By Amy Burtness, as told to Hallie Levine
I was diagnosed with myasthenia gravis in July 2019. In hindsight, I’d experienced symptoms for several years already. The main one was droopy eyelids that made it difficult to keep my eyes open and eventually led to double, blurry vision. I underwent three surgeries on the muscle in my upper eyelid. Each time, the operation would help for a few months, then the eye droop would begin again. I’m a systems analyst who spends her days on computer screens. There was no way those symptoms could continue if I wanted to keep my job.
Finally, my primary care doctor referred me to a major medical center where the specialist suspected a neuromuscular problem. She referred me to a neuro-ophthalmologist, who diagnosed me with myasthenia gravis.
Cycling Through Treatments
My doctor put me on pyridostigmine, which is one of the first treatments doctors try for myasthenia gravis. It’s a type of medication known as a cholinesterase inhibitor, which slows the breakdown of a chemical called acetylcholine. This can help to improve communication between nerves and muscles. But I experienced side effects like intense vomiting, which made it harder to continue. We then tried prednisone to curb my immune system. All that did was cause me to gain 40 pounds.
Meanwhile, my myasthenia gravis was worsening. I was diagnosed with generalized myasthenia gravis, which meant I began to experience other symptoms like trouble breathing, chewing, and swallowing. My care was transferred to a neuromuscular disease specialist for additional treatment options. My doctor wanted to put me on intravenous immunoglobulin (IVIg), which is a common and very effective treatment for myasthenia gravis. But my insurance company refused to cover it unless I first tried another class of drugs called immunosuppressants, like mycophenolate mofetil. I went through 3 months of misery on them, then switched to IVIg treatments.
I had hoped IVIg would be my miracle therapy, but it was pure torture. I was bedridden with side effects such as severe headaches and nausea. The nurse who came to my home to give me infusions called her manager during my third month to say she couldn’t in good conscience continue the treatment given how much I was suffering.
At that point, my doctor suggested a thymectomy. It’s an operation to remove the thymus gland and often improves symptoms. About 50% of people who have this surgery go into remission. The problem was I simply wasn’t healthy enough to attempt surgery yet. As a last-ditch effort, I underwent PLEX, or plasma exchange. I had to have plasmapheresis, a process where a machine removes harmful antibodies. I also had infusions of eculizumab, a type of drug known as a complement inhibitor. It works by limiting the activation of specific proteins thought to cause myasthenia gravis. Finally, after 9 months, I was stable enough to undergo an operation.
Prepping for Surgery
Surgery on anyone with myasthenia gravis is risky. I had to undergo a battery of tests, including a breathing test to demonstrate my lungs were strong enough for the operation. My doctors were also concerned that my recovery would be rough. I’d been bedridden and sick for years. It was hard to believe that just a couple decades earlier, I was a healthy athlete who’d played college basketball.
I also tried to prepare myself emotionally. I posted a lot on Facebook support groups for people with myasthenia gravis. It’s something I recommend anyone do before a thymectomy. I got encouragement and tips to help me navigate both right before and after surgery. I also reminded myself that I was getting the procedure done at a top medical center and my surgeons knew what they were doing.
Facing the Unexpected
A thymectomy is a major surgery. The thymus itself is supposed to be tiny. But for many people with myasthenia gravis, their thymus has gone nuts. That’s what happened to me. My thymus had become so large that it reached from my upper breastbone to my diaphragm. It also had ballooned to several times its normal size. The surgeon had to remove it in five different pieces. It literally had to be sliced out of my diaphragm because it had suctioned itself onto it.
When I woke up from the procedure, I was in the worst pain of my life. I had to stay in the hospital for 5 days and almost ended up on a ventilator. When you have surgery, your myasthenia gravis symptoms may worsen as you recover. My lung muscles simply weren’t strong enough to function. I began to breathe very shallowly and quickly. When I told the nurses, they told me I was having an anxiety attack. Of course, I was in the middle of a myasthenia gravis crisis. As soon as the doctors figured out what was happening, I was transferred to the neuromuscular ward. There, the staff was more aware of the symptoms of myasthenia gravis.
I’d like to stress that many, many people with myasthenia gravis undergo a thymectomy and don’t have the complications I did. But anyone who has the surgery should be prepared for a tough recovery, regardless.
Moving Forward
I wish I could say that my myasthenia gravis miraculously improved after surgery. My symptoms did improve. But I’ve been left with lingering nerve damage post-surgery. That’s not my doctors’ fault. They were given the Herculean task of battling a thymus five times its normal size. But I still have days where I’m bedridden. I stopped working in June 2020 and still haven’t gone back. I’m not sure I ever will.
As anyone who lives with myasthenia gravis knows, it’s an emotional and mental journey as well as physical. It’s important to get psychological help, whether it’s talk therapy, support groups, trusted friends, or a bit of all three. You have to get into a good headspace with where you are. This is particularly true in situations like mine, where you very well may live with debilitating symptoms for life.
Initially, I had so much pain post-surgery from nerve damage that I needed to go on several different pain medications. That left me with a brain so foggy I couldn’t even think. I was bedridden and too exhausted to even watch a movie. But as time went by, the pain got more manageable. I did physical therapy and got to the point where I could spend time out of bed.
Then last August, I had a setback. All my treatments have left me immunocompromised. I came down with a cold and was sick for months. Due to my insurance, I was transferred to a different medical center where PLEX was unavailable. My doctor started me on a new drug for myasthenia gravis known as efgartigimod. It was FDA-approved in 2021 and lowers the levels of harmful antibodies in the blood. It’s given every week as IV infusion. It’s helped my myasthenia gravis, but it comes with its own side effects, like headaches and nausea.
Still, I’m glad I had my thymectomy. I think I would have been much worse off without it. But I’d advise anyone considering one to research it. Doctors still aren’t quite sure how a thymectomy works to control symptoms of myasthenia gravis. One theory is that while for most people, the thymus goes dormant in adulthood, it becomes activated in people with myasthenia gravis and makes the antibodies that harm your body.
Most people don’t feel better immediately after a thymectomy, either. My surgeon told me that it can take up to 3 years for its effects to kick in. I’m optimistic that I’ll continue to feel better over time. I’m still housebound, but I celebrate small wins. Every hour that I spend sitting on my couch -- not lying in bed -- is a victory.
Show Sources
Photo Credit: Image Source/Getty Images
SOURCES:
Amy Burtness, 43, myasthenia gravis advocate, Sonoma, CA.
National Institute of Health: "Myasthenia Gravis."
