Thursday, June 18, 2015

Happy Myasthenia Gravis Awareness Month

June is Myasthenia Gravis Awareness Month and since I’ve been mentioning it so much over the last year on this blog I wanted to take this opportunity to explain this obscure condition that needs more awareness.

I had never heard of MG before June of last year until I glanced at the results of some of my blood work and saw the words “serological Myasthenia Gravis.” When I saw my neurologist last month, he explained that he was looking for the antibodies related to autonomic dysfunction but accidentally discovered that I had the antibodies typical for MG. I imagine that some or maybe many with MG fall into the condition through happenstance. If you laughed at “fall,” then you are acquainted with this peculiar disease.

Since then, I have learned so much about this condition. MG is a rare neuromuscular autoimmune disease that causes weakness in voluntary muscle movements. It’s not as rarely diagnosed as POTS and has an established history of research and treatment. Like other autoimmune diseases, the immune system sees the body as a foreign entity with MG and begins attacking itself. It does not attack the muscles but specifically the receptors that allow muscles and the brain to communicate. Maybe you can see the problem here. Here is a great infographic that does a better job explaining this. I’m just a Humanities nerd, so the science bit is always above my head:

As mentioned above, common symptoms include droopy eyelids and weakness in muscles that affect facial movement, chewing, breathing, swallowing, talking, and limb mobility. MG was termed the “rag doll disease” because of the severity of the muscle weakness. The ocular symptoms are the most common symptom in MG, and some only experience the ocular symptoms. The level of disability and severity of symptoms can really vary person to person. Men, women, children, and even animals can get the disease, and in some cases the disease can go into remission.

Like Dysautonomia, MG can be considered an invisible illness yet the symptoms can manifest visibly. As with most complex conditions, diagnosis can be tricky. I am going on year three of working toward a diagnosis but for some it can take many years. There are blood tests that can identify the antibodies that are specific to MG, EMG tests, breathing tests, and your medical history can help your doctor determine if you have this disease. Myasthenia is usually only fatal during a “Myasthenic Crisis,” which means respiratory muscles become paralyzed and the patient needs ventilation.

About two and a half years ago, I started having difficulty walking and my breathing problem that started with POTS was getting worse. I tried to brush it off for a long time. When I saw my doctor’s NP, she also brushed it off. Yet, my mobility was rapidly declining. I had to start driving between all of my classes I was teaching and eventually, after much fighting, fretting, and cursing my fate, I started using a cane. Then a few months later, I had to get a walker and then over the last 9 months, I've had to use a wheelchair. My breathing problem has also declined rapidly.

My doctor had me start taking Mestinon, a drug commonly used to treat MG. This drug provides the acetylcholine that the immune system attacks in MG so that the brain and muscles can communicate once again.  My breathing improved immediately with this drug, and I’m dependent on it to breathe normally. From August-November, I spent almost every day in bed, but since I started the Mestinon I only have to lie in bed on bad days or if I push myself too hard. It has given me some quality of life back. Yet, this is a short acting drug and has to be taken every 8 hours. It treats the symptoms but does not treat the mechanism of MG by stopping the body from attacking itself. 

I have documented my journey with diagnosis extensively on this blog because it’s been a difficult ride. I have the antibodies but the number is small and my EMG results were mostly normal. If one the antibodies for MG, it is considered a “serological diagnosis." Some with MG are "seropositive" with the antibodies and some are "seronegative" who test negative for all of the antibodies. I also did a spirometry breathing test earlier this year and that was very abnormal. My neurologist explained that it’s still indeterminate at this time. I’m also having some problems such as intense balance issues that are not typically related to MG. Like I said, the journey to diagnosis with many complex conditions is long and bumpy.

Treatment usually includes Mestinon, immunosupressants like Prednisone, IVIG, and plasmapheresis. In my experience with Dysautonomia, the treatments and research about the condition have been extremely limited and recent. There are “common” treatments for Dysautonomia but essentially every treatment is “off-label,” meaning there are no drugs to treat it and doctors use drugs for other conditions that have beneficial side effects for Dysautonomia. There are established treatments for MG and it has a much longer history of research. Yet, these treatments for MG can come with serious side effects. My doctor explained that this is why he has been waiting to be absolutely certain before diagnosing me. I know that many have also heard this from their doctors. I have learned an important lesson about the stakes involved in diagnosis this year. 

The thymus can play a role in Myasthenia and about 15% of people require a thymectomy. The thymus can become enlarged or have tumors, either benign and rarely malignant, called "thymomas." Removal of the thymus offers the best chance of remission of symptoms compared to other treatments. I have even heard recently of doctors removing the thymus even if there aren’t any problems with it because that can be beneficial sometimes. Just a few days ago, I had a CT Scan of my thymus to see if it needs to be removed.

Yet, the fact that there is treatment provides hope, and hope is a treatment in itself.

Maybe you found this blog and this post when searching about MG, or POTS, or chronic illness, and information and knowledge is a vital step in learning about conditions you may have and all of us raising awareness about invisible and rarely diagnosed conditions. Yet, knowledge is only a piece of the puzzle.

When you get a diagnosis, arming yourself with knowledge is only half the battle. Something that I wish someone would’ve told me when this chronic illness journey began in 2011 is that acceptance is your greatest survival tool in your arsenal in living with illness. There will be self-blame, guilt, and profound disappointment, but acceptance can help you live a full life no matter what illness throws at you. Someone in the great circle of being pointed their finger and chose you as the lucky winner to bear this absurd burden. I try to remember this is not my fault. I didn’t choose this, but I accept this burden and seek to make a life bearing it with grace and dignity. These are hard-fought lessons I am continuing to learn and I think they deserve a place among the science and physical realities of any illness.

If you are out in social media, check out and participate in the hashtag #IhaveheardofMG, a movement to give MG more awareness and a face for the condition. 

I leave you with this great video from 1935 that shows the early days of the discovery of acetylcholine treatment for MG. Wait until the end when the woman begins to mop. Every time I attempt to do house work I think of the smile this woman has on her face because I couldn’t do it without that drug either. 

Also, check out the links section of the blog for more information about Myasthenia.

Thursday, June 11, 2015

Going it Alone

Sorry for the silence on the blog the last few weeks. May was a bit of a rough ride for me. I had a  “probably should go to the ER” day  almost every week during the month and then got hit with some kind of GI virus which I termed “barfing disease.” It’s taken me a few weeks to feel more like my regular broken self instead of unable to leave my bed or eat food. I’m still fighting nausea every day. 

But I’m getting back to my exercise routine. I’m on the upswing and have a good feeling about June.

Mainly what terrified me the last few weeks as I could feel the deconditioning setting in as I had to spend most of my time in bed was the fact that I had to get my strength back because I will have to go it alone for the first time in almost a year this weekend. Deconditioning is your worst enemy with POTS and it sets in so quickly and is so hard to climb out of. My husband is going on a trip this weekend to participate in the Tough Mudder in Tahoe. He is going to get electrocuted, get hit with tear gas, and jump in ice water over a 12 mile course, and he couldn’t be more excited about it. He’ll have a great time. Plus, he gets to escape the 100+ degree weather we’re having.

So I’ve been crawling my way back and working hard since this will be the first time I will be solo since I’ve become home-bound and dependent on my husband for help with basic necessities. The last time I was solo, he went to Minnesota for our friend’s wedding in September. I was still able to drive myself to a store then, and I even drove myself to Costco to pick up my medicine. I haven’t driven myself to a store since then. That is not my reality anymore.

When he left in September, I had just gone on medical leave and had every intention of returning to work in a week or two. I was still trying to make everyone, including myself, believe I was still capably independent and able to have a career—not fighting an invisible decline that was steadily chipping away at my independence. In those first few days of his trip, it started to dawn on me how dependent I had become, how much I was struggling to do basic things. I realized that I had been living a lie for a very long time. That realization came at just the right time. I wrote this post about authenticity during those few days and made a deliberate shift in my life and self-perception—finally embracing authenticity and honesty. It was the catalyst that finally helped me accept and appreciate my limitations. And I haven’t looked back. Life is so much better for it.

I have this fairy tale image of how it’s going to go this weekend:

I’ll get our house clean while listening to a lot of jazz, play my keyboard, read, make myself meals, exercise every day, watch all four hours of Kenneth Branagh’s Hamlet and other nerdy things husband isn’t interested in, do some painting and writing, my friends will come over and we’ll drink wine, sing, play games…..

A fairy tale indeed. 

I’ll try to do some of those things but I’ll also have to be very careful not to max out all of my spoons so I can keep up with basic necessities. I can't do anything crazy like trying to drive or try to shower in the morning or get over ambitious with my exercise goals. I will take it one moment at a time, try not push myself too hard so I don't end up bedridden again and try not to panic as I wake up in the middle of the night not breathing. Slow and steady. My goal is to successfully make it through.

I want to say I was able to do it.

I am keenly aware that there are many Spoonies out there who regularly go it alone or who are single and do this alone every day. I applaud your tenacity and courage. It is no easy feat because there is very little room for error when you have to do this without help. You are true warriors. Living with chronic illness is a bit like caring for a body that is an impetuous toddler: your will is subjugated to the whims or tantrums the body will unleash at any moment. Any sense of control over your life is a thin veneer you feign to conceal what is truly fathomless chaos. Yet, we keep going and keep fighting. In the words of Elizabeth Taylor...

So all of you out there going it alone or who have access to invaluable help, you got this. We got this. Wishing you all the best