Explain Syringomyelia to Friends: Part 3: Why This Surgery?

This is the third blog entry where I explain why I'm having spinal detethering surgery to family and friends.

Syringomyelia can be caused by multiple different things. In my case, we believe it is being caused abnormal tension on my cord. Here is my understanding of why we are treating syringomyelia--which effects much of my spinal cord--by simply snipping the filum at the bottom (pasted from recent email):

Children born with obvious spina bifida are well known in
medicine, and treated young. However, tethered cord is not
a black-or-white condition. Children with borderline
("occult") spina bifida (a.k.a. occult tethered cord) are
sometimes treated by experts like Dr. W. Adults who
have grown up with spina bifida occulta (me) may not have
serious symptoms until mid age--when more cord damage has
accumulated. Most of these adults are diagnosed with
chronic pain syndromes like fibromyalgia or if they have
syringomyelia (SM) on an MRI, "idiopathic" SM. Most slowly
deteriorate without help, because adult-onset tethered cord
is not widely understood. This is changing (see research links
on lower right of my blog).

Dr. X takes advantage of the gap between vertebra to
minimize bone removal in the lumbar spine; he/she doesn't do a
full laminectomy. She only needs about an 8mm cut of the
dura (spinal sac). She says the procedure takes her about
half an hour. She has done thousands of the procedures
with very few complications. She considers my case
straight-forward, and expects I would have relief of some pain and
numbness. She reports that %90+ of patients like me have
neurologic improvement after detethering. I told her I'm
not getting my hopes up, but I'd be happy just to stabilize
my condition.

As I said in that email, my main hope is just to stabilze my condition, so I don't keep getting worse. There is a little permanent damage to my spinal cord, so I probably won't be back to the old crazy-endurance-sports me. About six months to a year after surgery, I should have a good idea of how it worked.

Explain Syringomyelia to Friends: Part Two, My History

This is the second blog entry where I explain to my family and friends the history of my spinal cord problems (syringomyelia) and why I'm having surgery.

Read Part One: The Surgery here.

I don't like to complain, but it is time I explained this to my friends and family.

For the last 3-4 years, I've been having increasing difficulty. What started out as a sore foot and then back pain ended up also including numbness, weakness, and for the last couple of years, constant pain.

I've had a lot of symptoms all over, so finding a diagnosis was difficult. MRIs in 2005 of my cervical (neck) spinal cord revealed a syrinx; a fluid-filled cavity in the center of my cord.

Cervical MRIs showing syrinx in spinal cord.

This condition is called syringomyelia (SM), and it was later found in lower parts of my spinal cord as well.

Now that I knew I had syringomyelia (SM), I had to find out why. SM is always caused by something else; it is the effect of something causing damage to your spinal cord. I didn't have the most common cause of SM (chiari malformation). I was told multiple times that it
was uncurable, so in 2006 I concentrated on working and living my life. My prognosis was a slow neurologic decline and chronic, sometimes severe, pain.

People often say, "don't take your health for granted", and I learned why. I was thankful that I didn't have something more deadly, but it was really hard to adjust. Going from running 30 miles a week, mountain biking, snowboarding, hiking, etc., to struggling to walk at times, was tough mentally. With a positive attitute and a lot of support, I dealt with it.

In the mean time, I was active volunteering and keeping up with the latest research. I met many of the top neurosurgeons in the country for this condition. The fact was, adult neurosurgeons didn't understand how to treat my type of SM.

This year (2007), I think I found an answer. In October, we flew to New York to see some experts, and they thought they might know what is causing the damage to my cord. It is a long an complicated story, but multiple neurosurgeons (3) now believe that my spinal cord is tethered
at the bottom. Being strung too tight has damaged my spinal cord over the years, and it is starting to show.

Read Part Three: Theory of Treatment.

Explain Syringomyelia to Friends: Part One, My Surgery

This is the first blog entry where I explain to family and friends what is wrong with my spine and why I'm getting surgery.

The Surgery
I am having the bottom of my spinal cord detethered by having my filum terminale cut. This filum is a thin cord at the bottom end of our spinal cords which does not contain any nerve, but anchors the spinal cord to your "tailbone", the sacrum.

The surgery is minimal as far as neurosurgery goes. I will have an L4 laminotomy (removal of part of the bone at the back of a lower vertebra). Some muscles and stuff will be moved aside, and then the dura (the sack that holds your spinal cord and fluid) will be opened. The neurosurgeon will find the filum amongst a bunch of nerve roots, and then cut it. I will be closed up, and the procedure will be done in an hour or so.

The neurosurgeon doing the procedure is very good at doing it in a very small space. The risks are low, but are primarly due to the fact that the dura is opened, exposing cerebral spinal fluid (CSF), and exposing nerve roots. The most common complication is a post-op CSF leak, which I hope we will avoid.

Read Part Two: History of my Struggles

Posted more research on Tethered Cord Syndrome

I just posted two more links to recent papers on Tethered Cord Syndrome:

• [paper] Tethered Cord Syndrome: An Updated Review


• [paper] Section of the terminal filum for occult tethered cord syndrome: toward a scientific answer

These can always be found on the links section on the lower right of this blog.

Whether or not to Detether

Now that I have a diagnosis of "occult" tethered cord syndrome (TCS), and I'm a candidate for a detethering (SFT) surgery, I have to make the decision whether or not to detether.

This is a tough decision, and I am taking a three-pronged approach to solving it:

1. Clinical: Are my symptoms bad enough or progressing fast enough to justify moderate risk?
2. Research: What are the published success rates with SFT surgery as a therapy for my type of syringomyelia.
3. Networking: What do patients who've had the SFT surgery have to say about it?

I've solved the first problem; my symptoms and progression warrant moderate surgical risk (as much as I hate to say it). The second problem is tricky, as the idea of tethered cord syndrome (TCS) which becomes symptomatic in adulthood (possibly causing terminal syringomyelia) is a new idea. (See Related Links on this site for recent research). Still, I'm reading, talking to physicians, and learning about current research.

The third prong of my decision-making attack relies on talking with patients who have had the SFT surgery. This is where I need your help.

Tell Me About Your SFT Surgery

There are two ways you can share your surgical story with me. First, you can send me a private message by clicking on my profile, and using the Email link on the left. The other option is to share your thoughts with all my readers by leaving a comment at the bottom of this article. This has the benefit of helping others who may be trying to make this decision.

I've also added a couple of (unscientific) polls which you can vote on if you've had the SFT surgery.

Trip to the Chiari Institute: Part Two: I am a Candidate

Announcing my Candidacy

Continued from part one...

After talking with the neurologist and then the neurosurgeon, I was informed that I was a candidate for detethering surgery. I was handed a card with a number I could call to schedule the surgery.

Wow. After years of searching for a diagnosis, and then being told repeatedly that there was no treatment or cure, suddenly someone thinks they can help.

I had been training myself not to get my hopes up, so I'd be disappointed when I was, again, told I could not be helped. I was working hard on accepting a slow neurological decline and a life of constant pain. Now, suddenly I was given a chance at stabilizing my condition. With that chance comes the weight of a decision that I can't make easily; should I have surgery, before my spinal cord is damaged much more, or should I not take the risk and be thankful for the function that I have now.

Trip to the Chiari Institute: Part One: Miscommunication

Clarification: this article is being misquoted as saying that I went ahead with the surgery and was positive about it. One, I did not have surgery at TCI due to these obvious issues with them. Two I'm not positive about the outcome of this surgery in my case even though I had another excellent surgeon do it. That other surgeon has a much less invasive approach so if anything my results should have been better. However, the surgery did not live up to the hugely positive claims made by TCI. I was left in much the same condition as before.

Introduction: The Chiari Institute

As of today, 2007, there are only a hand full of neurologists and neurosurgeons in the United States who specialize in Syringomyelia (SM), Chiari Malformation, and related disorders. In Long Island, New York, there is a center, called The Chiari Institute, which specializes in treating these disorders. The Chiari Institute is so well known among Chiari and SM patients, that people just call it TCI, and you know what they are talking about.

The good thing about TCI is that they know these diseases, and they have a lot of practice with their specific surgical treatments. On the other hand, they are very busy and it takes a lot of patience to get an appointment.

Why I Went to TCI

I decided to go to The Chiari Institute because I had met and talked to two physicians who worked there, at the ASAP medical conferences over the last two years. In specific, I talked with Dr. B, who was a neurosurgeon that recognized my symptoms as a possible case of Occult Tethered Cord Sydrome (TCS).

I had been looking for the cause of my SM, and no experts so far were able to explain it (beyond conjecture that it is idiopatic, congenital, and uncurable). When Dr. B had me walk on my heels, and had my wife pull on my legs, the symptoms it elicited were a perfect match.

Thus, I decided to fly myself and my wife out to New York to meet with Dr. B.

Miscommunication: We've changed your Neurosurgeon

We arrived at TCI early on October 22, 2007. After sitting down with a nurse and giving a detailed symptom and treatment history, she told us that I will not be seeing Dr. Bolognese, because he is out of town for a conference.

What?

This was after I explicitly said that I was coming exactly to see this physician, and that I was to know ahead of time if this was going to be a problem. I even had confirmation via email that I was going to see Dr. B. I had just spent thousands of dollars to come to TCI and get fresh MRIs, so we were not happy about this.

Part Two: Treating Neuropathic Pain Without Cognitive Deficit

As promised, I will review some of the medications I have tried for treating my neuropathic (nerve) pain associated with syringomyelia. Although it varies a lot from person to person, the type of pain I experience may be similiar to that which people with Chiari Malformation, Tethered Cord Syndrome, post-herpetic or diabetic neuralgia might experience.

I'll stress again that each person responds differently to medication, and that diet and exercise are the first line of defense in managing such an illness.

Here are the medications I've tried, in chronological order, along with a final grade (A-F) as to how helpful they were overall.

Paxil: F
When I originally started having neurological symptoms such as numbness and weakness, my primary care physician (PCP) stated that I must just be depressed, and wanted me to try taking Paxil.
Pain Relief: Paxil did not help my pain levels, although I took it for about a year.
Motivation: Paxil made me sleepy, ambivalent about life, and very unmotivated.
Cognition: If anything, my concentration was worse with Paxil.
Other Thoughts: Paxil did not help with my pain and other neuro. symptoms. It did make me occasionally manic, which was a bit fun I suppose. It lessened my libido, IIRC.

Vicodin: C
Narcotics are not very helpful with neuropathic pain. They do help you sleep, and can be useful occasionally to take a break from overwhelming discomfort. Vicodin midly worsened my concentration, and made me feel a little depressed if I took it for more than a day or two.

Neurontin (Gabapentin): B
Pain Relief: Neurontin was very helpful when I first started taking it. I could concentrate much better at work because it reduced my pain significantly. Your body seems to get used to it, however, even if you increase the dose. It feels like it still helps, but not as much as when I first started it.
Motivation: Not much of an effect, slightly sleepier.
Cognition: Tolerable, but seems to hurt short term memory a bit.
Other Thoughts: Very addictive in that withdrawls are painful. It is an anti-seisure drug, and if you quit taking a large amount quickly, you can actually induce seisures, or so I've heard. When I forget to take a dose before bed, I have crazy nightmares and sweats.

SNRIs: Effexor and Cymbalta: C-
I tried both Effexor and Cymbalta, which are similar anti-depressant drugs also used for treating neuropathic pain. One theory is that chronic pain causes depletion of certain neurotransmitters, and these reuptake inhibitors help restore the balance, making you feel better.
Pain Relief: Cymbalta was great for my pain, but I was an idiot, when I wasn't vomiting.
Motivation: These drugs made it a struggle to get out of bed and stay focused-- similiar to how I felt on Paxil, but with some pain relief at least.
Cognition: Wee! Happy times! I'm stupid! For me, these drugs made it hard to concentrate on work.
Other Thoughts: Effexor made me vomit when I started or increased a dose. I discontinued it in one week. When I tried Cymbalta, I added an anti-nausea drug, which at least let me avoid vomiting. I hung in there for a good month, and had pain relief. Even after the nausea faded, I still couldn't concentrate on work, so I quit taking it. If I didn't have a job, I'd try it again.

TCA #1: Nortriptyline: D
Nortriptyline is an old, second-generation Tricyclic Antidepressant that has found some off-label use for chronic pain and neuralgia.
Pain Relief: I didn't notice much pain reduction from Nortriptyline, which might have changed if I took it longer or at a higher dose.
Motivation: This one made me sleepy and less motivated.
Cognition: I felt foggy and less alert on Nortriptyline.
Other Thoughts: You need to take these drugs for some time (say, a month) before noticing their full impact. After trying this one for two weeks, we knew that the sleepiness would be untolerable, even if I did attain pain relief. I decided to try Desipramine instead.

TCA #2: Desipramine: B- ?
Desipramine works better for me than Nortriptyline. After working up to a 50mg dose over five weeks, I seem to be noticing a reduction in pain complaints. The side effects are tolerable: dry mouth, constipation, trouble sleeping (for a couple days any time I increase the dose), slight fogginess. Desipramine seems to have the energizing feeling I felt with the SNRIs (Cymbalta, etc.) without the sleepiness and ambivalence I experience on SRIs (Paxil, also SNRIs, Nortriptyline).
Pain Relief: Seems to be a bit better at five weeks, 50mg.
Motivation: Seems unaffected.
Cognition: A bit foggy/air-headed, but tolerable.
Other Thoughts: Everybody reacts differently to these medicines. This one works OK for me so far.


More to come
I finish this article when I get time.

Treating Neuropathic Pain without Cognitive Deficit: A Trial and Error Evaluation of Drugs

Disclaimer: Drugs are not the only way to manage pain. Exercise, relaxation techniques, as well as behavioral and phychological links, should all be explored. For serious cases of pain such as spinal cord injury, however, medicine is an invaluable tool to improve quality of life.

Central Neuropathic Pain: The Problem
Treating neuropathic pain is a challenging problem. Reducing pain without creating an imbicile zombie is even more challenging. There are number of aspects that make treatment difficult:

• "Pain" is a complex and unique target: Spinal cord injury/disease, for instance causes more than just a classic "funny bone" shooting pain sensation. There can be increased muscle tone (spacticity), burning, vibrating, throbbing, or stabbing sensations, and so on. In a case like Chiari Malformation or Arachnoiditis, there can be transient, local, increased intracranial or subarachnoid pressure. That is, your head or neck can be throbbing, and neural tissues *are* being impacted.


• Everybody responds to drugs differently.
As an oversimplification, your biochemistry is as unique as your fingerprints. A drug which relieves pain for one person's may only make another sick.


• Different people have different requirements. Some people would be happy to trade some short-term memory or alertness for pain relief. Others may have careers with little room for such concessions.
  


• Many pain medications affect reasoning, memory, mood, motivation, and energy level.


• Pain is subjective. This is obvious, but the fact that it is our perception of pain, and not just its cause (pathogenesis) that we wish to treat, makes the process more complicated. (If don't feel a pinch, did it happen?)

Requirements For Finding Helpful Medicines
Because of the variability in treating each case of "pain", finding drugs which help is largely a trial-and-error process. Finding a medicine which helps improve perception of pain thus requires:

• Knowing which drugs to try.You can't try every drug known to man, you must narrow your search.


• Trying until something helps. A helpful medicine is one which causes relief from pain while having tolerable side-effects.
  

My Personal Requirements
My pain management goals are, most important first:

• Improve cognition.My pain, untreated, greatly diminishes my ability to be productive at my highly-cerebral job. Try completing a long division math problem while someone slowly stabs you and you'll know what I mean. Research has shown that chronic pain causes cognitive problems ([1], [2]). In fact, chronic pain actually causes your brain to shrink over time.


• Reduce my level of suffering, or perceived pain.

Basically, I want to reduce suffering without causing cognitive issues. This is a balancing act, as reduction of pain definately helps my concentration and reasoning, but many medicines make me tired, foggy, or affect my memory.

What Worked or Didn't Work for Me?
Stay tuned for my next post, where I'll present a history of which medicines I've tried, and how they worked for me. Some made me sleep all day. Others kept me awake all night. Some made me violently ill (barf-o-rama).

Read part two.

MRIs: That will be $3000 plus $10 if you want to see it.

A quick rant about a pet-peeve of mine. Last year I spent thousands on MRIs. I never received a CD or a personal copy of the images. This year I plan on seeing a new neurosurgeon. All the neurosurgeons I've seen so far request that you "hand carry all films". This is because they don't want the hassle of handling your records when they'll probably only see you once.

When I just faxed in a request for a CD containing my latest MRI images, the records staff insisted on charging me $10 to get a "personal copy" of my images. It is not that I cannot afford the ten dollars. I just feel insulted that they provide records free of charge to other physicians, but must charge me even for the first copy I receive.

I told them to ask their neurosurgeons why they require their patients to hand-carry films. Then, I said, feel free to bill my insurance.