Predictive model based on ALSFRS-R score?

[Emanol]

Hello,

Does anyone know a good model of disease progression based on ALSFRS-R score? My father was told his score was 38/48, after 2.5/3 years since symptom onset. Could anyone point me to a good resource where rates of progression are plotted?

 

[affected]

Everyone progresses so differently - the score is not used predictively I'm afraid.

 

[Lkaibel]

Predictive is not a word much found in ALS. At diagnosis, my late husband looked like possibly he was in a rapid progression. Over the 15 months after Dx, however it was slowish. After that, progression did speed up considerably, but he was still able to speak and swallow when he passed and his lungs were only compromised the last few months of his life. That stop, start, stop pattern is actually fairly common in ALS.

 

[lgelb]

The FRS is a categorical scale, not a linear one. And losses appear sudden because a critical loss of motor neurons -- a threshold -- is reached.

We generally say ALS is most often fast/slow/fast.

The published models that cite five stages and the like don't explain much of the variance, don't account for different sites of onset/progression well, conflate bulbar, trunk, and limb features, and aren't really predictive as much as vaguely descriptive.

At a guess, your dad is on track for a >5y ALS experience but nothing is set in stone.

 

[Lkaibel]

Then there is the whole separate question of choices around that progression. Trilogy, feeding tube, venting are all examples of choices made. Even a power chair is a choice- one my husband chose against. All have a moderate to large impact on longevity.

 

[Jamesgol]

Emanol,
I am 3.5 years from onset. I score around 38. My progression has been slow but seems to be speeding up. Most of my involvement is limb only, with my left side being weaker than my right side. I have no bulbar involvement.

I am doing everything I can to go as far as I can. I use a ventilator at night even though I can breathe fine. I get help with EVERYTHING to maintain my energy level as high as possible. I eat well , in fact, I I've gained weight. I have a PWC , hoyer lift, and toilet/shower chair. I take Riluzole.

James

 

[lgelb]

Yes, you are right, Lenore -- the choices of intervention/adaptation can greatly influence quantity/quality of life, and at times that includes doing less instead of doing more.

James, I'm glad you're progressing relatively slowly at this point.

 

[affected]

On top of intervention strategies is our whole theme of preventing falls. Everything can change in an instant.

 

[dldugan]

I first noticed symptoms that I recall in 2016, but may have had some in 2015. My first EMG was in 2017 and the bad neurologist said he found nothing. In 2018 I insisted something was wrong and had a myriad of tests and another EMG. The bad neurologist and I disagreed and I asked for a referral for a 2nd opinion. He made one for me 5 months out. I cancelled it and made an appointment with a neuromuscular specialist 2 weeks out at a highly rated hospital. That’s when I got the DX, but I wasn’t surprised. This was in September 2018.

At this point 6 months from DX I have extreme left side weakness. Right side is starting to weaken, especially the right arm. I went from a cane to a rollator to a transport chair to a power wheelchair in 6 months. I’ve tried to stay a step ahead in preparing for the progression, but it has been difficult. Went on Trilogy in October. Have now ordered a hospital bed and will be looking at transfer options this week. I have been using the rollator for a few step to transfer, but that will soon no longer be an option. I am also looking for transportation and have located a local company for short trips. I will be renting a handicap van for longer trips. My daughter or wife will drive.

Seeing that my wife would be too overwhelmed staying in our Indiana home where I had a bed in the first floor den, we moved to Illinois to live with my daughter. Our house sold in 2 days so we are now planning on the next step. It is hard to stay ahead of this monster, but we need to try to do so.

 

[Emanol]

Emanol,
I am 3.5 years from onset. I score around 38. My progression has been slow but seems to be speeding up. Most of my involvement is limb only, with my left side being weaker than my right side. I have no bulbar involvement.

I am doing everything I can to go as far as I can. I use a ventilator at night even though I can breathe fine. I get help with EVERYTHING to maintain my energy level as high as possible. I eat well , in fact, I I've gained weight. I have a PWC , hoyer lift, and toilet/shower chair. I take Riluzole.

James

Glad to know! The thing is my dad does not really like to get help... he still dresses himself, cooks, sets the table, etc. I am glad he is still able to, but sometimes I also wonder if he should not save his energy for something more meaningful than the daily chores.

Since he has had no issues with breathing at all, no decline whatsoever, we have decided not to start with the ventilator at night, but that might change soon.

My father has also gained quite a significant amount of weight!

 

[Nikki J]

E, last I remember your dad was hovering between PLS and ALS? With PLS the general thought seems to be use it or lose it as opposed to ALS where conservation of energy seems to be the way to go.

When your dad does these things do they exhaust him? If they do they may be too much. If he still does them with relative ease then they are probably ok

 

[Emanol]

E, last I remember your dad was hovering between PLS and ALS? With PLS the general thought seems to be use it or lose it as opposed to ALS where conservation of energy seems to be the way to go.

When your dad does these things do they exhaust him? If they do they may be too much. If he still does them with relative ease then they are probably ok

It's tricky. He has no signs of LMN involvement at the clinical level, but a third EMG revealed slight if mild abnormalities (fasciculations in several non-bulbar regions, albeit without active denervation)