After months of lurking, I'm officially here

[Grandma D]

I've been lurking here for about 6 months, mostly to be able to say, "oh good, that doesn't sound like me!" But, as of September 24th, I became an "official PALS". Not a club I wanted to be part of.

My symptoms started with excessive yawning, and the inability to sing. Like I sang a solo on Easter Monday, and by Thursday I couldn't sing! Progressed to slurred speech. My PCP wasn't too quick, so I got myself to a neuro. He didn't know what was going on, but felt strongly that it was not ALS. No muscle wasting or tongue atrophy.

I followed up with a neuro at Mayo, had labs, EMG, and PFT, and all were within normal limits. But, because of my speech, she felt that it was ALS. Had a vocal cord EMG a month later, and that was abnormal. I was tested for heavy metals, and my arsenic level was high, but Mayo does not feel that it can be attributed to my symptoms.

Now I continue to have slurred speech, some slight difficulty with swallowing (which has improved over the past few weeks), jaw tremors, and weakness in my left hand primarily.

I am one of the "lucky" ones, who has also experienced hand and finger numbness, severe knee pain, and my symptoms and weakness get better throughout the day. I worked yesterday (as nurse), and I was exhausted when I left. Came home, mopped, did laundry, and walked 2 sets of dogs, and felt invincible when I went to bed! Mornings are the worse, I feel stiff and sore, and weak. My hands and arms feel arthritic. Then, by about noon, I feel much better.

I have already gone to clinic, and they said I am doing well and don't need to come back for 6 months. I am on nudexta and baclofen, which seem to help with the bulbar affect, and jaw tremors. I will start riluzole today, and I take acetyl L Carnitine as a supplement, recommended by Mayo neuro.

So now I begin this journey, with prayers and hope for a miracle, while at the same time, making decisions in the event of no miracle.

 

[lgelb]

A sad welcome, Grandma.

"Hope for the best, plan for the worst" is a wise general direction. In that vein, understanding that you have been diagnosed at Mayo, from what you have said, I would consider a third opinion at UF, USF, Miami or Emory just to be sure.

Best,
Laurie

 

[dldugan]

If you are going to the ALS CERTIFIED MAYO CLINIC in Jacksonville you are in good hands. There is also a certified clinic in Tampa that I plan to visit the Winter. Glad they seem to have caught it early.

 

[Nikki J]

Very sorry you have to join us but welcome. For what it is worth I offer my clinic’s guideline to activity- if it takes more than 1 hour to recover fully it was too much.

I go to Mass General which is an MDA clinic. While ALSA and MDA certifications may mean they meet some standards which they have is political and there are great clinics which are neither. I agree Mayo Jax is a great place to be though.

Look forward to getting to know you. How do you wish to be called? D? Grandma? Something else?

 

[Kristina1]

Sorry to welcome you, but welcome! I saw some inital bulbar improvement on nuedexta, and you may as well. It doesn't last, but it can give you a little more time with speech and swallowing than you might have had.

 

[KarenNWendyn]

Grandma D, I’m sorry to have to welcome you here.

Mayo Clinic has a great reputation.

However, I find myself skeptical of your diagnosis upon reading your symptoms. Maybe I’m missing something?

Slurred speech is certainly consistent with bulbar ALS, as is hand weakness that you mention.

However, you say your EMGs were normal, except on your vocal cords (I assume by “vocal cords”, you mean bulbar muscles). But a normal EMG on other muscles generally points away from ALS. Did they do an EMG on the hand that is weak?

You also mention hand and finger numbness, knee pain, and morning stiffness, with symptoms and weakness improving throughout the day. Since you’ve been a “long time lurker” here, you know that sensory symptoms point away from ALS, and usually the weakness of ALS seems to get worse towards the end of the day due to fatigue.

The morning stiffness and pain raise the question in my mind of an inflammatory rheumatic condition.

So while I’m really not trying to challenge the experts at Mayo Clinic, I’m hoping they have or will address my concerns, and I would urge you to get another opinion.

Meanwhile, no harm in the meds you’ve started.

I wish you all the best.

 

[lgelb]

As Nikki said, ALSA and MDA both certify centers. Hopkins is MDA, so it's hardly a second-tier certification. Some good but smaller resources are not certified by either because they don't have the bells and whistles.

At the end of a day, post-diagnosis, you want somewhere accessible (in terms of both distance and ways other than driving, e.g. email, video), caring, responsive so when you need equipment or some other help, things happen as promptly as they can given the other players. Neither the ALSA nor the MDA certification guarantees that.

It's about people, not checklists.

Also remember, you still need a relationship with a good PCP, to whom you may well transition entirely at some point.

 

[KimT]

Did they do a lumbar puncture and swallow test? If the only abnormal EMG was on your sternocleidomastoid (neck muscles), I would definitely get another opinion.

Sorry to welcome you here.

Regarding the pain aspect, I've had a ton of pain from the beginning that I never had before. None of my doctors can figure it out if it's part of ALS or possibly fibromyalgia or some other form of centralized pain syndrome. All I know is that it is much worse when I wake up, especially in my feet and right knee. My back has hurt for the past three years and it never hurt before despite all the sports I played and a very bad car accident.

I try to take care of my general health as best I can and treat the symptoms.

 

[swalker]

I am sorry to have to welcome you here, but welcome nonetheless.

You will find wonderfully helpful people here with lots great advice.

Steve

 

[Grandma D]

Very sorry you have to join us but welcome. For what it is worth I offer my clinic’s guideline to activity- if it takes more than 1 hour to recover fully it was too much.

I go to Mass General which is an MDA clinic. While ALSA and MDA certifications may mean they meet some standards which they have is political and there are great clinics which are neither. I agree Mayo Jax is a great place to be though.

Look forward to getting to know you. How do you wish to be called? D? Grandma? Something else?

Nikki- thank you so much for the welcome. My name is Dallas, but I am proud to finally be a grandma, he calls me grandma D. So Dallas, D, Grandma all work!

 

[Grandma D]

Grandma D, I’m sorry to have to welcome you here.

Mayo Clinic has a great reputation.

However, I find myself skeptical of your diagnosis upon reading your symptoms. Maybe I’m missing something?

Slurred speech is certainly consistent with bulbar ALS, as is hand weakness that you mention.

However, you say your EMGs were normal, except on your vocal cords (I assume by “vocal cords”, you mean bulbar muscles). But a normal EMG on other muscles generally points away from ALS. Did they do an EMG on the hand that is weak?

You also mention hand and finger numbness, knee pain, and morning stiffness, with symptoms and weakness improving throughout the day. Since you’ve been a “long time lurker” here, you know that sensory symptoms point away from ALS, and usually the weakness of ALS seems to get worse towards the end of the day due to fatigue.

The morning stiffness and pain raise the question in my mind of an inflammatory rheumatic condition.

So while I’m really not trying to challenge the experts at Mayo Clinic, I’m hoping they have or will address my concerns, and I would urge you to get another opinion.

Meanwhile, no harm in the meds you’ve started.

I wish you all the best.

Karen- you have questioned everything I have! The biggest thing being I feel much better in the evening, than in the morning, and seem to have more strength!
My local neurologist still does not think it is ALS, but defers to the experts at Mayo. Mayo, from Day 1, felt it was ALS. My first EMG was on my entire left side, and the only weakness was in my tongue. The neuro at that time said no ALS. Then, a month later, they did the vocal cord EMG, which showed weakness in the vocal cords. She then said it looked like ALS, I mentioned my neuro didn't think it was, and she said "has he heard you talk?" I had to request an MRI, ENT, everything. They were diagnosing based on speech. When I went back in September, she said there was no need for further tests, and got me into the ALS clinic. The people in the clinic are great, other than the doctors. The doctors, I feel, are cold and arrogant. And I have experience with docs, as I've been a RN for 39 years! I tried to go to the ALS clinic in Birmingham, couldn't get an entry appointment until May 30th, 2019. anyway, here I am, and venting a little bit

 

[KarenNWendyn]

Sounds like it’s behaving more like a pure progressive bulbar palsy. I’m keeping my fingers crossed it doesn’t morph into full blown ALS, given that it’s so atypical.

Well, keep an open mind, and keep asking questions of your doctors.

Probably a good idea to be taking the meds you’re on.

We’ll support you as best we can.