Below is the doctor's notes from my first neuro visit for MG May of 2012.  

May of 2012 double vision started.  The eye doctor I visited for the problem did the icepack test and told me he was 80% sure I had myasthenia gravis, ordered a blood test and referred me to a neurologist (this is all at Mayo Clinic-Rochester) where I worked.  I couldn't get in to see a staff neurologist for several weeks, so went to the desk and said that wasn't acceptable to wait when I was already having difficulty seeing, chewing, swallowing etc.  So I was scheduled with a resident--a 3rd year one almost ready to be a full fledged staff member. He spent 90 minutes with me (this was after the blood test result was positive and thymus imaging negative).  All of my medical records are online at Mayo and available for me to read.   I removed the MD names, and am putting it out here as an example of a plan for treatment (that I followed).  The biggest problem with the plan was not enough mestinon at first and I spent a few days in the hospital about a week after this meeting with breathing problems that went away when I took 60 mg every 2 hours.  I also started on prednisone before I was in the hospital, but at only 20mg/day and I needed much more to get MG under control.  Although my first neuro appt I mostly was complaining about the head (eye, chewing, talking....) problems, I had breathing problems but hadn't recognized them yet.   My comments in ()

IMPRESSION/REPORT/PLAN

#1 Seropositive generalized myasthenia gravis with primarily oculo-bulbar features (clear diagnosis so no question about MG itself, just the treatment plan

#2 Hypothyroidism  (already had for 10 years and being treated)

Mr. Hanson has mild generalized myasthenia gravis primarily with oculo-bulbar symptoms and antibodies against the acetylcholine receptor with no evidence of enlarged thymus on CT of the chest.

(thymus imaging is always done early to see if cancerous or problem.  He says "generalized" meaning more than just the eyes or head. He tested my hands and arms pulling against his and noted that mine, although beginning very strong, quickly gave way to his pull).   

There is nothing by history or examination that suggests a paraneoplastic etiology including Lambert Eaton myasthenic syndrome or an adult-onset myopathy or mitochondrial disease. In any case, he has had serological testing that is diagnostic for myasthenia gravis.

 (MG can be a side effect of cancer so doctor has to worry about that too in Lambert Eaton type  the myopathy and mitochondrial diseases are conditions that have similar symptoms to MG and so are ruled out).  

We spent a long time discussing treatment. Initial symptomatic treatment will be with a cholinesterase inhibitor such as pyridostigmine (mestinon). I recommend pyridostigmine 60 mg every six hours during the day.

(He also did a lot of neuro testing and strength, eyes, etc. and then spent an hour telling me about MG, the various treatments, and encouraging me to believe I would have a few months of difficulty followed by a near normal life but with ongoing meds for the rest of it). 

We discussed its cholinergic side effects including increased secretions, abdominal cramps, and diarrhea. These can be counteracted by adjusting the dose of pyridostigmine or with the addition of a low dose of glycopyrrolate depending on how he responds to pyridostigmine.

(mestinon has some harsh side effects, but works.  I didn't take glycopyrrolate for digestive distress side effect, but did take loperimide (imodium))

We also discussed the potential initiation of a corticosteroid. If he requires additional treatment, we would start prednisone at a low dose, such as 10 mg daily, and increase gradually up to 60 mg daily if needed. When he reaches a stable dose which controls his symptoms, this can be gradually tapered over a period of several months to an effective and tolerable daily dose or every-other-day dose.

 (Sometimes the first doses of high level prednisone makes MG worse so cautious doctors start slow.   I saw a staff neuro about a week later who did two things -- put me in the hospital and initiated prednisone at 20mg/day -- I walked across the waiting room back to the doctor's office and had so much trouble breathing that he figured I needed to be hospitalized.  I just needed more than 2 mestinon per day and the understanding that I could take them as needed rather than on a rigid schedule.  I took 20mg pred for a couple of months without anything improving noticeably.  I wanted to get better fast -- wife Margo was beginning cancer treatment--so I pushed the neuro and got to 30, 40 50 and finally 60 at about 4 months and then MG symptoms were gone--so back to 40 and 30 for many more months until Margo was through treatment and I could back off support).  

Complications of corticosteroid treatment include opportunistic infection, gastric issues, and abnormalities of bone mineralization, and these would be counteracted with additional medications including a proton pump inhibitor, trimethoprim-sulfamethoxazole, and calcium and vitamin D supplementation.  

 (I got osteoporosis, almost moved into type 2 diabetes and got cataracts from the prednisone at 60 mg/day but it got MG under control in a few months and was able to drop the amount to where things were less of a problem.  I visited endocrinology about the osteoporosis when it looked like I would be on prednisone long term.  I got fosamax, vitamin D, calcium and a test for hypogonadism -- another cause of osteoporsis.  I was found to have primary hypogonadism -- a disfunction of the testicles, and so put on relatively low dose testosterone gel at the same time I was beginning to tape prednisone.  That I went into remission, in my mind was associated with onset testosterone.  My neuro and endocrinologist did not think that would get rid of MG, but also were somewhat unsure as MG generally hits men as they age.  I remain on testosterone at enough to keep me in the lower range of normal -- and it has been good for muscle rebuilding, positive attitude and ambition and motivation.)

A potential complication of corticosteroid therapy in myasthenic patients is exacerbation of weakness. For this reason, it is important to titrate prednisone slowly and ensure close clinical follow-up. We will hold off on starting corticosteroids until we have had a chance to evaluate him after the initiation of pyridostigmine.  (the usual is start with mestinon and see what it does before the immune suppression.  A few folks never need more than mestinon but most of us do). 

It is possible in the future that he may require a steroid-sparing agent such as azathioprine, cyclosporine, or mycophenolatemofetil. These will have to be anticipated given that their onset of action is often delayed by several months. I recommend obtaining a hiopurinemethyltransferase activity level in anticipation of starting azathioprine at some point in the future if he tolerates corticosteroid therapy poorly or is unable to achieve remission with a tolerable dose of prednisone.

(the choice of Imuran, Cellcept, Prednisone were presented and IVIG and PLEX too.  The resident and his boss neuro were not the experts on MG at Mayo, so although they got me started on the meds, I was switched to an MG expert by the end of the first month who again spent about 90 minutes with me and my family preparing us for dealing with MG--and she was very positive.  Most of the folks we see here in neurology (i.e. parkinsons, MS, and other degenerative disease) we have only a delaying treatment plan.  With MG, we usually can roll it back so, although you have to take meds the rest of your life, you will be able to live normally.  She started me on prednisone about 3 weeks after diagnosis).  

In the meantime, he will be seen back in the clinic in a week, and we have set him  up to see Dr. xxx   in our department for follow-up thereafter for myasthenia gravis. I have discussed this case in detail with Dr. yyyy, who agrees with the plan. (the resident talked to his boss (mentor) who stopped in too, and told me he would see me until he got me in to an MG staff expert).   

PATIENT EDUCATION

Ready to learn. No apparent learning barriers were identified. Learning preferences include listening. 

Explained diagnosis and treatment plan; patient expressed understanding of the content.

(I managed to get a working relationship with the MG neuro's primary staff person (a nurse) who I could call and talk to and who then passed info back and forth with the neuro between appts.  I also convinced the neuro (a mid 50s woman) to try managing my progress via email.  Mayo was and is in the process of trying to handle access through a messaging system that allows the doctor to read and respond to emails in any slack time they have.  I tried to be concise with any questions I had, and often we agreed on a change of plans via email.  Almost always I got a response the same day from the neuro -- although sometimes it was in the evening when she likely was at home and should have been forgetting about her job.   In the year that I was under treatment, I did only 3 onsite visits with my neuro after the initial ones that got me underway with treatment.  It was almost always about changing meds with me pushing to take more prednisone to get MG controlled and her worrying about it pushing me into diabetes.  The compromise was I ate a diabetic diet that kept my blood sugar under 125.   The final discussion was about the tapering rate, me wanting to go faster than she liked.  We compromised by switching first from daily prednisone to alternate day, and then some weeks later to every third day and eventually every 4th day and every 5th day gradually dropping until 0 when we realized I was in remission.  That was quite rare for her -- 15%-- and great for me.  I attribute my success to a great institution -- Mayo Clinic, my own persistence in finding out everything I could about the disease myself, and figuring out an efficient way of communicating with the experts who could adjust things as well as answer my concerns).

I also used dailystrength to find out more from those who had followed this path before me, concentrating on those who had successful outcomes. 

Russ