Bulbar ALS - Second Opinion

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ColleenMarie

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PALS
Diagnosis
12/2021
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US
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Cary
Hello Everyone. A bit of an update and thank you for your generous thoughts / advice. I did see an ALS specialist today which was a relief. They are sending out my bloodwork to check for other issues. The Doctor felt that I could have Bulbar ALS and it may be slightly slow in progression. I am supposed to go back for a follow up and of course they ought to know more after my bloodwork results which may be done in a few days.

These are the comments he left.
61 y.o. woman with 8-10 months of progressive dysarthria but no dysphagia or other weakness. EMG was reportedly normal, MRI brain unremarkable. On exam today, patient's speech was slightly spastic but clear and understandable, no identifiable weakness or fasciculations but did have 3+ reflexes throughout including jaw jerk. Possible causes include upper motor neuron bulbar-onset ALS, PLS or stroke not seen on MRI. Will also need to check labs to rule out nutritional, infectious or autoimmune causes.

I am not sure what 3+ reflexes throughout including jaw jerk meant and only read this now that I am home?

How common is slow progression Bulbar ALS so I am not missing anything. My fear is that time is precious and I am either too stricken with anxiety to notice what changes or proactively doing something of my own to address this with clarity. For those of you who have experience with Bulbar ALS - how do you best monitor your progression? I am trying to be healthy mentally and live fully, support my family who need me but I am terrified. Having lost a friend to ALS, my mind is already in an anguished state remembering how hard it was for her and her family. Most people in my life refuse to talk about this with me. They think that if I am not falling or dropping something this is not ALS.

Any feedback is truly appreciated. I know everyone's time is precious so please know that words are not enough to express my gratitude. I feel very alone but having this forum has been about all the comfort out there I have had.
 

rmt

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CALS
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You sound similar to my husband. We are 3.5 years into symptoms currently and he has an Bulbar ALS/PLS diagnosis. His EMGs have been clean so far and he has no muscle wasting so far. We will have another EMG this spring/summer to see if it is PLS or ALS.

He started with progressive dysarthria and his speech was totally gone after about 2 years. I highly recommend voice banking ASAP if you are interested. My husband wasn't interested, but I still wish he had done it. He doesn't like the tablet and mostly writes on white boards for communication (a low-tech and easy solution).

He started having swallowing problems about 2 years into symptoms and now has a feeding tube. He moves more slowly than he used to, and says he doesn't feel as strong, but he still has complete use of his arms and legs. He has FTD with language issues, so communication is difficult now, but we make it work.

I'm happy to give you more details of our journey so far if you are interested. I know everybody is different but sometimes it is nice to talk to somebody who is at least on a similar path.
 

ColleenMarie

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Joined
Jan 5, 2022
Messages
16
Reason
PALS
Diagnosis
12/2021
Country
US
State
NC
City
Cary
I would so appreciate any details you are willing to share. Sadly, most of what I have read is dire, quickly progressing. Because your husband had slow progression, how long did it take to get something of a prognosis? In the first year, were there enough dramatic changes to speech that you both noticed or was it so slow that it remained the same until you got to the second year and speech increasingly was lost. I am going to try voice banking - that is a good recommendation. How has he (and you) both managed to cope, kept your spirits strong so that this is not so destructive psychologically daily. I feel like I am a wreck. While I have cared and lost terminally ill loved ones (other illnesses) in the past and I realize every moment is precious and we need to be in the moment for ourselves and those we love, it is hard for me to cope. Try as much as I can, I have trouble banishing it all since there isn't enough information. I am struggling to have the hope I used to have effortlessly and took for granted. Today my sister said that my skin looked so broken out. Anxiety and stress is insidious I guess. Try as we may to keep it at bay. Are there things you wish you did early on to help with the present you have now? Do you feel like there is enough help in spite of your husband's slow progression? I just feel like the meetings I have had were abrupt, dismissive and fast. Not surprisingly considering the healthcare times we all exist in with this terrible pandemic.
 

rmt

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Messages
208
Reason
CALS
Diagnosis
07/2019
Country
US
State
WA
City
Seattle
First, I'm so sorry you are here. But I'm glad you found this forum. It has been so helpful for me.

Honestly, we still don't have a prognosis! It really is wait and see what happens next. Even the ALS specialist (who is excellent and we love) has no idea what to expect.

The first ALS Specialist we saw (at about 1 year of symptoms) told us that even though the EMG was clean, it would definitely turn into ALS at any moment and my husband would be wheelchair-bound in 6 months. And yet, here we are at over 3.5 years and still mobile. Needless to say, we didn't go back to that doctor again! Nobody really knows what the future holds, so try not to get too wrapped up in getting a prognosis. But you do want to be ready for things that might come up and figure out what you might do when your functionality does change (feeding tube, remodeling you house, etc).

My husband's speech slowly got worse. I didn't notice the changes very much because I heard him every day but other people would comment on how much harder he was to understand. By the end, even I could barely understand him and finally he had to start writing everything down.

There are certainly things I wish we had done differently.

1. I wish he had taken Rizuole, just in case it might have helped. The doctor was on the fence about it, and my husband wasn't interested. And making recordings. I wish he had voice banked, or at least recorded himself some.

2. We only recently both started on antidepressants, and we feel so much better! I wish we had started taking them years ago. The stress and anxiety is so overwhelming and the antidepressants really take the edge off.

3. I wish we had talked about our fears and feeling more. I think we both didn't want to admit what was going on. It was so lonely trying to navigate all the feelings by myself. I've only recently started opening up to my parents (and we are VERY close). I should have done that years ago.

4. My husband is a veteran, so the VA has been a big help with his health care. But I wish we had stayed on top of his other health issues (blood pressure, restless leg, etc). Everything got pushed to the bottom when ALS/PLS showed up and we are only now getting things under control. And something as simple as controlling his restless legs at night has greatly improved our quality of life!

5. This forum is way more useful than most of our doctor visits. Seriously. The information from the people here is so helpful! You can get questions answered and the emotional support is so incredible. It is an amazing group of people.

6. Give yourself time and grace. It is OK to be sad. It is OK to be frustrated. It is OK to be angry. But you can still find wonderful, happy moments too. I wish I had focused more on finding the happy things, and not focusing on what was lost. We are doing that more now and it feels so much better.
 

ColleenMarie

Member
Joined
Jan 5, 2022
Messages
16
Reason
PALS
Diagnosis
12/2021
Country
US
State
NC
City
Cary
These are wonderful suggestions. Your generosity in responding to me is truly a gift. I am going to ask about an anti-depressant next week from my GP. I know that fear, anxiety, stress is not doing me and my psyche much good right now! Once I have my bloodwork back, also Rizuole. I will try to voice bank as well. Our house is not conducive to a loss of mobility, but you are right - it needs to be thought about. We lost family members in the last few years to cancer and it was just a terrible time in life before the pandemic started. I had my Father home with hospice and cared for him. My dogs even became sick and I needed a vet form of hospice. After this dark time, we emerged and then had less than a year before this wretched pandemic. I did think about being where I am now and the fact that each day passes, life is fleeting and we have to seize whatever pleasure, joy, gratitude and serenity it affords in the moment. It can be easier said than done. Of course, a lot of how we view life is with hope and that hope tends to tie into the future. The books we would read, the trips we would take, the celebrations we would plan. A big part of my life was always being with people, long conversations. Having to pivot and lose what we took for granted and assumed we would have is such a tough grief process. One more question I wanted to ask and it is simple, is exercises. Did your husband do speech exercises, things to strengthen the muscles around his mouth? I have heard people say to save your energy. Others say the opposite. Thank you again. It is an act of love to reach out and share with a stranger who needs assurance and comfort. I have found many of my appointments were quick cold, detached and I was outside getting into my car trying to process it. One neurologist gave me a print out to read and said good luck, good bye. Here there is such welcome warmth and empathy.
 

rmt

Distinguished member
Joined
Sep 18, 2019
Messages
208
Reason
CALS
Diagnosis
07/2019
Country
US
State
WA
City
Seattle
ColleenMarie, our house is not conducive to loss of mobility either. It is so hard to know when it will be time to figure something out in our current home or think about moving.

My husband did some physical therapy exercises for his face and speech for a while, but he doesn't do them anymore. I thought they helped, but he didn't think so. And I think it was frustrating for him. So he stopped and I'm OK with that. I know what you mean about conflicting information about doing exercises or not. You definitely don't want to overdo it. But I know some people with PLS have said physical therapy helps.
 
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