Waiting for 2nd opinion... Scared... Please advise.

[Luke]

Hi Amanda,you do need a certain number of posts before you can send private messages, but you are able to post on another member's profile (similar to a wall post on Facebook). To do this, click on Wright's name from his summary info next to one of his posts. Type your message in the box that appears on his profile. Bear in mind that this is publicly visible.

 

[Momcnc]

I can check and see what other testing was done. She was put through a bit. The Dr is at Hopkins so we had confidence in her. Se said that it would progress slowly and was not terminal. But it progressed very quickly...at least with respect to the throat area.
She still has no other symptoms...just can't understand her, a lot of trouble swallowing, particularly thin things like water, cannot really use her tongue to help scrape her palate( if that makes sense?), her mouth looks different, she complains of having a very dry mouth which I haven't seen on here?, she is definitely phlegmy though so maybe it is a difficult in explanation(?), she has a 46 percent oxygen rate (got the BPAP yesterday), but it is all confined to the throat area. Says nothing hurts and she feels great. She is coming around to agreeing to get the PEG.
Does anyone have any recommendations for Drs at Hopkins? Her current Dr has a great reputation but I don't think she specializes in ALS....I think she specializes in speech impairments and sees a lot of ALS patients as a result.

 

[Momcnc]

Just to be clear the slow progression etc... was when she was diagnosed with pseudo bulbar palsy last Fall....that has changed with the ALS diagnosis...

 

[Amanda81]

So she still has no other symptoms in her arms and/or legs? Just the bulbar area? This is what I've been wondering about because my mom really doesn't either. I do notice that she's slower... walks slower, gets up slower, etc. So maybe this is just the beginning.

I think the not knowing is the worst. I just wish it were more simple. More black and white. Because even with a diagnosis, it could be wrong and I am the type that will always wonder, "Well, what if it's this..."

And is Bulbar ALS different from ALS? If so, how?

So many questions! But you can't learn if you don't ask.

 

[Momcnc]

Nope...just the throat area..."just"
She said her arms and legs re fine. I need to ask her dr about the EMG. I did not know that was so definitive. You can't help but question the diagnosis, and hope it'll be something else. From what I've read bulbar starts in the throat area, and progresses faster. Still learning too though? Seems like sometimes it does not even get to limbs?

 

[Luke]

Progressive bulbar palsy is in the same category of diseases as ALS and sometimes evolves into ALS eventually. I am sorry to hear what your mom is going through and I hope you get more answers soon. I understand how frustrating limbo can be.

 

[notme]

ALS either starts in the limbs or in the bulbar region. Bulbar ALS doesn't necessarily go 'faster'--- but it is more worrisome, as the breathing and eating are affected. It can be years before other areas are involved. We have a few members here with bulbar only issues.

There are many adaptive aids available for speech, and a bipap will help greatly with breathing. There are also things for dry mouth. If swallowing is an issue, there is a spray for the mouth. One of the CALS in the other section can give you the name, it escapes me at the moment.

The EMG is definitive normally--as there are very specific changes seen in ALS. Still, as second opinion is always a good idea.

There are thickeners that can help with liquids, and a PEG feeding tube will likely be needed at some point to prevent choking.

Amanda, if your mom is having walking issues, you're going to want to prevent falls--a walker would be a good idea. Falls and ALS are a bad mix.

I'd suggest both of you post in the ALS portion of the forum for referrals to specific doctors. The PALS will know who is where. We have PALS from most states, unfortunately. The ALS association also has a list of specialists. It's a good idea to get into a multi-clinic if possible, as they can address all areas.

 

[frankb]

Hello, Amanda: - -Just caught your thread and, unfortunately, I don't have anything to add to these wonderful posts. But, WOW, what a group we have ! ! I am humbled at the response of our group.

 

[jellis86]

From Wright....

Hello Joel

I took a look at the EMG and you do not currently have any active denervation in any muscle that was tested. Active denervation means that your nerves are actively being damaged and they then lose contact (i.e. denervate) with the muscles they control. The normal (N) indication in the columns under PSW and fibs confirms that.

You do have a few 1+ values under the fasics column, which could or could not mean anything. The good news is that none were found in your tongue, which is where your symptoms stem.

There are also a few 1+ and 2+ values under the Amp column. Amp is short for amplitude . . . the amplitude of something called the motor unit action potential (MUAP). There is a measure of the electrical activity in groups of muscle cells called motor units. If the measured value is bigger in amplitude, the 1+ or 2+ or 3+ etc. will be indicated (the larger the value the bigger the amplitude). These larger values indicate that damage to the nerve controlling the particular muscle occurred (which led to denervation) at some point in your life but then new contact was made. This is known as reinnervation. Therefore, you have reinnervation in those muscles with the 1+ or 2+ values.

You also have some 1+ values in the Poly column. This indicates instability of the nerve, which can happen for a lot of different reasons (early denervation, inflammation, etc.).

Bottom line: that EMG is inconclusive and I'm sure they will want to give you another one in 3 to 6 months. As of right now, given your story and that EMG, you could not be given an ALS diagnosis; it just doesn't add-up (there are too many odd things that are happening, namely your ability to recover functionality).

I wish I could be of more help but I would insist that you keep me updated because I want to try to continue to help. In the meantime, ask your neuro to give you his/her impression of the EMG and make sure he/she makes it clear to you.

 

[jellis86]

Amanda and Momcnc,

Keep faith that it could be something else happening with your moms.

For now I live with an undiagnosed speech and tongue disorder and although it is difficult not knowing, I am okay with that for now!

One thing is for sure as Wright and many others have said.....it can't be ALS until it isn't anything else!

 

[Amanda81]

notme - Thank you for your input on Bulbar ALS and everything related. This is all so much to try to figure out. We are not-so-patiently waiting to hear from Washington University in St. Louis about our appointment for a second opinion. I don't think we have much say in which Dr. we're going to see. I have no idea how any of this works... we're just kind of going along and doing what they tell us. I hope we get some answers soon.

 

[Amanda81]

frankb - I know! I have been so surprised at the responses and support thus far. It is really amazing... I don't even know these people and they seem to care so much. It definitely lifts a small amount of weight from my shoulders to know that I, in the very least, have this forum to turn to for answers, advice, support, and a lot of other things. Thanks for writing

 

[Amanda81]

Joel - Thanks for the encouragement. No matter what happens, it is exactly where we are supposed to be. At some point you just have to accept what you're given, regardless of what it is. Good luck to you and keep in touch!

 

[Amanda81]

P.S. You have to post 15 times before you can private message - I learned from experience.

 

[Amanda81]

Hello everyone! We finally have our appointment set for our 2nd opinion. It is December 28th at 12:30pm with Joseph Black at Washington University. Does anyone know where I can find more information about him? A general google search just gave general info... address, phone #, degrees, etc. But I would like actual patient reviews. Thanks!