Update:

[Micheline]

Well I have called my ALS specialist twice this week. Still no answer on my biopsy. Now going on 3 weeks plus. I was asked to post my EMG results, but no one answered. I know everyone is busy.:smile: BTW: Happy Thanksgiving, for all canadians.

I think all I ever do is EMG's now. I am sorta used to them. Some of the doctors are "nice" about it, others just just get right in there.

On another note, Steve and I are/have been putting off telling our kids. Boy 16 years, girl 14 years. They have been helping alot. My son feels uncomfortable at times, do I blame him, absolutly not. My daughter has been very good.

BTW: We have our stairlift...yahoo! No more going down on my butt:grin: We will be looking into a better wheelchair, the one I borrowed is very old and only has one foot rest and no brakes. My walking is very limited now, a few steps at a time and then I need to rest. I need to see my GP about some AFO's, my ankles are getting worse, sometimes I get up and they are not straight (under me) and I have to rush to get them fixed or I will fall over. I have noticed muscle wasting in both of my thighs when I lay on my side everything droops. I have been getting cramps in my thumbs and index fingers, not liking that at all. Muscle aches in my thighs are not fun at all. Last night I couldn't sleep, I finally fell asleep about 1-1:30am up at 5am. That sucks even more!

We are torn, do we tell them that the doctor I am seeing now is an ALS specialist? Or do we wait? They have been updated on everything until now. So hard to decide, can anyone give some advise on this.

Micheline

 

[brendapals]

Micheline,
I don't know how much help I can be, but I will share my "telling the kids" story with you. I find out my diagnosed on 6/3/08, yes, it was quite shocking, but probably more so for my hubby. Our boys are 18,15 and 7. He really didn't want to tell them until he was sure I would be home for them, and not at work.
I guess he thought if they had questions, it would better for me to be here, than not.
Anyway, we spent one day with the kids at Chuck E. cheese, I'd rather have 10 root canals than every go there again!
Then I spent the next 2 days working. Then the weekend came, the time just never seemed "right", so on Sunday afternoon, they were all 3 in the "blow=up" pool from Walmart, and I just came right out and told them. My hubby was in the house. Yes, there was a LOT of crying, a few questions, a lot of answers, they suddenly understood a little more about my crying episodes, my choking episodes, things like that.
They all went with me to my first ALS clinic appt on 7/1/08. They were extremely supportive, and talked to everyone that came in the room.
Now that it's been 4 months, and not much is different, except my speech is worse, they really don't think much of it. My hubby is getting used to ordering for me at restaurants, and so are the kids.
I'm hoping to do some "voice banking" next weekend on our computer, wish I had done that 4 months ago. darn it. I heard my voice on my cell phone voice message, and it wasn't slurred at all. I got that phone in May.
That's enough food for thought tonight,
hope it helps and I will keep you in my prayers,
Keep the faith,
brenda

 

[Al]

Hi Micheline. Have you registeredd with the ALS Society of B.C.? They should have a loan closet of wheelchairs and other equipment that you may need eventually. I think you can register with a "possible" diagnosis. The kids know something is up. I'm for sharing the information you have now. They might have already googled your symptoms and have an idea already.

AL.

 

[Al]

Sorry Laurel. I was deleting your duplicate post and killed them both by accident and have no way of getting them back. I feel really bad. Should be more careful. Sorry.

AL.

 

[laurel]

That's okay Al. I was cursing my computer as I figured it had eaten them. But I would never ever curse you as you are a wonderful fellow. I'll re-write my post to Micheline in the morning--too much turkey tonight to do it now.
Laurel

 

[Al]

OK I'll blame it on the turkey leftovers. We had ours Sat. Thanks for understanding.


AL.

 

[joelc]

Micheline, Al is right, register with ALS Association of BC, they are incredibly helpful! Have you been to the ALS Clinic at GF Strong in Vancouver? If you haven't done either you need to ASAP - they are incredibly helpful and wonderful people at both places!

 

[CindyM]

Hi Micheline - I imagine that trying to decide when and what to tell the kids must be one of the toughest decisions, along with everything else this disease brings.

I have some feedback from a young (30 years old) friend. She has a neighbor with undiagnosed neurological problems and that neighbor's young teens suspect that, because their mother is not telling them much, their Mom must be really sick and it must be very serious.

My friend was able to assure these teens that sometimes, in neurology, answers are hard to come by and they should not assume their Mom is keeping anything from them.

What I get out of this, is that left to guess, they will make up their own answers. And if they suspect something really bad, maybe it would be best if they had help dealing with it. Just another perspective. You really have to go with your gut, I think, as you know your kids and their needs best.

Good luck and please keep us informed! Cindy

 

[Micheline]

Micheline, Al is right, register with ALS Association of BC, they are incredibly helpful! Have you been to the ALS Clinic at GF Strong in Vancouver? If you haven't done either you need to ASAP - they are incredibly helpful and wonderful people at both places!

I am going to the Gordan and Leslie Diamond Neurology Health care center in Vancouver. Great doctors I have been seeing. Steve and I have asked so many questions, it is almost like we are repeating ourselves. I was not sure if I could registar for the ALS in BC without a diagnosis? I think in the back of my mind I'm still waiting to get better, go to work, live like I did before.

 

[joelc]

I am still inclined to recommend getting an appointment at the ALS Clinic at GF Strong. They will be able to diagnose you better than anywhere else. I really hope you find out you don't have ALS but something treateable!

 

[laurel]

Neuromuscular Disease Unit

I am going to the Gordan and Leslie Diamond Neurology Health care center in Vancouver. Great doctors I have been seeing. Steve and I have asked so many questions, it is almost like we are repeating ourselves. I was not sure if I could registar for the ALS in BC without a diagnosis? I think in the back of my mind I'm still waiting to get better, go to work, live like I did before.

Hi Micheline,
My husband sees one of the neurologists at VGH's Neuromuscular Disease Unit--probably one of the two women neurologists in that unit that you have seen. They are both first rate diagnosticians. I think if you do get an ALS diagnosis they will likely refer you to GF Strong--they are linked up with GF Strong. Perhaps it is a positive thing that they have not referred you on to GF Strong. We did have to do some pushing to get treatment once my husband was diagnosed with CIDP as VGH sees so many patients that you can get lost in the shuffle it seems. I was surprised that they only tried IVIG once with you as many on the CIDP forum comment that they had several treatments before they showed any positive benefit. My husband was one of the lucky ones and responded with his first treatment--but we had to really assert ourselves to get that first treatment arranged. He got foot drop while he was waiting for the first IVIG, and I got very assertive to get him for treatment. But VGH is excellent once you get in the loop and things are organized.
Micheline how about sending Wright a private email and ask him to look at your EMG results? He probably didn't see them in your thread as so many people are posting these days. He is a sweetheart and will respond.
I was wondering what they said when they wanted to do the muscle biopsy? Did they mention polymyositis? Just wondering if they are considering that?
Happy Thanksgiving to you too. We had our dinner last night at a nice local golf course diningroom.
Concerning the kids, probably Al is correct. The kids already know something is wrong and likely are imagining all kinds of things. But maybe wait for the muscle biopsy results to come in as they may indicate something treatable. I am praying for that for you Micheline.
Laurel

 

[Micheline]

Hi Laurel,
They did rule out polymyositis a while ago. regarding my biopsy, she is looking for inflamation. I had no problems getting my first treatment on IVIG.

 

[SteveS]

To update this thread. Micheline and I finally decided to tell the kids since all the paperwork we are getting back from the GP has Limb Onset ALS as the diagnosis. We figured it was best to prepare for the worst and hope for an other diagnosis. They didn't take the news well as I assumed they wouldn't. My son seems to be in a bit of denial and my daughter has taken it very hard. We have made it perfectly clear that there is still hope that they find something else. The ALS specialist we are now seeing in Vancouver told me she was pretty sure, I would hope she does not do that that loosely with her patients. We are going back on the 31st for the start of what seems every test we have already had, my guess is to be 100% beyond the shadow of a doubt sure. If that is even possible reading through this forum and being the internet research specialist I have turned into. We still remain positive since there are so many diseases that mimic Micheline's symptoms but it is worsening by the day.

Steve

 

[wright]

Hello Michelle and Steve

I just read your post here indicating that no one responded to your post on another one of your threads, asking about your EMG results. I just went to that original thread to read it over and will respond to you here (I apologize I didn't see it earlier).

It appears that the EMG results you posted all came from the lower part of one leg. The extensor digitorum brevis is a muscle that runs on the top of your ankle . . . the tibialis anterior is a muscle that runs over your shin . . . the gastrocnemius is your calf muscle. Did they not needle any other muscles?

The only results you show are from the EMG and it shows that those three muscles are actively denervating (indicated by fibs and positive sharp waves). That doesn't give a full picture, though. The reason is that the report states you not only have denervation (they word it as a motor neuropathy) but it also states you are demyelinating. Does it indicate whether the demyelinization is from motor or sensory nerves? The demyelinating evidence would come from the nerve conduction study (NCS). Is the NCS in the report?

Demyelinization is not something typically associated with ALS, unless it is in the later stages of the disease process. Demyelinization is something that is associated with CIDP and GBS (although given the length of time you have had symptoms, it wouldn't be GBS but rather CIDP) and is typically used as evidence of a disease process that isn't ALS. I also noticed that one of your neuro's dismissed CIDP as a diagnosis. Is it because you didn't respond to IVIG? Sometimes people don't respond to the initial treatment and need subsequent treatments. I would press for more treatments. It is very expensive to do, so I'm wondering if you are having insurance issues?

I think I could give you more of a firm opinion if I knew the results of your other EMG's. Do you have that information? Are you having any other types of symptoms?

One other thing: lack of reflexes is also indicative of CIDP. That would also happen with PMA, which is a variant of ALS . . . but again, the demyelinization along with the lack of reflexes points more toward CIDP. I would remain hopeful and let the neuro's continue doing more tests and of course, lean on us as well.

 

[Micheline]

wright

Here is "some" of the information. Consult is 4 pages. So here goes! Sorry it is so long.

In the upper extremitie she had mild weakness of shoulder abduction and elbow flexion. Forearm and hand strenght normal.Deep tenton reflexes were brisk and symmetric in the arms. In the lower extremities she has flaccid paralysis of the hip flexion, knee extension, knee flexion. She had flicker of ankle flexion and plantar flexion, toe extension, toe flexion graded only 2 out of 5. knee reflexes were 1+ and ankle reflexes were absent.

sensory exam was notable only for slight elevation of vibration threshold in the feet and slight diminution of pinprick perception in the forefoot and toes.

Conduction studies:
Right median sensory, right ulnar sensory and right superficial radial sensory amplitudes were normal with normal sensory conduction velocities.in the lower extremity the right superficial peroneal sensory response was absent but was fely to be related to the size of calves.

Right median and right ulnar motor conduction studies normal with excellent amplitude motor response with no evidence for proximal condution block and no slowing of the motor conduction velocities. F-waves latencies were normal.

In the lower extremity, no motor response could be obtained in stimulating the right peroneal nerve at the ankle or at the knee.

Right tibial motor response was small in amplitude with slowing of the motor conduction velocity at 35 M per second but this was proportional to the marked reduction in amplitude of the motor response. There were no demyelinating features and specifically no significant dispersion of the motor wave forms.

EMG showed diffuse active denervationin at the paravertebral muscles at T12, L5 and L3.Active denervation was also present in the right biceps, and right deltoid. In the lower extremities she had mixed denervation in the right gluteus medius, right vastus mmedialis, right tibiali anterior.

Assessment;
Unfortunately the findings of diffuse denervation in at the upper and lower extremity musculature as well as denervation in the paravertebral muscles with minimal sensory findings is worrisome for a diagnosis of motoneuron diease. The rapid progression of severe weakness in her lower extremities with would b atypical for a genetic motoneuron disease such as SMA. (Been tested and no word that it was positive).

On reviewing the neurophysiologic studies today and the earlier studies of my first Neurologist, I do not feel that there is evidence for an underlying demyelinating polyneuropathy.

In summary, I am most concerned that this represents a lower motoneuron variant of ALS. The sensory findings are minimal.

That is it in a nut shell.:smile: