Living with ALS: Beyond the Ice Bucket

By now, you’ve heard of the Ice Bucket Challenge. Chances are, you may have even dumped a bucket of ice water on your own head and, hopefully, you’ve made a donation to ALSA.org. I’ll admit, there were a few times when I thought, jeeze, this Ice Bucket Challenge is really taking over my news feed. But, then I heard that ALSA.org had raised something like 4 times the amount it had raised year-to-date last year. And that number just kept growing. Last check, the Ice Bucket Challenge has raised over $70 million dollars. That’s awesome.

You see, we don’t talk much about ALS because, well, the disease is devastating. It’s hard to talk about. And that’s why it’s so important that we do.

Karen Shideleff was diagnosed with ALS three and a half years ago after feeling symptoms for about six months. Because Karen’s mother had familial ALS, Karen lived with the knowledge that she had a 50/50 chance of developing the disease herself.

I’m honored that Karen agreed to talk with me about her life with ALS and how the disease has affected her family. You can read a condensed version of our conversation below and watch the video at the bottom of the post for the full conversation.

Caitlin: To begin with, you lost your mother and grandfather to ALS and you told me you were the 25th person in your family tree to be diagnosed. When did you first realize that ALS was something that you might one day be diagnosed with?

Karen: When my mom was sick, my parents didn’t want to point out to us that it was a hereditary form of ALS because my three sisters and I were pretty young. We were 16,18, 19, and 20 years old when my mom was diagnosed. And we didn’t know our grandfather, so it wasn’t something we had lived through prior.

When I got into my 20’s I was in nursing school and I was taking classes in biology and genetics and I kind of put all the pieces together. It was very shocking to figure out that I did have a 50% chance of developing ALS.

Caitlin: And that’s because it was familial ALS, which I read accounts for about 10% of all ALS cases?

Karen: Right, so they say 5-10% of all ALS is familial. The rest is sporadic which means they don’t know why it happens, it’s a random occurrence. So familial ALS is rare, even within the umbrella of ALS.

Caitlin: So when you realized that there was a 50/50 chance that you would develop ALS, how did that change your life? How did it change your plans?

Karen: I was 21 or 22 when I figured it out and I was already dating my husband so at that point I made the decision that I didn’t want to have children because I didn’t want to take the chance of passing that gene on. It’s a really personal decision. I never had an overwhelming urge to have children and my husband didn’t either, so for us, it was a little bit easier of a decision to make. But, you know, it gets harder and harder as you get older and all your friends and family are having children. So it’s a decision we just consciously made throughout our relationship and our marriage.

And really, we just try to enjoy life. We’ve done a lot. We’ve traveled a lot, we enjoy spending time with our friends and family, first and foremost. And we’ve really just built our life around that. So I always say, I don’t have any regrets with my life. I don’t feel like I’m missing out on anything, which is good. I don’t know how many people can say that.

Caitlin: Is there a constant fear when you know you could develop ALS? You know, I think of the things people get anxiety over and something like this is just more than most people could imagine. Did you live with the fear until you were diagnosed? How did you handle that?

Karen: It was a constant fear. I would be lying if I said it wasn’t. And it’s still a constant fear for my sisters, unfortunately because they don’t have any idea if they’re going to get ALS.

But, every time you stumbled on something or you trip and fall or you get muscle twitches, anything that might feel like the beginnings of ALS really just sets it off in your head and you have to convince yourself, I’m just tired, or I just exercised too much. So, it’s very hard. It’s constantly on your mind. And unfortunately for me, it became a reality.

On the flip side, at least a diagnosis when it’s familial ALS, you already know so much about the disease, so I think the acceptance portion might be a little easier to come by. Whereas, somebody who just gets stuck with an ALS diagnosis and doesn’t know what it is, that’s a really difficult time to understand what it is and explain it to your friends and family. So, in some ways, I know it sounds ridiculous, but I’m glad I knew what I was walking into.

Caitlin: That doesn’t sound ridiculous. I think it must be damn near impossible to accept either way, but it does make sense.

Karen: Well, my friends and family all know what ALS is. So when I told them I was diagnosed, I didn’t have to explain what the disease is. And I had been fundraising for ALS for years, so even my co-workers and people I’ve been friends with all knew what ALS was.

Caitlin: So, how did you finally get diagnosed and what was it like to hear those words?

Karen: I noticed things were happening with my body and I knew something was changing. I was very active before I was diagnosed and I walked my dogs all the time and I noticed that my route that I always walked with the dogs was taking me longer and I noticed that my footsteps started to sound different on the pavement. This was maybe July of 2010. So, I just started to pay attention. And as it progressed along, I noticed my balance was getting worse. So, I took yoga, thinking, well, maybe I’m just getting older, trying to convince myself that it’s not what I think it is. I would say by Thanksgiving of 2010 I was pretty certain I had ALS. It was getting harder for me to climb the stairs and things like that.

In January I spoke to my husband about my concerns. And my doctor is actually my husband’s cousin, he’s an ALS specialist at Lehigh Valley Hospital, so the next week I got a visit with him and by the week after I was diagnosed.

Still, hearing the words, yes, your EMG shows that you are definitely showing signs of ALS, as much as I knew what it was, it still was pretty bad. My husband was with me and, you know, you think you can set up in your mind, get yourself ready for it, but hearing it just sucked. I cried the whole way out of there and the whole way home and even more when I got home.

Caitlin: I imagine telling your family members must have been an exhausting time.

Karen: It was. It was brutal. We didn’t tell anyone for a month. I waited and talked to my dad first and then my sisters. It just brings ALS right back into the forefront of everybody’s lives which is tough.

Caitlin: So how have your symptoms progressed over the last 3 and a half years?

Karen: So, ALS can present differently with different people. The two main ways of presentation are either bulbar presentation which affects speech and swallowing first and maybe some more respiratory problems and then there’s limb presentation which is more arms or legs. Typically it starts on one side. For me, I felt it in my right leg first and then my left leg started to have issues several months later, as far as balance, strength, and atrophy. But, there are differences between each patient, even my mom and I who have the same genetic mutation.

I had been working as a pre-op/recovery room nurse, but I started to not trust myself as much with the patients. You do these fall risk assessments with patients when they come in and when your fall risk is higher than theirs, it’s probably a good time to stop taking care of patients (laughs). But, in all seriousness I was afraid if a patient needed me to hold them up, we would both fall and get injured. So, out of responsibility to the patient and myself, I had to stop doing any hands on nursing. But, my employers were fantastic and they just kind of molded a job for me. I’m so thankful that I was able to work for people like that, that respected that I still had the brain of a nurse.

I went from no walking assistance, to a cane, to a walker. Once my shoulders started to get weaker and I couldn’t drive anymore, it really took a toll on me. So, I stopped working. And now I volunteer at the ALS Association. I am really involved with the chapter. I joined the board of trustees and I’m chairing the patient and family services committee, so it keeps me really involved which is great.

Caitlin: So, you’re in a wheelchair now.

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Karen: I am. I can still stand, like at the counter I can maneuver around, or if I’m holding on to somebody I can do a couple of steps, but because my hips are so weak, my stability is really shot. So, if I’m home alone, I’ll only stand if like the wheelchair is behind me and counter is in front of me. But, I spend the majority of my day sitting in a wheelchair.

Caitlin: You know, if you were really negative through all of this, no one could blame you. But, you’re really positive, and I’m wondering if that’s a choice you have to make every day. How do you do it?

Karen: As I was going through telling everybody about the diagnosis, I would jokingly say, “Fake it until you believe it.” Like, I just can’t go through these months being so incredibly stressed out without finding some joy, somewhere. And my husband and I are like, okay, we’re not watching any sad movies, only comedies. We have enough crap going on in our lives, we just need something light. And I’ve always been a glass half full kind of gal. Even as a nurse, I loved helping people and making a difference for them. So, if I can make a difference for another ALS patient, I try to figure out how I can still be helpful to other people.

And, you know, I have my moments just like everybody else, but I just try to be grateful for what I have in my life and that just carries me through for right now.

There’s no pity party here. Yes, it sucks and we shed our tears every once in awhile, but then you just put on your big girl panties and carry on.

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Caitlin: So, the Ice Bucket Challenge. It’s everywhere! What has it been like to see everyone talking about ALS?

Karen: You know, there are so many things that are amazing about it. So many people are talking about ALS and hopefully even if a small percentage of those people look it up and learn about ALS, then maybe they can explain to somebody the basics of what ALS is. Sometimes people will say to me, “Why are you in a wheelchair?” And I’ll say, “Because I have ALS.” And they just kind of say, “Well, I hope you get better.” Well, I’m not going to get better, but you’re not going to say that to somebody.

I’m so happy it’s getting national attention. I think the majority of people involved with the Ice Bucket Challenge know it’s fatal. No one survives ALS, and it knows no boundaries. It will attack men and women, young and old. Hopefully those are the things that are getting across to people. And that there’s no treatment and there’s no cure for this disease. And then the amount of money that’s been raised, it’s just crazy.

There are scientists trying to figure out what’s causing ALS and there’s just not a lot of funding. Our numbers are too small for big drug companies to care. I know that sounds horrible, but we’re a small population, we’re not profitable. So big pharma is not interested in helping to find a cure or a treatment. So, this bulk of donations that have come in is amazing.

Caitlin: What ALS does to a person is so scary. What is one thing you’d want people to understand about ALS?

Karen: ALS is not just affecting the patient. It affects their whole family and their friends. Eventually you become so incapacitated, you can’t do anything for yourself. You can’t feed yourself, you can’t dress yourself, you can’t bathe yourself. All the things we take for granted doing every single day of our lives, all of that gets taken away from you. Even something as simple as losing your driver’s license, losing that independence, it’s crushing. And on top of that, the emotional toll the disease takes is exhausting and it’s hard that nobody knows what ALS is.

You feel like screaming, “No! It’s not okay!” I have family that I’m worried about. I’m doing everything I can. I participate in trials and studies. I’m trying desperately to find a cure, not for myself, but for my sisters if they need it. Hopefully they don’t. But, there’s not a day that goes by that it doesn’t cross my mind.

Caitlin: Is there anything else you’d like to add?

Karen: For everybody doing the Ice Bucket Challenge, I think it’s great and I want to say thank you. It’s overwhelmingly crazy to me that millions of people are dumping ice over their head and saying ALS when they do it. My news feed is filled with them and they make me laugh and they make me smile. And if people are donating also, even better. Pete Frates, who is one of the people who started the Ice Bucket Challenge, I can’t say thank you enough to that gentleman and his family.

Let’s keep ALS out there. Let’s not forget what ALS is or what it does to patients like myself when the Ice Bucket Challenge stops and we’re still living, hopefully very well, with ALS.

Watch the f

If you haven’t donated yet, please join me in donating to Karen’s fund for the Walk to Defeat ALS. Click here to do so. Again, you can watch the video of my conversation with Karen here. Thank you!