What Statistic About ALS Awakens You to How Much Our ALS Community Needs Awareness?

[Fran Finney]

There are many TERRIBLE statistics associated with ALS! The one that horrifies me the most, and one that most of the public is still unaware of, is the more recent realization that as the disease progresses, in addition to physical losses, at least 50% of pALS experience cognitive deficits. We need more funds and studies to stop this progression ASAP!

Here is a link to and an excerpt from a (relatively) recent review article:
https://www.targetals.org/2022/03/08/its-not-just-physical-als-dementia-cognitive-impairment-and-memory-loss/
“The decline in social cognition and executive and verbal memory functions we see in some people with ALS cannot be blamed on physical deterioration alone. In fact, a 2020 study found a progression of cognitive and/or behavioral impairment in more than one-third of patients with early-stage ALS. Other research suggests that about half of ALS patients demonstrate mild to moderate cognitive and/or behavioral impairment, and about 10% to 20% of ALS patients meet the criteria for dementia. Roughly 30% of ALS patients develop impairment without dementia, and up to half of ALS patients are cognitively normal.”

[Dagmar]

It seems from your article and sources that dementia is primarily affecting those with a mutation in the C9orf72 gene . I have seen little on sporadic ALS causing dementia. Probably needs more study.

[Fran Finney]

I agree that we need much more research in this area! We know ALS impacts the central nervous system and the brain, and the interaction between ALS and cognitive functioning is very complex. FTD is not the only potential manifestation of impacted cognitive skills. My husband Hal was literally a whiz at mental math and complex reasoning before he had ALS. He was considered a “genius” by many and won national math contests. He loved to solve complex puzzles and write codes. Hal lost his ability to do quick mental math early in his ALS progression, and he also believed the speed of his thinking slowed along with the weakening of his body.

[Dagmar]

I am so sorry you lost your husband to ALS. Thank you for your commitment and ongoing efforts to raise awareness. We appreciate YOU 🙂

[Fran Finney]

Thank you, Dagmar. We appreciate YOU too!

[Kathleen B (Katie)]

Not sure if it’s a statistic, but I consider the fact that ANY kind did of loss of function in even 1 body part is SUCH an alarming loss of ability —arm, voice, leg, hand, face, core, hand, foot—because it signals a cascade of continuing progression for which there is little to no stoppage!  Even trying to learn why 10% can go beyond 5 years has not been determined—and that knowledge alone could help that survival percentage rise!  I am totally alarmed that so many talented, vibrant, wonderful people are still becoming an ALS statistic… very few outside our pALS & caregivers REALLY know the devastation of personhood this disease causes for those afflicted.

[Mike Leist]

In the days when Joseph Stalin was Commissar of Munitions, a meeting was held of the highest ranking Commissars, and the principal matter for discussion was the famine then prevalent in the Ukraine. One official arose and made a speech about this tragedy — the tragedy of having millions of people dying of hunger. He began to enumerate death figures … Stalin interrupted him to say: “If only one man dies of hunger, that is a tragedy. If millions die, that’s only statistics.”
The point he was trying to make is that statistics depersonalize things that happen to us, or around us. So instead, I’m going to tell you a quick story about me and ALS. Because ALS is deeply, deeply personal. But if you need something to make it personal to you, how about this: 1 in 400 people will get ALS in their lifetime. So you, or someone you know, will die from it. Because it’s 100% fatal. That should make my story more personal for you. My name is Mike Leist, and this is my story.
8. 8 years ago this month, in February of 2015, my life started to change due to ALS, slowly at first. I started to infrequently slur my speech. At first, it was only when I had a beer or two with my friends. I didn’t think much about it, and blamed it on some major dental work I’d just had done. Then it started to happen after I ate lunch, with certain types of words. I was a teacher, so slurring my words was concerning. Then one night I looked in the mirror at my tongue, and it looked like there were tiny worms under my tongue moving around. I realized something more significant was going on, and made a doctor appointment to get checked out. The first of many.
7. 7 years ago this month, after several doctor appointments and many tests, I was diagnosed with Bulbar ALS. When the neurologist gave me the diagnosis, my wife Lis was with me. He said, “You have ALS, a disease for which there is no cure. The life expectancy is 2-5 years. I suggest you get your affairs in order.” I was only 45 years old, and my world was shattered. Lis and I both sat in the office and cried a lot. On the drive home we had to pull over 3 times because I was hyperventilating. I kept picturing old couples holding hands as they walked together, and realized that was never going to be us.
We decided not to tell the kids immediately, but to wait until the end of the school year, which was only 3+ months away. Two were Freshmen and our oldest a Junior in high school, and this would be hard enough on them without having to focus on school. I told only a few friends at first, until school ended. Then after exams, we told the kids, and then co-workers, and my dragon boat team.
6. 6 years ago I had to stop teaching, because my voice had gotten bad enough that it was impacting my ability to do my job. Around 4 months after that, I lost my ability to be understood all together. So, I started using a text to speech app on my phone, and a Bluetooth speaker, to talk and amplify my voice. I also got a feeding tube, and had to start being careful about what I ate, because I was losing my ability to swallow. I had quite a few instances of choking that year, which was pretty scary.
5. 5 years ago I had my first non-bulbar functional impact, which was head-drop. I had to get a neck brace to support my head, and had to give up dragon boating, since keeping your head up while paddling is pretty important. I continued to lift weights, do cardio, and walk, but I could tell that I was losing strength in the weight room. I was also having issues with saliva, and trying different types of treatments for that.
4 years ago, I started losing my balance and falling. A lot. By August of 2019, I had to move into a wheelchair permanently for safety reasons. My functional strength was really starting to crater, to the point where I was having trouble texting on my phone by the end of the year. I got approval for, and began to use an eye gaze device.
3. 3 years ago, in March of 2020, I had a partially collapsed lung, and had an emergency tracheostomy. Because, I wasn’t ready to die. By the time I exited the hospital 19 days later, I was pretty much fully paralyzed. Since then, life has been pretty much the same. I try to make the best of a shitty situation by making the best of it, and doing the things I can enjoy, all with my eyes. I read, or rather listen to, a ton of books. I watch what I want, when I want. I do fantasy sports, and play games like cards or scrabble. I don’t know how long I can, or will want to do this. I’m 100% dependent on other people – my wife, nurses and home health assistants – to do anything physical.
0. There are zero treatments, or clinical trials, which offer any hope for anyone with end-stage ALS like me. There’s one treatment that’s offered some success with halting the disease with some people, but not all. And that treatment isn’t yet approved by the FDA. It’s called NurOwn, and is in a phase 3 clinical trial. But it doesn’t reverse ALS, or repair the damage that’s been done. Clinical trials will only typically allow people who have been diagnosed less than 2 years ago to even enroll. It’s going to be years yet – best case – before anything comes around that might provide even the hope of neuron regeneration. And in all likelihood, my story will have come to an end before that happens.

[Amanda]

Mike,

Your post brought me to tears. Unfortunately, I’m sure your story sounds all too familiar to many of our pALS.

Thank you for sharing.

Amanda

[Kathleen B (Katie)]

Thank you Mike, I now understand I’m a statistic…the example & clarification made total sense.  As an educator colleague, you were likely quite a gift to MANY students along your career path-you made a positive difference!  Your ALS story is indeed the reality of this disease, presented with concise clarity.Thank you.

[Dagmar]

This is a sad, but too-true statistic – – in this recent article, “Delayed diagnosis in ALS found in new AI analysis of patient records,” – – the time from first symptoms to diagnosis was a median of 11 months.

Doctors need to become more aware of ALS symptoms, the medical system needs to become unclogged (too many nurse practitioners??), and potential patients need to not ignore their initial symptoms.

We don’t just need to raise awareness for funding… but also for diagnosing.