Jen Brea: My ME is in remission

Question from @strategist about travelling by car.#125

Thanks @Jeff_w and @JenB and @Mattie for sharing so much

I posted yesterday about the development of a new symptom- extreme problems after being driven 270 miles by car. I had been doing the same journey for many years without a problem. I have never suffered whiplash.

I felt so ill it frightened me. I remembered @Mattie posting about his difficulty with car travel ( here or on PR) and exchanged posts with him. This was way before the raising of any CCI involvement. Combined with a diagnosis of hypermobility, and a mention of EDS, I thought it just possible that I might be experiencing neck instability problems and I might. I have spoken to my GP.

I am choosing to deal with it by replacing long distance car travel with train or air travel ( already booked my ticket for Cornwall this year), and using a Philadelphia collar to stabilise my neck for any travel.

I am watching what is happening with interest- another patient on PR had surgery last week- but I am much older with other health problems so am not pursuing this with any vigour.

It will be interesting to see how this pans out. It is essential that we know what it means for all sorts of reasons.

 

What is needed is adequate pre-publication validation of imaging analysis. NEJM would get that done adequately and if the findings are bona fide there would be no problem with editors. JAMA would be another option. If the claims of CCI are real then publication should be straightforward.

 

I think this has been somewhat misrepresented/misunderstood. A wider number of surgeons operate on CCI. However 1) fewer surgeons have made it their major focus and 2) fewer still focus on patients with EDS, a rare disease and 3) many surgeons are unwilling to operate on patients with EDS because of all of the many risks and complications that come with that patient population, e.g., mast cell flares, poor wound closure and healing. Fewer surgeons still operate on people with "complex Chiari," that is, both Chiari malformation and CCI. Jeff has seen (and I have seen myself) some horrendous and sad cases of people being operated on in their hometown in say, Alabama, by a surgeon who only does four surgeries per year.

The reason why Jeff has focused on these surgeons (and I'm putting words into a mouth so this is my presumption or is least how I understand why he and the broader EDS community rate these surgeons is) a) experience and # of surgeries per year, b) low complication rates, and c) an understanding and specialization in hEDS.

Understanding hEDS may not be the same thing as understanding ME (actually I know it's not! My one major complaint about my surgical team is they did not understand PEM and that was extremely frustrating). However, all of these folks understand mast cell activation syndrome and dysautonomia, making them a particularly good at understanding the kinds of challenges ME and hEDS patients w/ CCI are likely to face with anesthesia, surgery, and during recovery. These surgeons generally don't treat people with CCI due to rheumatoid arthritis or traumatic injury, for example.

There are other surgeons people do use and many have perfectly wonderful experiences. There are still not a lot of them.

The ones on Jeff's site are just the only ones he feels comfortable recommending based on his thousands of hours in forums watching patients before and after surgery. That's his choice.

Thank you. I really do try my best.

As the kiddos say, RETWEET

 

And what happened with the Enterovirus theory?

 

Just to temper expectations, both my internist and neurosurgeon are insanely busy. I fear for them publishing because of this, but I believe they will. However, I doubt it will happen in six weeks. Moreover, they want multiple cases, not just two. So they'll need to see the outcomes of a few more surgeries.

 

Not sure where I said that...

 

Maybe I should put this in this thread too:

I just did a Pubmed search on Craniocervical instability and fatigue. The result was:

No documents match your search terms

 

I am also happy to hear you have improved so much, @JenB and I wish the improvement continues.

Thank you for letting us know what was going on and your thoughts about it. I appreciate that you made it clear that right now nobody knows the cause of your improvement for sure, and that the idea that CCI & tethered cord were in your case two main factors contributing to your ME are only hypotheses.

I am glad to hear from Jeff that there are plans to publish case histories and to properly investigate the issue of potential CCI in ME.

I don't have the capacity to word my concerns at the moment, but to me it seems the hypotheses have still to be discussed more widely among medical professionals. Of course, additionally providing case histories with all relevant details (what exactly was diagnosed based on what? what exactly was measured in which way?) will be very helpful in this respect.

Please, @JenB and @Jeff_w , consider that on behalf of the majority of people with ME, it would be much safer to reserve the details of diagnostic procedures and surgeries for a speedy publication in a peer reviewed journal.

And there are those people with ME, their friends and families who will become even more distressed because they can't afford any of these special examinations and experimental treatments.

For me, the mere effort to follow the details and the incapacity to word my questions that cross my mind when I read the details is distressing, even though I am happy for each pwME who gets better, and I think it's worthwhile to investigate the CCI issue.

(Have been only able to skim this thread, and am a bit PEM-ed already, so hope my post makes sense.)

Edited for clarity.

 

Jeff said there were 17 imaging cases. Those are what need to be published. Until we see those published I would very much hope no further cases are operated on.

 

I personally find PubMed to be outdated.

I entered a search on Google Scholar using those same search terms.

I turned up 1,300 results.

 

I don't see any reference to fatigue in those citations.

 

That is because the text is embedded in the articles themselves.

 

Sure. I don't have a comprehensive list of everything I've read and seen, but this should get you started.

Here are a couple of overview documents by respected neurosurgeons:

Cranio-cervical Instability in Patients with Hypermobility Connective Disorders
Neurological and spinal manifestations of the Ehlers-Danlos syndromes (specifically the section on craniocervical instability)

The Chiari and Syringomyelia Foundation held a colloqium on craniocervical instability in 2013 - they have a webpage dedicated to it with links to videos of all of the presentations. I haven't watched all of them yet, but the talks by Ulrich Batzdorf and Roger Kula spend a lot of time detailing the range of ME-like symptoms that some (but not all) CCI patients experience. The presentation by Edward Benzel talks about the mechanism by which CCI might give rise to these symptoms.

Finally here's a study from earlier this year showing how a cohort of 20 CCI patients with these symptoms saw significant improvement after surgery.

Cervical medullary syndrome secondary to craniocervical instability and ventral brainstem compression in hereditary hypermobility connective tissue disorders

 

Google claims this one discusses fatigue, but I can't access that part

https://thejns.org/view/journals/j-neurosurg/53/4/article-p444.xml

 

By this logic this quote would be channeled "Everything that can be invented has been invented"
Obviously jumping to conclusions is ridiculous but you can't assume that a new idea can't be true since it has little published background. All successful ideas came out of ether (or more accurately someone's imagination).
Whats needed is to prove them.

 

Actually, I think the cause of my improvement is abundantly clear. I'll make sure to share more information in future Medium posts to explain why this is. The alignment of subjective symptom change and objective measurement via invasive testing was the most convincing experience of medicine I have ever had in my life. No, it's not a large N study, but within-case comparisons matter, too, and it's part of why I decided to have the surgery and why I knew that surgery was likely to result in remission in my case. But I'll explain all that more later. It deserves space and detail.

 

It was post #136 where you mentioned EDS. Which made me wonder to which audience you were talking to, the ME patients or the hEDS patients.

 

I also realize this discussion is perhaps premature for this forum and format. I will continue sharing my experiences online and as folks continue to pursue this diagnosis, they will also share theirs. I would not be at all surprised if there are 12-15 cases in a years' time. Hopefully then, there will be published research that can be discussed here.