Cure CJD

Heather Larson's experiences in helping find the cure for CJD

A question about consciousness & CJD

I am cutting a pasting a question left on another page of this blog today in hopes some people will stop by and leave their answers to it because I know I’m probably not the best person to answer this one.  I attempted to on the page where it was left, but I just didn’t have this same experience with my mom as she died of CJD.  I am also not altogether sure that this issue of consciousness and the talking rules out CJD (or proves the diagnosis should be CJD  —  and we all know there’s no clinical diagnosis).  So I’m cutting and pasting this here and I’m going to ask around to see if anyone can offer a better answer to someone in need than I can:

“My father-in-law is lying in a hospital bed right now. Mute, blind, incontinent, with a feeding tube, and unable to move except for the spasmodic jerking movements of his arms & legs. Sic weeks ago he was holding his great grandson and cracking jokes, going up to his cabin with his dog and working on the fence. He’s in the VA hospital at home now, after being flown to the Denver VA, where the doctors diagnosed him with CJD (but not definitively – of course). We’re waiting for the spinal test to come back (testing for the 14-3-3 protein). So far – they haven’t seen the indications they might see on an EEG. Basically – the only thing they are basing the diagnosis on is his symptom list, the rapid progression, and the fact that EVERY OTHER test they’ve done has come back completely normal (including a full-body CAT scan, MRIs, blood tests, etc.) I have a question for anyone who has been through this with someone: He has “woken up” about three times in the past few days, and said something completely normal (after not having said a word for over a week). The family is holding on to that fact – as a possible indication that this is NOT CJD. Has anyone ever seen momentarily lapses of apparent consciousness in patients with CJD (when symptoms are this progressed)?”

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August 3, 2010 - Posted by | Uncategorized | , , , , , , , , , , , , ,

9 Comments »

  1. Heather, please just let that family know that even though she may seem unconscious or even comatose, as in the case of my sister, she can hear and understand them, at least at times. My sister became paralyzed and for the remaining 8 days, we thought she was comatose. She was not. Tears formed in her eyes two days before she passed and she had been without fluids for 6 of those days when I was describing my love for her and other things.
    Just let them know she is hearing them and at times at least can comprehend.
    Karen

    Comment by klmh | August 3, 2010

  2. Thanks, Karen. Your response will hopefully helpful and it’s what I’m looking for. Please let some others know to leave comments about their experiences too.

    Heather Larson

    Comment by CureCJD_Heather Larson | August 3, 2010

  3. Heather – My husband lost his speech gradually from month 4 through month 6. In month 7 he lost swallow and developed myoclonus (muscle jerks). At the same time he developed aspiration pneumonia due to the loss of swallow — which was related to the lost of voluntary motor control — and I don’t remember him speaking after that except perhaps once in response to a photograph of our son.

    As with this fellow, they tested my husband for everything under the sun since he was only 52 and they did NOT suspect CJD for a long time. West Nile, Hashimoto, a kind of cancer that mimics dementia, and so on. The important thing to tell her is (1) leave no stone uncovered and make sure he is taken to a hospital like Mayo or Barrow or Johns Hopkins where they have seen many cases of CJD and other diseases that overlap, some which are treatable (2) 14-3-3 positive is not definitive since a positive could be caused by many other conditions.

    In my husband’s case, the diagnosis was made on the basis of the typical EEG plus the disease progression and symptoms as well as having ruled out every other possible condition. He had several inconclusive EEGs at the local hospital but when he went to Johns Hopkins, they took him off of all sedatives which were messing up the EEG results, and voila, his EEG was typical for CJD. UCSF claims that they can diagnose CJD from specific MRIs also. So the two things I have heard that are accepted as final diagnostics other than brain biopsy (not recommended) are EEG given under the right conditions and MRI done by and read by certain experts.

    Danny spoke for 6 1/2 months after disease onset. He probably had some awareness for another 1 1/2 – 2 1/2 months. After that, he no longer turned his head when we entered the room to the sound of our voices, nor gave any indication whatsoever for the remaining 3 months that he had any higher brain functions.

    Comment by Hatte | August 3, 2010

  4. My stepdad died a year ago from iatrogenic CJD within the span of 41 days. We, and some of the doctors, believe it was introduced during an elective back surgery when he developed a spinal fluid leak, which was patched with bovine tissue. His first symptoms presented 3 weeks after a neurostimulator implant last July. It is believed that the electric impulses from the stimulator triggered the prion, and the rest was history.

    The first couple of weeks was rapid deterioration of comprehension, vision and ability to swallow and respond to questions. The last week or so, he was on a lot of morphine to reduce the spasms and relax his muscle contractions. He was also in and out of lucidity. Toward the end, we asked the neurologist whether he could hear us, or understand us. She said she was almost sure that all of his faculties were gone by that point, but if it made us feel better, to keep talking to him – which, of course, we did. The way she explained the progress of the disease was, that it eats away at the outer brain (short term memory, etc.) and works its way through to the inner core – which is why long term memories seem to resurface.

    CJD is one of the most tragic, nightmarish diseases out there. I hope a cure is found soon. There is a company in California who has found some success in retarding the disease with the medicine used in malaria treatment – let’s hope they are successful.

    Comment by Kjirsten | September 17, 2010

  5. Kjirsten,

    I’m sorry to hear about your stepdad. Hatte, who left the comment above yours, also lost her husband to iatrogenic CJD. Your stepdad’s span of illness was much shorter than her husbands; I am always struck by how fast or how slow the disease progresses in different patients.

    Do you know the name of the malaria drug and who’s testing it?

    Heather Larson

    Comment by CureCJD_Heather Larson | September 19, 2010

  6. Reading this almost broke my heart. My father is currently in the same condition – has been for 5 weeks now. Today I was informed they think sporadic CJD is the most likely and they’re doing another spinal tap to check for 14-3-3.

    My father has also had moments of complete clarity in the first 3 weeks of hospitalisation, which gave us hope that he would pull himself through (the doctors were advising encephalitis was a possibility at this point) but in the last week, all he’s been able to do is cough, yawn and snort (I think this maybe due to the nasogastric tube blocking his nose in some way).

    I would say at this stage my Dad knows we’re there – he squeezes our hands when we speak and his mouth moves a lot, almost as if he’s talking but no words are coming out. And similar to your father-in-law, not more than 6 weeks ago, he was walking to the shops on his own and playing around on the internet.

    I believe we’re at the same stage as you as I type this. I’m more than happy for Heather to give you my email address if you think anything I can tell you may be useful.

    Hoping and praying that both our family members will be delivered from this evil.

    Regards,
    Suzanne

    Comment by Suzanne | September 24, 2010

  7. Hellow
    I have a terrible cjd phobia with depersonalization/derealization disorder. That fear started from early September. I am constantly thinking about little symptoms, such as little spasms, slight mental problems when when I am tired, stressed, have more intense dp/dr symptoms, etc Also I have pain or slight weakness of limbs and, most distressing, terrible anxiety and think that all these are cirtainly cjd. Can cjd have a so slow progression? Also, if someone has cjd in mind, will when hypothetically show the illness know it? Does a cjd patient know that he/she is not well? Finally, is the possibility of inhaling prions or amyloid from an Alzheimer’s patient real. Because one lives next to mine appartement. I do not have any family history or know anyone, or at least heard of anyone die from that. But I had a corneal transplant 4 years ago.
    I have a fear for this neurodegenerative diseases because they are completely out of our control and make us to lose our control as well. Thanks.

    Comment by bolko | November 20, 2010

  8. My mum is in the terminal stage of cjd; we don’t yet know which form and so I have asked for a blood test for genetic cjd to be carried out. Of greater priority at the moment is dealing with the rapid deterioration of my mother. She too is mute, incontinent, immobile apart from the jerking and twitching that goes with this hideous disease. It was a while before it was diagnosed, and I did get utterly frustrated with the attempts by doctors to explain her puzzling symptoms as caused by a urinary tract infection!I think she still knows us; she has a smile and soft eyes for us, but anything she tries to say is an inaudible and unintelligible murmur. I am terrified that she is locked away with knowledge and fearful awareness of her condition; this is not something any of her nurses or doctors can be sure of. However, the likelihood is that family and long-term memories are longer-lasting than the short-term memories. Now she is mute it’s easy to imagine she is thinking ok; however, I console myself with the fact that 2 months ago, when she was suffering more from cerebellar ataxia than any other symptom, she was forgetting things I ‘d just said and believed she was living in our kitchen, when she was of course in hospital. So logically, her understanding and self-awareness is going to be even worse now. I am devastated by her illness and its rapidity and am trying not to think of the possible implications for me and my daughters if it’s genetic.

    Comment by Hilary | January 13, 2011

  9. my dad died in october of sporadic fit and healthy 58 year old when it started i get where youre coming from i too hope they find a cure seeing my dad like that will haunt me we loved him so much x

    Comment by sarah trainer | January 29, 2011


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