Mixed Signals

And I have noticed some of your "stats" in your signatures have "bulbar onset ____, DX 00/00/0000"
Is this just saying you began to experience bulbar symptoms on one date, and then were given an actual diagnosis months or years later?

Yeah .. .that pretty much covers it. Though I dont show it , I had symptoms in July 07 and was not diagnosed until Oct 08. Yep it took that long to figure out.

Glen

Matthew ... .It seemed like you put some of your own time into reading about MG/ALS to reply to me, and that was really kind/considerate of you. Actually I had put the time in a couple years ago reading about it for myself, back when I was convinced I had MG. But my current diagnosis of ALS is firm and final.

Basically, the neuros don't account for my reaction to mestinon ... or perhaps they may attribute it to placebo effect if they think about it at all, and I wouldn't argue with that. In which case, I'd be glad to have them give me a placebo drug that doesn't interfere with beta blockers.

My current neuros ignore my double vision, too. Vision problems are not characteristic of ALS but they occur in a fair percentage of MG. Double vision while reading is also a symptom of a neuro disease called Progressive Supranuclear Palsy, one of the many ALS "mimic" diseases. That was my second diagnosed. (My first diagnosed was stroke. Third diagnosed was MG. Fourth and final diagnosed is ALS.) PSP is not related to ALS ... it is a "Parkinsonian" disease ... but has many of the same symptoms as ALS.

I think the atrophy makes my diagnosed of ALS conclusive, because muscles don't atrophy in MG (if I am remembering correctly). In MG, signals from the brain to the muscles are interrupted, but the nerves, brain and the muscles themselves remain healthy. Once the immune system is under control and stops preventing the signals from getting through, the muscles are able to function again. Recovery like that doesn't happen with ALS.

I agree with you ... if mestinon does give that boost to everybody, I don't see why everyone's not on it. (Maybe possible side effects ...)

And it does take a long time for many PALS to get a real diagnosis. My symptoms started in March 06, and I got my final diagnosed in May 08. There are so many conditions that our symptoms could be part of, some of them completely benign, some difficult but treatable, and some like PSP and ALS, progressive and incurable (so far).

Hi!

Hi Bethu and Matthew, i was previously given a diagnosis of MG as well, as I tested positive for the Tensilon test for one - two items out of the whole list. They put me on Mestinon for a few days, but the mestinon weakened me so much for the next few days that they had to take me off it. WAs feeling really good during the Tensilon test though, had so much energy and strength that wanted badly to go out and use all the energy that I've missed for so many months. Lol. I also entered into a crisis (2 so far), both resulted in stays in the hospital due to some breathing problems. Recovered miraculously from both, as in breathing stabilized enough for me to be discharged, but my strength never came back. The 2nd crisis also came when I was on Mestinon. But that happeend after a scare (a nurse pushing my wheelchair pushed me so hard, bearing in mind i do not have strength to hold on to my wheelchair, I flew off the wheelchair), was left gasping for air after that. Initially they had suspected Thyroid, MG, PLS and ALS. One by one they were all crossed, leaving behind ALS, after my emg showed some signs too. And that was after I had what they thought was crisis or deteriorating signs, and my condition sort of stabilized enough for me to be discharged after that. Am also not sure what's happening, but I've got a lot of info for MG during the time I thought I had MG. Have heard that there are often confusing signs and diagnosis can alternate between Mg and ALS as some of the symptoms are quite similar. Please PM me if you'll like more info. Have spoken to quite a lot of people with MG, and some of them have entererd into crisis before as well, so perhaps they'll be better off answering some of your questions! I'm not sure if everyone respons to Mestinon, as I didn't. It basically made my symptoms worse. Took me 2-3 weeks to get back to the state I was in before I was started on Mestinon. Based on what I know, people with MG generally get better with Mestinon, Predisone, Imuran etc.

But I do find it weird that the doctors are taking your dad off Mestinon when he has done so well after taking it. Have you checked with them the reason for this?

Hello all,

Just returned home from a truly dreadful few hours at the hospital. I had gone in to clean up his nails (nurses do not do any nail care, not even clipping!)... This is the third or fourth day off the mestinon. I came in and he was completely unable to hold his head up. It was resting on the table he has in front of his chair... He looked a lot like he did the weekend before all this happened and the respiratory crisis brought us into the hospital. He can no longer speak AT ALL and has gone from walking 100's of meters on Monday to being unable to even stand up. Worst of everything and anything was looking into his eyes as he struggled to stand up out of his chair and say something, the expression of terror and confusion and panic was truly heartbreaking.

I have NO idea what to do. I was REFUSED once again when I asked about restarting the mestinon. No matter what he has, what his prognosis is... I do not see ANY harm in restarting a medication that appeared to be providing some improvements and sustaining them long term.

I really have no idea what to do. Where to ask for help... who to tell at the hospital. I think it is truly criminal to watch a 62 year old man take steps further and further backwards, knowing that a potential treatment existed, and just continue to watch him decline. Last week he had worked up to being off the ventilator for 16 and sometimes 18 hrs per day, and requiring MINIMAL ventilation at night to needing the second highest pressure support and ventilation that the system can provide. I DONT think these are coincidences and I think they must be connected with the removal of the drug from his treatment. WHY is no body listening to reason. His night nurse was someone who has had him several evenings about a week ago... He was absolutely SHOCKED at the deterioration and said it was undeniable and obvious. He said he couldnt believe what was happening in the ICU was happening in a Canadian hospital.

I feel like i am totally helpless.

Get an attorney or threaten them with one? I had a situation with my daughter (long story) where a good friend of ours (a physician) told me, word for word, what to say in my efforts to advocate for better hospital care that would get them to sit up and take notice. It worked. Take in a video camera as well. Get that nurse's name and contact info. Ask for his records documenting time off the vent previously compared to now. Put your complaints in writing to every possible person, demanding a response, including the hospital attorney. Make it clear you are creating your own paper trail. Is it possible to have a second opinion from somewhere else when he is in the hospital? That was part of my daughter's issue. No one would step-in if she was currently hospitalized. So we couldn't get another doctor to intervene, we had to say "attorney". Have you tried to go up the chain of command yet?

Lydia

Matthew I think it is time to go to the hospital director of care and start making big waves, and start threatening to bring in the press.
Laurel

Matthew,

I totally agree with Laurel and Lydia. Has he seen and ALS specialist or MG specialist while in the hosp or is it just
the general nueros. Has he had an EMG or Single fiber Emg.

In agreement with the ladies. Documenting everything and using video is a great idea. And you will still need to get someone higher up to listen to you.

Perhaps you could contact these people. They could answer your questions about Mestinon and maybe advocate for your father at the hospital.

http://www.mgcc-ccmg.org/support.asp

Good luck!

Hi Matthew, I do agree with what everyone here's suggesting. Its tough diagnosing MG, have met many who have only got a diagnosis after a decade. Stand firm and fight for your dad, insist on knowing what are their reasons for doing so, ask them who would be responsible for the deterioration of your dad's condition, and document everything they've said down. It helps to take down their names, and what they say, so that they'll start taking you seriously. Wish you all the best!

Going After Hospital

I am going after a Hospital that constantly put Tim through a Life Threatening Situation. I took pictures, took all the notes that had been posted on a Wall. I made notes every time. The last time, the Nurse told me that this could have cost him his life. And was still abused. I had made a Formal Complaint the last time and they refuse to give me a copy of it. They had also made the same mistake in August when he was there. I am going after thin with all Guns Loaded. A Lawyer recommended me another Lawyer. I am working on it now. I have already requested Tims medical records. I have to go through Medicaid to get them, go Figure! I am sick of these hospitals and their neglagence!

Lorie

Questions to ask

Hi Matthew,

Found a chapter in Calgary, perhaps you can try contacting them.

Foothills Medical Centre
Adult services
E-mail: [email protected]

Address:
Area 3 University of Calgary Medical Clinic
3350 Hospital Dr NW
Calgary, AB T2N 4N1

Tel: (403) 944-4418
Fax: (403) 270-8830

Contacts:
Physicians: Dr. A.K.W. Brownell, Dr. T. Feasby, Dr. D. Zochodne, Dr. Korngut, Dr. Toth
Coordinator: Roula Simmons

Here are some questions you may wish to ask the neuro as well. Got this info from my friend who has MG.

She suggested asking them to do plasmapheresis. If he gets better, it's MG. If not, it's something else. Its something which should already have been done.

1. It is VERY dangerous to start someone on steroids while in a severe MG crisis. Have they taken him off of steroids too? He could then be in an adrenal crisis! Have they tested his cortisol? Again, the "protocol" is to do plasmapheresis.

2. Have they checked his arterial blood gases? What are his O2 stats? I know, you probably don't have these answers but this is what you would need to ask.

3. Have they done the MuSK antibodies too? Some people who have MG test negative for the antibodies bloodtests, but test positive for the MuSK.

Someone should ask these doctors if they want to have a lawsuit as a result of not giving your Dad appropriate treatment. Worse yet, they treated him for MG, took that treatment away and he got worse.

If you're still facing problems getting them to give you answers, then contact the Attorney General's office, they should be able to do things on an emergency basis.

The Ontario Site: http://www.attorneygeneral.jus.gov.o...t/overview.asp

Here's Alberta's AG: http://www.justice.gov.ab.ca/ministers/jag.aspx

They need to know that your Dad's life is in jeopardy and their action is urgent.

When in doubt, get a lawyer for an hour or two. That'll get those doctors off their butts.

Let me know if you have any questions. Hope the doctors wake up and start doing something right for your Dad, to get him feeling better soon!

Hi again to everyone on the forum...

I have to say what a nice relief it is to be able to come on here and talk to people about all this... Once I get paid (friday) i will be making a donation to the forum!

Just an update...

After 4 days off the Mestinon, his condition deteriorated steadily. He could no longer breath on his own during the day for more than a few hrs (previously he had worked up to 16), his speaking ability was totally gone, and he could no longer walk even a meter (10 days ago it was up to about 350mtrs).

Pleading with the Drs had no results and I was begining to think that perhaps they were right and he wasnt actually a myasthenic and the mestinon never was doing any good. I arrived Saturday morning in the middle of rounds and the first thing the Dr said was that they were restarting the drug because of his rapid decline... The dr was very specific and said that the efficacy of the drug peaks about 8-10 hrs after the dose and that by then we would see results if it ever was helping him.

Saturday night and still no improvment, in fact he seemed worse... He asked to be put into bed at around 7pm (usually he stays up until around 10) and he was still needing more and more ventilator support. Things really didnt look good.

Sunday morning however he was improving, seeming stronger and could speak a little bit. The Doc was unimpressed and thought that my Dad might simply just be trying harder during his assesment tests and that we may have been experiencing a placebo effect.

Things got interesting at around 5pm on Sunday. His speech has improved about 75 percent, he could speak in sentences once again, and his head and neck could once again hold themselves up without being propped up by his arms. I went and asked the DR what he thought about the change in condition and he said "i am still pretty certain that the mestinon isnt actually doing anything, I am stopping the orders for it"... I asked him to come into my Dads room and actually take a look at him. He was shocked and kept saying "neurology said that if we didnt see any change in 8 hours that we would never see one" ... He finally admitted that he really does not have much backround knowledge about MG or even MND...

My Dad has had every other test for MG... EMG, Tensilon, two or three different blood tests... even one that had to be sent to the UK... Everything has come back negative. All signs point to him not having MG except for his positive response to the mestinon.

DESPITE this... most of the hospital staff are still leaning towards ALS or another progressive MND. As if he had totally forgot what had happened last night, the Dr came in this morning and said "yes, voice is better... neck is a little better... I am still not convinced. Next thing might be to switch him out to sugar pills and not tell you guys and see if you keep noticing 'improvements'... This might be all mental here"

This is when I began to COME UNGLUED. It doesnt really matter WHAT condition my Dad has, good prognosis or bad, if a medication is helping him, do we really need to keep taking it away? When they took that drug away, I couldnt talk to my Dad. When they brought it back, I could. I think that, at the very least, the ability to speak and swallow food should be reason enough to leave any kind of medical therapy in place. I have lost ALL trust in both the Doctors and Nurses at the hospital. What kind of environment are they creating when I have to be wondering if medication that seemed to be treating at least SOME symptoms is secretly being replaced with inert substitutes to "test" my behavior and the behavior of my family in response. I think a large contributor to the problem here is that there is a lot of "long distance diagnosing" going on. The Dr sees my Dad for about 5 minutes each day, and many days not at all. They stand about 15 feet away from his door and discuss. The neurologist has seen him twice in 10 days, and was not present at all since withdrawing the mestinon, the corresponding decline, and then reinstating it and his improving... Am I out of line to ask them both to hold off on any sort of diagnosis until they actually spend some time with him and guage themselves how he responds to medications?

Connie: Thank you for the info. My Dad actually had Dr Toth from the foothills clinic come over to the hospital he is at and evaluate him for a week. HE is the one who gave the firm diagnosis of MG and said that it was a rare subtype which usually required a lengthly ICU stay if a respiratory crisis was reached. He went back to his "home" hospital though and we have since had 2 different neuros and 3 different intensivists.

Plasmapherisis has not been done. My Mom brought it up twice and the first time no one really seemed to know what it was, and the second they said it was "risky" and left it at that. He has had two courses of IVIG which seemed also to help him in his recovery. Until the MG medication was stopped, he was on an upwards curve of very gradual improvment. I think what has thrown everyone off is how slowly it was going. One of the Drs just decided that it shouldnt take this long and that he had an MND and not MG.
They do blood gases every few days. When he came in, his CO2 was so high that he has having hallucinations from it. He was that far into respiratory failure. Since being in the hospital it has been a little high but consistently at the same level which they think might just be "normal" for my Dad. For a while they stopped doing blood gas testing because he was breathing fine on his own during the day then being rested at night on the ventilator. I know one was done 2-3 days ago and it was fine.

The steroids were started, in very large does, about a week after arriving at the hospital. He was just on prednisone I think... They have slowly been tapering it off.

I think that part of what is happening is that they think I am in some sort radical denial phase where I am inventing/manifesting these "improvements" in my Dad out of no where and that really nothing has changed since his admission. His exercise therapists are on my side and have said that he has made great strength improvements... Before coming to the hospital, I was sure he had ALS. I was totally prepared for bad news, and had never even heard of MG. I had researched the symptoms he was having and they all seemed to fit with a MND. I am not desperately grasping at this obscure illness like they seem to think I am. I just want my Dad to be getting any kind of treatment that he can derive benefits from, no matter what his prognosis is.

Matthew,

I hope you have found someone to help you with all of this and that you will keep us updated.

Hi Matthew

Thanks for the update! You're most welcome, do wish there's more I can do to help.

First, would suggest getting Dr Toth back to have a look at your Dad. I'm sure you can contact the MDA chapter to see if there's anything they can do. Explain what the docs are doing to your dad, and get them to help you. I'm sure there's something that they can do for you if you let them know your situation.

Secondly, would also suggesting checking if they've tested your dad for the MuSK protein. There are some myasthenics who test positive for this, while do not test positive for the antibodies to the acetylcholine receptor.

Thirdly, to all the tests, sometimes all of these tests are negative or equivocal in someone whose story and examination still seem to point to a diagnosis of MG. The positive clinical findings should probably take precedence over negative confirmatory tests. MG is often quite difficult to diagnose, so a positive response to Mestinon is sometimes also taken as testing positive for MG.

MG usually declines rapidly, and can even start with a crisis. However, it can be controlled with the help of medication. Would also suggest that you get an attorney in asap, if they simply refuse to take you or your family seriously. Do keep us updated!