Continued from Path to Diagnosis: Part II (Cervical collar)
I was taken to pre-op where I saw my surgeon and anesthesiologist. The plan was to perform two invasive tests. The first, an invasive cervical traction test, would involve screwing two bolts attached to a metal handle into either side of my skull while briefly under anesthesia. I would then be woken up and a physician’s assistant would attach the handle to a pulley system that would lift my head with progressive pounds of force while the surgeon performed a fluoroscopy, took measurements, and monitored my symptoms. Second would be a 24 hour intracranial pressure (ICP) bolt test. I would again be put under anesthesia while a small hole was drilled into my skull. A thin wire probe would be inserted through that hole to directly and dynamically measure the pressure in my brain. I would then be taken to the ICU and monitored for 24 hours.
Now you might already be thinking, “Wow Jen, that sounds like a lot!” but this is one place in my life where my nerd comes in handy. Sure, I was a bit nervous, mainly about whether there would be any pain, but I also found the entire process weird, gross, and fascinating.
When I came to in the operating room, I was sitting up. My surgeon asked me, “How do you feel?”
At first I didn’t feel anything. I was just confused as I waited for the anesthesia to wear off. “Terrible,” I finally said. Not only was I not wearing the cervical collar that had been keeping many of my neurological symptoms at bay for the past four months, I was carrying the extra weight of the metal apparatus they had attached to my head.
“Now, turn your head to the left.” He asked me to do this because he knew from taking my history that this is what exacerbated my symptoms. I turned my head and slumped over. I could not move.
In these moments of non-responsiveness, which began after my thyroidectomy, it can look to an outside observer (I have been told) like I have passed out or, if my eyes are open, like I am dead. I am still “there” inasmuch as I can hear what is happening around me, but I have a hard time having any thoughts or interpretation of events. My cognition is still there, but at a rudimentary level. I cannot move or speak.
“How do you feel?” he asked again. He asked several times. I could not respond, so the physician’s assistant picked me up by my new pail handle, brought my head back to neutral, and lifted it slightly. Suddenly, I could talk, breathe, react again.
The surgeon asked me what I had experienced when I turned my head to the left. I told him that I couldn’t move or speak. I also said I stopped breathing.
“No, you didn’t,” the surgeon said.
“Yes, I did,” I said.
“No, you didn’t,” he repeated.
“Then why did you ask me?” I thought.
Then, they added twenty pounds to the pulley system, giving my head some lift. “How do you feel?” my surgeon asked. I thought I felt a little better, but it was hard to tell. At least I wasn’t struggling with the weight of the pail handle. Again, he asked me to turn my head to the left.
Again, I slumped over. The physicians assistant lifted my head and brought it back to neutral. “What happened?” my surgeon asked. I told him I couldn’t move or speak. I told him I had stopped breathing.
He said, “No, you didn’t. You’re being constantly monitored. If you weren’t breathing, we would know. You are breathing” or something to this effect.
Now, I was getting angry. I don’t lie. I wasn’t here because of my ME or POTS symptoms. I was here because I was constantly ceasing to breathe. I try to be as precise as possible about what I am experiencing under the rather naive assumption that someone will care or that the detail I provide will be useful. As though clues matter. They rarely do. My experience of medicine is doctors constantly throwing away information according to their own needs and biases.I was starting to think this neurosurgeon was just another case of that.
Then we went to thirty pounds of traction. At thirty pounds, something extraordinary happened. I did not anticipate it. It took me by complete and total surprise.
I was 28 when I got ME, after a severe viral infection. Since then, I have never known a moment of feeling well. In an instant, it was like I was 25 again, at the peak of my health. That 24–7 feeling of being sick, of constant brain inflammation, was gone. The unrelenting pressure behind my eyes was gone, too. For years, I felt like I was wearing an invisible leash, one that would violently slap me back into bed if I thought too much or did to much. That, too, was gone. Both cognitively and physically, I felt well for the first time in almost eight years.
“How do you feel?” my surgeon asked.
I could not say how I really felt. That I felt like getting up off that table and going running down the halls and up the nearest mountain. That I suddenly had boundless energy. Or that I felt like a superhero. How do you describe what it is to suddenly feel normal when you have completely forgotten what normal feels like?
“I feel great!” I said.
He asked me to turn my head and I did, right and left, just as fast as I liked. I was able to do it without slumping over, without triggering any neurological symptoms at all.
I knew in this moment that this was it. I had found what had caused seven years of that hell. I also knew that if the surgery was successful, there was a very good chance I would feel like I felt at thirty pounds of traction, 24/7, every day of my life. In other words, that I would be cured.
Some people have wondered, “how do you know if it was the fusion that put your ME in remission and not some other intervention associated with the fusion?” I will grapple with alternative hypotheses in future posts, but putting aside the fact that I have been under general anesthesia multiple times both prior to and after this test, and have taken all the other drugs associated with surgery, this is how I know. It was because the effects were specific, instantaneous, dramatic and total. It was not subtle.
However, I could feel my surgeon’s deep skepticism. I remember thinking, maybe he won’t operate on me. Maybe no one will. I didn’t care. At least I knew. I knew what was causing my symptoms. The peace and joy that came with that knowledge is hard to describe. I thought I would live and die and never find out.
The physician’s assistant released the traction. All of my symptoms came flooding back. Again, I had brain inflammation. Again, I had pressure behind my eyes. Again, I could feel that invisible leash wrap itself around my chest.
They started to reinitiate anesthesia so they could remove the pail handle and insert the intracranial pressure bolt. As they lowered me to a supine position, my head slid into a “bad” position.
“She’s not breathing,” the physician’s assistant said.
As scary as it was to be not breathing and to be drifting off, I knew that I was being monitored, and that they knew exactly how to help me breathe again (i.e., lift her head) which no other team, in no other ER or OR would. So I wasn’t afraid.
I smiled in my own mind, knowing everyone in that room had finally witnessed what I had been trying to tell them all along.
You might think my surgeon was kind of an ass for not just believing me when I told him I wasn’t breathing — yet another doctor who doesn’t believe his patients. After all, that is the mantra for so many of us: “Believe patients!”
He had reason to be skeptical: I did not present quite like his other patients. The dysautonomias (POTS, poor temperature control, etc.) he was quite familiar with along with Mast Cell Activation Syndrome (MCAS). The central apnea, a type of breathing problem that happens when the brain doesn’t send proper signals to your diaphragm muscles, causing abnormal gaps in respiration, was rare. Also puzzling was that I did not have a diagnosis of Ehler-Danlos Syndrome or any signs of peripheral hypermobility. And “fatigue,” as he called it (he was not familiar at the time with the concept of post-exertional malaise) can have so many different causes.
He explained his stance to me later: “I don’t want to drink my own Kool-Aid.” By this I think he meant that he doesn’t want to start seeing craniocervical instability (CCI) everywhere. I went to him, desperate and at what felt like the end of the line for me in terms of seeking answers. He is used to seeing desperation. So many patients with Ehlers-Danlos Syndrome (EDS), like patients with Myalgic Encephalomyelitis (ME), go for years, even decades, undiagnosed. Many have been accused of malingering, have been diagnosed with conversion disorder, or have otherwise been dismissed by medical professionals. There are emotional incentives to rush to judgment. The doctor wants to help. The patient desperately needs that help. In this context, it’s even more important to make sure to proceed deliberately and dispassionately in order to increase the odds that the diagnosis is right. So many problems can cause similar symptoms but ultimately require very different solutions.
He described his entire process as a way of trying to hedge against his own cognitive biases, biases all of us have. For him, the patient’s subjective experiences and symptoms do matter, but if he can’t also objectively measure or observe it, then he cannot take it as fact. I have met so many doctors over the years who have been supremely confident of, well, everything. Here was one who was essentially saying, “I have to work very hard not to lie to myself.” This is the first of the very many reasons why my surgeon earned my trust and respect.
When I woke up in the recovery room I remember telling Omar. “This is it!”
The surgeon later came into the room to explain the results of the test. In addition to the positive subjective symptomatic improvements I had experienced while under traction, he was able to objectively measure the relationship between my skull and upper cervical vertebrae — the craniocervical junction — via fluoroscopy.
The craniocervical junction is like a twenty lane highway. It’s where your brainstem becomes your spinal cord. A bundle of arteries, veins and nerves enter and exit. Just above it, the vagus nerve joins the brainstem. Much of signaling between your brain and your body requires this junction — the most mobile in the entire body — to be structurally sound within some fairly narrow parameters. It is kept both highly stable and highly mobile by the strength of your neck muscles as well as well as a scaffold of some of the densest, strongest connective tissue in the body.
The neurons of the upper cervical spine and brainstem are extremely sensitive to slight changes in pressure or stretch. Small abnormalities in the angle of the brainstem or even minor compression, as can occur with CCI, can cause major dysregulations of the autonomic nervous system and with it, the entire body. Here, a millimeter might as well be a mile.
On my fluoroscopy, the Basion Dens Interval (BDI), a measure of the vertical distance between the skull and the spine, was very clearly abnormal. Under traction, a normal person will have very little to no change in this measurement. If traction is able to lift the skull, this shows the skull was too “low” on the spine to begin with and that it is hypermobile. Anyone with a change of 2mm or greater is considered a candidate for surgery. My change in measurement was 4.8mm. This indicated that my skull was moving more than it should, as a result of ligament laxity. This craniocervical instability was causing cranial settling — my skull was sinking on my spine, crushing my brain stem and likely also causing problems with cerebrospinal fluid and blood flow (which could cause or contribute to intracranial hypertension).
The radical change in my symptoms I experienced while under traction amazed me, but this is something that my surgeon sees all the time in people who have CCI. Even more surprising, from a medical standpoint, was what happened during my 24 hour intracranial bolt test. This test helped explain the dynamics behind my central apnea and forced me to rethink everything I thought I knew about post-exertional malaise (PEM).
Read all the posts in my CCI + tethered cord series
Read this disclaimer. Crucially, surgery carries risks and it’s important to remember that in medicine, the same exact symptoms can have multiple, different causes. We have no idea how prevalent CCI is in our community and there’s been no research into its relationship with ME. We do know that it is more common among patients with EDS.