It’s June 1st. Today would have been my dad’s birthday. It is also Myasthenia Gravis awareness month. Do you know what Myasthenia Gravis is? I feel compelled to share my story in hopes of helping just one other person muddle through this disease, make one person aware, help explain to others, or just to give you, my fine readers, something to read when you might be otherwise bored.
“You Have Cancer.”
Scary words. I’ve heard those words. Sitting on the side of a gurney in a brightly lit ER clad in an open backed hospital gown on a cold Friday night in January I heard those words. Next to me, the one person who I know will always be by my side, my husband. My Super Husband. In his arms was our first-born. Our Son. Our Hot Rod. A wee little guy was he, not comprehending the words, which poured forth from the doctor’s mouth. I looked at him - at my Hot Rod, and then at my Super Husband, then at the doctor.
He wouldn’t make eye contact with me. Facts. Facts were what he wanted to speak about so he could leave the curtained area as soon as possible. Wow, I came in because my chest hurt. Because my doctor’s office said if there were chest pains, they would send one to the hospital, as they were not equipped to do EKGs. I had an EKG, blood pressure was read, pulse taken, pulse ox recorded, blood drawn, and CT Scans performed. I was tired. Tired of sitting in this hospital, nothing was wrong with me. I was twenty-five years old, and even the triage nurse told me he felt nothing was wrong with me. Turns out we were both wrong, but so was the doctor, so were many doctors.
At the time, I had never heard of Myasthenia Gravis. I didn’t realize that what this ER doctor, the radiologist and come Monday morning the oncologist, thought was cancer may have just been the onset of Myasthenia Gravis for me. As I sat listening to the doctor in the hospital, explain that my “Arterial Blood Gas” was low, only in the upper 80’s, that prompted them to do a CT scan of my chest looking for a blood clot, and instead they found a mass I kept peeking over at my guys. “You have lymphoma.” Those were his next words, and those words did hit home. My dad had died of lymphoma just a couple years prior to this, and I told the doctor so. “I have spoken to an oncologist and Monday morning you’re to see him.” Said this doctor, after glancing up at me then back at his shield, the folder which he held in his hand containing the test results.
Monday I did see the oncologist. I underwent biopsies. I visited other doctors and surgeons. I became frustrated and confused and eventually, I stopped going to doctors. The oncologist told me, when the biopsy results were negative, “It’s like drilling for oil in Texas, either there’s no oil there or they hit the wrong spot.” Yet the surgeon declared, “You just have reflux.” Those four words were the decisive factors, what made me decide to stop seeing doctors. Reflux. If only he had been right.
That mass which was found, the ‘cancer’, turned out to be Thymic Hyperplasia. If I had had my thymus removed that January there is a chance that, while I may still have Myasthenia Gravis, I might not be as badly off as I currently am. Could’ve. Would’ve. Should’ve. That’s not the case. It was not removed and now I know why that surgeon was happy to declare I just had reflux and no surgery was needed. Turns out, removing one’s thymus is a pretty big deal, normally performed by a cardio-thoracic surgeon and he was a general surgeon with, in my opinion, a mighty big ego.
Thymic hyperplasia and thymomas (cancer of the thymus) are common in M.G. and some research suggests removing the thymus, soon after diagnosis, may result in remission for some. That’s the hope - remission.
Fast forward several more years. At this point, we’ve had a new addition to our happy clan. A princess has been born and our family is complete. We’re happy, that hasn’t changed. Then something happened, something new. Who knew getting a pacifier into a crying princess’s mouth could be so hard? ‘What is going on’ I thought? Scared. My arms won’t work, neither would these bumbling fingers. Shaking, uncoordinated, bumbling, that was me. I was also three hours from home. I saw another doctor, I had to, I thought
I regained my coordination; I was able to finally put that pacifier in her mouth. I just rested a bit before I could. Rest. Another clue. More clues would come, frustration would set in, I would declare my mistrust of the medical profession more than once, and a few years would pass. And so, my new beginning would start. The beginning of living with Myasthenia Gravis would have to start with the diagnosis.
I went to my family doctor after that scary episode with not being able to put the pacifier in Princess’s mouth. I told him of the difficulty I had with moving my leg, my right leg and arm were more affected than my left and they still are. I told him of the electric shock sensation I had from my back down to my leg. The MRI was clean. The neurologist I saw at the time said he saw nothing wrong and didn’t need to see me back - after insurance denied his request for an Evoked Potentials test. Nevertheless, something is wrong again, and off to my family doctor I went.
My eye. That’s the problem now. This is another clue in this curious game of Diagnose Just Me…Crystal D. I’m pretty sure my family doctor felt there was nothing wrong with me at this point too. That it was all in my head, but darn it my eye has a lump on it, it’s unsightly, and it’s droopy. He sends me to the first medical person I later thanked, he sent me to an ophthalmologist. The lump, it’s common. The drooping, it’s not. After his test - look up, look down, and follow my light, look ahead, blah, blah, blah - my eye is drooping a little more. “I think you have a neurological issue causing the Ptosis.” Ah, that’s the fancy medical word for eye droop. He sends a message to my family doctor stating this, and my next referral was to the second doctor I would later thank, my neurologist.
Admittedly, I was very nervous about seeing another neurologist after the first one I saw all those years ago. The way he dismissed me, made me feel as though I were crazy and my symptoms were all in my head, it was very disheartening. That's another thing with M.G., we can look fine on the outside yet be turning to mush on the inside. My neurologist, however, was nothing like the first one. That first visit, in February, he knew what was wrong with me. Said he suspected I had something called Myasthenia Gravis but wanted to do some tests to be sure. This is another problem, the tests. There are blood tests for Generalized Myasthenia Gravis - an Acetylcholine Receptor antibody test (AChR) and a Muscle Specific Kinase test (MuSK). The problem? The AChR is only positive in 80% of myasthenics and the MuSK is only positive in a fraction of the 20% the AChR misses. That still leaves many with Generalized Myasthenia Gravis with false negatives - we are classified as Sero-Negative or Double Sero-Negative.
Diagnosis of M.G. can be difficult if one or both of the blood tests are negative. I only had the first test, it was negative. I wasn’t surprised. My Super Hubby and I joke that: “If it’s rare, incurable, or hard to diagnose I’ll get it!” My neurologist, he wasn’t surprised by the test, but he’s not what I feel is the typical neurologist either. You know the kind who ride in the exam room upon their high horse, never look you in the eye, can never be wrong and know everything. Again, he stated he felt I had M.G., and asked if I had ever heard of it. I didn’t realize at the first visit that I saw that name before. It wasn’t until I went home after that first visit and typed into my favorite search engine the words “Myasthenia Gravis” that this had come up before - when the mass was found in my chest. That was the first clue, my neurologist’s face lit up when I told him “Yes, when they found I had thymic hyperplasia I saw that before.” This was one more symptom to clinch his preliminary diagnosis.
Off with their heads!! Or rather…Out with that defunct thymus! I was officially diagnosed with Myasthenia Gravis March 23, 2011 and May 2, 2011 I was at a large university hospital having my thymus removed by a cardio-thoracic surgeon with my Super Husband by my side, my Amazing In-Laws here with our Hot Rod and Princess. I was in the hospital for 3 days, in the smallest ICU room I think has ever been made, with tubes coming from places I hope no one shoves a tube again, but I was not on a vent. Thank God, I haven’t had to be since then.
That was the road to diagnosis and while there were many times I cried after being made feel this was all in my head, I was a few card short of a full deck, or crazy it turns out I was just the Fruit Loop in a bowl of Cheerios the zebra among the horses and it took a few good doctors to listen, to look at me and to dig down in those medical books and recall those words few have heard since graduating medical school…Myasthenia Gravis.
Now, just because those words are written in my medical files it didn’t make this disease go away. I’m not sure if I’ll ever be in remission and my new normal leaves a lot to be desired. Gone, it seems, are the days I could just hop up out of bed, shower quickly, blow dry my hair, dress myself, brush my teeth, run upstairs and grab one kid and then the next and get them ready all in under an hour. Also gone is running about outside, chasing kids, seeing one of anything, not having to worry about being unable to swallow, drooling, slurring (or as I like to say speaking in cursive), head drooping, leg dragging, and the list goes on.
Now my life still revolves around our family, that hasn’t changed. But my life also revolves around medicine, doctors, hospitals, nurses, and paying attention to how I feel. I start my day with a handful of medicine, I end my night with another handful, and during the day, there are more pills. I receive plasmapheresis, for five days every other day then I get four weeks off and we start again. Previously I was receiving IVIg (Intravenous Immunoglobulin) everyday for 5 days and repeat in one month. Both of these treatments require donors, like my Super Hubby and hopefully you, to donate plasma or blood.
Plasmapheresis is treatment where one lucky contestant, such as myself, is sent to the hospital, a double lumen catheter (or as I lovingly have dubbed him - Frankie - short for Frankenport) shoved into my jugular, wheeled from there to dialysis, and the process begins. First, suck the heparin (medicine that keeps one from getting a nasty blood clot) from Frankie, next flush (squirt some saline into) Frankie, *cue nasty taste in mouth*, then attach a water hose…I mean medical tubing onto each side of the catheter, nurse puts in a bunch of numbers in the squeaky machine, said nurse hangs some bottles of albumin (a protein found in ones’ blood) then later on a bag of what looks like orange juice (this is the plasma), turns machine on and we’re off and running like a heard of turtles! That’s about as fast as I run any more also.
During this process your blood is removed from one side (lumen) of your Frankenport, or if you have veins of steel through vein in one arm, the plasma from your blood is removed by a centrifuge in the machine, and your blood along with some donors’ plasma is returned to you through the other lumen or vein of steel in the opposite arm. A little over 2 hours later, I’ve officially broken out in hives, received extra Benedryl, probably some antinausea medicine, and we’re just finishing up and I’m sent on my way to rest for the remainder of the day and sleep off the Benedryl. After about the 3rd treatment I start to feel a little better. I won’t be running any marathons. My idea of a 50 yard dash is still making it to the bathroom in time, but I can normally start to lift my leg a little more, my eyes aren’t as droopy and by the end of the 5th treatment I can eat without having to ask myself “To choke or not to choke, that is the question!” Plasmapheresis is used for Myasthenics in respiratory distress, for refractory cases *ahem*, as well as for other diseases.
IVIg is another process whereby blood is needed from a very generous donor. Okay, lots of donors. The thoughts are to overload one with immunoglobulin and your body stops attacking itself for a short time. For some this works, for others it doesn’t, and for some insurance is what stands in the way.
All of these treatments are expensive, and I’m grateful we have insurance and my husband has a good job with understanding bosses and co-workers. I’m grateful for my family and friends and their thoughts and prayers. I write this to inform, to help if possible. Knowledge is power, but the problem is that Myasthenia Gravis is a silent disease, which many have never heard of. During medical school this isn’t a condition touched on very often, one is lucky to have heard of it let alone actually studied it during medical school.
So, here are some facts about Myasthenia Gravis:
* Myasthenia Gravis by definition is Grave Muscle Weakness.
* Myasthenia Gravis is a rare neuromuscular disease.
* Known as “The Snowflake Disease” because no two myasthenics are the same, and symptoms vary from one person to the next.
* Only 80% of Myasthenics are have the AChR antibody leaving 20% undiagnosed or termed as Sero-Negative.
* Of the remaining 20%, when a MuSK test is performed it is only able to diagnose 40-70% leaving many undiagnosed, or termed Double Sero-Negative.
* Unknown antibodies are the cause for patients who are Double Sero-Negative.
* Myasthenia Gravis can affect only one’s eye and is known as Ocular Myasthenia Gravis or OMG.
* Myasthenia Gravis can affect all voluntary muscles - meaning if you can control it, it can be affected. Can you hold your breath? Open your eyes nice and wide? Hold your head upright? Swallow? Move your leg? Wow, we have a talented bunch here! Sometimes I can’t. Why? MG.
*Rest helps, a lot! If you don’t move it, you’ll lose it some people say. For Myasthenics, that’s not the case. If we move it too much we’ll lose it! Moreover, it’s oftentimes during the worst possible times. Busy day with your kids…you have errands to run, groceries to buy, and soccer is tonight at 6! Run, run, run…crash! Didn’t make it to that soccer game after all because you tried to be a normal mom. Now you can’t breathe, and you’re contemplating a hospital trip and it goes like this: Should I go to the ER? I can breathe, not very well but I can breathe. I can’t do that to the kids they’ll be scared. Oh goodness what if it gets worse?! I think I can, come on and breathe darn it! Who knew breathing could be so hard? Hey Super Hubby, if things don’t get better or they get worse, think you’d mind taking me to the ER? Yep, I took all of that ‘breathing’ nonsense for granted for years!
Now for some common symptoms (and my commentary of course in these parentheses I love so much!):
* Breathing problems - which can result in respiratory distress requiring intubation and hospitalization. This is the scariest of all symptoms for me.
* Ptosis (This is just a fancy medical word for eye drooping.)
* Swallowing Difficulties (I just love it when you I have to decide what’s for dinner based on ‘can I swallow that’ instead of ‘hmm, that looks good’)
* Choking (I have but one word…Spree! Those were nearly the death of me!)
* Slurring (I love it when people think I’m drunk because I can’t form words properly, she typed with sarcasm dripping from her fingertips.)
* Unsteady Gait (And yes, I have always wanted to be viewed as an alcoholic as I walked through Walmart in hopes of getting on that fancy website PeopleofWalmart.com)
* Neck Weakness (sometimes requires a cervical collar to help hold one’s head up - who knew your head could gain a hundred pounds in one day!)
* Facial Weakness (results in a grimace instead of that large smile you’re use to and everyone around you thinks you‘re upset because you can no longer smile…*sigh*)
* Double Vision (Why yes officer I did see that car and the one on top of it, I just wasn’t sure which was the ‘real’ one. I didn’t mean to play bumper cars on the highway…and this, folks is why I haven’t driven in about a year now.)
* Limb Weakness (Never before did I realize how many muscles you use to wash your hair! Showering is a process through which I become exhausted. Today you must choose, shave your legs, shave your underarms or wash your hair…which did you choose? I also am an owner of a rollator, but the kidlets like being pushed around on it when I do not have to be. Next up, I think, is a wheelchair.)
If you have these symptoms and have been made to feel as if your symptoms are ‘in your head‘ and you’re crazy by your doctor, it’s time to move on and find a better doctor. There are good doctors our there, though at times they seem as elusive as flying monkeys, yellow brick roads, lollipop kids and rainbows. Sometimes we have to search for them, but they do exist. I won’t be skipping down the yellow brick road any time soon, but maybe one day, or maybe I’ll find a little pot of gold at the end of a rainbow.
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