I don’t know how to get the word out about this. I am concerned about #MECFS patients who are a) having craniocervical fusions b) in Europe and c) are not actively engaged in patient groups on Facebook. [1]
My primary concern is that a significant proportion of patients who have fusions experience an initial improvement, but then have worsening symptoms roughly 5-7 months after surgery.[2]
In many cases, this is due to the “unmasking” of tethered cord syndrome, a condition many patients with craniocervical instability have. [3] me-pedia.org/wiki/Tethered_…
They likely always had it, but the increased upward tension on their cord after placing their skull *in the proper position* via intraoperative traction worsens symptoms. [4]
(Petra Klinge at Brown University is one of the world’s leading experts on tethered cord syndrome, so if you want to learn more, in addition to the MEpedia page above, you might dive into her YouTube lectures: youtube.com/results?search…) [5]
How common is tethered cord syndrome? I asked my neurosurgeon. Over the last six years, 30% of his craniocervical fusion patients have also had tethered cord release surgery either before or after surgery. [6]
There have been dozens of #MECFS patients who have had craniocervical fusion surgery in the US and Europe since I first wrote about my story. The most public patients have been me @jeff_says_that @kskarenscott @julierehmeyer. [7]
@jeff_says_that @KSkarenscott @julierehmeyer Jeff needed a second surgery ~ six months after his craniocervical fusion to address tethered cord syndrome. [8]
I had my tethered cord release just one week after my craniocervical fusion, when it became clear my tethered cord was so severe, it required immediate intervention: link.medium.com/bziuvw7A96 [9]
(I write a little more about my experience with tethered cord syndrome in this article, which is more of a theoretical piece: medium.com/@jenbrea/patho…) [10]
Julie is scheduled to have her tethered cord release surgery next week. She describes having an initial improvement after her fusion, but worsening symptoms three months later.
[11]
[11]
No one understands the relationship between craniocervical instability and tethered cord syndrome, or why both conditions seem to frequently co-occur. [12]
But one of the consequences of tethered cord, if you have it, is that it subtly changes the dynamics of your entire spine, resulting in a more kyphotic thoracic spine and “head forward” position of your skull. [13]
This, logically, might increase your likelihood of developing craniocervical instability or cranial settling at some point in your life. [14]
Now, I‘m going to say a few things that may be controversial, but they need to be said. These are just my impressions based on watching 100s of people navigate diagnosis, surgery, and/or recovery, with more than a dozen doctors on three continents, over 12 months. [15]
Any patients out there, feel free to disagree with me! Even better, you too can form your own independent impression, based on reading 100s of patients stories, if you join the conversation on Facebook (more on that later). [16]
#MECFS patients in Europe who are fundraising for craniocervical fusion surgeries are not by and large being informed by their doctors that they may also have tethered cord syndrome or other structural problems that require further surgery to address. [17]
This means that if you are lucky, and you have a “simple” case of craniocervical instability, a fusion surgery can be miraculous. If you have additional issues that need to be addressed, you may experience an initial or incomplete, but not permanent or full improvement. [18]
And even if you later become aware that you might have a second or third issue that needs to be addressed, you did not plan for this financially or practically, and are therefore screwed. It’s sad and I’m pissed. [19]
Generally, patients in the US do better. There are surgeons in Europe who, when it comes to craniocervical fusions, tethered cord releases, and other surgeries, do have good technique. However, I believe they lack the requisite diagnostic experience to maximize outcomes. [20]
US surgeons have been at this longer, some for 20+ years. They approach these diagnoses as consequences of connective tissue disorders. They know many of their patients will have several, *different* issues requiring multiple surgeries. [21] onlinelibrary.wiley.com/doi/full/10.10…
US surgeons know that many of their patients have #MCAS and understand how to plan anesthesia and pain management in light of that. [23]
*note, by US surgeons, I really mean, again, the co-authors on the paper above and/or the people Jeff lists on his website. mechanicalbasis.org/neurosurgeons.… [24]
And because only patients who have Medicare or a private PPO insurance plan can have surgery (they do not accept self-pay patients, unless they are international), if there are complications, if new issues come up, if further diagnostics are required... [26]
...the patient can generally have those tests and procedures covered by insurance rather than having to pay out of pocket again and again. Some people really do have to fight, but again, these are problems fellow patients on Facebook can help you troubleshoot. [27]
My craniocervical fusion was US$100,000, very similar to the price in Europe. I have a very excellent private PPO plan. I paid essentially nothing. [28]
However, I ended up having multiple procedures, multiple lengthy in-patient stays (10 weeks cumulatively) and a whole slew of imaging and invasive diagnostics. All told, I think my insurance has been billed $750,000 and paid out half. I have only had to pay about $7000. [29]
(Here’s what I remember off the top of my head having done. The hospitals are not allowed to bill me the balance. Yeah, I know, American healthcare is super confusing.) [30][...] 
None of this is right or fair. Many patients in the US and abroad do not have access to this level of care. Many things have to happen to change the situation, but first, I think it’s important to talk publicly about what is. [31]
In Europe, I’ve also seen surgeons take what I interpret to be a “one and done” approach. More extensive surgeries (e.g., fusion at more levels) so that you don’t have to come back and have repeated surgeries. [32]
There is some logic to this approach when everyone is paying out of of pocket. However, one benefit of the insurance environment in the US (conditional on having insurance) is that at each step, our neurosurgeons can take the minimum necessary action. [33]
They know their patients are not unlikely to need further intervention in the future. However, because you can never know 100% what symptoms a surgery will or will not resolve (or, in the case of TC, reveal)... [34]
...by taking a small step each time, the remaining clinical picture becomes more clear (e.g., what are the most disabling remaining symptoms, what can be improved with PT). You guard against performing unnecessary or unnecessarily aggressive surgeries. [35]
One other difference re: US patients and their outcomes. I think the #MECFS patients who pursue this course and who are patients of the Center for Complex Diseases are having the very best outcomes. [36]
Seeing so many different kinds of patients has made them excellent (not infallible, though—again, see Facebook!) at differential diagnosis. [38]
So while they do refer a lot of patients for surgery, they also diagnose underlying autoimmune conditions and infectious diseases. They test for Lyme, SIBO, small fiber neuropathy. [39]
They manage patients whose care include: CCF & other surgeries, IVIG, antivirals, antibiotics, anti-inflammatories, POTS meds, mast cell stabilizers, physical therapy, collagen peptides, stem cell therapy. My doctor, Dr. Kaufman, goes to every conference imaginable.[40]
Having that support both before and after surgery, and as other conditions have emerged––e g., the omohyoid problem I wrote about earlier this year, my two-month struggle with #COVID19––has been essential. [41]
Above, I shared my full suite of diagnoses. Again, many patients who have the privilege/luck of being thoroughly investigated end up having 2-6 treatable diagnoses. [42]
These include craniocervical instability and tethered cord syndrome, but also Chiari malformation, Eagle’s syndrome, internal jugular vein compression (for which I’ve encountered so far at least 4 unique causes), intracranial hypertension w/ TSS, MALS, all kinds of fun! [43]
I hope this gives you at least some sense of how complex this space is. For a very large proportion of people, it’s not going to be as simple as having a “miracle fusion.” [44]
I also believe, for many, it’s not going to be as simple as finding a single biochemical pathway and a magic drug, either—unless, perhaps, it’s an intervention that one does early, waaaaay upstream, at the time of the initial infection. [45]
The key takeaway is this: not everyone with #MECFS has a structural problem. But you do have a structural problem, it also doesn’t mean that a craniocervical fusion will be the solution to all of your structural problems. [46]
Because if you have one, you likely have more than one. Why? The analogy Dr. B uses in this talk is helpful. Something is wrong with the cement that supports and binds our bodies together. This means you can develop problems...just about anywhere. [47]
In addition to the lack of access due to insurance or geography, the complexity of our cases and the individualized care required imply some fairly challenging consequences for everything from designing research studies to implementing high quality medical care at scale. [48]
This is a truth that is hard to hear, but if it is the truth—and again, so much more research is needed—we have to deal with it. Our clinicians and researchers have to deal with it. And only if we can deal with it, can we start to find better solutions. [49]
And this, my friends, is why Facebook is so important. Because if you decide you want to open this Pandora’s box and walk this path, you basically need to become a motherf***ing ninja. Seriously.[50]
And if not a ninja, you need to become as well-informed as you can possibly be. That means following the Facebook groups, engaging in conversation with other patients, reading the relevant MEpedia pages, reading *all* my articles on Medium...[51]
...reading Jeff’s website, reading Julie’s story, reading the research we all link out to, looking for the names of the key physicians, searching YouTube for conference talks, reading the good, bad, and ugly of multiple patients’ experiences. [52]
AND MAKING NO ASSUMPTIONS. I can’t tell you how many times I encounter people who say, “Well, I assumed...” Don’t assume. Please, come ask. There are no bad questions. Everyone learns and it also helps us see where and how people are struggling.[53]
Depending on your cognitive capacity, it may take may take 3, 6, even 12 months to learn what I think in an ideal world, you would want to know. [54]
And we are all still constantly learning, which is why we can’t collate it all into a book (yet). Although, there are many written resources in the FB groups. [55]
I know this is not possible for everyone due to their cognitive abilities and general level of function. And maybe not everyone needs to become quite this expert, but these are major surgeries and they are for life. [56]
Everything above represents the common knowledge in our earliest, 12 month-old Facebook group, and it *barely* scratches the surface. [57]
My impression (and no, I have no data to back this up) is that people who are active on Facebook are by and large having BETTER outcomes than people who are not; than people who are only on Twitter or only on Phoenix Rising. [58]
Why? 1) Vital information like “a lot of people will need tethered cord releases” is common knowledge, 2) Many patients, when they become more informed, decide surgery is not for them, 3) Patients who decide to have surgery go in thoroughly prepared [59]
We forgot that there are people out there who might be making major medical decisions without this and other essential knowledge, and so I am writing this thread for them, in the hopes that I can encourage and empower others to become more well-informed.[60]
We also discuss non-surgical options all of the time. There are so many ways to manage or improve (depending on your diagnosis) and learning from patients’ non surgical experiences is just as important. [61]
So, join us. Diagnosis is a months’-long process as is getting on the surgical calendar. In the meantime, by being actively engaged (if you can be) you will learn *a lot* about this field and get to directly observe other patients’ range of recoveries and outcomes. [62]
You’ll get to see how the opinions, standards of care, techniques, and outcomes vary among neurosurgeons and learn from the patients of over a dozen surgeons on three continents, which not even the surgeons get to do. [63]
You’ll see hundreds of cases unfold in real time, with imaging, testing, conflicting opinions, alternative diagnoses, various interventions recommended, recovery experience, outcomes, etc. [64]
Here’s the link to the group. If you decide to join, mention this thread. Not everyone gets accepted and the acceptance process can be slow. BUT If you search, there are MANY MANY wonderful groups on Facebook for all of these diagnoses. [65] facebook.com/groups/1353765…
I wanted to offer Europeans and others outside the US a few rays of hope. First, there’s research underway in both the US and Europe that will, I hope, in the coming 2-5 years, offer a stronger evidence base across many questions. [67]
I am personally aware of no less than five studies. This will both improve quality of care as well as, I hope, make it more widely accessible. After all, many surgeons already have the technical skill to perform these surgeries. [68]
The challenge is a) they don’t—connective tissue disorder patients have complex clinical pictures that make “bread and butter” craniocervical instability or Eagle’s Syndrome or what have you more difficult to diagnose (remember, many of us have more than one pathology) [69]
b) the patients who need surgery are never referred because the pathway from primary care to neurology to neurosurgery is broken. (A lot of us get diagnosed with conversion disorder or other nonsense somewhere in the middle.) [70]
What this means, though, is that if the research happens and the evidence base is sufficient, care could become much more accessible to patients and available under national health services very quickly. [70]
We don’t need to invent a new treatment or train a new generation of doctors. Although diagnostic and surgical techniques WILL need to be refined and optimized for this patient population. [71]
My second ray of hope: while yes, the only way to get a craniocervical fusion outside the US is to pay an obscene amount of cash, in Europe and Australia, there are pockets within the existing system where treatment for some of these other diagnoses can be had for free. [72]
So it is possible that you are suffering right now from something that IS treatable, and for which treatment IS available in your country, but you have to know where to go and how to get there. You have to be a ninja, and you have to be prepared to get your heart broken. [73]
But there are thousands of patients walking on parallel, idiosyncratic paths. Collectively, we are drawing a clearer, more complete map. We are pushing our doctors to do better. We are here and we want to help you get to where you need to go. [74]
~ fin ~
(I may append additional info / QT some questions that may be useful to others.)
(I may append additional info / QT some questions that may be useful to others.)
