functional rating scale and prognosis

[gooseberry]

Does anyone know how the functional ratings scale decline or rate of decli e correlates to prognosis? I can seem to find any articles about this. We all know you cant reasonably predict time but it would be helpful to have some parameters.

 

[Dizmom]

I have that same question goose. Hope someone has an answer.

 

[gooseberry]

I have been looking extensively....trying to be prepared. There is no chart or anything I could find based of progression or rate of progression.

 

[lgelb]

There is no good data because it's a sucky scale -- which is why the papers you will find are more concerned about other prognostic markers, on which there is no consensus as yet.

It's a real issue because clinical trials suffer from not knowing if pts are going to make it through the trial, and what their "normal" rate of progression would be w/o the intervention under test.

Modern datasets will generally support an "average" monthly FRS drop of 1-3 points, but that is not evenly distributed. The better studies suggest that progression is fastest in the beginning and the end, and slower in the middle, FRS-wise.

So if the score dropped more in a given interval than it had before, that might -- might -- suggest that you were coming out of the middle. But your own eyes are the best judge with breathing and nutrition the two must-haves. And as I have said before, proactive BiPAP management (adjusting settings as conditions warrant) can mediate breathing decline longer than some people think.

There are also papers purporting to show patterns of progression, but they're not very good either.

Sorry to be so unhelpful.

Best,
Laurie

 

[gooseberry]

Laurie, that is what I have seen. Steve had big declines quickly early on, then stabilized. To me he is moving quickly again. Friends that saw him at the holidays say there is a huge change.

 

[Diane H]

The revised FRS is too easy to misinterpret. It is intended to measure function, not really progression. The questions ask about your difficulty breathing. Your score drops as the need for BiPAP increases. When you go on BiPAP or a vent, your score rises abruptly because you are no longer having breathing problems! This makes it a decent tool for assessing your functional ability when using equipment to replace strength, but doesn't track progression. Your score improves even though your condition has progressed so it doesn't correlate to life expectancy at all beyond that point.

I have also seen a version of the test that asks how long you have had ALS but won't let you enter anything over ten years! This is the ALS version of Age Discrimination! Should I call the ACLU? ;-)

 

[affected]

I know we want to know, to plan, to feel some kind of little bit of control over what will happen when and how to best face it.

But we really can't predict this, we just can't. It's the one constant thing I've found with ALS is that it is so unpredictable. Sorry Steph, we all wish we could answer this one.

 

[Barbie]

I studied those scales for years and never could find the answer you are looking for. We saw a sharp decline in the first year, then plateau and then drop--a pattern that repeated for years (for us) the final drop for my man was the breathing and that is what we all know is what does our loved ones in. know you have fought the breathing from the beginning so it is tougher for you.

I wish to god there was a better answer!

 

[gooseberry]

I just see the struggle to breathe getting worse daily. Today he fell again. I feel like I am beating my head against a wall trying to keep him safe but he refuses. No real injuries from the fall...which is good. I hope they can beat some sense into him next week during his assessment

 

[Dizmom]

I want to thank everyone, I suspected this was the answer. I have seen a rapid progression in the past 3-4 months. Last FVC was 17 (Jan). I have timed walks to that bathroom, more empirical data, and found that it takes 100% more time this month vs Dec. One foot is kind of dragging, and I have to steady him more often. I fear walking will soon not be possible and PAL says no walk no food. I wish he would wear his bipap more during the day but refuses but as I have heard and agree with PAL gets what he wants if I am able. When he does biPap then the walk seems easier for him.

Cheers everyone TGIF!

 

[Buckhorn]

Great question Goose - I have wondered the same. As Diane H stated, the FRS is a tool to measure functional ability, but there is very little correlation data about the actual progression of ALS, because progression of this nasty disease is so variable. I view ALS as the neurologic equivalent to cancer ........ simplistic perhaps, but in essence, there are many, many possible causes, various progression rates,
etc. Just no viable treatment options other than Rizulole.

 

[gooseberry]

Part of why I asked the question is to get some idea if anyone here knew of a correlation in decrease of rating and prognosis, but also because clinic,the va, basically any neuro he has seen performs this scale assessment. I just wonder what the point is.

 

[lgelb]

Depending on who you are, the point is:
1) To complete a chart
2) To establish eligibility for a clinical trial
3) Data collection/cross-correlation with other variables for research and reporting as institutions mine and combine their data for publications, reports to regulators/funders, grant applications

That's about it. It was never intended as a prognostic index.

 

[gooseberry]

#3 is the only one that has ever applied to Steve really since he presented with such reduced lung capacity. Just tough right now because the changes are quick and forever. Steve couldnt get into bed last night. I had to lift his legs and put blanket on him. His disease is different from many here but the limb piece and the breathing are showing swift changes. Yes I am grasping for straws, I know that. I just hate watching this disease destroy him.

 

[Buckhorn]

Goose, I am so very sorry. I am not accustomed to feeling powerless, and with ALS, that is what so many of us feel. That and heartbreak, frustration, anger, emotional exhaustion ........