking for at all and, at one point, even showed improvement in her brain. That is something I have never understood because I was seeing a decline at that time but the EEG was coming back saying she was fine. Dr. Sejvar today explained that sharp waves in the EEG present at some point in the illness but are less pronounced as the disease progresses. That explains why the EEG looked fine in her final two weeks with us – it was the end of the disease’s progression.

Hey – that’s one question answered. It’s not huge, but I had an “a-ha!” moment. I’ll take them as I can get them!

The second test my mom endured as they tried to diagnose her was the spinal tap, or the 14-3-3 protein test. That test came back negative. Guess what? Sensitivity of that test decreases as the duration of the disease progresses. That makes sense now, too, because it was done towards the end of her quick battle with CJD. So another mystery was solved today in my mind.

A new point of view
I was pleasantly surprised to learn a new point of view today – that of an OR nurse. She approached me after the auditorium began to clear out and asked me if my mom had had a biopsy done at Barrow. Yes, I said. She asked when, so I told her it would have been the end of October in 2004. Her face darkened and I could tell she was mentally trying to figure out if she would have been in the OR that day.

Then she asked me how I felt about the biopsy and if it was something I chose to do. I said not really, it was more of a matter of being told by Dr. Walker that this was what we needed to do. I didn’t question it at all. I explained to the nurse that this was because we had two possible diagnoses for my mom at the time; either she had cocci meningitis (valley fever infecting the brain), or she had CJD. She was being treated for the cocci but we needed to know one way or another what we were dealing with and that’s the case for doing the biopsy. You need to know if the disease is CJD or something else that you can cure. I do believe in doing the biopsy for that reason.

Remember: you cannot definitively diagnose CJD without an autopsy.

How the OR nurses feel
This is brain surgery and it does have some risk to the patient. Why possibly mame a young person for life if it doesn’t turn out to be CJD? That was the point the nurse brought up. She was curious because she didn’t know what the patient or the patient’s family is dealing with outside the OR. She says sometimes in the OR they get these orders and wonder if they are really necessary. It can cost the family a significant amount of money and is extremely invasive. (My mom’s was covered by insurance). For the nurses, the risk of infection is frightening and that is an understatement.

I could see where you would question the necessity of doing such a surgery as a nurse. Your health is now on the line by being in the room. You could be infected with the fatal disease. Of course you’d be asking yourself, “how important is this? Is it really worth me risking my life for another person?”

I guess I had never thought about that before. I’m grateful to that nurse for bringing it up. That’s yet another reason why we need a clinical diagnostic test so badly! The nurses don’t want to deal with the drama any more than the doctors and patient’s family want to deal with it.

In closing...
Jeanne White was there too with my father and I and I’m glad she came. It was also nice to see the doctors we had worked with two years ago when my mom was there. It was a reunion of sorts! I am glad to see so many people care about this disease enough to spend an hour with us today. The CJD Foundation was well represented, so I’m confident patients who come to Barrow will be taken care of properly. I think they already are. Barrow sees cases from all over the Southwest all the time. Dr. Walker even told me there were once two cases in one week!

We really have it easy in Arizona because of Dr. Walker and Barrow Neurological Institute. Dr. Walker knows what she’s looking at when she sees a case, she knows all about the Foundation and about the NPDPSC. CJD is also a reportable disease here in Arizona. We’re so far ahead of other states that it makes me feel good. That means all of us here can concentrate on other things related to CJD, like Grand Rounds.

I'm very excited about tomorrow

With the holidays finally past and the week nearly over, I finally get to steal a moment to blog about CJD. I haven't had a chance to read the full study yet, but scientists have found an exciting new way to make bovines resistant to BSE using genetic engineering. It's actually been big news in the mainstream, too! I was glad to see that. I got e-mails from friends asking if I'd seen it and phone calls as well -- and even from people who aren't in a CJD family like I am.

Every breakthrough counts! I think we may be entering a very optimistic age in prion science. The research is advancing quickly. There is hope.

Tomorrow, my father and I, along with Jeanne White (Jeff Schwan's aunt) will be at Barrow Neurological Institute for grand rounds on CJD facilitated by Dr. Sejvar of the CDC. Maybe this is a good place to post (once again) the links to my stories on Jeff and my mom:

Jeff Schwan

Phyllis Larson

Jeff's story resonates with me right now because a young man of age 18 is dying of this horrible disease right now in Virginia. While my mother with her age of 56 and genetic mutation didn't have much of a chance against this disease, Jeff should have at the age of 25. The young man in Virginia should have a better chance too. There is no reason why the very young should die of this disease. It boggles my mind! Not to mention Charlene Singh and the many others, some in their twenties, some in their thirties and some in their forties... Even 56 is young for CJD.

This is why we must keep searching for answers.

I wish you peace, hope, and advances in science for all of us in 2007!

Heather Larson