The beautiful thing about writing a personal blog — you know, aside from the obvious personal stuff — is the opportunity to share something useful that might pop up in a Google search (or Bing if you prefer one giant conglomerate over another). And while that’s certainly true of this posting, it took a little time to figure out how I was going to go about it, i.e. how personal did I want to be?
In the end, however, it was kind of a no-brainer. After many less than stellar searches of my own, it was obvious if I wanted to say something useful about syringomyelia (outside of the clinical definition), then I would need to be specific to my own personal experience as a quadriplegic. Because, ultimately, that’s what would have been most useful for me. So personal I will be.
The salad days
Up until about four years ago — aside from the whole chronic pressure sore thing (another story for another time, though there’s plenty alluded to it on this site — directly or indirectly) — things healthwise have been pretty stellar. I have a kick-ass immune system and for a C 4-5 quadriplegic — almost 30 years postinjury — I’m in excellent shape. Simply put, I take care of myself.
Still, somewhere around 2005 I started to notice some changes happening in my body, namely sensation loss below my level of injury (in my groin, er, *cough – penis – cough*). And while the loss was spotty at first — sometimes there, sometimes not — as time wore on it grew more and more consistent, sometimes lasting for months. Alarmed by the progression, I scheduled an appointment with my doctor and began the process of trying to figure out what was happening to me.
The first thing that was done was to get an MRI, but because my sensation loss seemed so specifically located in my pelvic region, instead of ordering a complete scan of my spine, only one of my lower back and sacrum were taken. A decision which, as it turns out, was grossly incomplete. Had I seen a neurologist familiar with spinal cord injuries from the beginning, instead of my physiologist, most likely a complete set of scans would have been ordered and my situation now would be markedly different. However, that never happened, and regardless, the radiologist at the time did see something he didn’t like, but when a request was made for further, more complete scans, my insurance provider denied it out right as “redundant”. I was then counseled by my doctor to “keep an eye on things”, and if they seemed as though they were getting worse we could give it another go then.
Fast-forward three years, and while the sensation loss didn’t appear to be spreading to other parts of my body (as far as I could tell, anyway, with my limited sensation), I was beginning to have severe neck pain, the kind of which I hadn’t known since the time of my injury. Wrongly or rightly — and perhaps some might say consistent with my character — I endured it for almost a year more before finally admitting I needed to see a neurologist or some sort of spine specialist about it.
Around February of 2009, after meeting with spine surgeon Bobby Tay at UCSF, a new set of MRIs revealed, not only a major chaiphosis in my neck, but a condition known as syringomyelia and a massive syrinx that ran from my level of injury at C 4-5 all the way down to the base of my spine, with a syrinx to spinal cord ratio of about 95%. It was suggested that, given its size, it had been expanding for many years (perhaps as long as 10) and was most likely the cause of both my pelvic sensation loss and my pain.
Admittedly, looking at my MRIs that afternoon, I was both relieved and nervous. Relieved, because at last I had a diagnosis and an explanation of what was happening to me, and nervous because, well… of two things, really; one, I’m intimately familiar with what happens to a spinal cord when it’s stretched or compressed for an extended period of time — neurons die. And two, I was told the usual solution to this problem was surgery.
And while I’m not somebody who dwells on past decisions or has regrets about things I’ve done, I do like to subscribe to the philosophy; do things for the you of today that the you of tomorrow will be thankful for. Because, c’mon, really, the last thing I want the me of tomorrow saying about the me of today is, “Gee, what a f—-n’ dumb ass”. So, yeah, it was a little difficult not to question what the hell I was thinking for so many years.
But I digress.
So, with my head spinning, I left that initial appointment with these three things:
- a follow-up appointment with a neurosurgeon.
- an understanding that my syrinx was probably expanding and therefore causing more damage.
- a desire to Google syringomyelia.
Be careful what you Google for
One of the first things I discovered about syringomyelia was that it kind of exists in that gray area of medical conditions, where it affects just enough people to draw the attention of the neuroscience community and generate a few websites, but not enough to be considered “sexy” and garner mainstream attention, celebrity spokespersons and research dollars. All of which could go a long way in advancing the further understanding of the condition and stimulate cutting edge (no pun intended) surgical techniques for treatment.
But as I’ve said on several occasions, and I think it bears repeating here, googling medical conditions is a sketchy prospect. On the one hand you might find what you’re looking for, but on the other, you might also find what you’re looking for. The point being, any condition you research is going to have a list of symptoms or facts ranging from the rather benign to the downright scary, and for whatever reason our attention always seems to fall on the scary. And mine was no exception. But since I was already dealing with symptoms that were categorically sketchy, the things I found out about syringomyelia — including the above — were less of a shock and more informative, giving me a much more pragmatic viewpoint than I had just hours before when I was looking at my MRIs in the doctors office.
Questions to answers to questions
No doubt, having an exhaustive breakdown of syringomyelia was immensely valuable, but in the end, this information felt somewhat broad. One of the great things about the websites I visited, however, was that most had forums, and for me this is where my diagnosis got personal and began to take shape. But unlike most forums out there (cooking, tech, what-have-you), medical forums — 99% of the time — will never be able to give you the exact answer you’re looking for. And how could they? Any medical condition one might have is going to come with an infinite array of unique factors that may or may not pertain directly to a clinical diagnosis. In other words, your mileage may vary. Which is fine, if you understand this going in, otherwise, you may come away with a skewed understanding of your particular situation and a very depressing outlook on your future. Because, medical forums are about people with problems looking for answers, they’re generally not — in my experience anyway — happy-feely places loaded with success stories. Which isn’t a bad thing — there are valuable nuggets of wisdom to be found from other peoples difficult experiences and/or questions, you just need to know how to apply them.
Opinions are like…
True (if you carry that expression out to its natural conclusion). But if I took anything away from my research, it was this; find a neurosurgeon experienced with syringomyelia and get several opinions. Obvious advice, to be sure, but it’s easier said than done. As I said above, syringomyelia is not the most common of neurological disorders, and even if it were, it’s not like I attend cocktail parties with neurosurgeons. No, aside from Dean Chou recommended by UCSF, I had to bite the bullet, tell family and friends of my condition, and ask if they could pass on my situation to their friends and family to see if anyone knew of any good neurosurgeons. Within two weeks — surprisingly or not so surprisingly — I had six, very well-qualified neurosurgeons to choose from. From these, I chose to schedule appointments with three; Bruce McCormack at the Neurospine Institute Medical Group, Larry Shuer at Stanford and Langston Holly/Ulrich Batzdorf at UCLA.
From my research, among the more important things I learned before going into these appointments, was that the standard way of dealing with syringomyelia was to insert a shunt into the spinal cord to drain the cerebral spinal fluid (CSF) from the syrinx. I pretty much knew that, unilaterally, this would be the recommended course of treatment, no matter how much I wanted to hear of some other new technique that would avoid placing this kind of hardware in my spine. Even still, I had questions; where would the shunt be placed, how long would the surgery be and what would it entail, who would be assisting with the surgery, i.e. a physician’s assistant or another neurosurgeon, how long would I be in the hospital, how long would my recovery be once I was released, and — you know, because one has to have priorities — would I be able to ski again?
But also — and almost equally important — I wanted to get a sense of who would be working with me, i.e. did we gel and what did my intuition tell me about them — understanding full well neurosurgeons aren’t typically the most effervescent crayons in the box. In the end, however, if you’re going to be cutting into my spinal cord, I’d more than likely go with skill over a sparkly countenance, but I’m also wise enough to know you can have both… or you should at least look for it.
Of the four doctors I met with, their experience, opinion on treatment and personalities varied enough to differentiate them from one another. The first doctor I met with, Dean Chou, suggested two shunts; one directly below my level of injury, and a second lower down, in case the syrinx was segmented. The shunts would run from my spinal cord and drain into the cavity around my lungs, requiring another incision and procedure from the front. Bruce McCormack recommended essentially the same thing, however, felt that two shunts were unnecessary due to the fact that syrinxes are rarely segmented. He also felt that the shunt should drain into the abdomen region rather than around the lungs, a more typical location. Both Larry Shuer and Langston Holly also recommended single shunts, but unlike the first two doctors both wanted me to have a CT myelogram before they could say anything specific about where the shunt would be placed. The difference between Shuer and Holly, was that Larry Shuer also suggested that, given the extreme size of my syrinx, an alternative, more experimental procedure, which didn’t involve shunting, might be a possibility — a terminal ventriculostomy. A suggestion that admittedly piqued my interest.
Personality wise, I felt comfortable with all four of these doctors — somewhat surprising given the aforementioned rep neurosurgeons have. But in the end I narrowed it down to Larry Shuer and Langston Holly, if for no other reason than they both requested more nuanced scans.[Side note: I met with both Dean Chou and Bruce McCormack at the beginning of 2009 and Larry Shuer and Langston Holly at the end of spring. At each of the initial two appointments, I asked them if surgery needed to be done immediately — my ski season was just getting under way and I wanted to know if either felt it would be unwise to put things off until it was over. Both doctors felt the best course of action would be to have surgery as soon as possible, but that given the look of my scans, waiting a few months would probably be fine. Each issued a caveat, however, saying that while my symptoms didn’t appear to be rapidly changing, a jarring accident could change that — there was really no way of knowing. So in the end, it came down to risk versus reward; and since I had no idea what would happen during or after any surgery, but I knew I had the ability to ski at that time, the coarse I chose was to ski my brains out that winter/spring and let the consequences be damned. Carpe diem]
To shunt or not to shunt
Truth be told, I was having a difficult time wrapping my head around the whole shunting part of the surgery. I understood surgery was necessary — that was a no-brainer — if I didn’t do anything things would continue to get worse, and if I had surgery, well, at least I had a chance things would improve. But shunts — aside from the risk of infection or the fact that they usually need to be replaced at some point due to dislodgment or clogging — seemed so… oh, I don’t know… clumsy. Which only hammered home how little was known about syringomyelia and the lack of cutting edge surgical techniques in its treatment. Aside from that, and probably more importantly, I didn’t really see myself as the kind of guy who had hardware inside him — I see myself as a strong, healthy individual and not one who needs artificial parts to function properly.
So what to do?
No doubt, the option to go with the terminal ventriculostomy, and avoid a shunt altogether, was appealing if for no other reason than that. But, the caveat with that technique — aside from the surgeon who suggested it had never performed one before (which truthfully wasn’t an issue, given that either way my spinal cord would be cut into) — was the simple fact that it wasn’t done very often. And when it was, the little information I could find, seemed to suggest it was only marginally successful. Still, the pull in that direction was strong — I really didn’t want a shunt. And besides, it could be argued, given my unique situation, I was the ideal candidate. But if I was going to roll the dice, and be somewhat of a guinea pig, I wanted to feel confident my reasoning was sound. Meaning; was I avoiding the shunt because I didn’t see myself as a shunt kind of guy? Or, given my extreme situation, was a terminal ventriculostomy a truly valid option?
Well, after much research, soul-searching, and the advice of a cousin of mine who’s a spine surgeon — I decided to take the more conventional route and go with the shunt. It wasn’t an easy decision, by any means, but given the risks versus rewards — namely if it didn’t work I’d have to get right back under the knife again — it seemed like the prudent thing to do. Of course, there was really no way of knowing what would be best, but it was a decision where — regardless of the outcome — I could live with my reasoning.
Yeah, but what about…
When ski season came to a close at the end of April, it was alarmingly clear I needed to get on with scheduling surgery. I had taken several hard spills over the course of the winter and spring, and whether or not it had anything to with my escalating symptoms of more sensation loss and weakness in my left arm (some would suggest it did), things were definitely getting worse and needed to be arrested.
However, here’s the thing; while I was mostly comfortable with my decision about the surgery — even though I had yet to choose a surgeon — what I wasn’t sure about was my postsurgical recovery. Definitely a concern, if for no other reason than it would help me mentally prepare for what lay ahead. The doctors said it would be about a month, but their experience was primarily with able-bodied persons — or at the very least, paraplegics — and their abilities and needs were far different from my own. As a fairly high-level quadriplegic, just getting in and out of bed could be problematic; I have to be lifted, which, under the best of circumstances, stretches my spine — a potential issue after major spinal surgery. Also, in order to do pressure relief while in my chair, I need to do a bit of a yoga move, where I lean over onto my knees, arms dangling at my side, with my head almost touching my toes to get the full pressure off of my butt — a serious stretch, which, again, seemed like a potential issue after spinal surgery.
So back to the forums I went, trying to get some sort of sense of what I could expect. And while I couldn’t find a single quad who’d gone through this type of surgery for me to bounce my questions off of (though they’ve got to be out there), I was able to get in touch with some paras, and what they said differed greatly from the prognosis my doctors gave me. Eye-opening, to say the least; recovery — based on their anecdotes — was a rather protracted and difficult affair. And while it wasn’t easy to hear, it was important to wrap my head around, because in the end it would serve to prepare me for either outcome — short and simple or long and challenging.
But obviously it wasn’t the short and simple that concerned me, it was the long and challenging. And not for the reasons one might think. I’m perfectly fine with physical challenges. Difficult or otherwise, it’s the story of my life. But if I’m going to couple a risky surgery, that carries with it a possibility of inflicting more harm than good, with that of a protracted recovery, well, then I better be ready to commit to a new direction in my life. Which I was (am). But I’m also all about committing to the present moment. And at that time — despite the growing sensation loss and weakness in my left arm — I felt I was at a crossroads. In other words, if there was something I wanted to do, I should seize the day and do it, as there were no guarantees after the surgery.
And so for about three days there, I seriously thought about packing up and going to South America or Spain. I’d never been out of the country before (aside from surf trips into neighboring Baja) and I was perfectly willing to accept any collateral damage that came with putting off surgery in exchange for traveling with the strength and physical mobility I still had. Don’t get me wrong, I didn’t want to lose any more function — I’m a high enough quad as it is — but like I said, the surgery was risky and came with no guarantees.
In the end, however, I decided to go with getting the surgery and put off my traveling until next year. I felt I had enough information about the recovery and faith in my own stellar health to roll the dice and attempt to arrest the problem where it was. Sure, others had a tough go of it and, yeah, I was a quadriplegic, but damn if I wasn’t going to be some sort of postsurgical, medical journal worthy, recovery phenomenon.
The first slice
On September 21st, almost 6 months after my diagnosis, I had my surgery at UCLA with neurosurgeons Langston Holly and Ulrich Batzdorf. Of course, I was out cold for the entire six hours of the procedure, but roughly here’s what went down (or so I’ve been told): In order to facilitate the draining of the syrinx, the surgeons cut into my back between my shoulders, cut through the dura surrounding my spine, removed some bone at T 5-6 to access my spinal cord (a location chosen for its proximity to my level of injury, but far enough away so if the spinal cord was damaged during surgery I wouldn’t lose any mobility function), inserted a 6 inch shunt into my spinal cord, which would drain the CSF into my dura and be reabsorbed back into my body, closed me up and then stapled the whole thing shut with 23 staples. Simple.
All told, I was in the hospital four days, released and spent two more weeks in Palm Desert recovering at my folk’s house before heading back to Berkeley to resume some semblance of my previous life. As far as the recovery went, I was spot on — there were no medical hiccups and I healed quickly. Of course, I’ve never had surgery before, so I don’t have anything to compare it to, but I’m going to go out on a limb and say, yeah, I’m some sort of postsurgical, medical journal worthy, recovery phenomenon.
The hospital stay itself, was a bit of a blur — drugs and anesthesia have a way of doing that, I suppose — but that’s probably not a bad thing considering the circumstances. Palm Desert, on the other hand, was an unexpected and memorable gift. I anticipated the benefits of being around family, but I didn’t really consider what that would mean outside of the obvious loving vibes, great food, etc.. The relaxed nature of the desert, the hot weather, not having to worry about making meals (I ate enough seafood to be rung out and used as a mercury source for a large thermometer company), allowed me to work on my only two self assigned jobs of healing and completing the first season of Dexter.
During those two weeks, I was able to get into my chair at least two or three times a day for about an hour at a time, and that was more than sufficient — any longer and my dysreflexia would kick in. As I said above, I don’t have much experience in these matters, but I’d say if you’ve got a surgery to recover from, you might want to call the Schmiesing’s in Palm Desert. Because truthfully, I’d say my speedy recovery owes as much to my folks’ unconditional hospitality as it does to the incredible healing abilities of my body.
Home again, home again
On October 6th, two weeks after my surgery, my staples were removed and I was given the green light by my neurosurgeons to head back to Berkeley. At the appointment, they asked me if I noticed any changes for the better or worse, and I found the question difficult to answer. All things considered, I felt pretty good, but until I was able to return to my normal life’s activities, I had no frame of reference. I hadn’t exercised in two weeks, my muscles had atrophied and I was still feeling somewhat dysreflexic. I didn’t feel any damage had been caused by the surgery, but again, it was difficult to say until things re-equalized — my sensation in general is difficult to pin down and really a matter of nuanced degrees.
My instructions for Berkeley were simple: no skiing (drag city), no pushing for at least two weeks, and then to take it slowly after that, seeing how I felt on a day-to-day basis. Two weeks on the nose, I was back on the track, trying to pick up where I left off, but it felt as though I hadn’t worked out for about a year. I was weak — my left arm especially, locking up every third rotation — and the time it usually took me to do a mile and a half, I could only do a mile. My incision healed magnificently, but the affected areas from the syrinx, not so much, and it became patently obvious this portion of my recovery might take longer than I hoped.
But how much?
That was then, this is now (December 11, second follow-up appointment et al.)
“So when you say subtle changes for the better, what exactly do you mean?” I asked my doctor. “Subtle seems kind of vague… like the word may. Like in a news story; “the bag may have contained heroin.” Which is true. But it may have also contained donuts. So is subtle something I could see?”
The doctor smiled slightly, getting my meaning. “Yes. There’s a subtle change in the size of the syrinx at the base of your spine and up towards the level of your injury. It shows up as more definition on the MRI.”
“Cool. A little better is better than a little worse. But is it going to drain more?”
“It should. Typically, in cases such as yours, and for reasons we don’t quite understand, the drainage at this early stage is slight, but in the coming months we should see a more substantial decrease in the syrinx’s size.”
“But what about my arm? It definitely feels weaker than before the surgery. Especially when I’m on the track pushing, it’s almost like it becomes paralyzed or something.” I demonstrated my pushing motion. “Every fourth push or so, I have to stop, wait a second, let it recharge, and then go again. It’s very frustrating.”
“Well, there’s nothing on the MRI to suggest it’s neurologically related to the syrinx, so perhaps it’s a fatigue issue with your deltoids. Try drinking some Gatorade a half-hour before you work out and see if that helps.”
“Gatorade?” I laughed, almost in disbelief. “You think it’s an electrolyte thing?”
“Perhaps, but it’s all I can suggest at the moment. Give it a try”.
Okay, so I was expecting something a little more neurological from my neurosurgeon, but okay, Gatorade, I can go with that. It’s a bit low-fi, but it’s simple enough to put into action.
“So, can I ski this winter?”
Say yes. Say yes. Say yes.
“I don’t think that would be a good idea, no. It’s still too early.”
“What about spring?”.
“Maybe. Let’s see how the MRIs look in March and we’ll discuss it then.”
I digested that for a second then asked, “And why can’t I ski exactly?”
The entire room burst into laughter; my dad, my good friend and, yes, my neurosurgeon… everyone but I. Clearly my extraordinary healing abilities weren’t appreciated.
“Because you just had major spinal surgery.” My doctor said, still laughing. “We usually like people to wait at least a year before doing anything extreme.”
“Yeah, yeah, I know that. But I mean, what are we afraid is going to happen?”
“The shunt hasn’t had enough time to secure itself and could come loose”.
“Fair enough.” I said, understanding March still gave me a couple of months to ski out the season.
He paused for a moment, looking me in the eyes, to confirm I understood the gravity of my situation, then asked, “So when in March would you like to schedule your MRI and follow up?”
“The first week.” I said without hesitation. “Definitely the first week”.
And there you have it, three months into this whole shunt thing and the doctor’s orders are to drink Gatorade and don’t ski… who would’ve thought (well, the skiing part, yeah, I would’ve thought). All in all, I have to say, I feel pretty good; the weakness in my left arm is still a major issue and I’m still having some pain in my neck from time to time, but I’m drinking the Gatorade and trying to work my way through it. Quite honestly, I think there’s more going on than an electrolyte deficiency, but either way, it’s encouraging to know that whatever is happening, it’s not related to the shunt. Because the fact is, like it or not, I’m now a shunt type of guy.
*Actually, there was a celebrity spokesperson on one of the sites I visited, a hobbit — or should I say Sean Astin — of “Lord of the Rings” and “Rudy” fame. Not exactly an A-list celebrity, I know, and truthfully I don’t even know if the PSA he did ran anywhere other than the particular website it was on, but he is a celebrity nonetheless, and I’ve got to give him credit for taking the time to do it. So, yeah, thanks, Sean.