Cure CJD

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

Which is worse — ALS or CJD?

I was watching the MDA Show of Strength on TV last night for a bit and couldn’t look away as the lives of patients with ALS were detailed. (Disclosure: I participated locally in fundraising last month for the MDA and my radio station’s local media partner is the ABC station that showed the program last night).

I have always been struck by the similarities between ALS and CJD. In a way, I am glad the course of disease in CJD patients is much shorter because I think it’s more merciful to their suffering. But I also see that ALS patients have a greater quality of life because their course of disease is shorter and seemingly slower.

Before you think that’s the most controversial thing I could ever say, let me explain. I saw an ALS patient on my TV who was slurring her speech but who could still speak to her husband. She was shown walking in her garden, although not without help from her husband, and not without a cane. As someone still able to communicate, she is still able to share her feelings and needs with loved ones. Her free will has not yet been entirely taken from her by her disease.

While her words may be slurred, she is still able to have the important conversations between herself and her family. This is a luxury we did not have with my mom. Her decline and loss of speech was so rapid, her “diagnosis” came too late. We were already unable to have those vital conversations, say goodbye, or explain to my mother what was killing her or that she was even dying. She just slipped away from us.

I once got into a discussion with a doctor about how I thought CJD was the worst disease a human being could get. He countered that ALS is the worst disease a person could get.

I think the two diseases may be apples and oranges, yes. But there are some frightening similarities. According the the ALS Association’s website, initial symptoms of the disease are:

*Muscle weakness

*twitching and cramping of muscles

*impairment of the use of arms and legs

*”thick speech”

*difficulty in breathing and swallowing

From: http://www.alsa.org/about-als/symptoms.html 

I can compare and contrast the two diseases all day and explain away why each of the above symptoms is actually quite different for CJD patients. But that’s not the point of this blog post. Maybe I’ll do that another time.

The point is this: 

How many of us in CJD families were flipping through the TV channels last night, pausing on the MDA Show of Strength thinking to ourselves, “When will CJD get national attention and become a household name?”

I know, many of you have told me over the years that won’t happen until someone extremely famous and well-loved dies of CJD. And you’re probably right. But why must we wait for that day to come? Kind of macabre, don’t you think?

Why is a disease with such similarities to ALS still slapped with a mad cow disease stigma, considered taboo, and still unknown to many doctors and nurses in this country? Why, when I tell someone my mother died of CJD, must I explain it to them and still leave them confused? Why, when I tell someone how my mother died, don’t I receive the look of pity and understanding I would have received had I told them, “My mother died of ALS?”

Well, I don’t want anyone’s pity. But I would like to see some understanding for those who walk into a hospital with a sick loved one like I did in 2004. I would like to see them able to receive a clinical diagnosis from a doctor who is well versed in treating CJD. I would like to see such a family greeted with understanding at hospice and at their funeral home.

I would like to see a person not have to bear the awkward burden of explaining what CJD is to their family, friends, and community when they should be allowed to merely grieve. I’d like to see the day when you tell someone that your loved one died of CJD and a person says to you, “Oh, I’ve heard of that. I know what that is.” This has happened to me so few times in nearly eight years, I could count those instances on one hand. Let’s move forward to a point where people have heard of CJD and don’t automatically connect it to “mad cow disease.”

We won’t get to that point of understanding if we don’t each work one by one to raise awareness about CJD. The ultimate goal is to reach a CURE for CJD.

A lot of celebrity, glitz, and glamour goes into raising millions for MDA. I’m still here writing a blog and answering scattered emails trying to raise awareness. I’m just saying!

Otherwise, here’s what you need to know for today: 

*CJD does not presently have a clinical diagnosis, meaning it is impossible to give a definitive diagnosis in a hospital setting.

*The only way today to diagnose a patient with CJD is after death through autopsy.

*CJD is a terminal disease. It presently has no known cure. There is no known prevention. There is no definitive “trigger” for those who face genetic mutations of CJD (like myself; my odds of carrying the gene are 50/50 so I don’t know if I am doing something “wrong” to trigger it in my body or not).

How about some ideas for raising awareness to close?

*Talk about it in your church, your communities, and at your kids’ softball game as you sit in the stands with the other parents. I believe this is called bonding and sharing the human experience. It is not taboo. It is what we are here to do.

*Contact the CJD Foundation and see if there’s something you can do within your own community. Can you talk to the doctors and nurses at your local hospital? Can you help them with CME credits through the CJD Foundation?

*Hold a fundraiser like a 5K, walk, carwash, or bake sale. Send the funds to UCSF’s Memory and Aging Center or the CJD Foundation or whichever research institution is your favorite. The MDA raises millions. How much are we raising?

*Start a blog about your experience. Or do what I did and just keep a journal as you go through this and share at a later date if you wish. We aren’t all ready to share as much and as soon as I did. I still have material I haven’t published about what I went through. Just write it down and get it on paper.

*Network with other CJD families. We are all on Facebook and Twitter anymore. Before social networking, we communicated via CJD Voice (then a Yahoo group) and email. There is strength in numbers and in finding out you’re not alone.

*Attend the CJD Family Conference, which is held every July in Washington, D.C.

If you have another idea, post in the comments below. If you are doing something right now (or have in the past) in your own community, please post it in the comments below.

~Heather Larson

@heatherlarson on Twitter

hotwords [at] mac [dot] com

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September 3, 2012 - Posted by | Uncategorized | , , , , , , , , , , , , , , , , , , , , , , , ,

10 Comments »

  1. Hey, Heather. I am intrigued by your story. My oldest sister, Danielle, died of fCJD over a year ago after becoming symptomatic around 2006. Seems she was the “first in the family,” since we couldn’t pin it to any other family member. My dad was taken by pancreatic cancer when he was 72 – no symptoms of CJD. My mom took the genetic test and the mutation was not found in her. So we can only assume it was my father’s side of the family. I have so many unanswered questions. But seems you’ve experienced this nightmare enough for two lifetimes. I couldn’t imagine going through it again. My sister’s nitty-gritty full on symptoms were a year before her delicate frame could take no more and she was gone from us. I wish for her sake it had not taken so long. I don’t think she would have wanted her loved ones to suffer so much from watching her disintegration. Horrible.

    Comment by nicole | September 3, 2012

  2. Hi Nicole,
    I am sorry to hear about your sister. We almost had the same experience with my mom. We had actually never heard of CJD before my mom was in the hospital! The neurologist took us in a conference room and raised the possibility of CJD and explained (for lack of a better explanation) that it was “like mad cow disease.” That’s when a light went on and one family member recalled that an uncle (whom I never knew personally) had died of “mad cow disease” (not possible for reasons that are now clear but weren’t to us then). That’s when the neurologist realized it must be genetic CJD and waited for the brain biopsy to come back to confirm it was suspected CJD. An elderly aunt (second cousin of my grandfather’s wife or something to that effect) told me there was a cousin somewhere along the line who had died of something that sounded like CJD too…I was never able to confirm that. So in total, there are three known deaths in our family, maybe four. My mother came from a large Polish/Catholic family and often told me she had 100 cousins. So there are any number of people in that generation who could have passed by now or who are reaching that age.

    Here’s the odd thing that I have yet to figure out — who lives and who dies? My grandfather is the carrier but he is still alive. Not one of his siblings died of CJD and he was one of fourteen children! My grandfather had only three children and only one of them is still alive; my mother died in 2004 and my uncle died in 2008. No one in my generation is anywhere close to being “old enough” to start showing symptoms of CJD and I hope we figure out a cure before we get there.

    There was a geographical and generational disconnect in my family between my mother’s uncle (who died of CJD unbeknownst to us) and the rest of the family. My mother’s family had moved from Ohio to Arizona when my mom was in high school and remained close to only one other uncle who also moved out West. Those who remained “back East,” as my mother called it, weren’t a part of my life growing up.

    If there is anything I have learned from this familial CJD experience, it is to be aware of who I am and where I come from. I am certainly trying to trace my family lineage on both sides, with some support on my non-CJD side from my aunts. I Googled my family name often until I was able to track down one of my mother’s closest cousins by email. (We have a unique name). He didn’t know of any other deaths but gave me useful information about other illnesses in the family and some other background information. But sadly, much of this huge Polish/Catholic family “back East” in Ohio that I heard so much about from my mother is hard to track down. I’ve always hoped they would find me through this blog, but that hasn’t happened yet. I, too, have so many questions!

    ~Heather Larson

    Comment by CureCJD_Heather Larson | September 3, 2012

  3. I got so much out of reading this story. My mom died July 13, 2012 which was just a few months ago. The doctors believed she had the sporadic form because of her age and some other symptoms that fit the puzzle for sporadic cjd At first I kept thinking thank god she didnt know what the diagnosis was at the time. Now I just feel cheated that I didn`t get the chance to say the things that others families say when their loved one is diagnosed with a terminal illness. When people ask how my mom passed so quickly (53 days after the first symptoms) I always start with saying its one in a million and you probably never heard of it and 99% of the time I`m right. I compare the impact of cjd like a fatal car accident. It just happened so fast. I probably have more in common with someone who lost their loved one that way. I keep reliving those 53 days to make some sense of my moms death. I don`t sleep much at night because I wake up to the words that my mom was saying before she lost her ability to speak and know who we were. It rings in my ears,” Is this a dream?’ Am I going to die”?. At the time we had no idea but we knew it had to be serious. I need to keep
    sharing my story. My mom was everything to me. She lived right around the corner. We would talk everyday at least once usually twice a day. When I found out she was going to die my first thought was you can leave yet because your not done raising me. I`m a 42 year old woman who is married with three children and I take care of my family the way my mom took care of mine.

    Comment by Lori LaRosa | October 19, 2012

  4. My father has just been diagnosed with cjd, last week. The progression is daily. Write me!

    Comment by James park | February 3, 2013

  5. I’m sorry to hear your dad has been diagnosed with CJD. It is shocking to see the daily progression of this disease. Just know there are no wrong decisions for you to make right now.

    Heather Larson

    Comment by CureCJD_Heather Larson | February 10, 2013

  6. I understand where you are coming from by the loss of a loved one and not having many people know what the disease is. My dad actually did have ALS and I have come across MANY people who don’t know what it is. I was a little hurt by the statements of one disease being worse than the other. I think any disease that does not provide us with a cure or understanding is equally devastating. I think our mission is to bring awareness to the forefront and push medical teams to dig deeper into these illnesses. The statement of ALS patients having a better quality of life is all dependent on the case. EVERY case is different. My Dad lived with it for 3 years and being confined to a wheelchair, having to be spoon fed and bathed by my mother, and speaking but barely being understood doesn’t seem like a wonderful way of living. My Dad prayed for his family to not see him deteriorate and for it to be a quick process. So point being, I think it depends on your definition of “quality of life” and your state of mind. But the reason I stumbled across this page was not to debate what is worse, it was to find out more information on the comparisons of the two diseases. I am curious to see in the future if doctors will tie the 2 together. There were symptoms my Dad had that were not common ALS symptoms which has always left me wondering if his diagnosis was 100% right. Kind of scary when you think about it.

    Comment by Andrea | January 28, 2014

  7. How can people NOT know what ALS is? I always thought it was a pretty well-publicized disease with plenty of awareness! Always goes to show…we can never advocate enough. And people see what they want to see, they look away from what they don’t want to see.

    I am sorry you found it hurtful that I have attempted to decide which disease is worse; that was not the intention. The intention is to get people talking and more aware of these diseases. These — CJD, ALS, MS, Alzheimer’s, FFI, GSS, Huntington’s, etc., — are horrific diseases. Most diseases are! But things that get attention are cancers (I personally raise money annually for the Leukemia & Lymphoma Society to honor friends who are survivors). The horrific neurodegnerative diseases aren’t getting enough attention because they are still perceived to be “rare” and aren’t as easy to talk about as cancer is. You say “cancer” and everyone instantly understands. Try and explain “brain wasting disease” or even pronounce “Creutzfeldt-Jakob Disease” let alone spell it… It’s disheartening to say the least.

    You bring up quality of life, which is very important. I’d like to think none of us would want to be sponge-bathed, spoon-fed, and wheelchair-confined. CJD may be more human in my family or at least give us a better quality of life simply because it’s over in four weeks. (The E200K mutation is fast as hell).

    I’d love to hear what you find in terms of comparisons between CJD and ALS. I wonder how different they are, really. I really do think a cure for any of the above named diseases would lead to cures for the rest. This may be like cancer in the way that if you cure one, you cure another. Or they just get “more curable” over time. Some Leukemias are much more curable than others…

    Of course, with CJD can we just start with a clinical diagnosis?

    These are terrifying diseases. They have many similarities. This is part of what made CJD so hard to diagnose in my mom. Doctors thought she had MS. They checked her for cancer. They dosed her with drugs for MS. She didn’t improve. She went through a few weeks of tests and drama before we got a diagnosis. She died six days after that. Even now, it seems like the final month of her life was a waste.

    So I keep this blog in hopes she didn’t at least die in vain.

    Plus, this has been good therapy for the past nine years. =) You only keep what you give away!

    Comment by CureCJD_Heather Larson | January 29, 2014

  8. Thanks for all the sharing.
    I myself have experience of CJD When my mother got ​​speech problem back in July 8, 2012 (same day of my father’s 73th birthday). I remember I had a bad dream many years before about that my dad would die in the age of 73. Instead, it was my mother who passed away two month later in an age of 66. First the doctor thought it was meningitis. It took almost to the end before the doctors could conclude it was CJD, and in that time it was to late to say good bye due my mother was unconsciousness and not able to react. We are 8 kids (7 brothers and 1 sister) in our family (in Sweden) which my mother born and raised. However, before I was born I had a sister that lived for some weeks before she died with heart failure. My younger brother got leukemia twice in teenage stage, but he still lives today and now well.
    The CJD was not genetic but sporadic (which was important to know in case other in the family has it, but also that you are now allow to be a blood donor if someone in your family has the disease, or any other similar disease I guess).

    The biggest problem with this disease Is that its very difficult to share with anyone except your family members, Because it is so rare and unusual. Also there is know scientific fact why it’s happens and why so few people gets it. It just happen for no reason which are quite annoying and frustrated.

    However, I believe internet could be a good place to map out how the people which suffer CJD have lived their life before and now, what nationality they where (my mother was part Finish and Swedish, and it says the CJD disease is a little higher rate in Finland) what the diet where (high rate of many disease is connected to food) and so on. Do any know any good site on internet for this mapping? I mean, if the science doesn’t have time or money to care for CJD, maybe we all could contribute with are own experience and knowledge.

    Best wishes,

    Fredrik

    Comment by Fredrik | July 24, 2014

  9. My mother pasted away from sporadic CJD at 4:10 a.m. on halloween October 31, 2012. Coincidentally, it was her favorite holiday of the year. We took mom to U of M on oct 1st to find out what was going on with her? She had went to her neurologist for spinal taps and MRI’s and they found problems with her brain stem? But still no answers?? Frustration set in with all of our family members? The symptoms were loss of gait, slurred speech, depression, and sleepiness. She declined very quickly:(. The doctors in kalamazoo, mi had no idea what we were dealing with? All we knew is that our mother was dying and there was nothing we could do to stop it:(.
    Mom worked at a deer processing farm for 20 years of her life. It’s possible she may have contracted this disease here or from eating tainted wild game that my grandfather was always bringing home for the family to eat in the 50’s and 60’s? No one will ever know? all we know is that she is at peace. Me and my sister and brother have a 20% chance of contracting sporadic CJD according to neurologists. I wish there was something we could do to prevent the onset of this incurable disease before it spirals out of control in our 60’s?

    Comment by Jennifer Akins | August 20, 2014

  10. Hi Jennifer,

    I am sorry to hear about your mother. If there’s anything that can be done now, it’s LEARN and VOLUNTEER. Learn constantly what can be done, what WE can do to help this disease find a clinical diagnosis and a cure.

    For me so far that is to donate my blood to the Red Cross to be studied. Hopefully, someday, those of us who have donated blood will have contributed to some kind of advance in what the Red Cross does so well — blood donation.

    I also volunteered to be studied in Dr. Geschwind’s study at the UCSF Memory and Aging Center. I did not have to have my status made known (I will either be in the control group or, well you know…)

    I know I’m from a family of genetic cases so it’s different. But I’d be proud to go down fighting for at least a clinical diagnosis, treatment, and/or cure.

    Keep fighting on,

    Heather Larson

    Comment by CureCJD_Heather Larson | August 26, 2014


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