Hi, read this on another forum, was thinking if any of you have any suggestions for this poor guy here, whose dad's been suffering in the ICU for 100+ days, but yet to get a diagnosis. Apparently his dad was placed on Mestinon for a while, and all his symptoms got better, but his doctors recently took him off Mestinon and his symptoms all returned very quickly. Now he has to be put on the ventilator, and is deteriorating very quickly. Doctors there seem to think that he has ALS but from his marked improvement while on Mestinon, I would be thinking of MG, as havent really heard of ALS getting better while on Mestinon. Anything to recommend?

An excerpt from what I heard from him:
"After being intubated he was almost immediately started on Mestinon as well as prednisone to help his respiratory recovery, and over the course of about 3 and a half weeks was given larger and larger doses. We immediately saw imrovements. He could hold his head up again, and after the intubation was brought to a tracheotomy we noticed that his speech has begun returning as well. He eventually was able to speak with little slurring and more air behind his words when he had his "speak valve" on. His swallowing abilities also seemed to return (we were in the midst of testing them with different fluids and foods). Four different neuros consulted all were of the opinion that it was a myasthenic crisis which brought us into the ICU and that the subtype of myasthenia he had often required 100+ day stays in an ICU setting to wean from the ventilator and make a recovery.

Things seemed to be improving at a slow but noticeable pace.

On Monday, a new doctor began ICU rotation and said that since admission, little progress has been made. He has totally stopped the mestinon and brought in a new neurologist who has said that MG is on the bottom of his list of possible diagnoses and that ALS is more likely. Today, 2 days after the last dose of mestinon, his speech is again very difficult to understand and he is again slumped over unable to hold up his head and is again having a lot of trouble breathing. Blood samples sent out showed negative for both of the possible tests for MG. We had initially been told that there is a percentage of MG patients who show negative to both of these tests."

3 days later:
"Just returned to work from visiting my Dad at the hospital. He can no longer hold his head up without it being propped on one or both hands. When I asked him if he could hold it up at all, he said he could but "really needed to concentrate on it", and even then could only go about 5 or 10 seconds. His speech has returned to the way it was when he was admitted to the ICU. This is all after 3 days off the mestinon."

The guy seems totally helpless and just thought that some of you may be more experienced with all these. Any ideas? He's located in Canada, City: Calgary, State: AB. Anyone he can contact who's experienced in diagnosing MG?

I just googled to see if there is an MDA clinic or organization in Calgary. There is a chapter. I found an e-mail & clinic list


calgarychapter@muscle.ca


Foothills Medical Centre
Adult services

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



Please pass this along. I hope this person gets help very soon.
Jenna

Connie, Why the heck does this happen?!

What they should be doing is plasmapheresis. If he gets better, it's MG. If not, it's something else.

You guys have attorney general offices that can do things on an emergency basis, just like here in the U.S. Here is 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 this man's life is in jeopardy and their action is urgent. I couldn't find that you guys have medical boards like we do.

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

First, it is VERY dangerous to start someone on steroids while in a severe MG crisis. Second, 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.

Second, 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 the caregivers of this man need to ask.

Also, have they done the MuSK antibodies too?

Someone should ask these doctors if they want this man to die and to have a lawsuit as a result of it because they didn't treat him appropriately. Worse yet, they treated him for MG, took that treatment away and he got worse.

Yes, ALS can temporarily get better on Mestinon but not over time. I would ask what OBJECTIVE evidence they are using to say it is ALS. EMG's?

This is crazy doctoring and I hope this man gets help soon. I'm sorry I can't help more. That was very sweet of you, Connie, to help out.

Annie

Thank you so much for all the info! I've passed the info along to his son, do hope he gets well soon and hope the docs start doing the right things.

I too find it appalling that this is happening in the hospital, and in the ICU. They have tests to verify if it is MG and they're not. According to the caregiver, his son, the patient has had EMGs done, the first two were clean, the latest one seemed to have pointed towards MG but yet they still persisted in taking him off the Mestinon. Requests to start the Mestinon again were rejected by the docs. They have no tests to prove that its ALS, the reason for taking him off the Mestinon was simply cos they only saw SLOW PROGRESS. Worse still, the doctors keep changing so they aren't even sure who is the doctor in charge. The whole family's stressed out, seeing the patient finally getting better and now deteriorating rapidly, yet they can't do anything.

Am really hoping that the family will find strength to really get the doctors to give them some answers.

Its really sweet of you to help too Annie! Will keep you updatedonce I hear from his son.

Connie, that was so thoughtful of you! I sure hope this poor man gets the help he so desperately needs. I hope his son gets back to you with some good news soon. Hope you are feeling better today! Take care.

Hugs,
Pat

Connie, I have something else to add. This isn't always the case with ALS but most often it is. There are always exceptions to any disease.

MG is a "head and down" disease because it usually starts with ptosis, neck or swallowing issues.

ALS is a "foot and up" disease because it usually starts with the feet.

I brought this up for two reasons. One, they should be doing an EMG on his lower legs to look for ALS. Two, the length this man has been ill should be a clue to them. If it was rather rapid, it is probably MG. Though some cases of ALS progress more rapidly, it's odd that the entire body is affected all at once like he has it.

Annie

Hey connie! This makes me sooooo angry! What more do they need? I know that only 10 days in the ICU costs over $100,000, so I can't imagine what his bill is going to be like!

What gives this new guy the right to rule out MG? Why do so many have to suffer b/4 they are dx'ed? Plus, the hosp is a breeding ground for all sorts of creepy crawlies......not so great if you are too weak to even hold your head up. I remember those days. They were so scary - understatment of the year!

I do not know what Mestinon is used for other than MG, but isn't a POSITIVE response to MG a HUGE indicator OF MG?Sounds like attorneys need to get involved b/4 more damage is done.

I pray that he gets the help he so desperately needs b/4 it's too late. Tell his son to fight tooth and nail - the squeaky wheel does get the grease - ya know?

Big hugs!
Erin

Thanks for all the info Annie. It was great help to his caregiver, who came back with all these info. From what I read, the patient did start with bulbar symptoms, before it spreading to his limbs. However all these deterioration happened within a span of 5mths. I too would think that its too fast for ALS, though the doctors insist that some ALS deteriorate so quickly within one year, and pass away the same year. I don't think anything showed up in his EMG to point to ALS, but his docs are saying its ALS simply because of his progressive weakness and deterioration, and his lack of fast improvement when started on Mestinon. The doctors seem to be giving lots of excuses, but here's what his son said.

How it started
"Around December 2008 he began having difficulty swallowing and speaking. The symptoms were manageable and tended to come and go. Things remained like this for a while and I didn't really think much of it. In April 2009 we went on a trip to Toronto/Montreal and i noticed had almost completely lost the ability to hold his head up, and his speaking had become much worse. He began complaining of seeing double and having other vision problems. He used to be an avid "city walker" and loved walking for many KMs throughout different cities we were visiting. This trip he wasn't able to go more than 2 city blocks without having to get into a taxi and head back to the hotel. I had noticed that these symptoms were improving with rest. Things began to rapidly deteriorate about a week after returning from vacation. To hold his head up he had to have it propped up with both hands. His speech had become extremely difficult to understand... It seemed to be one long slur most of the time. During all of this we were waiting for a neurology appt. which had come with an 8 week waiting period. By mid-may, he was no longer sleeping through the night and could barely eat or speak. One morning, he was almost totally unable to make it up a flight of stairs and became increasingly confused. He had begun hallucinating and was appearing to have problems breathing. We headed to the hospital and he was admitted immediately as he was in the midst of respiratory failure."

Tests done
"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."
*He didn't state if MusK had been testsed, I'll remind him to check again*

Current Situation
"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..."

What really amazed me was what the doctors said when asked if his dad could start the Mestinon again.
"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"

As to what Annie had suggested, the patient 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 they have since had 2 different neuros and 3 different intensivists.

Plasmapherisis has not been done. His wife 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. The patient 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 him. 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. His son said 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 from what his son thinks... They have slowly been tapering it off.

Questions I have, any comments?
1. The patient started showing marked improvement after about one day from restarting Mestinon. The doctors refuse to take that as the response from Mestinon, as its after the 8-10 hours specified by their neuros. Can it take longer Mestinon to take effect for some Myasthenics?

2. They mentioned that Plasmapherisis is "risky". I understand that it has complications, but is it considered risky since there are medications and others to help prevent the complications? Is Plasmapherisis suitable in his current condition?

The doctors seem to be thinking that the family is in a stage of denial, and so refuse to take what the family is saying seriously. I've asked him to try his best to get Dr Toth to see his dad again, and to at least inform someone from the MDA chapter of their situation, to see if anything can be done.

Hey thankss so much for everything! I wanted his son to come to this forum, am waiting for his private messaging function be enabled so that he can come here if he has time, to posts his queries directly so that it'll be quicker and more precise! In the meantime just wanted to help him wif some of his queries through all of you. Thanks so much for all your replies, I'm sure he's overwhelmed with the info that all of you gave.

I too, hope that the poor man and his family gets some real help from the doctors soon, a stay at the ICU definitely isn't cheap, and to waste time with all these doctors who don't seem to know what they're doing, definitely isn't a good way to spend all that money. His son seems to be extremely stressed out now ( I would be if my dad's being treated like that without any explanation), and the doctors are thinking that the whole family's in some sort of radical denial. I'm not sure if he's in a good enough state to ask for help from the AG office, or if he has enough strength to do so. But have already forwarded all the info from Jenna and Annie to him, am sure he finds it useful.

Will keep you updated once I hear from him again!

Wow Connie,

well done on all you have done, I've kept my nose out of this one, as hospitals in America seem so very different to here, and I have no idea of how things run there.

Thankfully here if you are seriously ill you end up in a public hospital,they are very good mind you, and incure no costs what so ever.