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

Please participate in Footwork For CJD

This is an event this weekend in West Virginia put on by my friend, Nikki Bland, who also lost her mother to CJD.  If you visit the Snively family’s blog, you can see a cool video they’ve put together of last year’s event.  Even if you aren’t in West Virginia, you can still donate to the cause either through Nikki or the CJD Foundation.  If you write a check to the foundation, please write “Footwork for CJD” in the memo so they’ll know you’re supporting the West Virginia walk.  You can also donate online at the CJD Foundation’s website through paypal.

Here’s the walk info:

JOIN US FOR A DAY OF REMEMBRANCE AND AWARENESS!

WHO:

The Creutzfeldt-Jakob Disease (CJD) Foundation, with the event organized by Nikki Bland and family

WHAT:

A walk where you will collect pledges on a flat fee basis for your participation.  The amount raised is up to you!  The day will begin with a remembrance lap.  Learn more about Creutzfeldt-Jakob Disease and meet families who have been affected by it.

WHERE:

Duvall-Rosier Field located on the campus of Fairmont State University, Fairmont, West Virginia

WHEN:

Saturday, November 14, 2009 – registration at 9:00 a.m. and walk to begin at 10:00 a.m.

WHY:

To benefit the CJD Foundation’s HelpLine, the only HelpLine for CJD patient and family support in the United States, The Annual Family Conference in Washington D.C, and The CJD Foundation Family Discretionary Fund.

Please contact Nikki Bland for more information at bland_nikki@yahoo.com or curecjd@gmail.com.  You can also visit the family’s blog at www.cjdhope.blogspot.com for more information.  To learn more about Creutzfeldt-Jakob Disease, visit the CJD Foundation’s web site at www.cjdfoundation.org.

You can also make donations payable to:

CJD Foundation, Inc.

PO Box 5312

Akron OH 44334