“I can be changed by what happens to me.
But I refuse to be reduced by it.”
Maya Angelou
Aristotle Onassis was a Greek/Argentinian shipping magnate, one of the world’s richest and well-known men, well known as the husband of Jackie Kennedy Onassis. In 1975, at the age of 69,he died, with bronchial pneumonia said to be the cause.
In fact, he had suffered complications from myasthenia gravis, a disease he struggled with in his last years. All his wealth and power could not get him the right medical intervention to save his life from this little-known neuromuscular disease.
I was 29 when I learned of Onassis’ death and first heard the words “myasthenia gravis.” Little did I suspect that 45 years later, those words would become a large part of my vocabulary…
In March 2020 I was working as a psychotherapist in my Pennsylvania office. The world was becoming aware that people were getting ill with a virus. On Monday, March 9, I left flyers in my waiting room for each of my clients to consider prior to our session:
TO MY CLIENTS…
IF YOU
OR AN IMMEDIATE FAMILY MEMBER
IS SICK WITH FLU-LIKE SYMPTOMS,
OR IF YOU HAVE BEEN EXPOSED TO THE FLU,
PLEASE LET’S RESCHEDULE OUR APPOINTMENT
FOR WHEN YOU / THEY
ARE HEALTHY AGAIN.
As you probably realize, this is an effort to
reduce the transmission of the coronavirus.
THANK YOU FOR YOUR UNDERSTANDING,
BARBARA
None of my clients cancelled. Covid-19 was upon us.
When I looked across the room during sessions that day, I became aware that I was seeing double images, which struck me as unusual but (inexplicably) not especially alarming.
By Monday, March 16, most therapy offices – mine included – had discontinued in-person sessions, which soon were replaced with virtual “telehealth” sessions.
And I had begun to experience more frequent, unsettling double vision, and to have great difficulty keeping my eyes open. That was soon followed by very uncomfortable episodes when food and drink, even water, got stuck when I ate or drank. Then my head began to hang down on my chest – my neck was too weak to hold it up! What was happening?!
In a virtual visit, my ophthalmologist had me do an “ice test”: I put a bag of frozen vegetables on my eyes for a minute. I was surprised when both the double vision and the involuntary eye-closing quickly (though temporarily) remitted. The doctor told me it was likely myasthenia gravis – those words I had first heard 45 years earlier. That felt ominous. She referred me to a local neurologist.
The neurologist told me–emphatically, arrogantly, dismissively– “You do not have myasthenia gravis!” A blood test quickly proved him wrong. I started on Mestinon, a standard oral MG medication.
So at the exact time that the world began to deal with Covid and its deadly outcomes, I was reeling with my own personal crisis.
Myasthenia Gravis?! What did that mean for me, for my future?? I was scared, too scared at the time to want to learn any more about MG.
I began seeing another neurologist in the practice who readily accepted the diagnosis, but she had no experience treating MG. Then I transferred to a neuromuscular physician at the University of Pennsylvania who was knowledgeable about MG. She prescribed the steroid Prednisone, an immunosuppressant. She also started me on Imuran, a non-steroidal immunosuppressant, which sent me to the hospital with pancreatitis.
So began my MG saga…
MG is an “autoimmune disease.” The first known use of those words was in 1952. An autoimmune disease is a condition where the immune system mistakenly targets and attacks healthy, functioning parts of the body as if they are foreign organisms. There are an estimated 80 or more autoimmune diseases. They all are chronic – once there, always there.
Unlike other autoimmune diseases which progress somewhat predictably, each MG patient’s trajectory is unique – just as every snowflake falling from the sky is unique. So MG came to be known as the “Snowflake Disease.”
It wasn’t long before I overcame my shock at the MG diagnosis that, at first, I didn’t want to face. It took me a long time to educate myself about the disease. These days I want to understand the condition that has had such a major impact on my life. I have read about it and attended virtual MG meetings. I’ve joined MG support groups on Facebook, not for medical advice but to learn through others’ experiences. Now I log on to these sites to ask questions, offer support, encourage others and share whatever knowledge I may have.
Still, in the last five years, I have met only one other MG patient in person. (He had ocular MG.)
Some medications and procedures are contraindicated for MG patients: magnesium (a muscle relaxer) and quinine (used to alleviate leg cramps) can exacerbate muscle weakness. Any anesthesia must be carefully monitored for safety. Some antibiotics should be used cautiously, if at all. Botox is not allowed. NSAID medications need to be avoided. I often feel like I’m dodging rain drops in a storm.
My current MG medications and treatments are Prednisone, bi-weekly Soliris infusions (at an infusion center), IVIG infusions three days every six weeks (at home with a nurse), and Mestinon.
Without medical coverage, the cost of the Soliris infusions is – wait for it – over $600,000 a year! I am fortunate to have insurance that covers most of that astronomical amount. That is not the case for every MG patient.
I began taking Prednisone in May 2020. I soon termed it The Evil Wonder Drug. Within a month it caused the ocular symptoms to disappear, and my neck muscles became strong enough so I could hold my head up. I was relieved!
But then… after a few months…
- I developed the classic steroid “moon face”
- I lost my eyelashes and most of my eyebrows
- My eyes and nose continually dripped
- Large bags appeared under my eyes
- The skin on my arms became paper thin and crepey
- The slightest bump resulted in dark purple bruises
- My hair was thinner, changed in texture and barely grew
- I suddenly developed severe cataracts and could not read without a magnifying glass
- My glucose levels spiked
- My mood was affected
I became very concerned about the drug’s potential to cause my bones and teeth to become brittle. And I was unnerved when I looked in the mirror and an unrecognizable stranger’s face peered back at me. I seemed to have aged years in just a few short months.
Under the guidance of my neurologist, I have tried several times to taper down on Prednisone – very, very slowly. When I’ve gone “too low,” I have ended up in the hospital with an “MG flare” where – to my dismay – the Prednisone dosage needed to be increased in order to control the symptoms. I don’t want to live with Prednisone, but I cannot live without it.
Although dealing with MG has been difficult in many ways, the only time it brought me to tears was in early April 2020, before Prednisone with its healing powers. When I attempted to use the microwave oven above my stove, my weak neck muscles made it impossible for me to lift my head to see the controls. I felt frustrated, disturbed, helpless. I quickly bought a counter-top microwave. Today I use that exclusively, even though I have a perfectly good above-the-stove microwave and I am capable of raising my head.
Over the course of living with MG there have been a number of times when weakness of my tongue muscles led to lisping. In July 2021 I had been lisping for a couple of days, but suddenly I discovered that I had absolutely no voice, could not utter a sound!
Fortunately, I was at my friend Susan’s house. So while she spoke with my neurologist, I wrote my questions and comments for her. He told me I needed to get to a hospital ASAP, that the situation could deteriorate quickly: my muscle weakness had already progressed from my tongue to my vocal cords, which potentially could extend to my diaphragm, inhibiting my ability to breathe.
In the hospital I was given infusions for a week and transferred to an inpatient rehab facility for two weeks. The experience was quite disconcerting.
The legendary professional tennis player Monica Seles recently announced that she had been diagnosed with MG in 2022. Having a public figure as the face of the disease may hopefully increase understanding about – and funding for – MG.
MG has left me feeling vulnerable, appreciably and prematurely older in function as well as in appearance. The disease is unpredictable – I never know when or what weakness will strike. One day I may be unable to raise an arm or extend my fingers; another day it’s hard to walk or talk or lie down; double vision has recurred; I can’t swallow food or water; my balance is off; I get winded easily; my hands become too weak to open a jar. I fell from a chair due to a loose seat cushion, and I was too weak to get myself up off the floor. At times I have trouble breathing. Heat intensifies MG symptoms. It is challenging.
MG may not be the most debilitating disease, but it certainly is among the weirdest.
In nature, each individual snowflake is exquisitely fragile. But just look at what those snowflakes can do when they stick together. For us Snowflakes too, we can find strength in numbers…