Mixed Signals

[connieS]

Hi Matthew, have more to add here, after hearing from my friend.

Plasmapheresis is "risky" but so is giving someone in an MG crisis steroids AND then tapering them before they have a chance to work. If they are not confident in their ability to help him, he should be transfered to a nearby, larger hospital (if there is one) who are. PlasmaP is not only "plausible" it is the ONLY thing reputable and credible neurologists will do when someone is in an MG crisis. It offers fast improvement and that is what someone needs when they are as bad as this man! Steroids can make someone worse and they could've killed him.

This is the deal with Mestinon. It kicks in about 1/2 - 1 hour after taking it. It can wear off 2-14 to 3 hours after taking it. It is basically effective for about two hours. When someone is in a crisis, having Mestinon helps but it is ALMOST like having water. They would have to give him a dose more often, like every 3 hours for them to start seeing an effect.

Have they even done breathing tests? The thing with ALS, and again this is mostly the case, is that it is progressive. It does not wax and wane a lot like MG does. And those arterial blood gas readings have obviously reflected the fact that his breathing is waxing and waning.

And if your dad has double vision that gets better when he closes one eye, then they SHOULD ABSOLUTELY suspect MG. Fluctuating double vision/ptosis is NOT a sign of ALS. It's like they are not being scientific about all this.

And what BS is this about blaming the patient and saying the Mestinon has a placebo effect?!!! Would they give someone with a heart attack a placebo pill instead of nitroglycerin? This is crazy and dangerous medicine and they have no idea what they are doing. If someone "normal" took Mestinon, they would get worse. And they do have objective ways to measure if he is getting better, like the clinical exam or the ABG's or EMG's.

And about EMG's . . . they would be negative on Prednisone. So when did they do them? While on prednisone?!

And antibody tests will often be negative when run when someone is on Prednisone. When were the antibody tests done?

I'm going to say this again: Find someone in a higher authority to help. Or an MG expert. Or anyone who can help him and soon before his condition worsens. If it's ALS, which I doubt from what I can read, then it's a really sad situation that can only have supportive solutions. If it is MG, then they can make your dad better.

And have they tested his thyroid, B12, erythrocyte sedimentation rate or other indicators of infection or anything else that may be making this worse like cortisol?!

If we could fly up to Canada to be a rep for your dad, we would. The way they are treating your family makes us ill. Literally. I'm sorry but am really angry at how they're treating your dad.

Let us know how things goes.

[jik992003]

Matthew, I am very sorry for everything your father is going through and hope that things get better.

[ktmj]

Matthew, good grief! Is there any way to get him moved to a specialized place that knows what they are doing?

We obviously don't know enough medically to know, but it sounds like the treatment, whatever it is, is horrible.

Best wishes - you obviously care a great deal about your Dad.

[matthewdbardsley]

Hi Everyone.

Today i got an official ALS Diagnosis for my Dad. They did a muscle biopsy and the "preliminary results" came in today and apparently they showed enough to have the Dr tell my Dad "you will definitely die in this hospital" ...

I think the muscle biopsy showed denervation? Is this a sure sign of ALS? With the improvements from before... I dont know what to do. I was told he has not much time to live.

[millinero]

Hi! Matthew...really sorry to read about your Dad's condition and more frustrated at the way he is being treated at this very hospital...well all of this happening within a span of just 3 months and your Dad ending up being on a ventilator with clinical improvements later on...is suggestive of something else....i am no doc but i do know this much that with MND too much doesnt happen suddenly.....it takes a while for Docs to diagnose ALS and that to after there is nothing they can relate the condition to......in your Dads case he was improving not deteriorating condition wise......you should seriously consider seeking an second opinion and get out of this hospital if it is possible.

[matthewdbardsley]

We were told a combination of "I have no idea... But it is definitely ALS"

What does that even mean?

Can a muscle biopsy confirm or rule out ALS? Up until now, he was on a steady and gradual slope up towards improvement... But after being so brutally informed of the potential for a progressive, untreatable condition... I dont know where any sort of motivation he may have had towards getting well would have gone.

[hopealive]

Mathew,

I have one piece of advice, please go straight to the hospital administrator with your concerns. I spent a week in the hospital with my mother a few years back. It was awful, no one was giving us straight answers, no one knew a think about ALS, it was dreadful. I visited the hospital administrator on my way out, in tears. He said I should have come to him right away. He would have made sure our time in the hospital was helpful and healing.
Good Luck,
Holly

[connieS]

Hi Matthew,

As I have said before, get in touch with Dr Toth again, as soon as you can. It doesn't make sense for your dad to have shown such great improvements in his condition if it was ALS. I have never heard of ALS fluctuating like that, within a span of 3 mths. It would be to your dad's benefit, to obtain a 2nd opinion, preferably somewhere where he can see a neuro who has experience with ALS and MG. A good bet would be to get the MDA to help you too.

If it is ALS, there would probably be not much that they can do. But if it is NOT ALS, then there is absolutely lots that can be done for your dad to get him to feel better.

It'll be a shame if its not ALS, and its something treatable, but got worse simply cos the doctors didn't know what they were doing.

All the best,
Connie

[millinero]

well Matthew you dont improve as far as ALS/MND is concerned...so do get a second opinion muscle biopsy can be possitive in so many things...not just ALS....with ALS u need to have UMN & LMN signs to be possitively diagnosed of it....and even then if u have them it could be attributed to other neurological disorders....and all of this happening within the span of 3 months....i dont think ALS proceeds in such a quick manner....so its better that u go some place else and get ur DAD a better treatment.

[lydia]

can't a muscle biopsy reveal some type of "grouping" of muscle fibers, in some tell-tale checkerboard pattern that is indicative of LMN problems?

[matthewdbardsley]

Hey everyone,

I thought i would stop and give a little bit of an update just incase anyone had been wondering.

My dad has been in the ICU for 102 days now, still without any clear diagnosis. We have been given MG three times, ALS twice, and very vague dxs without even a name twice. We have been told both that there is absolutely no potential for improvement and that what my father has is genotype of ALS which mostly attacks the respiratory system and when most people afflicted with it die they retain most of their ability to move their legs and arms, and the actual cause of death is respiratory failure or a respiratory infection.

We have good days where he seems to be generally improving and gradually getting stronger and we have days where he is completely bedridden. There have been 7 neurologists and COUNTLESS other doctors giving their opinions on this and no one really agrees. We have been preparing for the worst though...

My dad has been trach cradling for 16-18 hours a day now and some doctors have said he is totally ready to get off the ventilator completely and others say that he never will. We keep having setbacks (pnemonea twice, many UTI's,) and so we will begin to see deterioration which would be characteristic with a MND but then it seems to reverse once the infections are discovered and treated... At the begining of last week he was walking a few hundred meters twice a day every day, and going to the hospital gym each day for about 30 minutes. This week he cant really even walk more than about 5-10 meters. Do yout think a deterioration in a MDN could come on so quickly?

A lot of the confusion and uncertainty is the question of have we seen any improvements over this 100 days. It is hard to tell for me because I see him twice a day, every day! There was a plan to transition him onto using a bipap at night and a little bit during the day so he could at least come home and live the last of his life out at home where he would be happiest.

My main question is... the symptoms he had seemed to come on quite suddenly... everything kind of happened from December-April, and he was admitted to the ICU in May. With such a rapid progression occuring for the first five months, why would things have (at the very least) stabilized for the past three months? And I think there has been some gradual but very clear improvments since admission. One respirologist told my family that the improvment we are seeing is because of proper nutrition and being on the ventilator part time each day. I could totally accept that if the improvment was just for the first few weeks of admission but it has been going on up until this week. We have had no marked deterioration aside from when he is sick with something else on top of whatever this is... Could it be because of the general care he is getting in hospital?

[tdamess]

i am new to this als thing but not going to blame hospital but, sounds like too many cook's in the kitchen, he needs a primary care doc to look over everything all these doctors are saying and come to what condition he believes , ... he will have enough test, and opinon's from other doctor's.....or just transfer him to another hospital where one doctor will be in charge i dont know how it work's where you live nut, even the best hospital's make mistakes, he's been there a long time. ......hope someone else can help you ... that is what i would do best of luck for you and , your dad