One day in June 2012, an infectious disease doctor at Massachusetts General Hospital ordered 30 vials of blood be drawn from my veins. I didn’t have a diagnosis then, of any of my conditions. I remember laying prostrate on our couch when we got home. “Blood sick, Heart sick” was how I described it.
It was a feeling I would come to know well, in milder form, whenever I would try to stand for any length of time, especially stand still. At least 25% of people with myalgic encephalomyelitis (ME) also have postural orthostatic tachychardia syndrome (POTS). It’s a chronic condition in which your heart rate rises dramatically (greater than 30 bpm) when you move from a laying to standing position. I’ve had POTS for as or nearly as long as I’ve had ME. For seven years, my heart rate laying down has been roughly 75 — 85 bpm. Standing? Anywhere from 100 — 140.
POTS is associated with low blood volume, and for years I drank Nunn electrolytes, took saline IVs, and eventually, fludrocortisone, a mineralcorticoid that makes you retain salt, all to increase my blood volume and thus, my tolerance for being upright.
So when a few hours after waking up from craniocervical fusion surgery, I looked at my monitor and saw these numbers, it was a miracle. “Holy shit,” I thought. “My POTS is gone!”:
This did not last: one week later, I had a surgery on my spinal cord called a tethered cord release. Most people, if they have both craniocervical instability and tethered cord syndrome, don’t have surgeries one week apart. (I do not recommend it!) But in my case, in addition to tethered cord syndrome, a hematoma (a rare outcome of a blood patch two months earlier) was also compressing my spinal cord and causing new peripheral neurological symptoms, namely pain and numbness. My surgeon thought it needed to be addressed sooner than later. All I knew was the compression and pulling on my brain had gotten so bad, I could not stand up straight and had to sleep with my knees pulled into my chest. So we went for it.
After my tethered cord surgery, my POTS came back with a vengeance: my heart rate would shoot up as high as 160. At first, I was devastated. To kiss recovery, a dream I had frankly long given up on, only to have it snatched away…but then the POTS went away again. And then it came back. It kept coming and going. It was maddening not knowing at which equilibrium my body would ultimately settle. I set my Apple Watch to continuous heart rate monitoring. Eventually, I started noticing I only had POTS three days a week. Then one day a week. Then once every other week. And now?
It’s been 21 consecutive days without POTS.
I can stand up straight, for minutes on end, completely still, and nothing happens.
Well, that’s not entirely true. My legs having inexplicably turn purple. It’s a work in progress. BUT my heart rate doesn’t even cross 94. I feel fine.
A couple of weeks ago, I consulted with someone about painting and doing some garden work. We stood for an hour, slowly circling the perimeter of my house and discussing the scope of the project. For seven years, I have been mostly bed and wheelchair-bound, never standing for more than 3–7 minutes at a time.
“But is your PEM gone?” I can hear many asking. The truth is, I can’t know yet. I crashed in the ME sense several times while in the hospital, but I have not crashed since coming home. At the same time, I am far from living a normal life or knowing if I would have the stamina to maintain one. In spite of the neurological improvements I have made, I am still essentially as disabled as I was before. What keeps me in bed now isn’t POTS but I don’t know whether it’s PEM. I struggle with pain, fatigue and a general, indescribable neurological weirdness. (Fatigue, which I never significantly experienced before, is very different from PEM but can be nonetheless extremely disabling.) These surgeries changed the entire architecture of my spine and the biomechanics of my body. I am still learning how to sit and stand and move again. Most of it feels like part of the recovery process, which takes 3–12 months, but they don’t exactly send you home with an instruction manual. In theory, it feels like I could get up and run down the street without crashing. Yet I know if I tried, my body would come apart at the seams.
So I find myself asking questions like, “Do I still have an energy envelope? If so, is it just bigger now? Have I just not been able to be active enough to hit the edges?” I don’t know and imagine I may not for some time to come, but here is why I am hopeful:
- Prior to surgery, I was assigned very gentle physical therapy to try to strengthen my neck and shoulder muscles to make post-fusion recovery easier. I could only do the exercises every three or four days. True to form, if I did 10 repetitions of an exercise the first day, I’d only be able to do 5 the next. After my surgery, not only can I sustain a given activity two days in a row, the second day I can increase the number of repetitions, all without crashing.
- When I do exert myself, instead of the usual inflammatory cascade that ends in muscle weakness and sound sensitivity, I get an endorphin rush, something I haven’t felt in 7 years.
- And some other signs I’ll write about in future posts — if they stick!
What does this all mean? Again, this sentence from the conclusion of the Workwell Foundation’s recent meta-study keeps bouncing around in my head:
Pathophysiological processes consistent with autonomic dysregulation should be prioritized for etiologic studies in ME/CFS, independent of distal pathogenic causes and proximal multi-system effects.
In essence they argue that like POTS, PEM is a kind of dysautonomia.
The craniocervical fusion relieved the pressure on my brainstem. What is the master regulator of the autonomic nervous system? The brainstem. What was the cause of my central apnea, severe enough that my neurosurgeon described it as a “partial Ondine’s curse?” (Although mine would happen day or night.) Brainstem compression due to cranial settling. Where did Nakatomi 2014 and 2018 find dysfunction in ME patients? The brainstem and structures just above it. What are several US research groups now starting to study in ME patients using neuroimaging? The brain, but especially the brainstem.
POTS can have many different causes but in my case, the circumstantial evidence strongly points to some structural defect the surgery resolved (e.g., relief of a squished vagus nerve, better CSF flow), but I put my money on the brainstem. Where does the cause of my PEM (if not all PEMs) reside? I can’t be sure but if it turns out that my PEM is truly gone, then I’d put the money, at least in my case, on the brainstem.
Is this it, the answer to all ME?
Of course not!
But it may be a central mechanism for some cases of ME.
Are these treatments for everyone?
They are treatments for some people who have been diagnosed with craniocervical instability and/or tethered cord syndrome. They have risks, side effects, and lifelong implications.
It’s also important to note that the same symptoms can have many different causes, especially if those causes are acting on the same part of the body. The brainstem can be compressed, stretched, inflamed or otherwise compromised by CCI/AAI, Chiari malformation, intracranial hypertension, CSF leaks, toxic injury and infection to name just a few causes, resulting in very similar symptoms. (Or perhaps different symptoms, depending on which parts of the brainstem are affected.)
Is this the answer to Jen’s ME?
I can’t even think about that. Recovering from these surgeries and a body that has spent seven years mostly in bed is like summiting Everest and I’ve only just reached basecamp.
Why, after 28 years of life, absent any significant physical trauma did my skull sink on my spine and compress my brainstem?
I have no idea.
But I have some conjectures — more soon.
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.
Also, my hat off to Michael VanElzakker at Harvard/MGH (follow him on Twitter) who is studying the brainstem in ME patients, Dr. Peter Rowe at Johns Hopkins (search his name on YouTube) who has long been investigating structural causes of ME symptoms, Dr. David Kaufman who first suspected I had craniocervical instability, Jeff Wood (aka, ME patient zero) and of course, my neurosurgeon.