well this is silly but...

This may be silly but...

I've read that many PALS are now living beyond, and some well beyond, the 3 to 5 year prognosis. Here, other Forums and media have people diagnosed back in 2010, 2011, 2012 and 2013 still with us. Some are into their 10th year.

Here's may be the silly part. Is ALS as a disease getting weaker on it's own? Is it the new medicines? Twenty years ago PALS pretty much left us within that 3 to 5 year window. That's what gave Neurologists the well published 3 to 5 year prognosis found in nearly every article describing ALS.

History has/had diseases that devastated populations before modern medicines where the disease eventually weakened and faded away.

Are we fighting harder and the folks of 20/30 years ago gave into it?

Al,

That's a very good discussion topic.

My doctor is convinced I've "had it" since at least 2012. I sprained my ankle working at home. The ankle I sprained was the first weakness the doctors identified and nobody was even considering ALS. It healed and I was back to running again but not as far and not as fast. In 2013 I fell running. From that point on I could only walk. The twitches started in the foot of the bad leg in Summer 2014 as did the cramps in that foot.

I read a book by a preacher who had ALS. He lasted 15 years without being vented. He worked for 5 years after his diagnosis.

Now, could all this be that we are catching it earlier? Doctors are more aware of it. My old neuro noticed brisk reflexes before 2012 and I remember finding it odd that he asked me to walk on my toes and heels and squeeze his hands. I had gone to him for migraine issues and Meniere's.

I'd be happy if the disease got weaker on its own but my guess is that we are just getting better at identifying it early on or, at least, suspecting it. I think we have social media and the internet to thank for that. It's the same reason we have so many DIHALS people here asking questions.

I think it is being diagnosed earlier.

My aunt who died in the 90s had symptoms for at least 10 years probably longer before diagnosis and lived 6 more.she lived longer than any family since. I think bipap and quality of life measures have had an impact. Riluzole ( which my aunt did not have) is supposed to add 10 percent. It is probably a little of both but sadly we still see far too many living a year or so - some significantly less

i think you have a point, Karen. so many people have spent well, well over a year (even years) for a valid diagnosis. it seems that neurologists are now becoming more adept in committing to a diagnosis. i believe my slow progression is due to starting rilutek at a very early stage.
- - frank

I've known so many that lived < 2 years.

I would be interested in an actual study being done on this however, as it's so easy to think there is a trend because we look at a small sampling.

I think too there are people who are here for a short time and don’t make a lot of posts. If their loved ones don’t come and tell us we may not know they have died. I have lost so many facebook friends. Most of my sod1 a4v friends die horribly fast and young often leaving young children. ALS is indeed many different diseases.

I do think there is a wide range of survival time (and my experience with ALS goes back some 30-35 years). I had a cousin that survived ten years after diagnosis, most of that time on a vent. His sister survived nine months from diagnosis. I’m approaching seven years since diagnosis, nine years since my wife first noticed symptoms and I’m still vertical. Last week the VA ran me through MRI’s, nerve conduction test, and EMG again and the VA neurologist confirmed ALS. She noted obviously slow progression and asked me if it just was linear decline or plateaus. I couldn’t tell her; I’m not sure if I notice a decline until I simply can’t do something anymore.

Bill

I have wondered if more people are choosing to live on a vent. It kind of seems like if you can keep breathing you can keep living (barring complications like pneumonia). Or even if not that, perhaps more are getting timely interventions like ambulatory aides (to prevent falls which could speed progression), feeding tubes (before major weight loss), bipap, pneumonia vaccines etc etc.

But I agree with whoever said we may not be "meeting" on this forum those who've had a very fast progression and lost their lives early on.

After observing people in my ALS support group for about 18 months I found a wide range of knowledge about the disease, even after months and sometimes years post diagnosis.

My best friend from the group, a lawyer, refuses a feeding tube. She can still walk. She is 66 and said she has had a good life and is ready. She resisted the Trilogy until she nearly died but now uses it full time.

So I believe survival is sometimes connected to choices and even the will to live and be productive. Other times, progression is so rapid there isn't even time to make informed decisions.

I would not have gone to Mayo if my local neuro hadn't insisted. He knew my body and did his own EMG. We've been friends for years and he said he has a suspicion after I sprained my ankle in 2012. I think I just found out very early. The doc at Johns Hopkins said the same thing.

For some of us, the vent isn't an option for financial and family reasons.

This is a wonderful thread with lots of great insights being discussed. Thanks Kristina.

I can't believe that I would be overjoyed to discover I had syphilis! I did request the test multiple times but no joy for me. The chances I have it is so low it is laughable but I still hoped.

I wonder about progression almost every day. I used to be able to dance like a Cossack or duck walk - whatever you call it. But years ago I dreamed I couldn't do it anymore and sure enough I couldn't. Maybe it was age? Or was it the very, very early start of motor neuron misfiring? Did our progressions start years before but were so subtle we thought it was old age creeping in. And then, after multiple subtle signs we hit the tipping point and something is obviously wrong.

My progression has not moved too much past my right leg in a year but my gait is profoundly affected now and although I can manage precariously with two canes I really need a walker. Co-workers who saw me six months before are shocked at my progression. Fast? Slow? Probably medium.

As far as how long to hold on - I have many lines in the sand I don't want to cross. Vent and feeding tube are two of them. I still have lots to offer and just last week I wrote a program that will save a client hours of manual, tedious work every day. And I have had some great software demos where I really connected with the customer and moved the deal forward. So I feel productive but I am also ready to call it a day when I feel too much slipping away.

But, to round off my long winded post - it is not over until it is over for all of us in our ragtag group. I don't hold up much hope, but what if one of these incredible dedicated researchers discovers a cure that stops progression completely? Look at CRISPR and the gene that the researchers recently isolated that causes the repeat. Or maybe a drug someone tests, maybe even for something else that stops it dead in its tracks. Nothing is beyond possibility. Or a nuclear war starts a chain reaction and life on earth is extinguished anyway. Ditto with a massive asteroid hitting the earth. I mean so many positive things could happen.

One thing I know for sure, my life and mental outlook would be much worse if I did not have you people for support.

i am late to the discussion. but i like the failing not feeling thing tillie brought up. everyday i sit here thinking that i going to have to get up and do this or that, then reality sinks in and i just sit here realizing that someone will need to do it for me. my body has failed but my mind still feels like i am me. my wife says that she has to keep me in line.

it is odd almost humerous to tell your foot to move and to look down and see nothing happen. it looses its humor when you sit on the toilet for two to three hours telling your bowles to push and nothing happens.

i got to the point where at my last clinic when they asked what they could do for me i replied "fix me". my reward for being a down trodden wise a$$ was "straight to HOSPICE with you, do not pass go, do not collect $200.00".

I have thought that the rate of my progression was based on where my body was when i started the journey. I was a very strong triathlete with an FVC of near 200%. i think the further you have to fall the longer it will take to get there. i took no meds until the last 6 weeks or so to fight the air hunger. it seem that since doing so things are going downhill very fast.

My husband has been a slow progressor but I think that is changing, maybe..

I read in one of my groups about a 17 year old girl who died of this. I am going to assume genetics and I am going to assume fast.

What a nasty, horrid beast.

I think if I had more family support AND lots of money, I'd get vented, when the time came. Some of the most gifted people with ALS do all kinds of productive work with eyegaze.

Maybe not. I always forget I'll be 63 this year. Mentally, I still think of myself as much younger.....until I look in the mirror.

Lenore, I saw where three teens died from ALS. I don't know the details but that's just horrible.

My husband is 67, and had a life that leads him to say “I had a good run”. He said that right after diagnosis. I think if we were wealthy we would do some things differently now, but venting is something I doubt Brian would even think of choosing.

Brian was so very physical on so many levels. Some live in their bodies, others live through them. He was definitely in that later group. I remember him running around the house and yard, going and going and friends saying things like “Does he never just relax? “ I would say that was exactly what he was doing. He loves to move, loved physical work, loved looking good and worked at that too.

Is it terrible of me that touching him hurts a little, much as I miss touching? I think of what his body was, how much he valued it, and how it betrayed us both. He has no interest in sex now and any pressure on his body is uncomfortable so I am not depriving him of anything with my mixed feelings about his body.

All of that hard work and dedication for the body that failed him. I’m taking care of him, of all the ironies.