bulbar symptoms and more

I walk with the aid of a rollator due to spasticity and now I am experiencing hand weakness
and a bit of slowness when talking. My question is; when bulbar symptoms increase am I
going to see muscle sagging in my face? A visual change in my appearance?

And I know PLS progression is much slower than ALS but if one has already lost the ability to walk and will eventually lose the ability to talk, swallow, turn over in bed or hold a fork what does one do all day? Can you enjoy life in any capacity? Enjoy a movie, a pet, a new
couch?

My other concerns are: Will I be a candidate for the pump even though I don't take a high dosage of baclofen? I take 5 mg of baclofen at bed time and no more. This low dosage stops my legs from jerking. I want to avoid the side effects of increased dosages as long as possible. and....... does the pump help with swallowing difficulties?

Thanks for all the help

Hi Chris,

I hope you accept my advance apologies for my candor, but you are assuming far too much --- and way too soon.

Who says that your speech, hands, etc. will without question be compromised? I have had PLS for ~6 years (more likely, has sx. of PLS much longer than this), and have noted only minor issues with speech and swallowing. By all means, having PLS - with all its drawbacks -- doesn't mean you will be forced to live in the depressed and tortured state you have expressed below for the rest of your life. Nothing can be further from the truth. I enjoy my pets, a movie, my multiple live concert DVDs, my job, and other. Yes, PLS changes your life -- but it does NOT have to destroy your life! Speaking plainly, whether PLS ultimately gets the best of you is up to you -- and you alone. But you are anticipating symptoms that you may never experience!

And I'm on 60 mg. of baclofen - but I am not even CLOSE to pursuit of a baclofen pump. Why would you require a pump when you are only taking 5 mg/ day? Placing a baclofen pump (it IS a surgery -- with the same risks had by any other surgery!) would be overkill, in my estimation.

Mike

Welcome to Heartbreak Hotel.

Who knows what motor neurons will be attacked next. I just take it a day at a time. I sit here at the keyboard for my daily six feedings through the peg. I watch a lot of tv and nap under the bipap frequently. My only ALS pills are riluzole and Nuedexta. Oh, I use phytocannabinoid pain relief lotion for the jitters, which are made worse by the Nuedexta.

I don't know about jerking, but a magnesium deficiency causes leg cramps. I get 150% of recommended in my Boost+.

Hi Mike:
thanks for your response. I am really thinking ahead but it's what is happening to me
now that puts those questions in my head. In less than I year I am experiencing a
significant change in my hands (dropping everything, can't twist lids etc.) and now my
speech is changing. Makes me think things are moving fast. I am an up beat person
but lately I feel like I am waiting for the other shoe to drop.

I was inquiring about the pump for the day when I would be on large amounts of
baclofen with all the side effects. Believe it or not but that would be a positive and
makes me feel easier. The concept of not swallowing is possibly my biggest fear.
I've had PLS for approximately 4 or 4 1/2 years but, like you, I think I've had it
longer. I recall incidents in which I questioned myself.

I am still very active but the future sometimes looms over me. If I could get past
the things I use to do and took for granted and be grateful for the things I can still
do, but I am surrounded by all the same people and they are fine and I am not. So
maybe self pity and jealousy is playing an active part in my life.

I start the day off on a bad foot some mornings because I'll dream I'm running or
swimming, like this morning, and it is so real that when I wake up and face facts it
automatically brings me down and I have to get busy quick to change my thinking.

Thanks for your words Mike.

Hi Chris,

My PLS symptoms started in 2008 with somewhat slurred speech and eventually progressed to a very raspy voice. Then came the leg spasticity and other typical PLS symptoms in 2010 and beyond. However, I have not developed any major facial changes or drooping.

Regarding the baclofen pump, I take 80 mg/day by mouth. I hear great things about success with the pump to reduce muscle spasticity, but never heard of it helping with bulbar issues. Considering my baclofen intake, I'm a good candidate for the pump. However, after doing a little research, the literal size of the pump itself pretty much freaked me out. I expected something maybe the size of a half dollar, but these things are HUGE comparatively speaking. They are about 3.5 inches in diameter and nearly an inch thick! To me, that's way too big to insert under MY skin. Do a Google search on Baclofen Pump and go to the pictures of baclofen pump. You'll see what I mean. I'm very disappointed for myself, but with your low dosage, I (personally) wouldn't even consider one.

Chris, it's not all doom and gloom. Although we may need to make adjustments to feel more comfy, you can still enjoy life!

Hi Chris.

I have had PLS for 15yrs....slow progresser,use a mobility scooter when out but can still walk around home which is a disabled ground floor flat.
Regarding bulbar problems ...since the start I can get slurred speech when overly tired,sometimes a stutter which is weird as I have never done that before. Swallowing can be a problem if I have throat spasms...food gets stuck due to spasticity and tightness .
My main problems are jaw spasms which have been so bad I have broken a molar and caused nerve damage to the roots of teeth...I bite my tongue ofton and have a sore cut right now that is healing. I have weakness of the chewing muscles in lower face which when bad makes eating very slow and painful. I have had on my right lower face spasms that twitch and can when bad effect my lips.
Regarding physical changes ,I have slight atrophy on my left lower face ...why,i don't have a clue but I have heard of some with bulbar ALS have this.
I don't think that Baclofen works as good for bulbar symptoms...something I read saying it works from a certain point which is below the bulbar muscles. I have been offered botox which works rather well and will try when they get severe ....but I declined for now as botox can cause other problems in the long term my Doc said.
Everyone is different in progression and effect of symptoms but I found first several years were the hardest then things settled down which a lot of long time Pls'ers find.
I have had to give up a lot but found new things to occupy me ....I consider myself very fortunate ,there are many many others less so on this forum and around the world.
Life is what you make it.
Forgot to add...hands not great. My first symptom others noticed was dropping things,that was some months before leg symptoms. I type with 1 finger as my hand coordination is very poor,neuro noted ataxia in my clinical tests.

"Yes, PLS changes your life -- but it does NOT have to destroy your life! Speaking plainly, whether PLS ultimately gets the best of you is up to you -- and you alone. But you are anticipating symptoms that you may never experience! "

Thanks Mike. I needed to hear this from a fellow PLS er. Its been tough for me to muster a positive outlook lately.

Kathy

Hello Kathy, I've had to hear this from my wife nearly every day since I was diagnosed. We all need constant reminders, I think. I STILL need reminders. When one becomes more symptomatic, things inherently begin to feel more hopeless. We all need hope - and that's why we're here.

Chris, I hope my response does not come across as a mean-spirited one. It is not meant to be that way. Instead, I'm encouraging you to look at the reality of things: Even though you may have entered a period in which your symptoms have accelerated (or have seemingly accelerated), this does not mean that they'll continue to accelerate at the same pace. In fact, Caroline (Olly) and I have discussed this very issue before: At least the two of us -- both diagnosed with PLS - have experienced periods of more rapid progression, followed by plateaus in which the progression is followed by a relative period of stability (but even periods of stability involve some degree of progression, although lessened). If you have PLS (which you do -- you are diagnosed), it's more likely than not that you've entered one of those periods of acceleration that I've just described. Think back: Have you experienced similar periods like this one? My bet is that you have! Of all things, I sincerely wish you well -- and hope that you will soon be entering a more "stable" phase....

Mike

Tractor Boy said:

the literal size of the pump itself pretty much freaked me out. I expected something maybe the size of a half dollar, but these things are HUGE comparatively speaking. They are about 3.5 inches in diameter and nearly an inch thick! To me, that's way too big to insert under MY skin.

Click to expand...

Tractor Boy (great handle -- ), I have a spinal cord stimulator that was placed in 2006. It is 3 x 1.5 inches (W x L) and ~ 1 inch "deep".... I really don't know it's there -- after the incision healed, the rest went unnoticed.

Tractor Boy said:

Chris, it's not all doom and gloom. Although we may need to make adjustments to feel more comfy, you can still enjoy life!

Click to expand...

Amen! I'm beginning to get that myself, actually. You know, adjustment to PLS takes time (and this is true because PLS takes its time), but I'm learning to adjust as well.

Be well, all!

Mike

You know the thing is with me I play things down TOO much and people I know think i'm plodding along ok or some on the forum may think i'm a bit condescending . Mike is right there is plenty of evidence which shows in PLS like in MS there are plateaus ...these are when everything is at a slow steady pace,so slow it takes months to note any progression.
PLS is typical in that it is a slow progressing disease...be it a rare few who have a fast decline.

A few weeks ago I was in bed for 3 days unable to walk at all....been some years since that has happened and it was a shock. I think it was maybe due to stress with my son being so ill and having to take him to the hospital every week.

I keep getting phone calls about pensions...my son said I don't need one as i'm disabled...I said i'll be long gone before pension age.

My son has come to realise what it has been like for me...he can not work,go to the gym or out with friends ....he has lot 2 stone in 7 months,in severe pain in his bones and muscles and has fatigue/weakness...when he moans I just say now you know a little how I feel...shuts him up.

I know how hard it is starting on this PLS journey and its a huge shock (I was only 31)but like mike said you do adjust and can still lead a good life. Do a little of what you can when you can ,rest plenty and look after your general health as any virus or other illness can make PLS symptoms worse.