Limbo over, here we go!

So in March there was the Limbo post. The ugh, doctors are taking me a little more serious than I'd like post. I kinda thought they were all full of it. My eye doc suggested I see a neuro-opthamologist. He also thought Myasthenia Gravis was the cause of my eye drooping. He was also really rude, which personally makes me take anything he said less seriously. Which is nonsense, a lot of really good docs have horrible bedside manner. I just didn't want to hear it.

So things progressed. More issues. More weakness. Less ability to get through the day. So in August I tried a new med (see Swallow post). It was weird. It helped. But only briefly. Pyridostigm kicks in fast, about 15min. Then it lasts (for me) less than 4 hours. I can take it every 4hrs up to 3x/day (per my script, some people take more). The first time I took it, my boyfriend was at the house- always good to have another person present when trying a new med! His reaction was "you have facial expressions!" I was then able to take a shower without effort and wash my hair without a break. That was a BIG deal. Then we cleaned the fridge! And then I crashed. Pyridostigm helps, but it gives me a false sense of what I can accomplish, so it makes my day VERY roller-coastery. And I had upcoming tests that I had to be off of it as to not skew results (EMGs). So I decided to only take it if I had a day I REALLY had to get through. It has been a good back up. However when I take it regularly, the lows are a bit too low. When I do take it, it is hard to shake the I must get EVERYTHING done that I have not been able to get to in the next 4 hours feeling. So I decided to maintain a more even do what I can without the meds until we had more information.

Tests were coming back within normal range. There are 2 antibody tests for MG. Mine were both negative. My EMG/repetitive NCV was normal. The size of my thymus is in normal range but tissue still present (it shrinks as you age, often disappearing). Then yesterday was my Single Fiber EMG. It is a more specific EMG, where the doctor literally has the specificity to get a needle into the microscopic gap between a nerve and a muscle. This neuromuscular gap is where the problem arrises in MG so SF-EMG is often seen as gold standard for diagnosis. However, as MG is a big pain in the ass, there are people with an MG diagnosis that have a normal test result. I wasn't expecting much, I tend to be that rare person that "tests well" and nothing shows up despite my body being in revolt. So when the test was over, and the neurologist showed me a graph- saying that normal function would be to the right of this number, and he had another paper over the left side of the graph… I had about 2 data points right of that number… then he moved the paper… ALL my other points- at least 15 were to the right of the "normal" line… and just like that I had a diagnosis. He said my results confirm Myasthenia Gravis.

It was weird. There was actually a big weight lifted. No more tests. No more doctors looking at me like I was faking it. You can't fake a SF-EMG!! PROOF! Proof to that voice in my head that I wasn't just being lazy- that I needed to suck it up and just get shit done. I wouldn't call it happy, but there was a definite feeling of relief.

And then back to, FUCK. This is a real diagnosis. Ok, let's focus on the fact that it is usually really treatable. There are a few treatment options. I had already set up an appointment with a specialist in a neighboring town who comes highly recommended by MG people in my area and also does MDA (MG falls under muscular dystrophy association criteria) clinics. So I thought, I'd either be seeing him for a second opinion if SF-EMG was negative, or treatment options if positive. Treatment options it is. However, there is a lingering little fear voice in the back of my head. As the neuro-opthamologist pointed out to me, the G in MG stands for "gravis" as in grave as in deadly (I was having issues looking up the way he wanted, because I literally couldn't do it- he thought I was just not taking him seriously). With today's treatment options, MG is usually well controlled. USUALLY.  or a hospital stay on a ventilator. or death.

MG is an neuromuscular autoimmune disease.  Nerves send out acetylcholine, the muscles pick it up on their receptors and then contract. My immune system has decided to attack the receptors on my muscles that receive acetylcholine. So the nerves send an impulse, and the acetylcholine just sits there with no receptors to pick it up. This is why Pyridostigm works- it stops the body's enzyme from cleaning up the leftover acetylcholine, so it can hang out in the neuromuscular junction longer to get picked up. They aren't really sure why the body wages war on the muscle receptors. It is generally believed that the body was fighting something else, and then in a friendly fire type way got confused and blew out its own receptors. MG can effect any voluntary muscle. This results in weird symptoms. Difficulty holding up my head. Eyelids not working. Limbs weak. The bigger problems arise when swallowing is effected and you choke on your own spit or food. I usually only have this happen if I am laying flat and my head goes back a little, I tend to sleep (even with my cervical collar on) with my chin tucked towards my sternum as it makes swallowing a lot easier (which yesterday's neuro said they often teach people with MG as a strategy!). Most of the time if I can't swallow, it happens when I have been eating towards the end of a meal. I just have food sitting in my mouth and there is no swallow reflex. So I spit it out. The other thing that can happen, is when the muscles of respiration are involved. Previously this is how a lot of people have died of MG. They call it a Myasthenic Crisis. Nowadays, as long as the ER doesn't treat you like a crazy person (which happens!) they can get you on a ventilator and get the MG to calm back down to a normal level. From talking to (and reading about) other myasthenics, the hospital stay is usually a minimum of 3 weeks.

So there is a part of me that is still in AGH! mode. Big mixed bag of emotions at the moment. Limbo is over. Neuro next week for treatment options. 

http://www.myasthenia.org