Progression discussion

Status
Not open for further replies.

KevinM

Distinguished member
Joined
Mar 30, 2019
Messages
376
Reason
PALS
Diagnosis
06/2019
Country
US
State
FL
City
Tallahassee
Hi all. Rainy Saturday in north Florida, and as usual I have too much tIme on my hands and too many words on the tips of my fingers.

I saw this question posed on the public Facebook page, and it is one I would like to explore with our forum members. The question is “what is meant by slow progression? As the poster pointed out, we all have a good idea of fast progression, but I’ve not seen a medical definition for slow progression I realize there are many variations within each “category,” and it may be that only the passage of time will allow for a clearer idea of whether one is slow or not.

Timeout...we have a tornado warning and one has been spotted on radar a few miles from our house. Be right back...

Okay, back on the porch. The thunderstorm showing radar rotation went right over us, but apparently the twister didn’t touch down in our immediate area. Hoping the rest of the region fares as well.

So back to the question. Have any of your neurologists attempted to quantify slow progression? The easy (and incomplete) answer would be that if the average loss of function is about 1 FRS point per month from diagnosis, then anyone losing function faster than that would probably be considered a faster progressor, and anyone slower than that would be in the slower category, but that seems too simplistic. Plus, as has been pointed out here, there are plateaus, UMN vs. LMN predominance, bulbar vs. limb onset, etc. And progression is rarely linear.

This question also ties into when the clock actually starts ticking, more precisely from symptom onset or from diagnosis. I have read of many PALS that had mild symptoms for a year or longer—sometimes much longer—before seeking a diagnosis, which would seem to point to slow progression if one defines progression from symptom onset. However, by the time they did seek a diagnosis functional failure was starting to occur, and from that point on progression occurred more rapidly since enough motor neurons had died to reach a tipping point. So would they still be seen as having slow progression?

Perhaps there is no hard and fast definition because every case is unique, and only time will tell. One PALS friend of mine lost the use of her right hand and then arm within a year of diagnosis, but it was confined to that limb for almost five years before other failures started to occur. That would definitely seem to fall under the category of slow progression, at least for the first five years. Others have gone for years without losing complete use of any one muscle group (seems more frequent with UMN dominant PALS), instead having a slowly progressing systemic weakening. In a number of cases, FRS scores have remained in the mid 30’s to mid 40’s for several years.

Love to hear some thoughts on this, especially if your neurologist has defined it for you. Happily safe with house intact, Kevin
 

lisa g

Distinguished member
Joined
Apr 17, 2019
Messages
394
Reason
PALS
Diagnosis
04/2019
Country
US
State
FL
City
Fort Pierce
Good points Kevin, how does one really quantify slow vs fast progression? I have been told by 3 different neurologists that I am slow progression. But how do they know? They obviously don't know my body like I do. My frs has basically stayed the same for the last year but that being said I can feel the changes happening to my body. I can no longer squat down or run yet I can still walk, stand on one leg and stand on my tip toes. My hips are weak and my speech is slower yet neuros tell me I'm doing great and still have good strength. They can't understand that I feel like crap and I'm frustrated with the things my mind says I can do that my body says it can't.

I often wonder why qauge progression at all? It's an inevitable process thats going to happen. We sometimes over think the whole process. Too many times I'll feel a twitch or a spasm and get paralyzed with fear thinking is this it am I losing more of myself? Then I take a deep breath and say its out of your control, relax. Slow or fast, its still going to have the same outcome.
 

Fusia

Distinguished member
Forum Supporter
Joined
Oct 26, 2019
Messages
139
Reason
PALS
Diagnosis
08/2019
Country
US
State
MT
City
Big sky country
I'm glad you were safe and sound, Kevin, after the storm moved through your area.

Good questions about rates of progression, but I kind of agree with Lisa - I think each of our progressions is quite personal based on the functions we are losing given our individual lifestyle and activities that are important to us. For example, my current ALSFRS–R is 30, my diagnosis was 20 months ago, first symptom was 32 months ago. I consider my progression fairly fast, because I cannot do any of the things that I really love to do - hike, snowshoe, roll on the ground with my dogs, golf, ride a bike, travel easily, etc. But, if my score was 30 due to low scores in different categories, I might still be able to do some of those things, so I wouldn't feel that my progression was so fast. Similarly, if I hadn't been as active, I might feel better about my rate of progression.

All that said, however, my docs really haven't tracked this regularly and I've not talk to them about it much. So I can't really offer any medical- or scientific-based feedback that I know you are looking for.
 

Nikki J

Moderator
Joined
Mar 22, 2012
Messages
10,481
Reason
PALS
Diagnosis
04/2014
Country
US
State
MA
City
Boston
Lisa they are comparing you to the many PALS they see. Saying you are slow progressing is not saying you are fine but the fact that you can walk and talk puts you above most of their patients they have seen with a two year course. The fast progressing ones are vented or dead the medium ones are in a pwc and or feeding tube with or without speech technology.

i think progression is sometimes defined for study entry( the current c9 study is an example )and they look at frs slope but mostly I think they draw on their experience

I don’t know how much it matters. I know I have very slow progression for which I am grateful but I know it can be frustrating or even hurtful when they tell you you are doing so well when you can’t do the things you want to.
 

affected

Guru status reached
Joined
Apr 26, 2013
Messages
13,336
Reason
Lost a loved one
Diagnosis
05/2013
Country
OZ
State
AU
City
lala land
Really interesting question.
First part of your question/musings is that progression speed, in my understanding, is normally spoken of from diagnosis, not so much from first noticed symptoms.
I don't think slow progression is something that can be given as a prognosis. It is something that can be observed over time. More like a mid - hindsight observation. It can also change as some PALS are said to have had slow progression for x years and then have a sudden rapid decline.
My Chris was rapid progression and was gone 11 months after diagnosis.
However, it did take around 9 months from the true bulbar symptoms for him to get diagnosed (he didn't go to a doctor at all in the first 5 months or so). But by the time of diagnosis, he had already sold his business as he was unable to work due to swallow issues, no clear speech (he owned a cafe) and weak hands. So he could no longer talk to clients, nor carry out cafe level cooking duties.
Looking back after diagnosis, there had been some suspicious things earlier than that, and the FTD had subtly started at least a year prior to the bulbar symptoms.
Once the true bulbar symptoms set in however, things were rapid, but I would never count him as slow onset with a then rapid progression.
Not sure if that helps or muddies as it is kind of clear inside my head, yet hard to explain.
 

affected

Guru status reached
Joined
Apr 26, 2013
Messages
13,336
Reason
Lost a loved one
Diagnosis
05/2013
Country
OZ
State
AU
City
lala land
Nikki posted while I was still typing and says it really well. If all who have posted so far are compared to my Chris, there is an obvious difference in progression rates between him and each of you and this is the rapid v slow progression.
 

[email protected]

Distinguished member
Joined
Jan 3, 2016
Messages
291
Reason
PALS
Diagnosis
11/2015
Country
US
State
Iowa
City
Iowa City
My FRS has gone fm 38 in Jul 2016 to 31 in April 2021, which I guess qualifies as slow (that's with me doing the rating, a pro might rate me lower). But the losses are mostly in mobility, more recently in speech, swallowing and manual dexterity. I do most of the ADLs I did at the beginning, but with much decreased efficiency. I can sense increasing decline in strength and stamina, but still function and don't need constant attendance. I've never had a rapid drop in function, except possibly in legs. Not sure I want to see this slow decline continue to the end--that would imply a long period of possible paralysis, difficulty in communicating, etc. Would rather have the end stages go more quickly, for my wife's sake and mine.

Ed
 

EricInLA

Distinguished member
Forum Supporter
Joined
Oct 20, 2019
Messages
184
Reason
PALS
Diagnosis
01/2020
Country
US
State
CA
City
Los Angeles
This is a very interesting topic. I've often thought about my slow progression and what that means for my future. You can go a little crazy trying to figure it out. One of the neurologists who examined me thought I did not have ALS simply because the progression of weakness was too darn slow, and my hyper reflexes were symmetrical. But the experts at the ALS centers who diagnosed me both said it was ALS despite it being so slow (one said I was "elite" status for ALS, which I guess means I get free drinks at the ALS airport lounge.) In my case, I've had nonstop, strong, visibly freaky arm fasciculations for 3 years now. Literally. My arm has not stopped jumping for 3 years. It took a battery of neurologists 2 years to label me with the evil 3 letters.

I took to heart what Karen once told me - it's all about function. So despite my label, I take solace that I still have good function (unless someone hands me a pen and asks me to start filling out forms, or hands me a knife and an onion and asks me to chop). My legs are still strong so if there's a hurricane heading my way 😆, I can move quickly. But I live in LA so it will probably be an earthquake.

Bottom line is I don't think there's a definition of slow progression - rather, there are degrees of slowness. Even if there was a definition, there are lots of ways to be a slow progressor, so 2 slow progressors can appear totally different. Yes, we have measurements and standards - the FRS scores are one way, there are others more specific to bulbar or limb. But the ones we're all familiar with - age at onset, limb vs. bulbar, rate of progression in the early going -- I think are the best indications.
 

KevinM

Distinguished member
Joined
Mar 30, 2019
Messages
376
Reason
PALS
Diagnosis
06/2019
Country
US
State
FL
City
Tallahassee
Good (and witty) comments, Eric. Hard to run from an earthquake, though...

If you don’t mind me asking, did your neurologist(s) indicate if you were more UMN or LMN dominant? From everything I’ve read and heard from other PALS, those who are UMN dominant typically—though certainly not always—have a longer lifespan. Also, are your fasciculations limited to your affected arm?

As you might have read from my early posts, I was quite atypical in that I have had full body twitches from the start. You and others seem to do so much better than me at ignoring them. All the best, Kevin
 

Samkl

Distinguished member
Joined
Nov 1, 2019
Messages
117
Reason
Loved one DX
Diagnosis
08/2019
Country
AU
State
NS
City
Wollongong
Our PALS had respiratory issues initially, then weight loss followed by full body, symmetrical fasciculations.
 

wmilo

Distinguished member
Forum Supporter
Joined
Feb 14, 2018
Messages
145
Reason
PALS
Diagnosis
10/2017
Country
US
State
PA
City
Zelienople
Interesting topic, Kevin.

I'd have to weigh in on the side that says fast/slow is not terribly meaningful or helpful to describe progression. You would have to compare PALS with the same group of symptoms and when they were observed, which would be difficult. And it is a relative measurement anyway. Some progress faster than others is about all you can say.
 

EricInLA

Distinguished member
Forum Supporter
Joined
Oct 20, 2019
Messages
184
Reason
PALS
Diagnosis
01/2020
Country
US
State
CA
City
Los Angeles
Good questions Kevin. I would say I'm not ignoring my twitches, it's just that the brain sorta turns them off after a while. It's like living in NYC and after a while you don't notice all the noises of the city. But I definitely notice when I've overworked my muscles and they go from regular twitching to DEFCON 1.

I'm definitely LMN dominant, as I have fasciculations, limb weakness, and limb muscle atrophy. I do not have much in the way of UMN symptoms - no spasticity, no Hoffmans, no Babinski. Only symmetrical hyperreflexivity. In fact one of my neurologists still thinks there's an outside shot of multifocal motor neuropathy. But the others say no, because in that case I would be HYPOreflexive, instead of HYPERreflexive. Who the ---- knows. I just know the neurologists who work in offices with "neuromuscular" printed on the door have given me the 3 letters I didn't want.

My fasciculations are mostly in my arms, though they show up occasionally elsewhere.
 

KevinM

Distinguished member
Joined
Mar 30, 2019
Messages
376
Reason
PALS
Diagnosis
06/2019
Country
US
State
FL
City
Tallahassee
Thanks for the reply, Eric. The reason I asked about upper vs. lower dominance was because of your hyperreflexia. I, too do not show obvious UMN symptoms as of yet.

I asked about fasciculation location because I am still a bit confounded by my sudden onset of full body twitches as my first symptom that set me on the path of diagnosis. As Laurie commented to a CIHALS poster, “all the motor neurons do not start dying at once.”

That was my exact reaction when I first started the diagnosis process, until I learned from research and my ALS specialist that in a small subgroup of mostly, if not exclusively, males over age 50 this can occur months in advance of other symptoms. For me, they started three months before clinical weakness was detected.

As I’ve said before, I’m in a rare subset of a rare disease. Add in my rare Colovesical fistula and I’m one rare f—-ker. Kevin
 

KevinM

Distinguished member
Joined
Mar 30, 2019
Messages
376
Reason
PALS
Diagnosis
06/2019
Country
US
State
FL
City
Tallahassee
One other matter that has been sticking in my craw, but is more related to diagnosis (specifically EMG’). I thought it better to bury it in this thread to avoid too much back and forth.

I want to push back some on the forum responses that indicate that EMG’s cannot be done “too early,” and that a normal EMG absolutely clears someone of possible ALS. My ALS doc told me it is not that uncommon to have a normal or inconclusive EMG in the very early stages of the disease.

On the ALS Facebook page a poster had a normal EMG but is showing other symptoms, and asked the PALS/CALS if any had normal EMG’s before later being diagnosed. So far there have been 29 responses, all of which had normal EMG’s at first. One person had a normal one in April, a second normal one in October, and finally the following February an abnormal one that led to her diagnosis.

It is also true that UMN patients will many times have normal EMG’s until LMN’s become involved, so I think that needs to be a part of the responses as well.

Finally, and this has been said many times here, having the EMG performed by an ALS specialist is critical. Your basic local neurologist is usually not nearly as skilled in conducting and interpreting EMG’s as they might relate to motor neuron disease. Their bailiwick is detecting carpal tunnel or other problems, so having one done by them might show up as clean simply because of inexperience with ALS.

Okay, I got that off my chest. Kevin
 

Nikki J

Moderator
Joined
Mar 22, 2012
Messages
10,481
Reason
PALS
Diagnosis
04/2014
Country
US
State
MA
City
Boston
We have repeatedly told people that when they have normal exams and normal emgs then they need to move on. I stand by this. I believe the sticky specifically addresses the umn finding normal emgs question as an explanation for most if not all of those normal emg reports. It says this
Why do I see people saying their EMGs were normal when their ALS started? For those people who are accurately reporting their diagnosis the answer probably is that their ALS started in their Upper Motor Neurons and then progressed to the Lower Motor Neurons. Upper Motor Neuron disease is found on clinical exam so these people were not told they were fine. They had an abnormal exam and the neurologist knew there was something seriously wrong. If your exam was fine ( or just some brisk reflexes which are normal in many cases) this is NOT you.

People with umn issues do NOT have normal exams
 
Status
Not open for further replies.
Top