ASAP Conference 2009

The ASAP Syringomyelia and Chiari Conference took place in Madison, WI this year. I only went to one day of the conference, so I can only report on that part. I've been recovering from it since Friday night, and I've been trying to find the strength to type up all of my notes. (I took excellent notes. It was a lot like being in college. And I was a really good note-taker in college!) I will include a comprehensive overview here. (My thoughts may be inserted at times, but I will separate them from the facts presented.)

I attended the conference on Friday, July 17. We were very lucky to be able to go, even for one day, this year. My mom, Gus, and our friend, Susan, all went together. We were only able to go due to the generosity of my Uncle Greg and Aunt Corinne, who have a home near Madison. It was so nice to be able to visit with them on Thursday and stay at their place that night. We are so blessed to have such wonderful family!

So, we had an early start on Friday, because the conference day started at 8:00. Thanks to Provigil, I was able to stay awake all day.

The first speaker was Dr. Bermans Iskandar, from the University of Wisconsin-Madison. He was the host of the conference and did an excellent job. He had a dry sense of humor, and seemed very friendly and smart. He introduced the topic of Module 3: Complex and Controversial Problems.

-The older the patient, the smaller the tonsillar herniation. Children will have exaggerated hernations, but that decreases with age. So older patients may have much less obvious herniations.

-Chiari malformations come from many different causes. Is it really one disease? Is there really one treatment that treats all? A thorough understanding of the pathophysiology is needed for proper treatment. A Chiari caused by a volumetrically small posterior fossa will be improved with decompression surgery. A Chiari caused by cranio-cervical instability will be improved with a cranio-cervical extraction fusion. Treatment is not "one-size fits all".

-Sometimes increased intra-cranial pressure causes Chiari; sometimes Chiari seems to cause increased intra-cranial pressure.

-Imaging: Simple MRI's give a very limited picture. Even CINE MRI's, which are the gold-standard for imaging Chiari's, provide inconsistent radiology findings.

>Thoughts: Throughout his speech, Dr. Iskandar inserted advertisements for tourist spots in and around Madison. He did it all with a straight face, too. Quite charming!

The next subject was Occult Spinal Dysraphism and the Tethered Cord Syndrome, presented by Dr. Jerry Oakes. He presented a quite strong stance against the current trend in treating Occult Tight Filum Terminale. I will present the information as fact, just as he presented it. This doesn't make it so.

-Occult Dysraphism is another name for a primary tethered cord.

-Anatomy: In vitro, the spine grows faster than the cord. Therefore, the cord ascends. The cord normally ends at the L1 vertebra or between L1 and L2. The nerves connected to S1-S5 are responsible for bowel, bladder, and sexual function. Therefore, all symptoms of tethered cord are related to bowel bladder, and sexual function. Because of normal variations in anatomy, 3.8% of normal patients will have termination of the cord at L2-3.

-Signs of primary tethered cord in children (when it's usually identified):
---Skin abnormalities at the location of the tethering. 60%
---Bladder disturbances. 20%
---Weakness of legs. 10%
---Deformities of bones in feet, hips, or spine. 5%
---Pain. 5%

-Adults frequently have pain.

-To diagnose tethered cord, an MRI of the lumbar spine is done. The following findings require surgery:
---If the conus (base of spinal cord) is below the L1-2 space and there are symptoms, the filum should be sectioned.
---If the conus is below the L1-2 space and there are accompanying changes, such as a fatty filum, bony deformities, skin abnormalities, or a terminal syrinx, the filum should be sectioned.
---If the conus is above the L1-2 space and there are symptoms AND accompanying changes, the filum should be sectioned.

*Since a section of the filum terminale surgery carries very little risk, the temptation is to abuse and overuse the operation. (This statement strongly implies that recent developments in treating tethered cord are unnecessary and irresponsible. He was ready for a fight.)

>So, that speech was a lot to hear. The room was quite silent. Dr. Oakes had spoken quite assertively.

Next, Dr. Arnold Menezes, from the University of Iowa, spoke about "Complex Chiari: To fuse or not to fuse".
>So exciting! I hope he wants to fuse me!!!

-Cranio-cervical junction fusions are only recommended for a small percentage of Chiari patients. Fusions leave the patient with a limited range of motion, and they carry a high risk for complications and ongoing problems.

-Cranio-cervical fusions are only indicated in 4 cases:
---A reducible bony abnormality, such as a retroflexed odontoid leading to basilar invagination. 25%
---A previous decompression surgery done from the front. 44%
---Occipital-Cervical instability. 26%
-----This is when there is a significant difference in the size of the tonsillar hernation between flexion and extension.
---Musculo-skeletal abnormality, such as EDS or Marfan's syndrome. 15%

-Symptoms of cranio-cervical instability are postural:
---Occipital headache
---No headache in the morning
---Worsened while upright and with exertion
---Dizziness and nausea when upright
---Feeling of impending crisis with exertion
---Relieved with head support and horizontal rest

-Treatment

---A properly-fitted brace is both diagnostic and therapeutic. If the brace relieves symptoms, this is diagnostic of instability.

---Types of fusions: Instrumented (Can be either rigid, with plates, screws and rods, or semi-rigid, with loops and cables.) Non-instrumented (Use of patients own bone to fuse.)

*Every fusion surgery requires bone placement for a successful bony fusion. Bone must fuse with bone ultimately. A brace needs to be worn for 3 months post-surgically to ensure a solid bone-to-bone fusion. Bone to metal fusions are not adequate. Either the bone will give out or the metal will.

---A good fusion should use limited instrumentation to preserve as much range of motion as possible. Usually, the skull needs to be fused to C2.

*Reiteration: There must be bone placement into the fusion for a successful osseous fusion.

>It was great to hear Dr. Menezes speak so assuredly about fusions. I even got to talk to him during the break, and he was so friendly! He looked at my nametag. Maybe he'll remember my name and decide to take my case. I would feel confident with my head in his hands.

Next, Dr. James McCarthy from U of W-Madison spoke about Scoliosis and how it relates to Syringomyelia. I'll include some abbreviated notes on this section, as it wasn't one of the most important sections.

-When Scoliosis is present with Syringomyelia, which includes 60% of SM patients, the syringomyelia should be treated first, as it often resolves the scoliosis.

-An exception is when the Scoliosis is larger than a 70 degree curve. This needs to be treated with a full spinal fusion.

Next came a question and answer session.
>Each of these sessions were quite painful to get through, since so many people presented their personal problems to the panel, which was explicitly not allowed.

The panel included the previous speakers and Dr. Kula, the neurologist at The Chiari Institute.
>There was a very definite clash between Dr. Menezes and Dr. Kula. It was quite tense at times.

-The first question (my question that was submitted online before the conference) was: What can be done to prevent the need for a fusion revision due to a sub-optimal cranio-cervical fusion?

Menezes: For a fusion to be optimal, bone should be used in the fusion, not just metal. It also should only be performed when indicated.

Kula: Fusions often fail due to a lack of adequate bony material in the area of C1 and C2. Osteoporosis and Vitamin D deficiency should be treated to ensure a bony fusion

-Question: Why are so many neurologists unaware of Chiari malformations?

Kula: There are often minimal neurological findings in patients with Chiari. Besides a downbeat nystagmus, which may or may not be present, the neurological exam is often perfectly normal. For this reason, neurologists don't see the need for treatment unless they are very well-informed.

Next, there was a break. After the break, we began module 4: pain.
>This section was not very helpful to me. I will only mention the few points that were made.

The first speaker was Dr. Miroslav Backonja, from the U of W-Madison pain clinic.

-In Syringomyelia, the pain is often neuropathic.

-Neuropathic pain is caused by diseases affecting the somato-sensory system (injury or lesions of the nerves).

-Hyperalgesia=an increased pain response to normal stimulus.

-Hyperpathia=an increased pain response to repeated stimuli.

-Most people have descending modulation, which makes it possible to ignore pain. People with chronic pain tend to have a loss of pain inhibition.

-A multi-modal and multi-disciplinary pain management clinic will approach both problems: Medications will be prescribed to decrease hyperexcitability of the nervous system, including hyperalgesia and hyperpathia. Cognitive-behavioral therapy will be used to help with pain modulation and develop coping skills.

>I'm not even going to include information from the next speaker, Dr. Angela Mailis-Gagnon, because I found her to be so unhelpful.

Next was the question and answer session on pain. The first question was about chronic opioid use.

-First, Dr. Backonja clarified that opioids are legally prescribed medications, and narcotics are illegally sold and taken drugs.

-Moderate use of opioids is often necessary in treating moderate to severe chronic pain. As long as the use is controlled and in moderate doses, it's generally safe.

-Unfortunately, some people are not helped with narcotics. Some people experience severe side effects. These people won't be able to tolerate narcotics.

Question: How to deal with persisting headache after decompression.

-The Alexander techniques for cervical alignment can alleviate headaches. Improving posture through physical therapy and lifestyle adaptations can help.

-Being in pain heightens the sensitivity of pain. It lowers the pain threshold and heightens pain awareness.

Next was the lunch break.

>I was very fortunate to be able to eat lunch with Laurie, a fellow Chiarian and blogger. It was great to meet her. I'll include a picture when I get around to loading it onto my computer.

Next was module 5: Clinical Research.

First, Dr. Paolo Bolognese presented on Intra-operative Ultrasound Techniques.

-Color Doppler Ultrasound is used intra-operatively in every operation done at The Chiari Institute, but nowhere else.

-Most decompression operations are done with only a B scan real time ultrasound. Color Doppler Ultrasound has three parts: B scan real time ultrasound, color coding, and pulse waved doppler. The pulse waved doppler measures the velocity and the flow of fluid.

-This technique was actually invented by Dr. Bolognese, and described its use in a paper published in 2003. Pulse waved doppler was originally used in cardio-vascular surgery, to measure the flow of blood through veins and arteries. Dr. B decided to try this to get a more accurate view of the flow of cerebro-spinal fluid flow intra-operatively.

>He showed pictures for comparison throughout the presentation. The differences were quite impressive.

-Color Doppler Ultrasound is used in every operation at The Chiari Institute, but nowhere else. This is because it is very, very expensive, difficult to use, and operator dependent. It requires a particular expertise.

*Using a B scan, you can only make assumptions about the flow. CDU is the only way to actually measure the flow.

>Here, Dr. B mentioned what a funny word "assume" is in the English language. hehe

-A B scan can show brisk movement of the structures, even if the flow is not really going anywhere, just back and forth. This kind of flow is called "stump flow".

-With CDU, you can measure the CSF flow before decompression, then make sure that the flow is improved after decompression by tailoring the operative technique to the individual.

-Even a CINE MRI, which is the gold standard for measuring CSF flow, is often inaccurate. Measurements of flow with CINE are affected by cardiac and respiratory variations. Everything that is not in sync with the heartbeat is lost.

-The only other doctor that has mastered the use of CDU and uses it regularly is Dr. Lu, in China.

-At TCI, they have been able to establish that in cases of Occult Tight Filum Terminale, the tension in the cord creates stagnant flow within the filum. This has been demonstrated using CDU in each operation.

>This was great for me to hear. It really validates the existence of OTFT, and the need to detether in these patients, despite what other doctors may say.

-So, CDU allows for much more insight into CSF dynamics. However, it is difficult to master and expensive to use. It provides anatomical and functional feedback throughout the operation, allowing for a personally tailored decompressive operation based on individual anatomy.

>We all know that each Chiari malformation is unique. This demonstrates how TCI is able to tailor the operation for the individual. They use more aggressive tonsillar shrinking often, because it makes more pathways for CSF flow. They determine whether or not to open the dura during this operation.

>In the last couple minutes, Dr. B honored Dr. Milhorat. He said that he was an incredible mentor and friend, and he was very sad to be operating without him. He looked so sad. It was very sweet. I was in tears. They were clearly very close. It's a shame that Dr. Milhorat couldn't be at the conference, because of recent controversy.

>I think Dr. B did a great job presenting on the topic. He was also quite the comedian. It was good to see him keeping his sense of humor. Unfortunately, he came in soon before his presentation and left soon after. I heard it was because his kids were alone in the hotel room at the time.

Next, Dr. Nicholas Wetjen spoke about outcomes after decompression for Syringomyelia. I don't really have anything to report on this section.

Dr. Frim, from the University of Chicago Hospital, spoke next. He discussed neuropsychological issues in Chiari and Syringomyelia patients.

-The most common neuropsychological problems in childhood are brain fog, school problems, and ADHD.

-Most Chiari 1 patients are broadly average both cognitively and emotionally. However, they tend to have specific deficits. these deficits include memory retrieval deficiency, low processing speed, reduced executive functioning, and mild cognitive impairment (memory type). The lowest scores overall were in verbal memory.

-This classifies most Chiari patients as learning desabled.

-Further study should be done to find out if decompression surgery helps with cognitive functioning. Dr. Frim is performing a study like this right now, testing neuropsychological functioning both pre-operatively and post-operatively.

-Cognitive rehabilitation could be done to help with Chiari-specific cognitive weaknesses.

Next, Dr. Brandon Rocque spoke about trends in surgeries. This was a pretty basic presentation discussing the differences in decompression surgeries.

The next speaker was Dr. Allison Ashley-Koch. She works at Duke, studying the genetics of Chiari. Her work is a continuation of the work of Dr. Marcy Speers, who passed away in 2007.

-There are multiple causes of Chiari, that may all have different genetic causes:

---Push down: Hydrocephalus
---Suck down: CSF leak causing hypotension
---Squeeze down: Reduced volume of the posterior fossa
---Shake down: Trauma causing acquired Chiari
---Pull down: Tethered cord

>The rest of her speech just said that there are some possible leads, but they haven't really figured anything out yet.

The next speech was on canine Chiari and Syringomyelia. Small breeds, especially but not exclusively the Cavalier King Charles Spaniel, are most susceptible.

Lastly, there was the final question and answer session.

-The only valid question was about the long-term prognosis for Chiari patients post-decompression.

Frim: We don't know, because it's still a fairly new surgery. We can only compare to similar surgeries that have more long-term evidence. For example, patients with tethered cord that are successfully de-tethered have early deterioration of spinal cord function. And patients with shunts have early cognitive problems that are usually associated with old-age. All of these patients have a more "condensed" life-span. We don't know if Chiari patients will have similar experiences.

>And we'll end with a joke. One panelist was explaining that there are many roads to Chiari. Dr. Frim mumbled, "There's construction on all of them."

Sorry it took me so long to post this. I've had a bit of a rough time since then, which I'll post about soon.

Knee problems

This pictures shows the anatomy of the knee. By looking at it, I can guess at what's wrong with my knees. The LCL's must be very loose, causing the fibulas to dislocate...all the time. I'm sure that they are so loose that they're never really in the socket. The fibulas just slide around all the time. I wonder what kind of damage this is causing. It sure hurts a lot!!! It hurts all the time...not just when I walk, not just when I stand, not just when I move my legs around. It hurts when I drive, when I sit, when I rest, even when I lie down!!! That's how I know I need to do something about it. I hope it is something that can be helped. I've been wearing Ace bandages on my knees, and it seems to help a bit, but not enough. What if it's too late and the damage has already been done?

My shoulders and my hips also have this kind of hypermobility. It just doesn't cause too much pain, yet. I better watch out, because eventually, they will all be in trouble.

I also have a weird sensation in my calf. It feels like a bug is crawling around on my leg. Really annoying.

My other issue is having patches of skin sensitivity. Right now, it's an area on my left forearm. It doesn't look like anything is wrong, but if you touch it, at all, it hurts a lot!!!

Stupid body is falling apart. I'm so tired of being so sick. It's so hard get get through even the easiest days. I can't wait for surgery. I wonder when I'll hear from Dr. Menezes. I wonder what he'll say. I guess I'll keep busy with other doctors until then.

The shortness of breath and knee pain have been worse lately. I need to figure out what kind of doctor to see for the pain in my knees. They hurt even when I am at rest. I'm wearing knee braces. They hurt all the time. I may see a rheumatologist. Who knows?

I'm still choking on pills pretty often. I really need to lie down to swallow anything.

I have a pretty bad spinal pain, too: pain around C-4 to C-5 (under my fusion), pain in the middle of my T-spine, and pain at L-4 (where I had my detethering).

I'm not sure if the chiropractic treatments are helping, but I'm still going to every treatment (3 days a week), even though it is a strain on my body to fit it into my schedule.

We're leaving on Thursday for Madison to attend the ASAP Conference on Friday. We chose to go to only one day, but we'll be seeing quite a few important doctors, including Dr. Batzdorf, Dr. Menezes, Dr. Bolognese, and Dr. Grim. Very cool. I'm just worried that it's so early in the morning. (It starts at 8:30 am.) Yikes! I've been sleeping 'til noon lately. And I never get dressed until the afternoon. We'll see how it goes.

I'm exhausted and hoping to be able to make it to work today.

Been a while

Gus has nagged me for not writing in my blog lately. I know it's been a while. I've been distracted by my word game that I'm addicted to.

I've seen Dr. Don, the chiropractor, a few times. Financially, he's been extremely generous. He'll be getting very little pay for my course of treatment. He's recommended 24 treatments in 12 weeks. He did some x-rays. They showed that my scoliosis in my thoracic spine, which has always been considered mild, has progressed to "significant". It looks really bad on the x-ray, although I don't know the degree of the curve. I just know that it looks horrible. It curves to the right, quite a bit, and I seem to lean to the left, perhaps to compensate, in each picture. Shoot. Let's hope that it doesn't continue to progress. Although, with Ehlers-Danlos, most things are progressive, since the ligaments wear out more and more over time.

The chiropractor is doing two kinds of treatment: electro-stimulation of acupuncture points in the ear. It hurts a bit when he does it. He also uses acupressure on neuro-reflex points on my head. Nothing jarring. Nothing cracking. Nothing forceful. And it seems to decrease my head pain, at least a little bit. I really like Dr. Don, so I'm going to go through with the whole treatment.

Another example of the EDS worsening is my knees. They've become very painful. I need to seek treatment for this soon. Otherwise I won't be walking too much longer.

I haven't sent my package to Dr. Menezes yet, but it really is together and ready to go now, so I'll get it out tomorrow. I'm also sending out a request for the surgical report that was missing in the package that was sent to me.

My mom was out of town for the 4th of July weekend. I wasn't well much of the time at all. I participated in celebrations on the 3rd of July. That night, my body crashed. I couldn't get out of bed for the next few days.

One week ago, I passed out while driving to work. I just barely was able to pull over onto a small shoulder to wait to be rescued. Today, I'm feeling really tired and out of it, so I'm a bit worried that it will happen again tonight. I'm doing what I can to prevent it. Lots of Provigil. We'll see if it helps.

I'm so very tired and my knees are killing me.

That's all for now.