"Early stages but progressing" he said

[initforever]

My apologies if this is not the proper thread, however it is my first time coming out from behind the curtain. I have been following and learning about ALS since we first suspected the possibility months prior to receiving a diagnosis. My PAL was confirmed and reconfirmed 5 months ago. On several threads it has been discussed about slow /rapid progression however I've not seen a "definition" that explains the difference. Is it determined by a timeframe, by the loss of muscle use or the inability to do certain things? In my heartfelt opinion it's all too fast and too soon.

 

[affected]

Welcome to the family! I'm so sorry you have to join us, but at least you have found the best!

It is about the time frame and we don't really know how fast progression will be, but we can look back and see it.

My husband was gone 11 months after diagnosis. Every single week there were more and more things that were failing on him, that he could not do. That was rapid progression. I was running constantly to try and keep up with more equipment, different ways of doing things, more services and help as nothing stayed the same.

Some PALS (person with ALS so it's singular and plural like sheep ) will lose some ability and then stay at that stage for months or longer before that part gets worse. So for example walking is hard so they get fitted with an AFO and can walk safely for many months.

We can't say in advance what any PALS will be. My Chris remained rapid progression from a certain point, but some PALS will progress rapidly then suddenly plateau for weeks or many months without any seeming reason.

Definitely it's all too fast and every loss is too soon. We try to live in the day, as that's all any of us have no matter whether disease is a factor or not

 

[soonerwife]

Welcome initforever! Sorry you have to be here.

My PALS has Bulbar Onset which started in his tongue. His neurologist considers his progression slow. Slurred speech started in July '14. He was diagnosed in Oct '15 and had clinical weakness in his ankles at that time.

Bulbar is USUALLY a quicker progression. They were surprised at his being as slow.

He now can not speak, has a feeding tube that he uses for 100% of his nutrition and is barely walking with AFOs and a cane.

They say every case is different...

 

[bkite]

Hi and welcome to the forum. Sorry to hear of your PAL. As far as measuring speed of progression, there is no consensus. You read in clinical trial studies about the use of the ALSFRS-R. The ALS Functional Rating Scale revised. This is available on line and you can chart your PAL's scores every month or two with it. The scale has a graph with different curves of progression, and you can observe which curve your PAL'S scores line up with.
The Seattle ALS Clinic that I attended never talked about the above scale and only looked at lung function test results. And they didn't talk about an end point. As my FVC dropped to 70, we began planning for feeding tube placement. When it got to 60, night time ventilation was started.
Hope this helps.

 

[lgelb]

Welcome, IIF. Sorry you have to be here.

I have moved this thread to "Newly Diagnosed," so don't worry about where to post things -- we will put them where they should be if needed.

There is no formal definition of slow vs. rapid progression, though you can see others' FRS curves on PatientsLikeMe if you care to. The FRS scale is a very rough snapshot of function and only measures a few things, so we don't take it too seriously, though a certain score is required for some clinical trials.

However, based on published data over time and experiences here it is fair to say that living less than two years after diagnosis vs. living more than four or five are the ends of the spectrum and that most people fall in between. You will see that a "median" [half more, half less] life expectancy is 2.5 years, but those numbers are kind of old.

So here, rather than worry about trend lines, most of us prefer to focus on what will gain your PALS the best balance between quantity and quality of life.

Those things include, but are not limited to: avoiding falls with appropriate mobility devices, respiratory support [BiPAP] as soon as it's needed, maintaining weight with a balanced but not obsessive diet, hydration without bathroom visit concerns, physical activity that's not exhausting, active-to-passive range of motion exercise, a bed setup that allows for deep, refreshing sleep, some kind of hobby or activity that's enjoyable, interaction with nature, pets, family and friends.

Best,
Laurie

 

[initforever]

Thank you each for your input. I understand that each person's journey is unique unto themselves yet I just wanted to make sure I had not overlooked/missed something that would help in getting ahead of each month becoming a "new normal". As I'm sure many of you understand, it's been hard to get our arms around today's "new normal" before the next "newest normal" throws itself at you.

 

[affected]

Do read the stickies on being newly diagnosed and anticipatory planning as they will hopefully give you a lot of tips on 'getting ahead'

 

[Jlynn]

One day at a time. At the beginning of my brother's diagnosis I would try to think too far ahead. My brother was actually the one that suggested we just do one day at a time. It seems to work for us (most of the time). So sorry to have to welcome you here but you really have come to the right place.