Jen Brea: My ME is in remission

Question from @strategist about travelling by car.#125

Jeff_w said:
It was pretty horrendous. Dizziness, nausea, light and motion sensitivity, and a PEM crash afterwards.
Click to expand...

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.
 
WillowJ said:
Hopefully there's a better option?

The two problems with this are
1) the new editor (neither of the previous editors think he's doing a good job)

2) word on the grapevine is this journal has a policy of not publishing anything related to ME(/cfs).

Or maybe just put it somewhere there would be robust post-publication review. As pre-publication review doesn't seem reliable or meaningful.
Click to expand...

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.
 
Milo said:
N
i am a little skeptical by the fact that only 4 or 5 centers from around the world is aware of the problem and that in order to be diagnosed with CCI you need to see these specific doctors. It is a red flag to me.
Click to expand...

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.

This said, I think @JenB has used great caution in sharing her experience and I appreciate that.
Click to expand...

Thank you. I really do try my best.

We desperately need science and medical care. Said the 17 millions of us.
Click to expand...

As the kiddos say, RETWEET
 
Jonathan Edwards said:
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.
Click to expand...

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.
 
Jeff_w said:
The second step will be a study testing for the presence/absence of measurable CCI-related brainstem compression in people with ME. A potential biomarker study, if you will. Michael VanElzakker, at Harvard University, is working on this.
Click to expand...

JenB said:
You can't test hypotheses until you formulate them and most hypotheses come from empirical observation. We're in that latter stage, with more cases to come. I think that's very exciting. I am sure there will be a case series published and after that, perhaps a more systematic study. It will also be interesting to see if/how the VanElzakker and Younger brain imaging studies connect.
Click to expand...

I'm not clear from these comments if any study along the lines of what Jonathan Edwards suggested is being planned. We really need it to be.

What is needed is a blinded study of imaging done on a decent number of people with a ME diagnosis and healthy people. Presumably the imaging could be done in one location following any specific instructions, and then sent to the specialist doctors with an independent party keeping track of the diagnoses made. That wouldn't be a terribly expensive or time consuming study, especially if the specialist doctors donated their time to review the imaging.

The utility of the surgery is then another question, but we don't need to wait for the outcomes of surgeries to get a sense of whether CCI is a significant issue in people with ME.

Edited to clarify meaning
 
Last edited:
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.

JenB said:
You can't test hypotheses until you formulate them and most hypotheses come from empirical observation. We're in that latter stage, with more cases to come.
Click to expand...

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.

Milo said:
Patients are desperate to get better and will go to great lengths to access treatments and rule out other diseases. This desperation have led people to abandon their homes to live in the desert (mold theory), order medication from India and injecting without medical supervision, and endless experimentation with extreme diets, fastings, supplements, chelation, antibiotic therapies, just to name a few.
Click to expand...

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.
 
Last edited:
JenB said:
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.
Click to expand...

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.
 
Michiel Tack said:
Could you share some of the videos and papers about CCI that suggests this? Many thanks in advance!
Click to expand...

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
 
Jonathan Edwards said:
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
Click to expand...
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.
 
Last edited:
I agree with Jonathan Edwards and others that individuals telling us a lot of details of their personal medical history, especially if those writing those personal histories intermingle them with speculation/hypothesis-making about causal pathways can be unhelpful. It ends up causing a lot of confusion among other ME sufferers.

I stripped out a few of the landmarks Jen has told us on her medical journey and posted them in my last post simply to highlight the fact that she had said herself that it was specific neurological symptoms that had occurred more recently (and that most of us don't have and are not part of any ME definition) that led to a CCI diagnosis.

I did that to remind people that most of us with ME don't have those specific neurological CCI symptoms and that thus far, there is no evidence that for most of us ME symptoms are caused by structural neck problems. We need to wait for proper research before anyone draws such conclusions. The research on brain stem talked about at a recent ME conference was about myelination of nerve cells, not about bone/ligament structural issues.

I would actually discourage @JenB from writing more and more details about her personal case history, especially doing so in the context of ME advocacy. I don't think it helps the rest of us to have such a high profile individual publishing such an unusual personal journey towards remission and intermingling it with a lot of unproven hypotheses.

If we are going to make progress with ME research and treatment, we need properly researched information and hypotheses made by scientists who are expert and can set up and carry out appropriate research to test those hypotheses.

I don't think it helps our advocacy cause to have a high profile given to individual stories of recovery/remission that are told in such a way as to give prominence to speculation about the cause of remission, whether from CCI surgery, avoiding mold, avoiding gluten, standing on circles shouting no, yoga, rituximab, drinking celery juice, ketogenic diets, meditation, living in a desert, or whatever.

It is absolutely right that we celebrate individuals' remission. But please can we avoid ME advocacy groups giving prominence to speculative hypotheses based on a few individual cases. I fear it does our cause harm.
 
MSEsperanza said:
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.
Click to expand...

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.
 
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.
 
You must log in or register to reply here.
Back
Top Bottom