Husband got Dx this morning - Early ALS Bulbar onset, slow progression

[brooksea]

Erica,

If you can, as others have said, get a second opinion at the very least. My husband got 3 opinions, unfortunately, all the same.

The EMG results are puzzling though.

I'm hoping you are able to find another opinion that will take you down a different avenue to something treatable.

 

[rose]

I've hesitated posting this, but I know people are going to keep reading this about the EMG and panicking if they think it might apply to them or their loved ones as well.

Generally speaking, the only EMG testing done for the bulbar area is the tongue. Not always, but usually. The tongue is innervated by the 12th cranial nerve.

Erica's husband does not have that much tongue weakness, but he does have a weak epiglottis. The epiglottis is innervated by the 9 & 10th cranial nerve, not the 12th.

If the facility he was tested at does not test the other areas for their patients, then the doctor is most likely taking this into consideration, as, after all she would know better than anyone what nerve is responsible for his problematic areas, and whether or not it was included in the EMG testing.

I'm not suggesting that they should not go for more opinions, not at all.

My personal experience was that I did have an EMG of my vocal cords, before one done anywhere else, and it was abnormal (vocal cords are 9 & 10th cranial nerve as well). A couple of months later I had an EMG of my tongue which was normal, (eventually it wasn't, but at first it was). So, before something like this becomes more global, it is possible to have a normal EMG, but receive this diagnosis, especially if the specific area of weakness was not tested with EMG. Even though testing is good, a doctor's exam findings play a huge part in what is determined.

I hope he is able to go to another facility for more opinions, but I understand, having gone through it, why they would be told this.

 

[Zaphoon]

Excellent post, Rose!

 

[t34gib]

Erica, So sorry for the diagnosed, but a second opinion is absolutely called for. I too, have just been diagnosed with Bulbar Palsy, probable ALS. I only have a speech problem with mild swallow prob. Slow progression was also mentioned. The thought of losing my voice is horrifying. But there are other means of communication and people in my circle have been very supportive and if that is what it comes to, so be it. Someone told me the other day on this forum, take the next two weeks and find your new normal. I am finding that day by day, I think of it less. I still think about it a lot, but trying to just move on. See what happens over the next 6 months. I hope your husband is very slow. Your daughter deserves to have her father for a very long time.
Nancy S

 

[lydia]

Erica,

I am so sorry to hear this, I just don't understand it and had been thinking of you two and thinking there was a mistake...please get a second opinion just to be sure. Thinking of you, your husband, and your daughter.

Lydia

 

[brendapals]

Erica,
I am also sorry to hear of your husband's diagnosed. It is shocking, to say the least, but slowly, and on your own terms, you all will adjust to a "new normal". Our boys are now 18, 15 and 8. I was diagnosed in June 08. My speech is the only "big" difference I have, I have noticed over the last couple of weeks my "grips" are not as strong, but it's really no big problem.

I will post more later, we have had a horrible ice/snow storm and our power was just out for 48 hrs, and I have lived to tell about it!:lol::lol:

talk soon,
I'll keep you in my prayers,
-b

 

[bryan12]

best wishes to you,
stay strong

bryan

 

[perplexed]

Thanks, everyone for all your best wishes. Rose -

Your informed input is so valued. In fact, my husband's tongue EMG was inconclusive as he "wasn't relaxed enough." He never had one done of the epiglottis. I have suggested it in my cover letter to the 2nd opinion. When I raised the question with the diagnosing neuro of a brain MRI being sufficient to detect weakness at the bulbar cranial nerve area, she said it was. The Dx was made, she said, simply by clinical symptoms (swallowing difficulty, saliva pooling and weight loss), by the presence of clean EMGs, and by the absence of anything else so far that has presented itself in explanation. So a bit of omission and a bit of commission. She said that while the clinical Dx is "suspected ALS," she is "absolutely sure" while saying in the next breath that absolute certaintly means the opposite.

My user name is very apt at this time.

I posted elsewhere a plea for PALS to advise what is most helful from their families at the time of diagnosis, so any suggestions are welcome. I can't imagine what he is going through inside right now. He's not communicative.

In shock, trying to take things one step at a time, and reassure my daughter that she will be safe. Oh yes, and drinking wine heavily come evening.
Perplexed

 

[wright]

I am sorry I haven't responded to this thread sooner and I am more sorry for what you, your husband and your family are having to go through right now.

You have been getting wonderful advice and information on here, though. I don't know if I have too much more to add, other than it is important to get another opinion.

There might be a matter of semantics here with your neuro, too. Some neuro's will call PLS . . . ALS, so he might very well have PLS or some sort of pseudo-bulbar palsy, which could explain the clean EMG despite his symptoms. The outlook for both of those are much, much better than ALS.

Rose could also be correct in that it has yet to spread to the tongue and that is why the EMG is clean, so please make sure the next EMG is more thorough (make sure they include the limbs as well).

I wish you the best and I will try to get on here more often to lend my thoughts should you need them.

 

[perplexed]

Re: Husband got ALS diagnosis this morning - Early ALS Bulbar onset, slow progression

To Everyone who wrote me, I just found this forum again after wiping out my OS to get Windows 7. Thank you all so much for everything. I don't know if this will find you all, but to recap: it's been almost two years since my husband was misdiagnosed with ALS (yes, I'm suing the doctors as a wake-up call so hopefully they will not repeat this kind of medical negligence and incompetence, not to mention the patrony and condescension with a patient's family - won't get anything as it's Canada but it will at least go on their records). They caught it so late after misdiagnosing for 5 months that the cancer was in Stage IV. He will probably never eat again, and life expectancy very different from whether they would have caught it at the outset. This would have involved cat scans in the correct area (hey sir, you can't swallow? Let's just scan that abdomen, not the throat, where the CANCER IS), and not disregarding that every single test came back negative except the MRI which was DONE INCORRECTLY.
So as you may imagine, I'm balancing extreme anger and resentment with keeping my family together and being positive for my daughter, who's being great, though at the onset of teenage-itis.
So - thank you all for all your help and love, and I send it right back.
Erica

 

[BarryG]

Re: Husband got ALS diagnosis this morning - Early ALS Bulbar onset, slow progression

Erica, it's nice to see you here again although I wish that the circumstances were better for all of us. Sorry about your husband's misdiagnosis and subsequent correct diagnosis and I hope that you can get some satisfaction in your law suit. I know that there are many good doctors but I'm sure there are some bad ones too and I'm sorry that you found them.
Take care
Barry

 

[Al]

Hi Erica. Sorry how things worked out but hope your suit is successful. From my understanding some judges are becoming a little sympathetic to patients lately.

AL.

 

[rose]

Re: Husband got ALS diagnosis this morning - Early ALS Bulbar onset, slow progression

Because Erica and I had kept somewhat in contact (until she lost her login ~ sounds like something that would happen to me) I was aware of the cancer diagnosis, but did not know how well her husband had fared from treatment. It still burns me up, and I mean really infuriates me, and I'm a CALM person, when I think how they begged for different tests to be run, and other options to be considered, and their neurologist just brushed away their efforts to be heard. Between this example, and tag0620/Tracy's husband's incorrect diagnosis by respected, prominent specialists, it is clear that patients and those in their family who are active in the diagnosis process, be vigilant in their determination to reach the truth.