How does bulbar ALS progress?

[BarryG]

Hello All;

My name is Barry Godsalve and I live in Hinton, Alberta, Canada. I am 53 years old and was diagnosed with Bulbar onset ALS in January of 2008. My wife first noticed that my speech was occasionally slurred in June of 2007 so I went to see a neurologist in August. After a bunch of EMGs, MRIs, and arm/foot wrestling with a number of different neurologists they finally were able to give me a definite diagnosis. My speech and swallowing are getting progressively worse so that I have to use a Palmtop 3 Dynavox with people that are not familiar with me (and sometimes with my family as well).

Jennifer and Beth, I too dream of being able to talk again and am sometimes jealous of everyone who can talk normally. The worst part is not being able to have a conversation with others over a meal and not being able to crack jokes like I used to do.

I've just started to notice a weakness in my right hand, especially when it's cold (BTW is was -38 degrees Celsius, which is the same in Fahrenheit, here yesterday morning). It's getting hard to hold a pen and write legibly so thank goodness for keyboards.

I have been a member of this forum for a few months but haven't posted before so I thought I would share my experience so far and I appreciate hearing about others who are in the same boat. Anyway, I try to think positive thoughts.

I want to wish everyone a Merry Christmas and Happy New Year!!

[brendapals]

Welcome Barry, to our wonderful forum family!

I was dx June 08, with bulbar onset. I am slowing down quite a bit on my speech, and very, very slurred. Other than that, I'm doing pretty wonderful. Still driving, still working as a nurse ( albeit 2 days a week ), still going to my kids' basketball games, even did a little bit of shopping this week, do laundry, dishes, and leave the heavy stuff to my hubby and boys.

I'm sorry you had to find us, but I'm sure will you soon see that this is the best bunch of folks anywhere! You will find support, friendship, people you may never meet in person pulling for you, in whatever's going on!

A very happy holiday to you and your family,
take good care,
brenda

[Al]

Hi Barry. Welcome but sorry for the diagnosis. Is Hinton where they had a big propane incident a long time ago? I seem to remember the name and it was in a propane fire training film.

AL.

[Jennifer51]

someone a few replies back mentioned psuedo bulbar...can anyone in very simple terms explain the difference between psuedo bulbar and just bulbar onset als.
My first symptons were slurred speach..had a bad emg, and have very very brisk reflexes and some weakness that I had not noticed...I was told it was psuedo bulbar, but I really dont understand the difference...what ever it is I hate it.
First symptoms in Jan 08, now can barely talk, and legs are very weak but still walking..just.....having a lift fitted tomorrow

[BarryG]

Hi Al;

You are probably remembering the CN rail accident in February 1986 where a freight train went through a red light and slammed int a VIA Rail train. Twenty three people were killed and unfortunately those kind of things forever associate a place with the accident. Hinton is a town of about 10,000 people about 3 hours west of Edmonton and an hour east of Jasper. The main industries are forestry and coal mining.

[Al]

I can relate. We had a train wreck in Mississauga in 79 and evacuated 250,000 people because of burning tank cars. It was a busy week for us. No loss of life due to the superior skills of the Fire Dept. lol.

AL.

[wright]

Jennifer

Pseudo-bulbar means that the upper motor neurons that innervate the lower motor neurons of the bulbar region (i.e. the brainstem), are affected. In other words, pseudo bulbar means you have upper motor neuron problems that are causing your bulbar issues. Therefore, the EMG would be clean.

Bulbar onset would mean that the lower motor neurons of the bulbar region are affected and that is what causes the bulbar issues. These neurons directly innervate your bulbar muscles and therefore, the EMG would be dirty.

You said you had a dirty EMG: what was dirty? If you only have pseudo bulbar neurons affected, your EMG can't be dirty. The only way an EMG is going to be dirty, is if lower motor neurons are affected . . be it lower motor neurons of your brainstem or lower motor neurons of your spinal cord.

[Sybil]

I have not been diagnosed as yet but many tests in last month. Very quick onset. Mobility very linmited. Walking with walker. Falls. Upper arms seem okay. Speech going down hill fast. How long am I likely to have speech?

[BarryG]

Hi Sybil, welcome to the forum. Unfortunately each case is different so no one can say how long you will have speech. In my case my speech problems started in July of 2007 with a bit of slurring which turned into hoarse and nasal speech and now is not much more than a mumble. Some people say "I can understand you just fine" while others just stand there with a confused look on their face so I'm not sure if it's always me (sometimes my speech is better than other times) or if the others person's hearing ability might also be a factor in how well they understand me. Whatever the case I have a bunch of communication devices and strategies ranging from the low tech (writing things out) to the high tech (electronic speech devices).

I am sorry that you have mobility problems also, I haven't really got to that point yet although walking is now more difficult and using my right hand for writing, buttoning etc is more trouble.

Do you have an ALS clinic that you attend? Have you seen a speech language pathologist to discuss your speech problems and get help with them?

All the best and if you have any questions we're here to help

Barry

[BethU]

Sybil ... so sorry for your health problem. I hope it doesn't turn out to be ALS.

As Barry said, it's really impossible to tell how each case will progress, but his suggestion to see a speech therapist is spot on. They can offer all kinds of tips on keeping your speech intelligible as long as possible.

What I am finding with both speech and fine motor skills is that when I lose a function I've been doing all my life, I can often outwit ALS and figure out other ways to do the same thing and keep the function going for months longer. Such as turning keys with my knuckles instead of fingertips, etc. With speech, therapists can work with you on separating syllables, emphasizing final consonants, etc.

Speech therapists will also work with you on swallowing and may spot issues before you are aware of them. They can order swallowing tests and show you ways to prevent problems.

Good luck.