A Small Syrinx can cause Big Problems: Tell the doctors

Someone on the asap.org message boards started a thread asking people with "small" syrinxes to report their symptoms. I decided to post my response here, along with extra late night rambling.
Many of us struggle with physicians who seem to believe that symptoms correlate strongly with syrinx size, even though there is no supporting research. I say the patient's own senses are a powerful diagnostic tool; listen to your patient.

I have 3-4mm syrinx at widest.. c4-c7 and t4-t12.. No chiari visible on brain mri. (diagnosis 8/2005).

I have gone from being very athletic and active to struggling to walk at times (poor proprioception, weakness in legs)

I have pain in the neck (from c7 up), and a lot of muscle spacticity/tightness long my spine and in my legs. I have headaches with pressure sensation in head and neck. Almost constant throbbing sensation in head and neck. A numb patch in throat comes and goes and I have trouble swallowing sometimes. For a while, I had some annoying frequent urination and leaking sensations. I often have weak/shaky legs, numbness in feet, heels, hands, elbows (comes and goes).

It is *hard* to find a doctor who believes that the syrinx (or its cause) is the root of my symptoms, even though the cervical location is consistent with the numbness in my hands and arms, my first warning symptom. Although I was in tears in pain multiple times last week, my neurologist will only prescribe neurontin and a little flexeril if I beg.. She doesn't seem to believe that my syrinx(s) causes the symptoms I'm reporting. She told me I should see a PCP if I have "tight muscles" because she's a "nerve doctor". This spacticity is messing up my body. I exercise and stretch an hour a day.

No neurologists or PCPs specialize in SM, so finding a doctor who will treat appropriately is trial and error. I don't want to be popping pills, but I don't want to feel like dying either. Surgical treatment of SM without ACM is just starting to show some minimal promise. We need to improve this situation...

Web button for fundraising

You may have noticed the button I just added to this site.



I invite any fellow bloggers or website authors to steal it and use it to help raise funds for ASAP. You can steal the image and link, or just paste this HTML into your blog (which hosts the image of my unreliable home server):

<a href="http://asap.org/donation.html"><img src="http://smnw.selfip.org/asap-button.png"></a>

Highlights from my Spine MRIs

Click on images to enlarge.



First up, we have a lovely (axial T2-weighted) cervical spine MRI image showing my cervical syrinx. The donut looking thing in the middle is my spinal cord. The hole in the middle is the syrinx. The syrinx is a gap filled with cerebral-spinal fluid (CSF) which has been forced into my spinal cord by some sinister and yet unidentified force. These first couple of images are axial slices. That means they're 2-d slices made in the plane parallel to the floor if we were standing up. To simplify, it's the same cross section you'd see if you chopped someone's head clean off at the neck.






Next on our lovely slide show we have another axial slice, this time lower in the thoracic spine. Again you can see a white dot in the center of my spinal cord. This is my long-skinny thoracic (middle back) syrinx which runs about from T4 through T12.




Now on to the sagittal images. These slices are taken in the sagittal plane, which is what you'd see if you sliced someone clean in half from head to toe. This is a (T2) MRI of my cspine again. This time you can see the dark spinal cord coming down from the brain (to the right of the vertebrae and discs). You'll notice the white bubble in the center of it around C4-C6. That's the same syrinx that looks like a donut in the first picture. This talk of donuts is making me hungry.





Next we have a sagittal (T2) mri of my thoracic spine. Look for the thin white line in the dark spinal cord. When I first heard I had another syrinx from T4-T12, I was upset; that sounds really long. I felt a little bit better when I saw that it's fairly thin. It does get a little bit wider as it settles around T12, but it's hard to find in the MRI images.




This is a sagittal image (T2) from my thoracic and lumbar spine (lower back). Of note are the white line in the spinal cord at the top, and the seen-better-days L5-S1 disc at the bottom. It's turning black and it should be white; it's degenerative and bulging. Many people with SM experience multiple DDD (degenerative disc disease) and disc herniations. My guess is that it is from muscle spacticity. My paraspinal muscles and neck muscles just don't relax. They are way to tight.


Finally, here is a link to a movie which shows a sequence of axial cervical spine (neck) images. It moves from my upper back towards my head, one image per second.

t2-ax-cspine.avi

(Right-click on the link, choose "Save As", and save it to your Desktop. Then, open it from there.)

Enjoy. This stuff is putting me to sleep and giving me a headache. Good night. Zzzzzzz....

Starting a Support Group. Who, Me?

Today I explain why I'm starting a support group, something I've never considered before.

Dealing with Syringomyelia over the last couple of years has had its difficult moments. I've gone from being an accomplished hiker and mountain biker to struggling to walk at times. I used to take my health and ability to concentrate for granted. Now I'm thankful whenever I can move or concentrate on something without the distraction of pain.

I do not suffer without support. I am so fortunate to have married the girl of my dreams last year, expanding my family with more people who care about me. I am so thankful for all my friends and family; they are a wonderful support system for me.

Why, then, is it difficult to talk to them about my condition? Why would I ever need more support than what I have now?

For one thing, it is hard to explain the cereberal spinal fluid dynamics of hydromyelia to someone when they just want to know why you're not going hiking like you usually would. A simplified explanation often falls short. "Just take some ibuprofen if your neck hurts," or "come on, tough it out," are likely to be responses. My friends and family care about me, but I need to explain the predicament I'm in so they can understand. If I say "I have a disease and it's bad so I can't do stuff," that only piques curiosity.

To be honest, I do not mind explaining my condition to people. In fact, I need to vent about it on a regular basis. I start to feel a little alienated, however, when I explain what is wrong with me. Suddenly they understand, and that makes me different. It is hard to explain what I mean.

I also think about the people I talk to about this. My wonderful wife takes the brunt of my complaints and worries with stride, but it is hard on her. She feels bad because she cannot solve my problems. She cares about me deeply and when I talk of pain, she feels it too. When she is sad, it makes me sad. You can see how this could become a cycle of negative moods.

To break this cycle, to help those I love, and to help myself, I've decided to start a support group. I will volunteer my time to help people in my area with syringomyelia and chiari malformation meet and talk. Those who suffer need extraordinary support, and I believe they can help keep balance in their own social lives by getting together.

I threw up a web site for the new Syringomyelia and Chiari Northwest Support Group today, let me know what you think.

A Neurosurgeon Who Knows What a Cine MRI Is

On Monday I saw a new neurosurgeon, Dr. V, at Harborview/UW Spine. Dr. V is a younger physician who studied with Dr. E, a top expert in Chiari malformations and hydromyelia (a.k.a. syringomyelia). It was refreshing to talk with a neurosurgeon who was informed on the latest research in my condition.

The first neurosurgeon I saw back in July of 2005, however, was not as familiar with it. Dr. S, at Swedish Hospital, basically said that my SM could be worse, but it was not curable. He said this without looking at my brain MRI (which Dr. Y's office did not forward to him). He suggested I get a full spine MRI in six months or so, and we called it a day.

Not curable, huh? If I wasn't such a self-preserving geek I may have taken that as the gospel and moved on.

Recent wisdom on syringomyelia (SM), however, indicates that a syrinx does not usually occur on its own; there typically is another condition which forces the cerebrospinal fluid (CSF) into the spinal cord. Furthermore, if you remove the primary condition, the syrinx may resolve or shrink. About 90% of the cases of SM are secondary to an Chiari malformation (CM), where the lower brain plugs the base of the skull like a cork, forcing CSF into the spinal cord.

Consistant with my luck, I have the rarer form of SM. My MRIs do not show a Chiari malformation. There does not appear to be a lack of space at the cervico-medullary junction. I do, however, have some of the symptoms of CM. CM can affect things innervated not by the spinal cord, but by one of the crainial nerves. This is due to the pressure and compression parts of the brain are subjected to with CM. Since my syrinx only goes as high as C5, it should not be causing some of my symptoms, such as nystagmus (twitching eyeball), or the numb patch in the back of my throat, or the difficulty swallowing, each of which come and go. These symptoms are exascerbated by bending over (increasing intracranial pressure). I also get "pressure" headaches in the base of my skull, temples, and so on.

I had a couple of decent head injuries (head hits metal post and head hits concrete, don't try at home). These can cause a small bleed in the brain, which is known to cause scarring and arachnoiditis. Basically, my body would respond to the trauma by growing new membranes or scar tissue which attach to the brain. This tissue can block CSF flow.

Knowing all this, and reading some of the latest research on CM and SM, I began to think I needed to have a CSF flow study to see if we can find any CSF flow obstructions. Cine MRI, a technology developed to visualize blood flow through the heart, is being applied to diagnose syringomyelia.

When I mentioned Cine MRI to my first neurosurgeon, Mr. S, he indicated that he'd heard about it, but remained pessimistic. From the little I know, performing and interpreting the results of a Cine MRI makes reading a regular MRI seem easy (which it is not). I imagine Mr. S just didn't have personal experience with Cine MRIs.

As I worked through the admissions and referral process to get into UW/Harborview Spine, I hoped that they had a neurosurgeon who knew how to use Cine MRI to analyze CSF flow. When Dr. V suggested I do a flow study, without me even mentioning it, I was very happy.

Neurontin (gabapentin) for Pain and Spacticity

One of the first things we did when I started seeing my new neurologist (Dr. D) was to reevaluate any medications I was taking and see if we could improve the management of my pain and muscle tightness (spacticity). At the time, I was taking minimum-dose Paxil (10mg/day), and Vicodin. I was taking the Paxil because I still had some left from my old primary care physician (PCP) Dr. M, who thought my strange neurological symptoms may be due to depression. The Paxil never did help my pain and neurological symptoms. It may have helped me sleep and kept me happier as I dealt with chronic pain, but it never served its intended purpose. We decided I should quit taking it. Second, we looked at my use of Vicodin to manage pain. I complained about the side effects of Vicodin. I was taking it daily for pain, but would wait until I got home from work if at all possible. I would take the lowest dosage possible, but I still experienced grogginess, an exascerbation of my weakness symptoms, and feelings of depression. Being a narcotic, Vicodin tended to make me feel like crap mood-wise if I took it for very long. It really was not the right drug for the job.

Dr. D prescribed Neurontin for me, and I initially took 300mg once and then twice a day. At first I felt euphoric and woozy, but those side-effects wore off within 3-4 days. As I got used to Neurontin, I found that it was much better at managing my pain, and caused fewer side effects compared to Vicodin. I quit taking Vicodin regularly; my pain level was usually tolerable.

While I was taking Neurontin (300mg) only twice a day, I noticed my pain would usually come back in the two to three hours before my next dose. I started taking Vicodin again occasionally after work. I also was still having problems with spacticity. Even though I spent over an hour a day stretching, and had a stand-up desk at work, the back and joint pain from my ever-tightening muscles was a serious problem.

At my next follow-up appointment with Dr. D a month later, I told her how I was doing. We decided that I should start taking the Neurontin three times a day for pain. I mentioned I was having a hard time with spacticity still, and she prescribed a short-term muscle relaxant for me to try out (forget the name now, will fill in later).

Over the next week I started taking the muscle relaxant before bed and increased my neurontin dosage to 300mg three times a day. Although I'd be a little light-headed at work in the morning at first, I had the best week I've had for a long time. I almost felt normal for most of the day. It was wonderful. I sent Dr. D a memo thanking her for listening to me and making a big difference in my symptoms. As my body became used to the increased dose of neurontin, and as I quit taking the muscle relaxant, some of the relief I had faded. I continue to do well, however. I am more productive at work, and happier at home.

Success: A new doctor, a new city.

After wading through the admission beaucracy for OHSU, I finally got scheduled for an appt with Dr. D.. After finally getting Dr. Y in Seattle to send a referral, I was quickly admitted to see my new Neurologist. Upon visiting OHSU for the first time today, I was amazed to see how big the Portland campus is. I'm glad to say that my first appointment with Dr. D. went very well. She listened to me, and was professional and yet personable. We both have a similarly silly sense of humor and the appointment was actually somewhat fun. I think I passed the basic neuro. exam as usual, and she scheduled me for complete spine MRIs, which I completed this evening. I had the joy of laying as still as possible for over two hours in a noisy tube (don't swallow, and don't scratch your face even though it itches like mad). I returned to the Hostel we're staying at in Portland around 10pm. My (newly married and getting used to the word) wife was already in bed. I didn't feel like laying down at all after that MRI marathon, so I went out for a pint of tasty beer at the local McMenamins pub, and now I'm heading to bed. With a job interview starting around 8:00 am, and a medical fun fest the rest of the day, it was a long but good day.

Calling Dr. Y's Boss: Give me a referral please.

I was stuck in a tough spot. My old neurologist, Dr. Y in Seattle, had refused to give me a referral to OHSU Neurology. OHSU Neurology had refused my PCP's referral after a bunch of time and effort. What to do?

I decided to call the P.clinic where Dr. Y practices and explain my problem to a manager or someone who would listen. After reaching a curt (rude) dead end with Dr. Y's office the last couple times I tried to call, I really didn't want to call them again.

Luckily, I got ahold of a friendly, professional care manager, Ms. BW, at the P.clinic. She listened to my story and went and talked to Dr. Y's office. She got them to do the referral and called me back within the half hour. Yay, I get to go through the OSHU referral hoops again. I suppose putting Dr. Y's office through that process is punishment enough for being rude to a patient. Ha ha.

I wonder if Dr. Y read my letter yet. In a way I feel a tiny bit bad for being such a squeaky wheel. I hate to complain in general. When it comes to getting healthcare, unfortunately, you sometimes have to escalate issues and file complaints. The stakes are too high to just give up.

OHSU Neurology: Clinic Manager Calls Me Back

I just got a call back from Jean an OSHU Neurology. I explained the difficulties I had with his office, as described in my previous post. He was apologetic but ultimately unhelpful. To summarize our conversation:

• OSHU Neurology does not take referrals from Primary Care Physicians, only from other Neurologists.
  
• He refused to give his manager's name and number, which I requested should I need to esclate the issue further.
  
• He apologised that his staff failed to tell me that I needed a referral from my Neurologist.
  
• I agreed that it was useful for a new neurologist to have the notes from an old doctor, so they don't have to start over. I mentioned, however, that all there was to know was my diagnosis and history of symptoms, which were provided. I only saw my old neuro Dr. Y once after diagnosis, and Dr. Y refused to do any more referrals.
  

So I have a new neurologist who won't take patients without the old neurologist's referral, and an old neurologist who refused to do a referral. How is that for focusing on the patient?
What would you do next?

OHSU Neurology: Beaucracy ad adsurdum

My current health care plan is this:

1. Get a neurologist in Portland (where I'm moving to) and get a full MRI work-up.
   


2. See a specialist neurosurgeon in Seattle.

The neurosurgeons are on board and ready to see me. They'd prefer that I get a full set of new MRI's done first, but they are willing to order the films if they have to. I'll need a good local neurosurgeon to help me manage my SM in Portland anyways, so I decided to find a neurologist before seeing the neurosurgeons.
This should be a straightforward task. I have a solid diagnosis of a serious neurological condition, so there's no doubt I need a neurologist.
I decided to go to OHSU's neurology clinic since they are probably the biggest center in Portland. Unfortunately, getting in to see a neurologist at OHSU is near impossible. They have the most absurd and useless admissions process I've ever seen. Here is what has happened so far:

• I called the main OHSU neurology line and said I needed a new neurologist in Portland for my diagnosis of Syringomyelia. They told me to have my doctor call this number (503-494-7967, see ohsu.com) for referral instructions.
  
• January 18, 2006: I called Dr. Y's office in Seattle and requested a referral to OHSU neurology, giving them the voice number to call for instructions. Dr. Y's office latter called me back and said they will not do the referral (after already doing two to UW and OHSU neuroSurgery) in an rude tone.
  
• January 19, 2006: I called my PCP Dr. M and requested a referral to OHSU neurology.
  
• January 23, 2006: I'd heard nothing from OHSU Neurology or Dr. M, so I called Dr. M's office to see how the referral was going. His staff was on top of it, but they were getting zero response from OSHU Neurology despite leaving two messages and waiting two days.
  
• January 23, 2006: I called the main OHSU Neurology line and explained that I was having difficulty with their referral process. I also had called the number they gave me for referral and was told to leave a message. I asked for the name and number of the Supervisor. The staffer said that I should avoid calling the supervisor and creating a "messy situation", and that she would instead tell the woman in charge of the (503-494-7967 part of the clinic) referrals to call my doctor back. I kept my tone professional, politely asking again for the name and number of the supervisor, just in case I should need it. (Jean Ellis 503-494-2286) I then hung up and waited.
  
• January 24, 2006: I called my PCP Dr. M. They finally got a response from OHSU Neurology. They said that OHSU requires the referral to be mailed, not faxed, and that they would fax a reply and mail the referral immediately.
  
• January 25, 2006: Roxy from Dr. M's office called. They received a fax (no phone call) that said that I need to follow up with my Neurologist in Eugene (none such person exists) first. WTF!?
  
• January 25, 2006, ~11:20 am: I called the OHSU Neurology "supervisor" Jean Ellis at 503-494-2286 and got his voice mail. I left my name and number and asked him to please call me back ASAP, preferrably today.
  

Ahh, another fun day dealing with healthcare beaucracy. Isn't it amazing!? It's even more fun when you're in pain. Yay.

Fed up with Doctor Y's Office: The Letter

Before leaving Seattle to get married and go on a long sabbatical/vacation, I saw my (former) neurologist Dr. Y. We discussed my recent diagnosis of Syringomyelia, and I told him I'll need help managing it while travelling. He seemed to agree, but when I called him from abroad and from NYC his office was less than helpful. I felt fed up and let down and I was in pain. After returning to the west coast, I wrote this letter to Dr. Y which I will send tomorrow.

Censored version with names removed
From Patient: A
To: Dr. B Y Fax: xxx.680.2246

January 23, 2006

Dr. Y,

You may recall my vacation plans from our last appointment: I married in August in Oregon and then my wife and I have been traveling (US and EU). We are currently travelling across the US and plan on settling in Portland. Travel has been difficult at times with a chronic, painful condition, but I'm thankful to be able to do so.

I called your office on December 7, 2005 from NY to give you an update on my condition (it'd been almost 3 months) and ask for a massage prescription. I was very disappointed with the lack of care I received from your office. I had told you I would be traveling and may need to call from the road. I realize that there are limitations to what you are able to do without your patient present. I entrusted you to be the primary physician to help me deal with my difficult neuro. condition. I discovered that, because I happened to be away from Seattle, I had nobody to turn to for help with my painful condition. All I was told was that “if its that bad, go to the ER”. I do not feel this is appropriate; I have a chronic condition, not an emergency, and the ER is quite expensive. Massage is effective treatment for spasticity, and there is no contraindication here. This was unacceptable. My doctor friends told me to find a new neurologist and I agreed.

What I expect from my neurologist is a strong ally in dealing with Syringo Myelia (SM). I am grateful that you were able to help me diagnose SM, and appreciate your past efforts to listen to me.

On January 18, 2006, I called and requested a referral to the OHSU neurology clinic in Portland, where I plan on living. You refused to give a referral even though you diagnosed the condition. Wonderful. Thanks for the help.

I will try to send a book titled “Contents Under Pressure” in the hopes that it will educate you and your staff about just how difficult it is for the patient to find quality care with a rare neurological disorder such as SM. Very few doctors (only neurosurgeons, really) understand the condition, you have to get a referral to see a specialist, and the health establishment presents numerous obstacles to the patient who is in a lot of pain and slowly loosing motor control. I'm only 30 years old. Just try to imagine it.

I felt that you should receive this feedback first.

Sincerely,

name
cell
email

I ordered two more copies of Contents Under Pressure, one of which I will send to his office and urge them to read. The book portrays some of the frustrations people face dealing with the health care beaucracy when they have a rare, difficult to diagnose disease such as SM. The author actually does not have a syrinx, but he has a related condition called Chiari Malformation.

Introduction

In 2005, I was diagnosed with a rare spinal cord disease called Syringo Myelia (SM). From the ASAP website:

What is syringomyelia?

(sear-IN-go-my-EEL-ya)
Syringomyelia, often referred to as SM, is a chronic disorder involving the spinal cord. For reasons that are only now being understood, cerebrospinal fluid enters the spinal cord, forming a cavity known as a syrinx. (Doctors sometimes use other words such as cyst, hydromyelia or syringohydromyelia) This syrinx often expands and elongates over time, destroying the center of the spinal cord. As the nerve fibers inside the spinal cord are damaged, a wide variety of symptoms can occur, depending upon the size and location of the syrinx.

Syringo Myelia (SM) has presented a number of challenges already:

1. Getting a diagnosis is very difficult. My Primary Care Physician (PCM), Dr. M, basically told me there was nothing wrong with me and treated me for depression. The symptoms vary quite a bit, and the condition is rare, so few know about it.
   
2. Finding a specialist. Neurosurgeons are the experts when it comes to SM. Finding one who knows the latest research on the condition is not easy. A neurosurgeon is needed to evaluate the condition and see if it can be fixed, and a neurologist is needed for management of the symptoms.
   
3. Seeing a good specialist (neurologist + neurosurgeon). The health care system is difficult to navigate. You need a referral to see a specialist (even if your insurance doesn't require it). My neurologist, Dr. Y in Seattle, has actually refused to refer me to a new neurologist in Portland. Today OHSU neurology told me that a referral from my primary doctor (PCP) would probably not be good enough. Some medical staffers are courteous and professional (e.g. Dr. M's office), others can be downright rude and unhelpful (e.g. Dr. Y's office).
4. I haven't made it past step 3. yet, because I've been getting the run-around from doctors for the last month. Good thing I pay $350 /mo. for health insurance!
   

Syringomyelia (SM) is a serious condition. It typically causes chronic severe pain, numbness, loss of sensation. It can also cause paralysis, stabbing chest pains, eye problems, crippling muscle spacticity, and joint damage. Knowing that, you'd think doctors and their staff would take you seriously. You'd think.

This blog is a way for me to vent about some of the bullshit I go through as I try to find good healthcare for this rare condition. I hope to spread awareness and urge the beaucratic health care system to refocus on the patient.