It’s Rare Disease Day — Which Is Problematic

It’s February 28, 2021 — 2/28 annually is “Rare Disease Day,” a day when people flood social media with graphics and hashtags like #RareDiseaseDay. I know of people who have truly rare diseases like a necrotic bone disease or epidermolysis bullosa. I know many, many more people through 16 years of online support groups who have seen a case of CJD in a loved one.

I both like and don’t like seeing awareness of CJD raised on this day every year.

There’s a caveat for many of us who aren’t feeling seen and heard on this day — those with familial mutations of CJD.

Yes, for those of you who lost someone to iatrogenic or sporadic CJD, it is a rare disease FOR YOU.

But for those of us who have lost multiple family members to CJD because our families carry one of the many genetic mutations of CJD, of fCJD, this day is a double-edged sword. Yes, awareness is (kinda) good (read to the end). But being force-fed “awareness” by calling CJD “rare” is bad.

CJD is NOT RARE.

There are three reasons for this, one I already mentioned, is that there’s no way something is “rare” when it happens over and over to a family.

The rest of my explanation as to why CJD isn’t rare is for you non-familial folks, more specifically, the sporadic cases. You can’t call something “rare” if you aren’t collecting enough data points. This has been a problem for the past 16 years I’ve been involved with this and it needs to change.

The first aspect to this three-fold problem is there isn’t an autopsy done on every single death by suspected CJD; CJD is only diagnosable to this day upon autopsy. (Unless something changed? Did I miss it? Did we manage to discover a clinical diagnosis yet?)

The second problem is, here in the U.S. at this time and for the past 16+ years, not every cow meant for human consumption has been tested for BSE.

And third, not every hunter opts to test their wild game for Chronic Wasting Disease (CWD) — as they should to ensure their safety — either.

So let’s recap:

When it comes to CJD autopsy, testing of cows for BSE, and testing of wild game for CWD — there isn’t enough autopsies or tests for prion disease being done to collect proper data in order to be able to call any and all prion disease “rare.” We have long suspected that if we did collect all this data, it may not be “rare” at all. (But your beef still will be — rimshot! Yes, there is still humor in prion world, or more accurately, my very bad jokes and sarcasm).

I’ve been doing this long enough that I don’t have hope that there ever will be proper testing done (in the U.S.) in order to collect data points. It should happen. It needs to happen. But I may not live to see it happen. Whether we are buying cow at the store or eating wild game we caught in the wilderness, we should be testing it. We aren’t testing because of cost and time. Families don’t obtain autopsies for a variety of personal reasons. Hunters just want to eat; they don’t want to wait on test results. (This is a very deadly risk to take but people take it because either they are hungry and have to eat or there’s just a disconnect about the seriousness of prion disease due to long incubation periods).

In lieu of having collected proper data, therefore, we cannot call prion disease rare. It’s NOT, nor has it ever been a “one in a million” disease. I know that sounds easy, convenient, accessible, and catchy. People love to be special, we love exceptionalism. We want to be considered rare and unique. But it’s just not true when it comes to CJD. If it were true, then I’d have at least 3,000,000 family members. (To date, I know of three who have died; I am still doing genealogical research to find more, so this may change).

CJD took my mother and uncle — each at age 56. It took their uncle at age 69. So do NOT ever try and tell me this is a “rare” disease. While I can support anything that raises CJD awareness, I choose not to do so without writing a blog post like this to make people think. It may be “rare” for some and that’s their lived experience.

But my lived experience is that CJD is NOT rare; it is generational. It doesn’t skip any generations. It is terrifying to look around a holiday table and wonder which of us will get CJD and which won’t. My Great Uncle Walter (whom I never met) was one of 14 children. My Grandfather Bernard — Walter’s brother — did not die of CJD but passed the mutation down to 2/3 of his children for certain so far (I am not sure if the third one has passed or not).

My lived experience tells me most doctors are aware of CJD from medical school and CMEs, but they have not all witnessed or treated it. I have given a speech on CJD to doctors at Barrow Neurological Institute to help them with CME credit on prion diseases in a partnership with the CDC. (They gave me a whole five minutes to speak after the CDC doctor spoke! I focused on the importance of autopsy in my speech). That may be all some experience in regards to prion disease.

My lived experience tells me many people who have donated blood or work in that field are aware of CJD as it’s part of the blood donation questionnaire. But they may never see someone suffer through it like I have.

This all brings me mixed feelings about today, “Rare Disease Day.”

I want people to care. I just want them to care in the right way.

We have a CJD Awareness Day on November 12, but a lot of CJD families hop on “Rare Disease Day” to promote awareness of CJD. We do many things to promote awareness, such as wearing shirts we make, or like I have done with this blog and my first CJD blog.

I’ve been doing this for 16 years; I no longer care about awareness.

I CARE ABOUT A CURE. I care about a clinical diagnosis. I care about research, drug trials, and a TREATMENT. I care about donating my body to UCSF when I’m dead. (This is good for science no matter what kills me and COVID-19 sure tried).

To truly obtain these things, yes, we do need “awareness.” But we aren’t going to get it without spreading the truth — and true horrors of this disease.

I want you to know about CJD. But there’s so much more I want you to know than just that it exists.

I want you to know it’s horrific and steals abilities from a person, dehumanizing them to the point they can no longer walk, speak, swallow, or know when they need to go to the bathroom — that’s just for starters. I want you to know that it quickly takes my family members in a matter of weeks — which IS RARE in the world of CJD.

Most families suffer losing a loved one for more than a year or 18 months. Imagine being a caregiver that long. Imagine watching your loved one suffer like they are trapped in their own declining body that long. They can’t speak to you about it; their brain is literally shrinking and filling with holes.

I want you to know that, while it is genetic in my family, prion diseases are also highly infectious. The bulk of prion disease has gotten around the planet because it’s highly infectious. But it also has spontaneously appeared on the planet, as it did more than 200 years ago in New Guinea. I want you to know that prion disease has been on the planet for that long and yet we still know so little about how to treat, diagnose, or cure this horrific disease. We still don’t know why proteins misfold.

I want to make you know and care about it to the point that you join me in doing something about it. I’ve been trying for 16 years.

I will close by saying something vital for all to understand.

Those of you who deal with sporadic and iatrogenic cases, you are blessed to only have to deal with it once — you only lose ONE.

For me, “raising awareness” isn’t a hobby or a trend; it’s life and death. It’s mission critical in my life to help find a clinical diagnosis, treatments, and a cure for CJD. Because there’s a 50/50 chance I die of this in less than 20 years. Getting likes on Facebook for sharing a “rare disease day” graphic is cute and all, but this is my life. There’s a 50/50 chance my generation of my family watches one another die of this in fewer years than I’d like to admit. I am trying to leave some hope for the next generation after mine.

Hope vs. No Hope. Let’s define “hope.”

There are some things I have hope for and some I do not. I say this because every couple months or so I get an email from a very hopeful family who thinks there is treatment, a cure, or something they can do for a loved one who has (somehow) been “diagnosed” with CJD. If you want to be hopeful, look for clinical trials in your city/state/country. For example, in the United States, we can search for clinical trials at the ClinicalTrials.gov website.

Do a search. Go ahead, I’ll wait.

Yeah…so two quinacrene studies? Thanks but no thanks. I thought we’d given up on that long ago…

There’s also a study from 2010… Well, it’s 2013 now. You get the idea. You’re going to have to be in the right city a the right time in order to get your loved one in on any clinical trials as they suffer from CJD. Then there’s the ethical question of prolonging their life — a.k.a their misery — through a clinical trial. I am not sure I would personally want mine prolonged in that fashion. Of course, the E200K in my family kills so quickly, I’m not sure patients like ours would get more than 2 months out of a trial at most. You never know. You could be the first person saved, right? Someone had to be the first to receive FDR’s polio cure, right?

So that’s the kind of “hope” we end up having.

When I say on here that I “have hope,” it’s hope for the next generation of my family. My generation may get lucky. You never know. But if you’re coming here with a relative who is currently suffering from CJD, they are dying. I am sorry. There’s not much modern medicine can do for them.

I joined a research study at UCSF. It might help someday. This is about all I can do at this time. But I felt, out of hope, that I had to try and offer myself up to be researched. UCSF is doing a great deal of research studies and clinical trials.  If you are in or near or can somehow get to UCSF in San Francisco, California, then go for it.

Take a look here:

http://memory.ucsf.edu/research

As you can see, there are a ton more clinical trials listed than the government website lists. (Ok, two more but at least they are current). Also, UCSF is on a budget. For instance, I paid my way out there and back last year to enroll in the study. I paid for all my travel expenses. I chose not to stay overnight at the hospital so I wouldn’t cost them more money. I really want the money they have to go towards researching my brain health, not towards putting me up for a night and feeding me hospital food. I spent that night drinking with friends instead. 😉 No, they didn’t pay me to participate in the UCSF study.

Hope is the place to be. But you have to be realistic about it. I hope I can enter more research studies. I hope UCSF can find something useful out of what I have given so far, and will continue to give of myself in years to come. Medical research is slow and expensive. Be patient and be realistic, but never lose hope.

Research studies to join at the UCSF Memory & Aging Center:

http://memory.ucsf.edu/research/studies

There are six clinical trials listed right now at UCSF, all for Alzheimer’s Disease:

http://memory.ucsf.edu/research/clinical-trials

As you can see, there are currently no options for a patient with CJD to undergo a clinical trial in America that I personally know of right now. Maybe there are some I am unaware of at another research institution. Please tell me in the comments and give a link if you know of one at any time. (It is Sunday, April 7, 2013, so if you comment anytime now or years from now I will still see your comment).

You’ve seen someone die of CJD so do you have PTSD?

I want to write my “holy crap it’s been eight years” blog post. Before I get to that, a more pressing issue that I think will help others much more.

In eight years, I only experienced what I think of as a “PTSD flashback” once. I didn’t think it would happen again unless I faced the same trigger. I believe it was 2007 (the years melt together) and I was in whichever class it was at Boston University in which I had to watch the film “Hilary & Jackie” for an assignment. This particular movie features a character who suffers from multiple sclerosis and who therefore has on-screen seizures. Seeing this in a movie — not even real life — was enough to push me over the edge because it looked so similar to what my mom went through. It gave me an anxiety attack. This only happened to me that once and I never thought of it again until this week.

My mom was diagnosed with CJD on November 2, 2004 — election day. Bush vs. Kerry. Since I didn’t know my mom was “really” dying yet, I went to work that day in the newsroom where I worked. I got out at 11:00 am and went to vote, then I went to the hospital to wait for the diagnosis. I waited all day and it came around 5:00 pm. I was just very determined to work and vote that day in spite of what I may face later. I think at that point I was still clinging to the idea that I might still have a normal life.

That was eight years ago. I get through each anniversary in my own way. Each year is different. I think the first one (2005) was the worst since it was only a year and my dad was remarrying so quickly. This year, I just felt like I was the most at peace I have ever been about it all because I had done my part to help research at UCSF and with the Red Cross. I feel like I have taken more control of my own destiny therefore and that I have at least done what little I can do to help fix this. Despite what I have done and the eight healing years that have passed, I had trouble this week.

I had what I will call a “PTSD flashback” on Tuesday on the way to vote. I felt like I did in 2004. I felt that pain like it was fresh and I was back in Phoenix on election day 2004 and it was Bush vs. Kerry not Romney vs. Obama in 2012. I had to get a grip on reality and realize I was in 2012 and Wichita, Kansas. I was far away from the experience of eight years ago, so why did it feel so fresh?

Again, I ignored this. How could this possibly happen to me again after five or six years or however long it’s been? I’m not keeping track. I didn’t see someone have a seizure. Do I have other triggers I am not aware of?

Many of us who have witnessed what CJD does to the human body, to our loved ones, have talked amongst ourselves about how we suspect we have PTSD. Has a medical professional diagnosed me with this? No. But I know what it is. It seems to happen to me on a situational basis. It’s so rare that I don’t get it. We all have problems dealing with the anniversary of the death of a loved one. So I chalked it up to that and forgot about it again. I had other stuff going on in my life this week, thank goodness.

Then Friday morning, a friend of mine and I are together and he gets the call — this is it for his mom. Seeing him go through that emotional roller coaster in a span of five minutes was apparently another trigger for me. Friday would have also been my mother’s 64th birthday, Saturday the eight anniversary. I had another “PTSD flashback” in which I felt like it was 2004, my breathing got heavy, and I had to tell myself over and over that it is 2012 and I am in Kansas. Click your heels, there’s no place like home. 

I got through it. I knew instinctively what it was and I was able to keep myself from going over the edge. I anchored myself to my surroundings in every way I could. I kept my eyes open, noticed every detail around me, kept telling myself over and over, “It’s 2012 and I am in Wichita, Kansas.” It was my mantra. It kept me anchored to the present.

Is this good enough? Hell no. If you think you are suffering from PTSD after losing a loved one to CJD, get help. This can be very serious. I’ve received a lot of help over the years, which is how I have been able to help myself on instinct.

The reason why I am even bringing this up is because of two things:

• For the same reason I even have this blog, I bring up the things everyone else is afraid to talk about.

• People don’t talk about CJD. But even fewer people talk about PTSD. I don’t think it’s anything to feel shame or embarrassment about. I’m not saying I don’t feel some weird negativity about it like I am flawed because that’s exactly how I feel.

Why do I feel this way? I don’t get it. I have never been to war. I think of PTSD as something that happens to veterans or people who have been victims of extreme violence. So what gives?

I think this page I found explains it best about how it feels and how I’ve managed to deal with this in the past week:

http://ptsd.about.com/od/selfhelp/a/flashcoping.htm

I understand that seeing another human being have a seizure is going to be enough to throw me into flashback mode. That has only been a problem one time in eight years and isn’t something I am likely to see much of in my lifetime. I do not understand my other “triggers” that happened this week. I think these were calendar-related triggers. Election day and my mom’s birthday? Apparently those are days that are enough to bring on some heavy emotions. My mom also died the day after her birthday. I guess that’s a lot. Yes, eight years have passed since she died. But this was only the second election since she died, so maybe I am just not “used to” election days in my post-CJD world.

Seeing someone die of CJD is very emotionally scarring. It’s different than anything most people have been through. Seeing someone have a seizure isn’t just seeing a person have a seizure. It is really (to those of us who have lost someone or several family members to CJD) seeing that person die a rapid and debilitating death that takes away everything that makes you human in an instant. There’s an extra element of trauma with CJD for caretakers that you don’t get with other diseases. I think it has to do with the overall lack of understanding from the medical community that makes everything so much more stressful.

I don’t think there’s ever been something else in my life that has involved more stress and fear. Not car accidents, earthquakes, fires, floods, tornadoes, any scary medical situation or dangerous situation I have ever been in. Nothing — and I mean nothing — has ever felt like it did to see my mother suffer in the way she did for that month and two days in which she suffered from CJD before death. It has been eight years and I still don’t understand my own emotional scarring from it.

That’s why I am writing this today and every day. Something I write here might help someone else go through this. Maybe this can also help others who are trying to understand someone (friend, coworker, loved one) who is in the position I was in/am in. Be gentle with that person if you are going through this and be kind to those going through it. That’s all I ask.

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

More proof we need a cure for prion disease

I’ll keep this short because I am tired. I think the energy has just been sucked out of me by reading the many comments on the ABC News Facebook page in regards to the story they posted about downer cows being found at a California plant that supplies the popular In-N-Out hamburger chain.

 

I think what shocks me the most is a few things — and I can post at length about this at another time. And I’ve meant to do this for a while about other social media screen shots I’ve saved over time. (A later post will cover misconceptions and poor journalism regarding “mad cow disease” issues at length). 

1. The American people are absolutely ignorant about what the real danger is here. They aren’t picking up on the term “downer cow” or what that is or why it is important — although it is a term recognized by CJD Families.

2. ABC news has given one line on a television newscast (which I saw Diane Sawyer read on my very own TV tonight) about how this plant also provides beef to the school lunch program. In-N-Out gets all the bad press. Excuse me, adults choosing to buy fast food is one thing. But children who have no choice who are eating this at their schools? That should be the lede of the story. I think I saw less than five people comment on ABC’s Facebook about this. Scary.

3. My pet peeve is we’re all experts in this country after two minutes spent on Google U. Read a book! More specifically, obtain a copy of Sheldon Rampton and John Stauber’s “Mad Cow U.S.A.” Read it. Learn it. It will make you a more effective consumer.

4. I see people posting on this ABC News Facebook page that they’re so glad they pay more for superior cuts of meat. Nice thought, but it doesn’t mean a thing. Until every cow in the U.S. that is meant for human consumption is tested for BSE, it doesn’t matter which cut of meat you’re eating. It doesn’t matter that it is hormone-free, grass fed, corn fed, or even blessed by fairies and unicorns. It still has not been tested for BSE to your knowledge and you will never know whether it has been tested or not. Just because something is judged to be superior and you paid more for it does not mean it does not contain an infectious agent that causes prion disease.

And I shouldn’t have to tell you this. You should be a responsible consumer. But you aren’t. 

This is one thing I have learned in nearly eight years since my mother’s passing and since becoming subsequently interested in prion disease.

People really don’t care and the American people are so absolutely ignorant about all this — media included — that you cannot protect 33 million + people from themselves.

This is why the only answers was, is, and will always remain to be the FIND THE CURE FOR CJD.

This is also part of the reason why I rarely comment about beef. The larger part is named Oprah Winfrey.

~Heather Larson

@heatherlarson on Twitter

hotwords [at] mac [dot] com

Thank you for your emails about CJD

I have received two separate emails from CJD families in different foreign countries this weekend. Each person is asking me to tell them about treatment options for a suspected case of CJD. At first, I was confused and wondering who is giving people the idea that there is a treatment.

Then I realized that we all go to the hospital expecting swift diagnosis, treatment, and a cure for illness. I really want to see the day we live up to our own human expectations. I’m happy to be a small part of it.

With that said, I will now struggle to answer each email. It never gets any easier. I know someday it will because some day I will be able to give them the answer they want. Of course, if that were really true, people won’t be emailing me at all. That is the goal. Someday, we will have a clinical diagnosis, a treatment, and a cure.

Right now, we don’t.

As it always goes, I will email these nice people and answer their questions. I am sure they won’t email me back ever because that’s usually how it goes. Sometimes a miracle happens and a patient is diagnosed with something other than CJD and that is when I get a return email at some later date, which is nice. I am always happy to hear there is one less case of CJD. I wish there were no more cases of CJD. Until then…

I regret to inform you there is no clinical diagnosis of this disease. The only definitive diagnosis comes from autopsy. I also regret to inform you there is no treatment. There is nothing to treat or cure. Not right now. I have heard there may be a drug for humans in 3-5 years, but I wouldn’t get excited yet because medical progress is slow.

Other families have told me they have used drugs to make the person with the suspected case of CJD comfortable. In my experience, this was not possible. There was nothing we could do to make my mother comfortable. However, the course of disease in my family is extremely quick. My mother and uncle died at about the one-month mark. Those of you who do not share my family’s specific E200K mutation will unfortunately find the course of disease may last for months.

I can’t imagine how hard that must be. I’m being completely candid here; I reached my breaking point the morning of the day my mother died. I don’t think caregivers get nearly the credit and support they need. But that will be another post on another day. Today, there are emails to return.

~Heather Larson

On Twitter: @heatherlarson

hotwords (at) mac (dot) com

Long time, no blog. Here’s why I took some time off from blogging about CJD.

I took some time off from blogging this year (nearly four months now) to let some ideas marinate, among other things. I do have a great deal to write about for you. This blog and the many CJD families have not been far from my mind at all. Quite the opposite.

CJD is something I think about daily. 

 

I visited UCSF recently and took part in their study and have continued to network with CJD families all over the world thanks to the magic of the Internet — this blog, Twitter, Tumblr, Facebook, email, etc. I think that I have some real hope for this first time in seven years of doing this and not just optimism.

I know from reading your messages that you come to me looking for hope.

No one is coming to my blog and then sending me emails about anything negative. We’re all in the same boat looking for answers and that boat may have neither oars or a compass, but we are collectively heading down the same river together. We all want to find answers, hope, and the best treatment for our loved ones as well as ourselves.

I started blogging about CJD after my mother died of CJD in November of 2004. My initial goal still stands and that is to create a place online that shows this disease is happening to people and that is deserves attention, advances, and a cure.

If you don’t speak up, you disappear. 

I also wanted to be able to fearlessly share my experiences, thoughts, feelings, emotions, and knowledge. This is why I posted the quote about courage with this post. It takes courage to speak up like I do, which is something that comes naturally to me as a writer and radio personality. I have always had something to say and desired to share my experience with you. This didn’t stand out to me as anything but normal when I began doing this. But after receiving many different emails over the years I have realized there is a lot of fear surrounding the discussion of CJD. I think many consider this to be a taboo subject.

So it’s becoming a new goal of mine to keep sharing so that others may also have the desire to share. CJD does not need to be taboo. There does not need to be a stigma, shyness, or shame surrounding this disease. There is no reason to be afraid of a CJD patient; give that person your love and support and especially support the loved ones who take care of that person.

The more we talk about our experiences as CJD families with friends, neighbors, coworkers, churches, doctors, nurses, health care providers — whoever — the more we will make others aware. The more we make others aware, the more they will care and the easier it will be for the rest of us to get funding to research institutions who need it.

I want this blog to highlight the people who are doing something about this disease. There are many people working towards the cure for CJD and they deserve to be written about. The only way to solve this problem of prion disease in the world is to find the cure. I hope my courage will infect others who will also be inspired to join the fight.

~Heather Larson

On Twitter @heatherlarson 

email: hotwords (@) mac (dot) com

(8/21/2012 edit: No, the irony of using an Oprah Winfrey quote above on a blog about prion disease has not been lost on me. I am well aware of how Winfrey’s failures have castrated the American beef consumer’s rights while only bringing us Dr. Phil. Thanks again for that, Oprah! She’s got some good inspirational quotes though). 

Started a CURECJD Tumblr account

In an effort to always be trying to reach a new audience in order to educate others about the horrendous disease CJD, I started a Tumblr account about it.

You can access it here as — of course — CURECJD.tumblr.com.

I think there are people who read lengthy blogs and then there are those who watch videos and digest smaller amounts of info…Tumblr is for that latter group. So the Tumblr account may be a more abbreviated version of this blog plus whatever reblogs I can come up with after I discover someone posts something on Tumblr worthy of reblogging…

Social media is just another useful tool in fighting the good fight. If you’re on Tumblr, please track the tag “CJD” on your dashboard and reblog the good stuff you can find. You never know who you end up reaching and how you can make them start to think about CJD.

You can follow me on Twitter @heatherlarson

Watch Christine Lord’s video tribute to her son, Andrew Black, a UK CJD victim

Asking Downing Street for help

I admire these people for heading to Downing Street to demand answers. The way Christine Lord has fought for justice for her son has inspired me for years now. I read this today and I can only think one thing. I just hope that the U.S. government can learn from the mistakes of the British government. To learn more about that, I recommend reading Sheldon Rampton and John Stauber’s “Mad Cow USA.”

Here’s a link to the BBC News story:

http://www.bbc.co.uk/news/uk-england-hampshire-13372323