Slow Progression Questions

[Clearwater AL]

ALS/PLS starts as a singular disease.

The disease starts to impair motor neurons in the primary
motor cortex onto the brain stem then the spinal cord.
It starts there... not from somewhere else up.

From there… as motor neurons die, the ability of the
brain to initiate and control muscle begins to fault or fail.

Slow progression may be slow fault rather than faster
progressive failure.

Other neurological disorders may coincide. There is no
accredited link that other neurological diseases may
cause ALS.

ALS is ALS.

 

[Nikki J]

I am not saying that ALS is other named diseases. The comment I made is a quote from my neurologist and others. What they are saying is ALS has different causes and different courses which is part of the reason that it has been so hard to treat.

there are multiple identified FALS genes. You might say I have C9orf72 disease. I have friends with SOD1 disease and FUS disease etc. on the non FALS side there are PALS with a lot of inflammatory markers and some with none. Going forward we are going to see more trials targeting specific subpopulations. this has already happened with trials for therapies targeting the genetic mutations and inflammation

 

[EricInLA]

Chester,

I am exactly like you. In fact, your initial description describes my situation word-for-word, which is unusual for a disease in which everyone is different. But it sounds like we're in the same boat (except I'm relatively young - just turned 50) I've had symptoms for 2 years now, but was only diagnosed 3 months ago.

For me, slow progression is a blessing, because I have 3 teenage boys and I want to be here for them, and see them grow and thrive, for as long as possible. I may feel differently when I get to the later course of the disease and become a burden for my wife. That's what I fear the most. But I've learned to take the advice of my doctors, and friends on this forum, and simply forget about that and live in the moment. The truth is we can't accurately predict what symptoms we'll get and when, so why be consumed by those thoughts? I take my Riluzole and live my life. I do have my moments, but have been able to transition from the initial shock and grief into a (relatively) normal routine and life. I'm still working as well, and find it helpful for my mental state.

At the moment, my biggest frustration is not being able to travel because of the CoronaVirus. Our family is adventurous -- we love exploring new places -- and it eats me up knowing that one of the few summers I have left -- at least with my physical capacities intact -- will likely be lost to this virus. But I try to find the silver linings.

By the way, this forum is an excellent source of information and empathy.

 

[Clearwater AL]

Nikki and all... all I was saying is where it starts and how it starts. That is a
singular point.

I understand all the other contributing factors.

It's like PMA... the jury is still out if that is ALS, a form of ALS or something
entirely separate. Example....

In the World Federation of Neurology El Escorial ALS diagnostic criteria, PMA
is not considered a form of ALS but is labeled as “suspected ALS,” and
patients with that diagnosis are excluded from ALS therapeutic trials.
PMA is rarer than ALS.

Yet, it nearly mimics ALS more so than others.

Yes, ALS has different causes and different courses. But, the starting
is a singular point in the Primary Motor Cortex.

Hope this generates some discussion and more members chime in.

 

[wmilo]

Hi, Chester. I am about 2 1/2 years from dx; lower limb onset. I am still independant for dressing, toileting and showering, but with significant effort. I get around with a rollator, but my balance is poor and I need to hold on pretty much all the time. Still eating without assistance but difficulty using utensils and having hand/arm tremors and shaking at times.

My two cents for what to expect is to plan for and implement now as much as possible accomodations for showering (roll-in if you have the money and space, but at least grab bars); ADA-height comode; widen doorways or look for the hinges which expand wider, and the like.

Regarding blessing or curse, I think that is a glass half-full/half-empty perspective. For myself, I believe slow progression is a blessing: I have more time to enjoy family relationships and to experience the amazing creation that is the Earth. I have become more aware of all the ways that God has helped me during this journey. I recognize that many PALS are facing much more difficult circumstances and that my attitude might change over time, but for now I hope to keep an "attitude of gratitude".
Best regards,
Bill

 

[Ed340hp]

Chester, I am also a limb onset slow progressing PALS. I am at five years from diagnosis, but looking back I was fighting off slowing down for a few years before the diagnosis with exercise. My GP Doc noticed muscle atrophy in my hands, and a few months later the Neurologists and UCI ALS Clinic Doctors confirmed ALS. I was unaware I was in a slow progressing status until I was in a (failed) clinical trial for a year, and it was mentioned in the open label follow up trial appointments.

Be aware that many PALS experience pauses and then accelerated progression. Some of the pauses can be attributed to adaptation to exploit compensation of other muscle groups for atrophy in one area. Atrophy is asymmetric, and coordination and dexterity (balance) can suffer long before strength suffers in dominant muscles The Doc's say fast twitch muscle lower motor neurons seem to atrophy first, impacting counter balance moves, but upper motor neuron commands control coordination, with atrophy degrading balance. I mention these distinctions because you can feel strong (lower motor neurons) and still fall from a loss of coordination (upper motor neurons). In my experience exercise helps, because it's too easy to give in as you lose range of motion, and blood flow circulation to the limbs may help to adapt and pause progression for a few months or longer (and even gain back some ROM during a pause, why I recommend PT and OT, if you can get it, for daily chore and transfer technique, and ROM or strength). Safe transfers to exercise equipment can be less dangerous than outdoor exercise.

A big thing to remember is you have to be aware of other non-ALS health issues. PALS fall (and slow progression means you have more opportunities to fall), causing other injury complications so protect against falls, but only you can assess your overall health concerns and many Doctors are hesitant to tell a PALS if they have other health concerns. You will hear that you are your only reliable advocate, and as you fight through the progression away from work and play you will realize how true that advice is in everything you do in the future.

 

[wmilo]

After reflection, I want to expand my comment above:

I would not want anyone to think or feel that I am suggesting that because I choose to see my slow progression as a blessing, therefore I would see someone else's fast progression as a curse.
The very idea of something being a "blessing" or "curse" to me is only meaningful in the context of our relationship to God; otherwise, it just is what it is with no blessing or cursing attached.
What might constitute a blessing for one person, might - based on their individual circumstances and personality - be more of a curse for someone else, and no one else can judge that.
In my world view, we each have our own path, and we will all reach the end of that path some day, without exception. We are in a rocky stretch now, but our relationship with God as we travel it matters more than how long it is.
I know there are many different views among the forum members and I don't wish to antagonize anyone whose views may differ from mine. I just wanted to clarify that I did not want to imply an "either/or" dichotomy regarding how we evaluate our disease progression.

Regards,
Bill

 

[KarenNWendyn]

Chester, if you’ve had symptoms for two years and are still able to do most of your activities of daily living, then you’re probably on the slowish side in terms of progression.

What can you expect? Progression isn’t linear. You may continue to progress at a fairly slow rate, or your progression may speed up, or you may have an occasional plateau. But ALS eventually progresses one way or another, and you have to be prepared for that.

Since it is involving your hands, there will come a time when you need help with self care. Since it is in your legs, there will come a time when it will be unsafe to walk. I would look at your home environment and think about what may need to be modified to enable you to get around in a wheelchair. It’s possible you may never need one, but I think it’s important to anticipate and prepare.

As far as a blessing or a curse, that depends on your values and priorities and how you utilize the time you have left. Slower progression does allow more time to prepare. Focus on what you can still do and enjoy, and do it to the extent our crazy world will allow. Once you lose the ability to do something, you’ll never get it back.

 

[sweetmozart]

Hi Chester, my husband got his in his arms first as well. I remember, he had the same muscle atrophy between the thumb and forefinger that you mentioned. He would probably be considered an outlier, but he was diagnosed in 2001. He is still fighting. He's in a power chair now, but can use his desktop with a regular mouse, set to highly sensitive He's not on a ventilator. So, it is possible to go on much longer than the 3-5 you may have been told. Take care!

 

[mpnatx]

Chester, I too have slow progression. After five years, I am still able to walk and talk. Everything has become weaker, but very slowly. I often plateau, then after, I go down a little more each time. I'm waiting for the other shoe to drop.
Haven't left the house since March even though Texas is reopening. The leaders in Texas don't believe in science.
At one point, I was concerned that I may have been misdiagnosed and asked the Neurologist to do another EMG. He told me "It's academic". I actually agreed with him. I know I have ALS.
I've Learned that ALS is not the same for all. It's a tough disease that takes over, eventually.
Be careful and read as much as you can here!

 

[KimT]

Marty,
Our governor sounds a lot like your governor.
I was so convinced I didn't have ALS, at one point, I let the doctor use me as a lab rat for students to do EMGs on. I guess I was hoping for something else to magically show up. I even had genome sequencing and so many other tests I can't even remember them.

 

[jethro]

@vitsra there is a crucial difference between slow progression, "plateau" and plateau. it is hard to differ it, but there is a big difference. there are no lifte time endless plateaus. also, there is a big difference between als_consequently ongoing atrophy, and als_caused atrophies. anyway, this is not a topic. but there is one interesting thing: how nelda knew that her "als stopped 6-7 months ago". excluded plateau? what used bedlack's reversals to revers als? nothing. sorry, NOTHING in common! those who can read between a lines will read.

 

[Babycakes]

I also have slow progression which started in my right hand. I was diagnosed around 2 years ago and had first symptoms around 4 years ago. I’m in the oral Radicava study as well and Ive taken Riluzole for a few years. Now I have weakness in my right arm and very slight weakness in my left arm, right leg and back.

Im in my 30’s with young kids. I have used the time since diagnosis to do lots of things I love. I stayed working til last summer. I’m really glad I did that. The diagnosis was a huge gut punch and I was glad to have a place that felt normal — where I could forget about it for a few hours of the day. I chose to not share much til I went on disability, but everyone is different. We decided to move closer to family and now I’m full time mom with disability
Plan for the future, but enjoy and live in today

I also take a few supplements. Theracurmin, NAC, Magnesium, multivitamin, CBD when I remember.
I personally have really appreciated ALS news now, ALS TDI, ALS Untangled

best,
Kim