Cure CJD

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

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  1. Thanks for your blog. I agree that it is important to raise awareness of prion disease, but also to ensure that people are given the facts, not simply scared by the sensationalistic tendencies of media. I hope to contribute to this education too, through blogging (which I am just setting up) and twitter. FYI, I am a neurologist and prion researcher just starting up my own lab in Edmonton, AB, Canada. (BTW – you mean 14-3-3, not 4-3-3)…

    Comment by Valerie Sim | April 28, 2009

  2. Hi Valerie,

    I can’t wait to see your blog once it’s set up! Thanks for finding the blog and commenting. It is so great to see a neurologist and prion researcher with an online presence on blogs and twitter. I am looking forward to all your contributions, both on and offline. I did mean 14-3-3. I must have written in a rush, which is usually how this blog happens.

    Comment by CureCJD_Heather Larson | July 3, 2009

  3. Pre 1968, vets in Australia successfully used a treatment for a brain wasting disease PEM – Polioencephalomalacia – in livestock, which has similar symptoms to CJD in humans.

    I wonder if doctors know of this treatment, which is also for human use.
    ainee.

    Comment by ainee | November 9, 2009

  4. Heather,

    Great work I lost my Mom to CJD > E200K in 1997. I found out one week before my Moms death that I also carry the mutation. Im very sorry for your loss and pain > always stay strong<<<<<

    Comment by Robert | December 30, 2009

  5. Hi Robert,

    So sorry to hear about your mom and that you carry the mutation. I still don’t know what my own verdict is. What a horrible time for you to find our your results. We should definitely email. Glad you found the blog.

    Heather Larson

    Comment by CureCJD_Heather Larson | January 2, 2010

  6. Heather,
    It’s believed that prions are the cause of CJD.
    From my research, I believe prions are not the initial cause of CJD and other brain wasting diseases, but lack of oxygen to the brain, due to injury and/or toxin could be.

    I wonder if the medical researchers are doing any research to see if prions are anaerobic – from my research, I think they are.

    In Sickel-cell anaemia, the cell changes comformation when it is exposed to oxygen. I believe the lack of oxygen causes prions to misshappen or missfold to cause the brain wasting.

    I wonder if you or someone you know, would be interested in my research and findings.
    Regards
    ainee

    Comment by ainee | January 4, 2010

  7. I seem to very vaguely remember there may have been a bit of research about something with oxygen that was done at BYU…I’ll have to ask around and see if anyone has a hold of that article still.

    Comment by CureCJD_Heather Larson | January 5, 2010

  8. Dear Heather,
    I believe a treatment which was used successfully in Australia as far back as 1968 for a brain wasting disease in livestock, could well be a treatment – also for human use – for Neurological illnesses, including CJD, in humans.

    In 2001, our sheep were diagnosed with a brain wasting disease. Research found the sheep symptoms are similar – give or take a few – to those of human illnesses, including CJD.

    Similar symptoms – why not similar treatment?

    I’m happy to share my research and findings.

    Comment by ainee | February 4, 2010

  9. Have you stopped eating Beef? I am so afraid to cook and feed my family beef after a dear family friend of ours was recently given the death sentence that is CJD. I know that not all forms of this “monster” are linked to beef consumption, however it is a drastic shift that I have been scared into to making. Anything to alleviate the potential.

    Comment by amanda | May 15, 2010

  10. 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)?

    Comment by Shelley | August 2, 2010

  11. Hi Shelley,

    My mom’s EEGs were also pretty normal and didn’t show her neurologist what she expected to see given my mother’s other CJD symptoms. As for the rapid progression of your father-in-law’s illness, that is not common in CJD unless it’s genetic (to the best of my knowledge at this time, and I say that from the bias of someone whose family goes through the rapid progression of the E200K mutation). All those same tests came back normal too with my mom, even a PET scan. She was as healthy as could be save for her CJD symptoms and a fatty liver — the latter of which no one could explain.

    I’m going to have to defer to others for your question about the lapses in consciousness and the talking…that’s a very tough one to answer. I saw my mother smile and almost laugh at a joke her brother made the day or so before she died. At that point, she’d gone from akinetic mutism to being (what we thought was) completely unconscious. Her eyes weren’t opening anymore and she’d stopped reacting to us — or so I thought. This smile and laugh I thought I saw could’ve been involuntary, or it could have been her last real “interaction” with us. I’ll never know. But she wasn’t speaking to us. So it’s completely different from what you’re experiencing. With CJD, the disease affects every patient differently depending on which parts of the brain are being affected by the infectious agent. So there is no textbook case or cut and dry answer to your question in that respect because no two cases are alike.

    I think what I will do is invite some friends from CJD Voice to answer your question, and see if anyone with expert knowledge may be willing to comment.

    I am so sorry for what you are going through and certainly hope your father in law doesn’t have CJD. I hope you find the answers you are looking for.

    Heather Larson

    Comment by CureCJD_Heather Larson | August 3, 2010

  12. […] Follow me on Twitter @heatherlarson […]

    Pingback by A question about consciousness & CJD « Cure CJD | August 3, 2010

  13. I’m sorry to hear about your father.

    I have often heard from family members of patients with CJD that there are occasionally moments of lucidity.

    Comment by Brian Appleby | August 3, 2010

  14. Hello again. I thought I’d update the thread since my last comment was August 2nd and we didn’t know for sure if my father-in-law had CJD. The spinal test came back positive for the 14-3-3 protein, and he died on August 8th. Very rapid progression. The autopsy results confirmed that he had an “ocular” variant of CJD. I think they call it that because his vision was the first thing to be attacked by the disease. At any rate, the realization that such a disease exists – and can take someone SO quickly, and in such a way that you often don’t get much of a chance to say goodbye – has reminded me how important it is to say everything you want to say, to everyone you love… NOW.

    Comment by Shelley | September 27, 2010

  15. Its 2 days since I went to my final genetics counseling session and was delivered the news that I have the E200K mutation. I have inherited it from my father who died from CJD in September 2010.

    I was told that it was likely to be sporadic becuase he lived 5 months from the initial onset of symptoms. The first symptom I noticed was memory loss.

    I am absolutely terrified. After watching my father die from this disease – to now find that it is genetic has basically taken away the value of my life.

    All I can think about is what happens if I develop an early onset? Who will look after my young children currently aged between 2 and 11. how will they cope without a mum?

    When the doctors delivered the result and asked me did I regret finding out. I answered that I regret the result but still felt that I would want to know the result even though it is not what I wanted.

    Interestingly no onw else in my family wants to be tested. Some of those people don’t have children but would rather risk passing the gene on than finding out they carry the gene.

    In this day and age IVF can assist in ensuring that you only have children that dont have the gene. I wish I had this option but I have all my kids already.

    Starngely I read earlier on thsi blog about copper – my dads full blood sibling (50/50 chance of having the CJD mutation) reported high blood levels of copper. I didn’t want to say to him that maybe this indicates his body is failing to process copper properly and perhaps this is a symptom of cjd.

    My dad ate a high protein diet and popped many vitamins. he was fit and otherwise healthy. I do note that a few weeks before his symptoms came on he had 2 vaccines – one was the flu vaccine and one was phneumovax. The phneumovax vaccine is made from bovine materials although the manufacturere insists that the bovine material is sourced from countries that do not have vCJD. I know that vCJD and familial CJD are different but perhaps the ingrediants of these vaccines had a trigger effect some how.

    What also worries me is how health professionals lead you to believe that familial and sporadic cjd are very rare but looking around the web at blogs it is not that rare – and appears to be on the increase.

    Thanks for the opportunity to share!

    Comment by Amy | April 2, 2011

  16. My father died in Feb. 2012 after a very hard fought battle with sporadic cjd. Does that really exist or does it mean they just really don’t know what caused it?

    Comment by Cara Albright | July 23, 2012

  17. It really exists Cara. They can diagnose it definitively, only with an autopsy after death. My father-in-law died in August, 2010 of sporadic CJD. They were able to say for certain that’s what it was, when they performed an autopsy afterward. It’s a horrible, horrible way to watch a loved one die. How long was your father showing symptoms before he died? I’ll pray for comfort for you & your family, because I still remember the pain of watching Jim deteriorate over a period of only a few months.

    Comment by Shelley Haslip | July 23, 2012

  18. Cara, I am sorry to hear about your father. I wish I were great at explaining biology; I’m not. It is tough to track down how a loved one contracted a prion disease because of the long incubation period. It isn’t like dealing with a disease like e coli or salmonella with a much faster incubation period.

    Thank you, Shelley, for sharing your father-in-law’s story in the comments. It is my hope more people will have the courage to leave comments about their loved ones so that we can show the way this disease is affecting society and that this is not a “rare” disease to be ignored.

    ~Heather Larson

    Comment by CureCJD_Heather Larson | July 28, 2012

  19. Hello everyone. My mother-in-law entered the hospital last week. We are awaiting results of her spinal tap, but the doctors are thinking she may have CJD. So far her MRI and EEG have come back completely normal, but all other tests are coming back normal too. Her symptoms started two months ago with muscle weakness and pain. Over the last month she started falling regularly. She often told me she felt “weird” and “scared”. She said it felt like she couldn’t put her legs where she wanted to when she was walking. Her conversations became short and she seemed depressed, but she was still able to converse. I just thought she was upset about having to think of Christmas gifts for the kids Then over the last week she quickly progressed to hallucinations, memory loss, only recognizing people from time to time and weird movements. Does this sound like CJD? Does this sound like a normal progression? We are so hopeful that she could “wake up” and be normal again, but every day we lose a little more hope.

    Comment by Elizabeth | January 8, 2013

  20. Hi Elizabeth,

    It sounds likely your MIL has CJD, however, I am not an expert or medical doctor. I can only share my personal experience. What I will say is that there are hundreds of rare neurological disorders, so DO keep hoping for the best! People do find my blog, ask this very question, and then come back later to tell me something else was wrong with their loved one. I sure hope it happens that way for you.

    The hard thing about CJD is the lack of a clinical diagnosis. The only way we can presently diagnose this disease is through autopsy. Going through the hell of diagnosis is unfair because tests often do come back normal. But CJD does show a certain pattern on an EEG. I hope you get the answers you need as soon as possible!

    Much love,

    Heather Larson

    Comment by CureCJD_Heather Larson | January 8, 2013

  21. Thank you Heather. I will definitely update when we know more. Thanks for having this blog. It’s hard to find any new information out there, but it’s nice to talk to others who have gone through it. -Elizabeth

    Comment by Elizabeth | January 8, 2013

  22. That’s what I’m here for.

    Comment by CureCJD_Heather Larson | January 8, 2013

  23. One quick questions, does anyone know why it takes so long for the 14-3-3 protein to come back. My mother-in-laws spinal tap was over a week and a half ago and they are still waiting on results. They did start her on high dose steroids 24 hours ago and she is a little more lucid. Hopefully this is a good sign.

    Comment by Elizabeth | January 10, 2013

  24. Elizabeth, so sorry to hear about your mother in law. I just lost my dad in Feb to sporadic cjd and I wouldn’t wish it on anyone. Not sure why the tests take so long to come back but they did for us too. The waiting is so hard. Hopefully the steroids are the answer but we went through the same thing. We were so happy at how well he responded to the steroids but unfortunately didn’t change the outcome for us. If you don’t mind me asking where is your mother in law from. Just curious, we are in Columbus, Ohio and have heard of a few other cases here since my dads. We will pray for a good outcome for your family, please continue posting to let us know the results.

    Comment by cara | January 11, 2013

  25. Cara,

    Thank you for the response. I’m so sorry to hear about your Dad. The waiting is very hard:( It’s hard not to get my hopes up, especially for my kids and my husband. My mother-in-law is from Racine, WI. (town sandwiched between Chicago and Milwaukee) I know CJD is so hard to diagnosis. They did her MRI again and everything still looked normal. I wish she was lucid enough to talk to the kids, but her hallucinations are just too often.

    Thanks again for your response and I will definitely keep you all updated.

    Comment by Elizabeth | January 11, 2013

  26. Depends on the hospital and day of the week…I can’t remember how long the 14-3-3 test took. If the steroids are seeming to help, that could be good. I know my mom had zero improvement from those. (This could also depend on the type of steroids given, dosage etc). Nothing helped my mom. In the first weekend she was hospitalized, they thought she had MS so they gave her immunoglobin transfusions. That may as well have been a placebo because it did nothing.

    Comment by CureCJD_Heather Larson | January 11, 2013

  27. Thanks, Cara, for replying to Elizabeth! I love to see the support from everyone on here!

    My mom was from outside of Cleveland. She grew up in North Royalton.

    Comment by CureCJD_Heather Larson | January 11, 2013

  28. It is so hard to see someone go through those hallucinations. My mom had those toward the end and she saw what she feared most — snakes. I wish testing were easier and more rapid. This is one of the goals of CJD research — to come up with a way to diagnose hospital patients in a SIMPLE manner rather than torture them with weeks of testing. The families also suffer through this as well. So sorry to hear about what you are going through. Hang in there. You have support here. If you are on Facebook, this is an excellent support group to join if you haven’t already: http://www.facebook.com/groups/112378108841431/

    Comment by CureCJD_Heather Larson | January 11, 2013

  29. Elizabeth,

    My dad doctor gave him meds for hullucinations and paranoid thoughts. They really helped. His Dr. was Dr. Appeby, out of the Cleveland clinic, he was wonderful and knows alot about the disease. How long has she shown symptoms?

    Cara

    Comment by cara | January 11, 2013

  30. Heather,

    I love what you are doing to help fight CJD, what more can we do as families who have been touched by this aweful disease? We started a foundation in dads honor and are raising money for research but I feel like there is more we can do. People need to know that this disease exists and that we are all at risk until they can find a cause and a cure. What do you know about the cjd foundation and the work they are doing? Is it more of a support group?

    Cara

    Comment by cara | January 11, 2013

  31. Thank you Heather for the Facebook link.

    Cara, we actually just moved from Cleveland, OH this summer. We lived there for 2 years so I’m very familiar with Cleveland Clinic. They are great! My mother-in-law started showing symptoms about 2 months ago (beginning of November). I remember when she was visiting she complained about muscle weakness and pain. She blew it off as “getting old” and I guess I thought the same thing. Things progressed from there, slowly at first and the last week, very quickly.

    Thanks again for the support!

    Comment by Elizabeth | January 11, 2013

  32. Elizabeth,

    That doesn’t sound at all like how dads started so that is encouraging to me. He started being very forgetful, we thought it was dementia at first. We will keep the prayers coming.

    Cara

    Comment by cara | January 11, 2013

  33. Thanks for the post and for sharing, Cara! So sorry about your dad.

    -Heather

    Comment by CureCJD_Heather Larson | January 12, 2013

  34. Just out of curiosity, what does she see when she hallucinates? You don’t have to answer if you don’t want to.

    -Heather

    Comment by CureCJD_Heather Larson | January 12, 2013

  35. I have always heard great things about Dr. Appleby.

    -Heather

    Comment by CureCJD_Heather Larson | January 12, 2013

  36. Cara,

    I ask myself the same thing all the time! I think having a foundation and raising awareness in your dad’s honor are great ideas. You can pick your favorite research institution and donate to it while urging others to do the same.

    I think the CJD Foundation has its place in helping educate, raise awareness, and — for me — informing me about the autopsy available through the NPDPSC/Case Western.

    What we need is a wealthy benefactor here to donate the millions needed for research so we can get more done in less time,

    I enrolled at UCSF in their genetic study and donated my blood to Red Cross research as well.

    -Heather

    Comment by CureCJD_Heather Larson | January 12, 2013

  37. You’re welcome!

    Comment by CureCJD_Heather Larson | January 12, 2013

  38. Heather,

    I haven’t been to see her, and I’m not sure if she is hallucinating or what you would call it. Some examples are, she eats a banana, but there is no banana in her hand. She folds clothing, but there is no clothing. She is often confused about who people are and is just in a different world. Something, besides reality is going on in her brain most of the time. The other day she went to kiss and hug my husband, missed him, and kissed and hugged the air. She scared a lot too. I’m not sure if she is verbalizing what she is scared about, but she gets pretty upset.

    Thanks again for your support. We still have not heard anything on test results.

    Comment by Elizabeth | January 12, 2013

  39. Heather, I just read that you live in Wichita, KS. I was born in Wichita and most of my extended family lives there:)

    Comment by Elizabeth | January 12, 2013

  40. That is interesting. My mom was scared and saw snakes on her legs.

    -Heather

    Comment by CureCJD_Heather Larson | January 14, 2013

  41. That’s cool — small world. I got a radio job here and have been here 2 years now.

    -Heather

    Comment by CureCJD_Heather Larson | January 14, 2013

  42. Hi everyone. i just wanted to come back and update. My mother-in-law’s tests all came back negative, even the protein 14-3-3. It has now been 3 weeks since she was initially hospitalized (very severe symptoms) and 2 months since symptoms started. She is actually making a turn for the better. She is attempting to eat again and she even stood today. My husband was able to speak to her on the phone. Her memory and thoughts are still very jumbled, but she is doing much better. I wanted to thank you all for your support and will keep you all in my prayers. I know you can never rule out CJD for sure, but we are hoping all of this improvement means she is on the road to recovery and that she truly does not have CJD. Thank you again to everyone.

    Comment by Elizabeth | January 21, 2013

  43. I am glad to hear your MIL is doing much better, Elizabeth!

    Every once in a great while, someone like you comes along and finds my blog. You get a different answer than the rest of us were given. This gives me hope.

    I’d like to see CJD become more rare. I really would. I’d love for it to disappear entirely! I think that’s the goal. I don’t care which way it happens, whether it just goes away mysteriously, we find a cure, we find a way to prevent it — whatever. This disease needs to just GO AWAY.

    Every answer that is found, as in the case of your MIL, helps. I really do see such similarities in all these neurodegenerative diseases that I think every answer that is found brings us closer to finding The Answer that gets us closer to cures for these things.

    I hope she continues to improve! Please keep us updated. It’s not an easy road to go down even where you are now. But at least you are getting answers! And at least she is getting better!

    This is the goal. Round of applause.

    Heather Larson

    Comment by CureCJD_Heather Larson | January 26, 2013

  44. Hi! I am looking to do IVF with PGD because my husband carries both CJD and Huntington’s disease. We want to have children but I wanted to see if anyone had any experience with doing this. Also with testing for 2 different diseases I am concerned if it can even happen. Can anyone put me in contact with people who have had success with this? Thank you!

    Comment by Brittaney Gan | May 3, 2013

  45. Huntington’s is new territory for me, I can’t imagine how horrifying it is to carry both mutations. If you have the money and determination for IVF, I believe it can be done. I was told at UCSF it would cost $35,000 minimum. Never wanted to have kids but they threw that option on the table for me during genetic counseling. What they told me about IVF is you can be certain you are not creating a fetus who would carry your mutation. I’m not sure what RBD is, pardon my ignorance on that. What you need is someone who can understand the science of these two particular mutations. Have you checked with the hospitals in your area? I will put this question out there to some people and see if they know the answer and let you know on here. I appreciate the question.

    Comment by CureCJD_Heather Larson | May 8, 2013

  46. And by hospitals, I meant teaching hospitals!

    Comment by CureCJD_Heather Larson | May 8, 2013


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