Path to Diagnosis: Part II (Cervical collar)

Continued from Path to Diagnosis: Part I (An empty sella)

The vascular neurosurgeon who diagnosed me with intracranial hypertension told me I might have craniocervical instability (CCI) but this was not the first time I had heard of this diagnosis. The first time I had heard of CCI was on this now epic Phoenix Rising thread. On it, a patient named Jeff, who now writes about his experiences at mechanicalbasis.org, described getting diagnosed with CCI, a condition which can cause cranial settling and brainstem compression, and how having surgery cured him of his ME. This, he explained, was what had been causing all of his symptoms of both ME and POTS. “How many others are just like me, but undetected?” he asked.

I read his story with interest because, well, it was fascinating. It was absolutely conceivable to me that a compromised brain stem could cause the symptoms of ME or something very similar. After all, many of the symptoms in the Canadian Consensus Criteria (CCC) and International Consensus Criteria (ICC) can be symptoms of dysautonomia, and the brainstem is a major component of the autonomic nervous system.

So I took all this in as academically interesting. However, I did not think it had any personal relevance to me. I was a viral onset patient. (I later found out, so was Jeff.) Prior to my thyroidectomy, I had classic ICC-ME with no symptoms that obviously suggested a structural problem, much less one involving my neck. Besides, at the time I was making some improvements. At the beginning of my illness, I was almost entirely bedbound and would crash from relatively minor activity like talking on the phone or attempting to go for a wheelchair walk. These crashes would last for weeks or months. Thanks to a combination of drug treatments and the avoidance of mast cell triggers, I could now sit in a chair for a few hours a day, leave the house once a day in my wheelchair and later, travel around the world for the release of Unrest (although this involved spending a lot of time crashed in hotel rooms in foreign countries!) In short, I had moved from the severe to the moderate part of the spectrum. While I was still profoundly disabled, I was responding to drug treatment. I also had numerous laboratory findings consistent with the ME research literature. For these reasons, I thought that my illness must have a biochemical, systemic cause.

So, pursuing a craniocervical instability diagnosis wasn’t high on the priority list and frankly, the images of Jeff in a halo scared me (though he later explained that very few CCI patients end up in a halo, as he did, prior to surgery). The thought of never being able to turn my head again normally also scared me. Moreover, surely if I had something that could be clearly, objectively measured, like a problem in my cervical spine, it would have been diagnosed by now. Surely this was something my neurologist, who diagnosed me with conversion disorder, would have screened me for, given all of my neurological symptoms.

Then I had my thyroid surgery. My neck was hyperextended to intubate me while under anesthesia. I woke up with an excruciating pain in my jaw. I could not sit up without falling over. A month later, I had what felt to me like a severe viral infection with low-grade fever. All of the severe ME symptoms I had had when I first became bedridden would come raging back, erasing the modest gains I’d made from drug treatment. With them came two completely new symptoms: an inability to breath when putting any pressure on the back of my head, such as when laying flat and falling over, and becoming immobilized and unresponsive (but not losing consciousness) when I turned my head to the left.

On the advice of a friend with hypermobile Ehlers-Danlos Syndrome (hEDS), ME, and neurological symptoms, I began wearing a hard cervical collar. (Again, I do not have hEDS.) I had already been to the emergency room at UCLA without success and had a neurology appointment a full month in the future. The best they could offer me was, “Don’t lie on your head.” Right.

Since my thyroidectomy, I also had the problem that whenever I would accidentally hit my head against the railing behind my bed or otherwise put too much pressure on my head and neck, I would start leaking what I think was spinal fluid. Now, I cannot be sure that this is what was happening, but immediately after injuring myself, I would taste something slightly salty, slightly sweet, and thicker/gooeier than saliva dripping down my throat. I would lay flat, rest, and it would go away in a few days. The flow also seemed to increase when I was upright. If I got my neck in the wrong position or tapped my head, that same taste would come back. This happened multiple times.

Not only that, but I eventually started to stop breathing, even when sitting up with my head facing straight. We once went with me breathing 5 seconds/not breathing 15–45 seconds, on again, off again, over and over for a full hour before we could figure out how to position my head so I could breathe normally. My life was becoming untenable and no one thought any of this was an emergency.

So I ordered a Philadelphia collar from a local medical supply store in Los Angeles (and later, a Miami J collar on Amazon).

I cannot recommend that anyone order a cervical collar. There are some real downsides (Jeff explains this further in his FAQ), namely a deconditioning of the neck that can worsen any instability and cause you to become dependent on the collar. But I had reached a point where in my own estimation, I very clearly had no other choice.

The cervical collar did not really provide any traction to speak of, that is, a lifting of my head, but it did provide immobilization of my neck.

Wearing a cervical collar did nothing for my POTS or PEM, but it had a dramatic effect on some of my other symptoms. I slept in it, and so was no longer waking up in the morning with my entire right side numb with the sensation that my arm was dangling out the socket. I could spend the day sitting up again. I stopped getting spinal fluid leaks. I still couldn’t lay on the back of my head, but I stopped having central apnea, at least while sitting up. (Central apnea is a type of breathing problem that happens when the brain doesn’t send proper signals to your diaphragm muscles, causing abnormal gaps in respiration.)

On the recommendation of a friend, I consulted with a pediatric neurosurgeon at Stanford. He told me to wear the collar religiously for eight weeks and see how I felt after a period of strict immobilization. He explained that many of his patients with Ehlers-Danlos Syndrome (which again, I do not have) will prophylactically wear a cervical collar when they have a viral infection and experience worsening neurological symptoms, but often the need is temporary. So I kept the cervical collar on, now doctor-recommended.

I also emailed a new ME specialist I had recently begun to see, Dr. David Kaufman. (Dr. Kaufman is also Jeff’s doctor. Jeff had introduced him to the idea of CCI, based on his own research.) I told him about all of my new symptoms, how they had started after my thyroidectomy, about what happened when I laid on my back or turned my head.

He said, “I think you might have craniocervical instability Jen” and wrote a prescription for an upright MRI.

I tried ruling out every possible alternative explanation for my symptoms: intracranial hypertension, Eagle’s Syndrome, spinal fluid leak. I prioritized these diagnoses because their solutions — a venous stent, removal of an overgrown styloid process, a blood patch — all seemed much better than a craniocervical fusion. But then I reread Jeff’s thread and we spoke on the phone for three hours.

The similarities between our stories were undeniable. He too developed ICC-ME (and Ramsay and every other type of ME) after a viral infection. He too had had a severe worsening of his ME and POTS symptoms and the onset of new symptoms after a surgery. Dr. Kaufman had said to me, “craniocervical instability.” Then my vascular neurosurgeon (who has never met or spoken with Dr. Kaufman) said “craniocervical instability.” When, now three months post-thyroidectomy, I started to stop breathing and losing consciousness while sitting up, even while wearing my hard cervical collar, I knew it was time to stop avoiding the one thing I wish I didn’t have. A third neurosurgeon, one who specializes in craniocervical fusions and other surgeries related to the “neurosurgical complications” of Ehlers-Danlos Syndrome, reviewed my imaging.

Before I knew it, I was in another hospital being wheeled into an operating room for yet another invasive test. This test would turn on its head everything I thought I knew about my case and change my life forever.

Continued in Path to Diagnosis: Part III (Traction)

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.