Severe declining of ALSFRS-R and FVC

[jethro]

i saw at bedlack's presentation where he states that when alsfrs-r starts to decline, it doesnt stop. as i remember, that "point" was" app mid 25 alsfrs-r. of course, it is individually. alsfrs declines lineary, fvc functionally.
does somehow FVC follows alsfrs-r? my fvc dropped app 10% and alsfrs didnt dropped such.
my alsfrs-r score didnt declined much in a last 12 months (all declining happend in a last 6 months, so it is not good idea to write such a thing now).
in a last year my fvc dropped from 97 to 84. i guess, most of "dropped" points happened in a last few months.
average falling alsfrs in a 1st year is 0,6 pts/month, 2nd year is app 0,34pts/month.
any comment?

 

[lgelb]

Three of 12 ALSFRS-R questions relate to breathing, so of course, they are related. But the FRS is not linear. It's categorical. People's scores therefore decline in a gradual but not completely predictable way. If you look at the questions, the "tipping points" for each number of points (0-4) are in no way evenly spaced. For example, the difference between two and three+ pillows is way smaller than the difference between three+ pillows and can only sleep sitting up. The worst level in that question, "unable to sleep," would mean death.

Nor do the 5 options for each question cover the waterfront in terms of possible situations, so it's sort of a "best approximation" assignment of scores in many cases.

For anyone interested, the scoring is easily obtained by Googling.

 

[Kristina1]

im not 100% sure what you're asking here? but fvc is the breathing component in ALS. So it will affect your FRS scale in those 3 breathing questions Laurie mentioned. But I've heard of PALS whose breathing was affected last, so they had been in a wheelchair for years before needing breathing support. Or the other way around, breathing being affected early on and the PALS dying while still being able to walk and what not-- my doctor had had a bulbar onset patient like this that she mentioned to me when we were discussing progression and death in ALS.

In my personal opinion I don't find FRS scores to be very useful beyond their purpose as a [frankly somewhat crude] tool to quantify/measure patient progression in research studies. The answer choices for each question tend to be somewhat subjective and usually encompass a large range. By range I mean, taking question 1 for example, I have been on "intelligible with repeating" for a LONG time. In the beginning I qualified because people would need me to repeat a word once every few sentences. Gradually I have had to repeat myself more and more. So that answer has the potential for a really wide range of speech intelligibility. And I find most of the questions are like that, **especially** around the third answer. For the longest time I went down about 1 point per month, but my score got to 24 and then stayed there for 6 months, losing a single point this month. And it's because most my answers are the 3rd or 4th one, and there's been a long range of decline that still fits under the same answer.