How I decided on surgery for occult tethered cord syndrome

As some of you know, I decided to move forward with tethered cord surgery (SFT) this August with Dr. Paulo Bolognese. I will be blogging about my surgery journey in case it helps others walking this path. Of course, my story is only one of many, but I have certainly benefitted from hearing about a range of experiences and hope that mine adds some value.

Since my last post on this topic, the decision on whether to get surgery has consumed me. In this piece, I share my decision-making process, my blockages, and how I resolved them.

1. Wrapping my head around the diagnosis. Initially, I had doubts about my occult tethered cord syndrome (OTCS) diagnosis. Equal parts of fear, denial drove this, and simply not putting in the time to understand the diagnosis better, as I typically do when staring down a new condition.

Unfortunately, my handwringing thrust me deeper into my limbic system/paleo mammalian cortex. Trying to nail down all of the uncertainties, falsify the diagnosis (as any good scientist would do), and develop every imaginable contingency plan A-Z turns out to be rather taxing. I call it my “Danger Danger Will Robinson mode” because my brain sees danger everywhere, like the robot in Lost in Space.

After a month of putting my head in the sand, I knew I had to understand better why Dr. Bolognese gave me an OTCS diagnosis. I figured if tethered cord syndrome is a congenital condition, why is it only showing up now? Might something else explain my symptoms? Why are people with hEDS more prone to having OTCS?

Tethered cord syndrome occurs when a fibrous thread, called the filum terminale, becomes inelastic. The filum terminale is a thread-like structure made of connective tissue that serves as an anchor for the spinal cord – it reaches down into the sacrum, where it attaches (see image below). The spinal cord needs some flexibility and fluidity for everyday activities – turning the head, twisting the torso, bending down, etc. With an inelastic filum, the spinal cord cannot freely move inside the spinal canal.

People with hEDS are hypermobile in their joints, but paradoxically, the filum terminale is taut like a stretched rubber band. When cut, it even snaps like one (see Dr. Bolonese’s surgery video below). This puts the spinal cord in a state of oxidative stress. During my consultation with Dr. Bolognese, I remember wondering but how do you know my filum is taut and thin? Wouldn’t you need to cut into my spine before drawing that conclusion?

I spent an inordinate time looking at each of my symptoms in isolation, most of which are pretty non-specific. I needed to convince myself that there were no alternative explanations for them. Listening to talks by prominent neurosurgeons allowed me to appreciate better that if you have hEDS, a neurogenic bladder/constipation, orthopedic and neurological problems, well, that starts sounding like tethered cord syndrome (even if you can’t see it on an MRI). My atomistic approach prevented me from seeing the bigger picture for what it is.

I also gained a much better understanding that OTCS can lead to permanent neurological damage, which I knew in theory. However, when symptoms are manageable in the present, it can be hard to project them out into the future. I hated the idea of deciding from a place of fear, surgery as prophylaxis for what may (or may not) come.

Here is a 15-minute video from Dr. Petra Klinge, one of two other surgeons I would consider for SFT surgery. It is not technical and does a great job explaining “the triad” of symptom categories associated with occult tethered cord (syndrome urinary, orthopedic, and neurological).

Dr. Patel, another highly-respected surgeon, discusses EDS and OTCS in this Q&A session.

Here is another more technical video from Dr. Bolognese. The entire talk is interesting, but the most relevant parts to occult tethered cord start around the 12-minute mark.

2. Waiting for more signs

I hoped I might see some additional signs and symptoms to indicate that I do, indeed, have occult tethered cord syndrome. My surgeon was very clear that I have OTCS and need SFT surgery. He said he did not need any additional evidence to feel comfortable making the diagnosis. He based his decision on my “extreme” neurogenic bladder, positive questionnaire, and soft signs on my MRI (posterior positioning of conus, loss of lordosis). I have to remember that Dr. B has done this surgery on over 1000 people; he made the diagnosis based on decades of surgical experience with this condition.

I am not a fan of clinical diagnoses and was concerned that having hEDS influenced his decision too much. I had a negative heel walk test (for the most part) and didn’t have many leg symptoms, or so I thought. The absence of leg and gait symptoms filled me with doubts. I secretly hoped that I would see some more signs to become more comfortable with the diagnosis.

It is hard to know if a symptom or condition fits your chronic illness picture. You can read about symptom sets associated with conditions online and look to your peers for their experiences and descriptions. However, until you experience something yourself, it can be hard to know if you have a condition. While patient groups can be a lifesaver, they can also throw you off the scent. For example, I saw videos of fellow patients who lost their ability to walk or worsened their gate issues by walking on their heels. Many descriptions also include migrating pains – dull-like in quality but still miserable and at times extreme. People also describe numbness and tingling – also, weakness.

Since deciding to go forward with SFT surgery, the scales have fallen off my eyes. I do have leg symptoms, some of which date back to early childhood. Some symptoms are worsening, but I now see they were there all along, but I had been minimizing them. I often do that – I get to thinking that everyone else is so much sicker than I am and that I couldn’t possibly have what they have.

I do a poor job at describing symptoms, but here goes. I have searing, burning feet, especially on the soles – it reminds me of when I had a phototoxic burn from doxycycline; my feet burn and are tingly. The burning has worsened and now extends up into my calves. I also have wicked and wild fasciculations – tiny ones throughout my calves (I call them my Demogorgans, like what Eleven had in her legs after the Mind Flayer got her in Stranger Things). My legs have a heavy sensation – more cumbersome than anything, but as if someone replaced my muscles with a different substance. I joke that they feel like electrified meat sticks. Like walking around on some big Slim Jims with blinking Christmas lights inside.

My legs also get a “buzzy” feeling – not like zaps, but like faint electricity zinging around. I find I have to move my legs to discharge it – is this how restless leg syndrome feels? When symptoms are bad, my whole body feels like this. I also get a numb and tingling feeling in my coccyx that can shimmer into my inner thighs. Finally, I get intermittent growing pains – something that dates back to my earliest childhood memories. It is a dull, aching feeling that seems to emanate from my lumbar down to my legs, making them also feel weak and painful, despite my muscles being plenty strong. I also get strange migrating thigh pains. But, nooooo, I don’t have leg symptoms, lol.

My head and neck symptoms also continue to dog me. These are among my most disabling symptoms: suboccipital headache, pressure, neck pain, paracervical pain, and all around feeling very poorly. I am unable to be in any position other than a fetal one. My spine feels like it is burning. I am grateful my surgeon will assess my neck instability and cerebral spinal fluid pressure the day before my surgery. My dysautonomia is also worsening – my systolic blood pressure is often in the high 70s, and I am lucky if my diastolic breaks 50 mm/Hg. My heart rate was 41 bpm a few days ago. Is this related to tethered cord syndrome? Who knows.

Of course, I do not dispute my worsening bladder, bowel symptoms, or lumbar pain.

In combination with my hEDS diagnosis, these symptoms leave me feeling more confident about the diagnosis and being on the right path with SFT surgery.

3. Living with the decision

As I explored in my last piece, fear over the possibility of a bad surgical outcome was a major sticking point. My deliberations coincided with some problematic stories coming from fellow patients on this path. I was terrified of retethering and needing more surgeries. I also was concerned that detethering could create instability in other areas, leading to more surgeries. Since that time, a couple of the higher-profile cases I was following mostly resolved. One person had retethered and had a successful 2nd detethering, and another is doing far better after having a shakey couple of weeks of healing. A third friend is struggling, but she had three surgeries, so I am holding out hope that her recovery will accelerate at some point.

I knew I had to be able to answer the following question: will I regret the surgery? Since making the decision, the answer is no. I understand that I don’t have a choice about the diagnosis and may need more surgeries (but will do all I can to avoid them). I also am willing to accept the idea that this surgery may lead to instabilities in other areas, such as my neck. I am hopeful that the invasive traction and intracranial bolt test I will be doing the day before my surgery will give me a better indication of what to expect after detethering surgery. I know many are hesitant to get traction done because it could weaken ligaments, but I am the type who does better with more information, not less. I want to know what I am up against and am willing to take that risk. I will also do all I can to prevent re-tethering and need neck surgery.

I also need to be prepared for being worse off after surgery due to some unforeseen circumstance. I accept this as well.

4. The no-action alternative.

As an environmental scientist, I often view big decisions through an Environmental Impact Report (EIR) lens. An EIR always includes a “no-action alternative” to measure proposed changes. What happens if you do nothing? Not making a decision is a decision in itself.

By rejecting the surgery, I would have to accept the idea that my condition could worsen and become irreversible. I knew this in theory and even discussed it in my last blog. However, sometimes you need to feel these choices viscerally. These past few months reminded me that sometimes wisdom isn’t found in a cost-benefit analysis or on a piece of paper with yes and no columns.

A significant shift came for me when I saw a video made by a woman around the same age recovering from SFT. She talked about how she was also diagnosed with EDS late in life (I was 49). She was filled with gratitude for her post-surgical gains and was accepting of the permanent losses. She described how delaying surgery for ten years due to misdiagnosis cost her valuable time. She explained how the signs and symptoms were there all along, but doctors kept sending her home, telling her there was nothing wrong until she lost her ability to walk. She implored watchers not to wait for more signs and symptoms to appear and to get the surgery done sooner than later.

We are familiar with how a broken back can paralyze people in an instant. Tethered cord is like a slow-moving version of this, increasingly becoming paralyzed and losing bladder and bowel function.

In the video, these words hit home: “If you think you have a tethered cord, do not delay. Do not delay getting to a neurosurgeon who understands it and understands Ehlers Danlos Syndrome and other connective tissue disorders – one who understands OTC and how grave it can get. Don’t put it off; those neurosurgeons are hard to get to, and once you are with them, unless you are very emergent, they cannot get you in to see them the next day for surgery. You have to get on their list. Don’t delay; help get the word out, early diagnosis, early diagnosis, early diagnosis.”

To learn more about how EDS is associated with various spinal conditions (including tethered cord), check out this article (in lay terms). https://www.ehlers-danlos.com/2017-eds-classification-non-experts/neurological-spinal-manifestations-ehlers-danlos-syndromes/