When I was a kid, we used to visit a place by a river. Over that river was a rope swing. Every time we went there, I had to psych myself up to take my turn.
You had to grab onto the rope, wrap your legs around it, and then wait while someone gave you a push.
If you let go too soon, you’d fall on your ass and roll into the water. If you held on too long, your legs would wobble with the strain of holding on until you were forced to jump off at the point you started—on the shore. Embarrassing.
Your best choice was to wait until rope swing was out above the middle of the river. The water below seemed miles down. You swallowed your fear. Then and only then did you let go.
The plunge was terrifying and exhilarating, the cool water wrapping itself around you like a flowing robe, your hair slick against the back of your neck, the feeling you could do anything now. Anything.
Five and a half years ago, I was 47 and at the top of my game. I had a glamorous career in Silicon Valley and had just published my first book.
My husband and some friends took me out to celebrate. We chose a food and drinks place that had a large outdoor courtyard.
It was chilly, so I sat under a large heat lamp. At some point, a worker came and began fussing with the thing. I later learned he was changing the propane tank.
This unbalanced the heat lamp. It tipped over.
I wasn’t aware of any of this at the time. All I knew was that out of the blue, something slammed into the back of my head. I fell forward onto the table.
Within a few minutes, I regained my composure. I sat up and chatted with people around me, shaken but otherwise okay.
But after about fifteen minutes something strange happened. The muscles jumped inside my neck like they were doing a kind of dance.
One ambulance trip to the ER later I was home with the diagnosis of “neck sprain.” It was supposed to take six weeks for me to get better.
But that’s not what happened. As the weeks wore on, I got worse, not better.
We went to doctor after doctor. Nothing was broken, and no discs had slipped. So as far as they were concerned their job was done. But my neck wasn’t done with me.
The “neck sprain” fountained into pain, vertigo, more pain, migraines, gallbladder distress, rashes, and a hundred other strange and terrifying symptoms. My husband and I were both falling apart from the strain.
At one point, I shared something new that had started that day, and he blurted out, “No more symptoms!”
I stopped telling him. But the symptoms piled up. After awhile, I couldn’t digest food. Everything made me sick except fish in broth.
I lost a third of my body weight in three months. My husband took to pouring the soup down my throat, as I couldn’t lift a cup on my own.
I spent most of the day lying flat on my back.
I couldn’t stand to be touched. The inside of my skin hurt. I felt as if someone or something was grabbing my hair and pulling it tighter and tighter until my scalp was on fire. I couldn’t read, watch TV or even think too hard without being hit with something I can only describe as full body panicked nausea.
I didn’t know it then, but I was one of millions in similar situations—set adrift by a medical system that’s not designed to deal with chronic pain, or really chronic anything.
Most med schools have zero required training in pain management, even though 10% of the population has disabling chronic pain. Doctors are taught to look out for hypochondriacs and drug seekers. What better way to describe someone who keeps going to doctors and insisting they need help for their pain?
We went to the Stanford Hospital ER in the hopes they’d check me in. After spending hours lying on a gurney in a freezing hallway, they gave me an MRI. It was normal. I was sent home.
After that, I lay in bed, staring at the ceiling. It was over. No one would save me. The pain was so extreme I couldn’t hold on anymore.
I told my husband I was going to kill myself. I didn’t want to die, but at some point I’d gone through a door and was in a room where death was preferable to spending one more minute in a body like this one.
He locked all my medicines in a cabinet. Kept an eye on me all night.
A doctor friend intervened. He got me into UCSF hospital the next day. This saved my life.
But the doctors there were no different. The scans were normal, therefore it was nothing. However, they did give me enough pain medication and muscle relaxers that I was able to function again, at a low level. I went back to work from a hospital bed in our apartment.
Three years went by. We struggled along. Then, I found a neurologist who gave me a diagnosis: cervical dystonia.
After all this time, I had something real, and treatable. Hallelujah.
But the more I learned about dystonia, the less sure I was. Yes, I did have the main symptom, constant muscle spasms. And my head was tilted and stuck in a turned position, which is how cervical dystonia looks.
But what about the other stuff? Why the insistent pain when I tried to do almost anything—reach for the faucet, put on socks, sneeze? Pain that turned into muscle spasms that got worse, not better, over the following hours and even sometimes days, until I wanted to scream.
I met people with dystonia and they didn’t have stuff like this.
And there was more that set me apart from them.
I couldn’t bend my body, so I needed help showering and getting dressed. I also couldn’t move my left arm without pain, and had lost the use of my left hand. This was in some ways the most debilitating of all, as I’m a lefty.
No more writing, typing, or doing anything that required the agility of one’s dominant hand. (Of all the cards I’ve been dealt, the cruelest has to be that it’s my left side that’s gone weak.)
Still, I clung on to the diagnosis, a shipwreck victim clawing at my one tiny piece of wreckage. I tried the main treatment for it, Botox. It didn’t work.
But I didn’t let the diagnosis go. I couldn’t. The alternative was what I’d lived with for the previous three years.
A new perspective
A little over a year ago, we moved from the San Francisco Bay Area to New Jersey. The one thing we didn’t expect was that I would find a better doctor. But I did—a young Romanian neurologist.
Our first appointment was unlike any of the dozens before it. She let me describe everything I’d been through. No interruptions, except to ask questions.
Her eyes lit up. She had a theory about what was going on.
“Is this a real thing?” I demanded to know.
Later, I’d be embarrassed. But I’d heard so many things from so many doctors—“psychogenic” disorders, somatization disorder, dystonia…I never knew what was coming next.
Anyhow, she was nice about it.
“Yes, this is a real thing,” she answered.
Her hunch was that it was something in the realm of anti-GAD antibody autoimmune disease. In more plain speak, Stiff Person Syndrome (SPS).
SPS is a rare autoimmune disease marked by extreme muscle spasms. But it has other symptoms as well. The name says it all—it makes you stiff.
Both dystonia and SPS are incurable. But the difference is that SPS can be treated in ways that sometimes work, whereas dystonia has a much spottier track record on the account.
For dystonia, there are just a handful of medications, plus the Botox. Other than that there’s the last resort: deep brain stimulation. This involves drilling holes in your skull and outfitting you with a remote control for your brain.
With this new diagnosis I could start treatment right away. No brain surgery required.
The more I learned about SPS, the more I recognized my experience. It put everything I’d been going through in a new light.
For one, SPS can make you anxious. So anxious you jump at loud noises—something that had been happening to me from the start. I was learning that this is a part of the disease, attacking the brain and depriving it of the chemicals it needs to moderate your response to danger.
Then there was the arm stiffness, Every doctor had dismissed it as “frozen shoulder.” Not one saw it as a symptom that could be part of my larger problem. And of course I was stiff all over.
My neurologist was the first to take these seemingly unrelated symptoms and build a picture from there.
The good, the bad and the ugly
I had my first round of treatment in a local hospital. It involved an intravenous drip of immunoglobulin, known in the biz as IVIG.
The morning after my first dose, I woke up and for the first time since the accident I didn’t feel anxious. It was like having the sun break through after living under a cloud.
As the days progressed, I found I could walk better than I had in years. My pain was down as well. I was still disabled and in need, but I wasn’t down as far as I’d been.
I started PT. I could hold and squeeze a ball with my left hand without pain. Turn the kitchen faucet on and off. Carry a cup from the sink to the table. Tiny things to most people, but huge to me.
Then I joined a Facebook group for people with SPS. That’s when the happiness began to wear off.
Yes, some people in the group were reporting great results from treatments such as IVIG. But it often stops working.
True, there are plenty of other treatments. You can get stem cell injections. You can take a designer drug called Rituxan. You can try different formulations of the immunoglobulin. You can travel to Italy or Russia and get a series of treatments that some say puts you into permanent remission.
But then there’s the flipside. There was a lot of talk in the group about pain. Much of it sounded worse than mine—and I didn’t believe this could get any worse. Many people said that after awhile, they couldn’t sleep. My one place of oblivion. I couldn’t bear the thought of losing that.
And unlike dystonia, SPS is a “progressive” disease. This is the kind of “progressive” you don’t want—it means you get worse.
In fact, SPS can kill you. Your body gets stiffer and stiffer until you’re unable to take a breath. You die in a horrible way, gasping for air while your throat closes up.
Since my treatment, the results have waned. The worst pain is at bay, but I’m not walking as well, and doing things hurts again.
I don’t know where I’m going. Up? Down? It’s impossible to say. But for the first time since the accident, I have a working diagnosis that makes sense.
I’m out above the river now. It’s time to let go. Will I sink or swim?