a cure for your pain or the biggest fraud since Madoff?

Diagoras, who is called the atheist, being at Samothrace, one of his friends showed him several pictures of people who had endured very dangerous storms; “See,” says he, “you who deny a providence, how many have been saved by their prayers to the Gods.” “Ay,” says Diagoras, “I see those who were saved, but where are those painted who were shipwrecked?”

–Cicero, On the Nature of the Gods

The day after the 2016 election, I was perched on an examination table at the National Institutes of Health Clinical Center for my first appointment in a syringomyelia study. I had mailed the MRI CD showing a silvery cavity from C5-T1 within weeks of my syringomyelia diagnosis, alarmed I might need surgery I could never afford. The study seemed like a good way to get top-notch care for free with the added bonus of contributing to science.

I signed the informed consent for surgical risks: paralysis, loss of sensation, infection, hydrocephalus, repeated surgery–and wondered how in the hell I would answer if the doc said I needed brain and spinal cord surgery thousands of miles from home.

Instead, when I finally saw the study docs and discussed a second syrinx discovered in my thoracic spine, he asked me point-blank, “How awful is your life?”

It sounds terrible typed out like that, but I swear it wasn’t. I got his meaning right away: What’s this thing doing to my quality of life? Is it worth opening up my skull and spinal cord?

“I’m a pretty adaptable person,” I said. “Life is different, but it’s not awful.” I thought about how I used to walk without a mobility aid, how much I missed activities that were off-limits now, the burning electric pain, the weakness in my hands. “It’s not bad. Just different.”

“Then it’s probably not time to operate.”

It wasn’t the sole basis of his decision and I’m leaving a lot out, but the point is: I agreed.

I did not and do not want surgery. I have read the statistics on Chiari-Syringomyelia surgery, and they aren’t great. Some people recover and go on to live free of the pain and functional losses. Others wind up going through the whole shebang a second or third time. Some people’s spinal cords wind up like Swiss cheese:

SyrinxShuntsSpinalCordSection
photograph from a research study of a postmortem specimen of spinal cord who had been treated for Chiari 1 and syringomyelia with shunts. There are several shunts stuck inside the spinal cord.

If surgery helps, that’s great. If I can avoid surgery, even better.

That’s just me. I can’t speak for anyone else.

Back in my syringomyelia patient groups, though, total strangers said my doctors didn’t know what the hell they were talking about.

“Post your MRIs,” they said, and I did.

“Oh yeah, tethered cord. Definitely. And craniocervical instability.” I had no idea what they were looking at.

“Your doctors are gaslighting you.”

But I didn’t feel gaslit, at least not about surgery. I had felt that way at the doctor before, but not this time.

I knew better than to argue with a patient support group, though. A few months prior, I got booted out of a local Chiari group because I didn’t want to follow up with their fave rockstar East Coast neurosurgeon who had operated on virtually all of them. I didn’t want to see him primarily because he had operated on virtually all of them. It seemed a little strange to me how the group labeled every other neurosurgeon incompetent. Most neurosurgeons know nothing about Chiari, went the common refrain. Only this doctor can help you.

It’s true Chiari-Syringomyelia isn’t as widely known as it should be, but it’s also true there are numerous quietly competent neurosurgeons out there who can decompress like rockstars, too.

I never imagined with all my skepticism that years later, I would send my imaging to a different superstar neurosurgeon, believing he was one of only 3 or 4 people on earth who could help me.

___

I wish I could remember why or how I finally learned what craniocervical instability is, but to be honest, I think it was Instagram. I had just gotten my Ehlers Danlos Syndrome diagnosis and followed a lot of people with EDS hashtags. It seemed like everyone (not really, of course) was sporting neck braces and flying to New York, Spain, or Maryland to a short list of unicorn neurosurgeons. Only these doctors understand CCI, they all said. Since some of my symptoms weren’t explained by EDS or Chiari-Syringomyelia alone, I decided to check it out.

I chose a neurosurgeon who accepted DICOM files online, filled out his questionnaire, and uploaded my upright MRIs and rotational CT scans.

But here’s the thing: When I got my report back, it didn’t even sound like it was about me. The person on those pages sounded like they were dying. And even though my measurements were not extreme, the recommendation certainly was: fusion all the way down to T1.

Something like this:

IMG_2113
XRAY of cervical spine and skull from back and side; showing rods and screws from C1-T2, from the case study: A Case of Occipito-Thoracic Fusion for Skull Base and Cervical Multiple Myeloma January 2015 DOI: 10.4172/2165-7939.1000217

I was terrified. When I contacted the neurosurgeon office to ask how it works if you decide to get surgery, they replied, “We can get you here as soon as next week.”

Next week?

I’ve been hanging around neurology offices long enough to know that “next week” is not normal. It set off every internal alarm. Plus, he wanted cash payment, no insurance.

I contacted my geneticist, got a referral to a local neurosurgeon, and here is how it went:

I loved that doctor. He told it like it was, but he was deeply kind, too. Never once did I feel he blew me off. In fact, he watched me so intensely he noticed few things nobody else had–and got me referrals.

Sometimes a “no” is not gaslighting. Sometimes a “no” saves your life.

Fusion surgery is indeed disabling. Losing range of motion is hard enough, but for someone with EDS, fusing one segment of your already-unstable spine means the next segment picks up the torque forces … and becomes unstable. Next thing you know, you need surgery again.

Thank god I was too broke for rockstar surgeon guy because what if I had the cash, hopped on a plane, and got all that hardware in my spine for … nothing?

And in fact, fast forward a few years, and my unexplained symptoms have explanations now — none of them CCI.

When I tell this story, people get mad. I get it. Plenty of people really do need CCI surgery and I am in no way denying it. At the same time, though, patient groups can make you think a treatment is more successful than it is — or that a doctor is more “rockstar” than they really are.

___

Survivorship Bias

Survivorship-bias
from Wikipedia: an airplane illustration, viewed from above, with various red dots on the wings indicating damage

During World War II, planes that survived battle got examined for damage to determine the best spots to place armor.

Abraham Wald objected. Mechanics only saw planes that made it back. What about the planes that got shot down? Look at that damage, and you’ll find the weak spots. That’s where your armor goes.

Survivorship bias: when you don’t see what’s fatal because you never see the fatalities.

Chiari & CCI groups don’t hear from people who settled lawsuits and signed NDAs. They don’t hear from patients who died.

If such cases get mentioned, groups explain them away: Surgeons who take on the riskiest cases are going to have more negative outcomes.

On the surface, it sounds reasonable, but if you go digging, you find stories like then-4-year-old Katie Bryant , who developed an infection and syrinx after neurosurgery she did not need:

“I have good news and I have good news,” the blue-jean clad Bolognese gushed in his Long Island office, sharing Pringles with Katie and her twin brother Brodie.

Katie’s operation, Bolognese reported, was a roaring success. The Bryants heaved another sigh of relief when he said Brodie’s MRI had no sign of Chiari.

Then, Bolognese said something that chills them to this day.

“I think we should do the same [tethered cord] surgery on Brodie so he won’t develop Chiari later on in life,” Bolognese said.

“My husband and I looked at each other in shock, and thought ‘Is he kidding?'” Bryant recalled. “I knew right then and there we had made a terrible mistake.”

Tethered cord, CCI & Chiari-Syringomyelia surgery often go together and Dr. Bolognese is one of the most famous of the “rockstar” surgeons.

You will find patients like Jennifer Ronca, abandoned on the operating table under general anesthesia while her neurosurgeon took off on vacation.  Imagine waking up braced for pain and finding out the surgeon never showed up.

The hospital — and many people I’ve encountered in patient groups — contend that case got blown out of proportion. It’s just a sensationalistic, overblown story about normal human error.

That’s what NorthShore Hospital argued, too.

But Ronca was traumatized:

“I cried again,” Ronca told The News when she learned the hospital got off without a fine. “The Health Department never called me to hear the facts from me. It’s just another betrayal.”

Ronca said since the ordeal, she has suffered recurrent nightmares and pain that plunged her into a deep depression, for which she was hospitalized in October.

“I’ve had a lot of issues trying to come to terms with what they did to me. The Health Department just assumed there was no injury,” she said.

And if you start looking for the lawsuits, you will find far, far worse: people left paralyzed or dead, accusations of human experimentation (some of these surgeries and techniques are not standard of care) and more.

If you focus only on the good stories, you miss the risks — and the damage.

I am not out to tell anyone what surgery they need or don’t need. I won’t tell you which neurosurgeon to see. I won’t tell you who to trust. But I can share with you some documents I received from a public records request about one of the most famous surgeons, Dr. Bolognese (not the one I consulted), whose name appears on every list and has been promoted by the EDS Society:

https://www.dropbox.com/s/l8x5jelirr30tol/BologneseNYDEPTHealthReadableText.pdf?dl=0

A quote from that document^:

“This program may represent one of the biggest frauds in the history of modern neurosurgery, of “‘Madoff’-like proportions.”

Remember what my local neurosurgeon said: I’d call it a bank transfer. 

Read for yourself and decide. I can’t tell you what to think and I won’t. I have no personal experience with this particular doctor and no vendetta. His name comes up a lot in CCI circles and there just happens to be a lot of press out there about him. I want people to know the information is out there. All you have to do is look.

And there are plenty of neurosurgeons — maybe even a local or semi-local one if you’re lucky — who can help you. It’s not true you have to choose from the unicorn list. You have options. (Adding a note here: I understand the picture may be different outside the U.S., especially with nationalized healthcare systems.)

Here is my advice:

If you decide to pursue CCI surgery, get a second opinion. Heck, get a third. If possible, get at least one consult at a research hospital. No matter what: Do not rush to go under the knife for anyone who won’t take insurance. If a surgeon doesn’t take insurance, think of it as a red flag — maybe nothing is amiss, but maybe something is. File public information requests for complaints. Search court databases for malpractice suits and read the cases (oh my god, the cases). Information is power.

Your neck & spine are the only ones you’re ever going to get. Be cautious. Be wary. Inform yourself.

Note: Later, I will update this post with a Dropbox folder with more information. I want to include some of the lawsuits, but even though they are technically “public,” I don’t think the patients imagined a day where someone would retrieve the cases and blast them on the Internet. Redactions are in order. I’m working out some technical issues and will upload then or perhaps do a second piece.