hi everyone,

you can read the post "do i have it?" for background but after i couldn't walk all afternoon and evening a week ago last sunday, i woke up the next day and couldn't take a deep breath. it got progressively worse throughout the day like annie mentioned it could and by evening we were racing to the hopital.

i ended up in ICU 2 nights and critical care 5 nights and regular one night. turns out my CTA chest is normal and all the blood is negative. but i am responding to 45 mg of mestinon 6x day and prednisone 30x5 days, 20x 3 days, 10x 3 days.

the ice pack test on my eye was positive, does appear to totally open. i need a walker right now and i am way ramped up on the pred. they are still wary to be totally sure of saying it is absolute even though the doc said the breathing was very scary in the ER and looked consistent with myasthenic crisis and on day 2 he was wondering about IVIG (?)

he still wants me to pursue a single fibre emg outpatient but if i am responding so well, can someone please tell me what is the wisdom in that? i mean other than if they will pull my meds (god help me).

this has been absolute hell. any thoughts? is this a diagnosis? ALL the symptoms, including choking, fatigability x5, eye droop and responsiveness to mg drugs or am i deluding myself here???

not even close to well. . . .

debra

Wow Debra - -so sorry to hear about your crisis!

Yes it does sound like MG - - and Annie will be the best to advise...but there isn't much point in doing the SFEMG when you're on pred and mestinon I wouldn't think.

The good news is that you have responded to the drugs - even though they haven't gotten you back to where you were. It takes time.

IVIG is an immunoglobulin therapy that has helped many here who have experienced a crisis. (I am lucky enough to only need mestinon so far so I am not speaking from experience.) I'm sure others here will be glad to share their first hand experience.

In the meantime, take it easy and I hope things improve for you as the days go by.

Sue

Debra, I'm so sorry you got that bad. The inability to lift your leg up was a huge clue. It's easy to deny that you are getting worse. I wouldn't recommend a crisis for anyone with MG but it is a very good learning experience!

There are few diseases that cause a fatiguing of muscles. MG is the major culprit. Sometimes a diagnosis of MG is done in an ER with a clinical exam, an ice pack test and response to treatments (according to PubMed sources).

The one thing they did wrong, besides not giving you copies of ALL your tests, was not giving you plasmapheresis. That is the "standard of care" for an MG crisis (besides being in the ICU). Sure, it might be different for those who know that, for example, IVIG works better than plasma BUT that is what they should have done - antibodies or no antibodies. If you got better on that, it's a done deal (usually). Neuros will sometimes use plasmapheresis to diagnose a patient when they are antibody negative and their SFEMG is negative or borderline.

MG can temporarily get weaker on steroids. That happened to me during my crisis, besides having steroid insanity. It took an entire month of bed rest for me to get better. And a couple months later, I had an exacerbation where I had to rest again for 10 days. This is the time when you have to be very careful about not doing anything. You can trick yourself into thinking that you are over it and okay. Your MG is now unstable and it needs lots of TLC.

You need to make sure you are taking calcium and Vitamin D while you are on Pred and after. It doesn't matter if you are only on it a short time. Flax or fish oil isn't a bad idea either.

I cannot recommend highly enough that you get yourself a darn good pulmonologist. They can check how your breathing is doing, by either breathing tests and/or arterial blood gases (ABG). Did the do ABG's on you? Do you know what your oxygen saturation was? They can make sure you are improving, along with your neuro, and put in orders for breathing tests like MIP and MEP if you get an exacerbation.

The wisdom in making absolutely certain you have MG is that you don't want immunosuppression (if you have to have that) if you don't have MG. It looks pretty crystal clear to me that you have it but I know I would want to know for certain. They sure as heck are going to take you seriously now. Once you scare a doctor, they don't "pooh-pooh" you again! I guess doctors in the UK don't scare easily, eh Rach?!

Please get copies of everything, including doctor notes from your trip in. Part of this learning about a disease is learning what to look for and what happens when you have a crisis.

You cannot go out in the heat right now. Period. In fact, stay as "neutral" in temps as you can. I have fans in my bedroom and bathroom (for while I dry my hair); they really help. You can't do anything besides take care of yourself. If you do "normal stuff (like drying your hair)," chances are that your recovery time will be much longer. And if you start to get worse on the reduction of Pred, you have to notify your neuro immediately.

And if you start to "crash" again, dial 911. DO NOT have someone drive you. EMT's are equipped to help you and will put you to the head of the line in the ER. Those triage nurses are sometimes completely worthless and may not understand what an MG crisis is!

I know this is a lot to take in. It's rather unnerving really. As long as you listen to your body, take it easy and REST, you should be okay. If you have the SFEMG, which I would recommend, make sure you are off of Pred for awhile and it's often recommended to be off of Mestinon. HOWEVER, you need to follow your neuro's lead on this and only you both can decide if you are stable enough to do that. Because, as Sue said, those drugs can make the test look normal. But being off of drugs right now will just put you back in the hospital.

That was nice, Sue, but there a LOT of people here who can give great advice on a crisis, MG diagnosis, etc. Including you. And different perspectives are excellent to have, especially since MG is so different for each of us.

I hope you will recover soon. Use this time for fun things like movies and music. No singing or dancing though!!! And I had my exacerbation after only talking, so be careful about that too.


Annie

They did the ABG but they didn't tell me anything. . .just put me in ICU. They did sent in the respiratory therapists almost daily to assess and the first day after one blow out hard, it took a full 10 minutes to recover and they did not continue. i know on the spirometer, it was only 500ml the 2nd day since i could see that number. they didn't want to say much--just that my saturation (?) i think was 97-100% all the time so that was good (you know, the one that is on the monitor that you can watch with your heart rate that they take with your fingertip).

btw, my heartrate stayed around 95-100 at rest all week and would go to 125 if i lifted my arms, ate, peed or anything. no one spoke of that either. i also had some tachycardia for no reason and swinging b.p.

i don't understand about plamapheresis. can you explain how that would help?

and my neuro is booked and on vacation through monday and i don't have an appt til the 27th to see him and they are trying to refer me to a specialist in the group and i haven't even an appt yet for him since there has been no communication due to the vacation. i'm kinda freaking out.

great info guys, thank you. . .i have a lot of paper to get and i know nothing of pulmonology and although i was in pulmonary ICU, by day 2, the head was unimpressed with my numbers and my tests being negative so i was moved out.

right now, the breathing is up and not fluctuating on the pred. i get scared watching it go up and down on the spirometer they gave me. . .i just want to get in somewhere so that i am being followed before i run out of meds in case i crash. i don't want to have another crisis. it was VERY scary.

debra

Debra, I know how scary it is and it's not fun to be in a hospital under any circumstances. You need to find a very calm center where you can stay relaxed. It will help tremendously. It can even help to say to yourself "It's okay, I'm okay, I'm safe." Even if you don't actually "speak" those words, or others, it helps to reassure yourself.

Okay, I have a question . . . did they do the arterial blood gases while you were on oxygen? Were you on oxygen? O2 saturation is NOT always a good indicator of how your MG is doing. Even ABG's can be deceptive. If you were on oxygen and they did your ABG's while on it, like they did with me, it skews the results. It's like having your glucose checked right after eating a hot fudge sundae! The results won't be accurate.

I am very surprised they didn't do bedside MIP and MEP, in addition to total lung capacity. And those bedside readings are not as good as the pulmonary "booth" testing.

Do not do the incentive spirometer they gave you too much! You use your breathing muscles to check your lung capacity. Did they give it to you to "check" how you are doing or as something they want you to do regularly? Atelectasis (Ah-tell-ect-uh-sis), which is a small area of collapse of the lung in someone who can't take a deep breath in, is something I've had quite often and they "prescribe" the incentive spirometer in that case as something to do regularly. NO, you don't have to be all freaked out about atelectasis because it's not super serious and you weren't told you have it!!! You could drive yourself crazy by checking on your "lung stats" all day! Go by pulse and how you are feeling. I wish you had an oximeter.

If you are getting close to the end of the steroids and you think you need more, call your neuro's office and ask them if they can extend the prescription until you see him!!!!

Why doctors don't seem to know how to talk to patients in the middle of an MG crisis is beyond me. They were useless during mine too. Just because you aren't eight years old doesn't mean you don't need reassuring!!! As long as you rest, stay coolish (body temp and temper-wise) and listen to your body/MG, you'll do fine. Get as much sleep as you can, that will really help. Anything you can do to make your immune system happy like not getting stressed out or sleeping a lot or whatever will help.

You can always go back to the ER if you need to!!! Don't hesitate to do so.

Plasmapheresis, which I've never had, is similar to dialysis, which I do know about since my Dad had that. It basically cleanses your blood of antibodies. Some doctors don't like to do it because they are afraid of doing anything invasive in case it hurts the patient! Oy. What do they think an MG crisis does to us? Gently massage our muscles?

I hope you continue to get better.

Annie

surprisingly, i was never on oxygen. but the strange thing was they would have me push against their hand with a foot and then i was short of breath and panting so long that they were just like "if that is wearing you out that easily, you are in ICU"

and they did a few bedside tests for my lung functions. i don't remember the names but one had a very short piece of plastic and you blew as hard as you could and then the same but sucked in as hard as you could the next time. then there was one with a long blue tube one you blew one hard blow. they would just tell me try again or that was pretty good. . .but by the time i left i heard my numbers were great.

they did tell me to do the spirometer a few times/hour but not because i had a collapse. they said to build my lungs and to check how i was doing. . . the nurses told me to do it tem times an hour. i thought she was nuts i asked the dr. and he said a couple of times was plenty and to not wear myself out.

one of the things i don't understand is why the doc put me on pred on day 2 then stopped it for 3 days then back on one day then off for 2 then on the big taper. what was that about? my breathing was up and down all over the place. and then he comes in and asks after it has been documented by every nurse before and after each mestinon dose how weak/strong i am, if i think the mestinon is working. are you joking???? i couldn't breathe or lift my legs, uh YEAH it's working!!!!

and why do you recommend the sfemg if i have to go off meds and everything is working? isn't it just for the docs to have a "definitive" diagnosis? and isn't it 90%? what if, god help me, i don't hit that one either?

debra

Hi debra,
Plasmapheresis or more exactly plasma exchange, is the exchange of the plasma, which is one of the blood constituants.
The effectiveness of that treatment is based on the fact that most of the antibodies are present in the plasma, so if you remove the plasma, you remove the antibodies.
That exchange is done by centrifugation of the blood which separates plasma from the other constituants. Based on the individual physical caracteristics, about a gallon of plasma is replaced.
Blood is usually extracted from one elbow vein and reinjected in the other arm after passing through the centrifuge and pumping systems.
One exchange last from 75 minutes to 2 hours depending on the vein capacity to sustain high blood flow rates and on the quantity of plasma to be exchanged.
When done in crisis conditions, one exchange is performed every 2 days and is repeated 4 or 5 times.
The effects are shown rather rapidly, a couple of days but unfortunately they depend on the quantity of antibodies which are hooked somewhere in the body and since they are not circulating, they cannot be removed.
I hope this helps...
Maurice.

thank you maurice : )

great info. . .i really get it now why that would be totally helpful. i wonder why they chose not to do that why i was in the hospital. i was there a very long time. . .

my stamina in very poor. i walk across the room with my walker and have to sit down. why am i so weak?

debra

Debra,

I'm sorry you had to go through all that you did. I'm sure it was a very scary period.

I had sort of a mini crisis last November when my neuro put me in the hospital to give me a high dose of steroids by IV. When that didn't work, he ordered plasmapheresis (up to 5) and they were done every other day. I wasn't nearly as bad as you (wasn't in ICU but was having some breathing problems from time to time). I was scheduled to have my thymectomy surgery in about a month, but my neuro and surgeon moved up the surgery while I was in the hospital and after I had finished 4 of the plasmapheresis. They were of the thinking to do it now while I was somewhat better rather than sending me home and the possibility of me getting worse again before the surgery. When my neuro ordered the plasmapheresis, 2 house surgeons came up to my room to "get me ready." They ended up putting a catheter in my groin that had 2 tubes dangling from it. That wasn't pleasant. I learned later that this is not the usual way it's done. The actual treatments don't hurt, but I did find it was more of a mental thing. It was done in the dialysis unit and gave me a weird feeling knowing all my blood was leaving my body and being put back in with new plasma. I was a little tired after each treatment, but I had been in a hospital bed for over a week at that point, so was just weak overall.

It's now 5 months later and I'm doing well (knock on wood) and am just taking 30mg of Mestinon 3 times a day. Just ended a 5 month taper off Prednisone.

From reading everyone's story on NeuroTalk it seems neuros and medical institutions certainly approach each case differently.

I'm hoping each day finds you a little stronger.

Kathy

wow kathy, steroids by IV that is intense. i can only imagine how odd the blood leaving your body must feel and then returning but in a different way. it sounds like it really helped you though, right?

a 5 month taper of steroids sounds like an awful lot but i guess that is a post-surgery thing? i am now on my last dose of 20mg today and go to 10 mg for 3 days and then stop. i really hope that it is sufficient and my breathing is maintained.

i had physical therapy on saturday and some things went well but my vestibular issues went poorly and i became dizzy and nauseated and my heart started to race so we had to stop.

the mestinon is working great for strength but stamina is still very poor. i guess it will take time.

happy to hear you are doing well and thanks for your input, debra