The science of craniocervical instability and other spinal issues and their possible connection with ME/CFS - discussion thread

[JenB]

So I appreciate the likes but really am honestly asking these questions, not being rhetorical. I am severely ill. I was able to read posts on this thread from bed for several days but was too sick to sit up or formulate my questions. Took great effort yesterday to sit up, join this group, and write my post, and I paid for the effort. I would be most appreciate if anyone could answer.

The post about the surgery in Seattle increases my concerns.

Thanks.

Thank you for the effort. I am so sorry you are doing so poorly. I will go look for your original post now.

Is that the article I posted in Twitter a few weeks back about the surgeon who ignored the swallowing problem his patient was having post-op, ultimately resulting in her death? That was absolutely horrible and heartbreaking. I don’t know of anyone who has ever gone to that surgeon but he is still practicing.

As Michiel has pointed out, this is a complex surgery. The rate of complication can be very low but the complications can be dire and there is the risk of death. I don’t really know how to help anyone weigh those risks but will grapple in a future post re: how I did for myself. It was a very difficult decision for me to make and I did it by becoming as informed as I possibly could be about the surgery and its risks. I also had the opportunity to develop trust with and confidence in my doctor, both via direct interactions and by talking to many former patients. That matters, too (the surgeon you pick), which is why you want to if at all possible talk to more than one doctor and several of their patients.

 

[JenB]

Hi, Jen, Is it okay on this forum to embed Karen’s video/tweet without her permission? I thought that was frowned upon wrt to forum rules. Also, I understood Karen was misdiagnosed with ME but has EDS.

Karen says she has ME. It is very possible to have both ME and hEDS. Many people in our community do. Several clinicians have written about this.

I am not sure why embedding something from a public platform is against the rules but will defer to the mods on that.

 

[vsou]

Those are certainly legitimate concerns. But perhaps we should also look at this from another perspective:

Worldwide we can estimate there will be around 22,000 ME/CFS patient suicides each year, as a result of the sheer hell that ME/CFS can be. (One study showed the suicide rate in ME/CFS is 6 times that of the general population, so from that you can work out this 22K figure).

Thus we are not in a neutral situation; for every year of delay in finding a cure for ME/CFS, 22,000 more die.

Now it's possible that if more ME/CFS patients start exploring CCI/AAI surgery, there inevitably will be some cases of negative outcomes, and perhaps even the occasional death due to post-surgery complications. That's to be expected from any major medical treatment. But that thought has to be balanced against all these annual deaths from suicide, not to mention the misery of millions more who don't kill themselves, but nevertheless live for decades with this horrible disease.

This is true. However, people should know what they are signing up for. My experience, which includes being a patient of Dr. Kaufman previously, as well as Dr. Kogelnik, includes times that I have been prescribed treatments without adequate warning/information/disclosure of risk/benefit. My husband attends all my medical appointments, so this is not a case of me being too ill to know what the doctor said. It doesn't sound like the family of the girl who died from surgery in Oregon had adequate information about risk/benefits either. I agree that patients should be allowed to make their own choice about what they are willing to risk to get well. However they can only do that if they have adequate information. I am really concerned with this idea I hear in some posts that medical professionals will always be professional, can be trusted to have patients best interest in mind, etc etc. This has not been my experience. I am surprised to hear this from ME patients, and don't know where is coming from.

 

[Guest 102]

Karen says she has ME. It is very possible to have both ME and hEDS. Many people in our community do. Several clinicians have written about this.

I am not sure why embedding something from a public platform is against the rules but will defer to the mods on that.

Just that Karin was reported in a recent article in UK as having been misdiagnosed with ME, I think @anniekim tweeted that a couple weeks ago? Yeah, I have wondered about those kind of public links too, I think the issue with posting other people’s health links is they may have changed that post in interim. I know there is some issue with discussing/linking/photographs to other people. Best to check with mods.

 

[JenB]

This is true. However, people should know what they are signing up for. My experience, which includes being a patient of Dr. Kaufman previously, as well as Dr. Kogelnik, includes times that I have been prescribed treatments without adequate warning/information/disclosure of risk/benefit. My husband attends all my medical appointments, so this is not a case of me being too ill to know what the doctor said. It doesn't sound like the family of the girl who died from surgery in Oregon had adequate information about risk/benefits either. I agree that patients should be allowed to make their own choice about what they are willing to risk to get well. However they can only do that if they have adequate information. I am really concerned with this idea I hear in some posts that medical professionals will always be professional, can be trusted to have patients best interest in mind, etc etc. This has not been my experience. I am surprised to hear this from ME patients, and don't know where is coming from.

Informed consent is really important. I definitely had that from my surgeon, in addition to the research I did on my own. But no, I can’t guarantee that all surgeons go through that same process with their patient. I also research the hell out of everything that it is hard for me to relate to the idea of being uniformed, but I know that many people are far to ill to do this or it might not occur to them. It’s also very hard to know what you don’t know.

The Seattle case is unusual and heartbreaking because the father of the girl who died is a doctor. He fought really hard for her and no one listened to him. I would be surprised if he wasn’t aware of the risks or at least, he certainly had the social capital and expertise to do his due diligence.

 

[Barry]

I am not sure why embedding something from a public platform is against the rules but will defer to the mods on that.

Not sure it is.

 

[Barry]

I also research the hell out of everything that it is hard for me to relate to the idea of being uniformed, but I know that many people are far to ill to do this or it might not occur to them.

It is also true that researching things in depth and analysing what you find is a skill in itself, and by no means everyone has that skill even when they were healthy.

 

[Jeff_w]

I have been following this thread with interest. What I don’t understand and hope someone here might be able to provide information about is whether it is accurate that CCI can only be accurately diagnosed by a handful of doctors worldwide? And, if that is accurate, why that is the case if diagnosis and treatment of CCI has been established for some time? (And if it is not accurate, why is that what is being reported?)

No, that is a misunderstanding, thanks for raising this point.

To clarify: When CCI occurs as part of a high-impact traumatic accident, it is recognized and treated on an emergency basis by neurosurgeons skilled in skull base surgery. This has been the case for decades.

When CCI occurs in the absence of blunt force trauma, that is when things get difficult, as most neurosurgeons will not test for it or treat it.

However I have concern about the possibility of CCI surgery being performed in cases in which it should not be. I have read that some think that no surgeon would perform that surgery unless it was clearly medically necessary.

No neurosurgeon worth their salt would ever operate on a patient without substantial supporting evidence from scans, in-person testing, etc.

Nobody should rush into surgery. A good neurosurgeon won't rush you. If you go to a neurosurgeon who wants to operate in the absence of extensive testing, or who you feel is pressuring you to take a surgical path, then that would be alarming and -- to my mind -- a deal breaker.

A neurosurgeon who specializes in non-traumatic CCI will do extensive testing. Also, their patients tend to be chronically ill and very complex, with comorbid POTS, MCAS, etc. Thus, they are very careful in terms of diagnosis as well as the practical precautionary aspects that come along with delicate patients.

On my website, I suggest specific neurosurgeons for all of the above reasons.

 

[anniekim]

Just that Karin was reported in a recent article in UK as having been misdiagnosed with ME, I think @anniekim tweeted that a couple weeks ago? Yeah, I have wondered about those kind of public links too, I think the issue with posting other people’s health links is they may have changed that post in interim. I know there is some issue with discussing/linking/photographs to other people. Best to check with mods.

Yes, a Daily Mail article 2 weeks ago about Karen’s surgery reported her as telling the newspaper she was misdiagnosed with ME initially but now has a diagnosis of EDS. However, of course if Karen is still saying she has ME then I can only presume the Daily Mail wrongly reported it as her telling them she was misdiagnosed with ME.
https://www.dailymail.co.uk/health/...res-grandmother-walking-time-three-years.html

 

[Guest 102]

Then it is equally possible that all of us are misdiagnosed and that neither our criteria, nor our experts, nor any of our research/lab findings to date is up to the task, in which case we truly do know nothing and cannot ever know if any of us actually has ME.

ME is a clinical criteria. I met the criteria. *In addition* I have a whole lot of labs. If I was misdiagnosed, then CCI is such a twin sister wife disease to ME, capable of causing the same symptoms and labs as ME after an identical onset, that we should all be studying it right quick.

As I said before on here, in absence of a biomarker, all of us actually have *presumed* ME, no matter when we were diagnosed, or who we were diagnosed by. I have no doubt I have ME, nothing since 1982 makes me think I don't. But it is still presumed.

I guess I wonder if, since CCI is fixable, and ME is not, would you not now be happier if you *had* had a misdiagnosis of ME and a correct diagnosis of CCI?

Misdiagnosis happens. Am sure it happened in 1980s too, but probably less frequently than before Oxford criteria. And now we have a multiplicity of criteria. I know that you met ICC-ME and have confirmed diagnosis from top specialists. But you were still presumed ME, like all of us. And now you have made a remarkable and unusual recovery.

But most pwME, even those v severely ill for years, simply don’t have these particular alarming new symptoms you and Jeff developed after your respective surgeries. With all I am hearing about CCI, and other new cases, would there now perhaps not be a push on making CCI a rare differential diagnosis? Then people like you and Jeff would get access to surgery faster.

Or maybe that is too simplistic. And CCI is in fact holding some key to complexity of classic ME. (EDIT - to add last sentence)

 

[JenB]

I have been following this thread with interest. What I don’t understand and hope someone here might be able to provide information about is whether it is accurate that CCI can only be accurately diagnosed by a handful of doctors worldwide? And, if that is accurate, why that is the case if diagnosis and treatment of CCI has been established for some time? (And if it is not accurate, why is that what is being reported?)

I am writing as a severely ill patient diagnosed with both ME/CFS and EDS; as someone without a strong science background and with severe cognitive symptoms, who is nonetheless trying to keep up with what is going on well enough to make wise medical choices.

I appreciate the work Jennifer Brea has done to advance ME/CFS advocacy, and am happy that her health has improved, and that others have had improved health outcomes also.

However I have concern about the possibility of CCI surgery being performed in cases in which it should not be. I have read that some think that no surgeon would perform that surgery unless it was clearly medically necessary. I am genuinely very happy to hear that some people have had such positive experiences with medical professionals that they believe that.

Unfortunately, I have had very different experiences, despite the fact that I have been ‘fortunate’ enough to see expert doctors and specialists at very well regarded medical institutions. I have had multiple doctors offer me treatments, including but not limited to rituxan, that I do not believe were appropriate for me, without caution/full disclosure/appropriate consideration of my complete health condition.

Of further concern are the numerous lawsuits against at least some of the doctors who perform the CCI surgeries which allege exactly that issue: that inappropriate unnecessary surgeries were performed.

The idea that I have to trust the a handful of surgeons, said to be the only ones who have the expertise to interpret the necessary imaging, to tell me whether I need irreversible surgery; when those same surgeons stand to financially gain from that surgery and have numerous lawsuits against them saying that they performed unnecessary surgeries, is unacceptable to me.

For what it’s worth, I researched CCI surgeries years ago in relation to EDS, before there was any discussion connected to ME/CFS, and had serious concern at that time also.

I think that I am part of the population people on this thread are concerned about: so ill that I am desperate and have difficulty understanding all the scientific developments, with enough resources to possibly access medical treatments discussed. I appreciate that you are providing information here and are looking out for individuals who could be potentially harmed by inappropriate treatment.

I also appreciate everyone for sharing their experiences, including those who have had good outcomes from CCI surgery.

I know that this topic hasn’t been an easy one, but I think that it is extremely important to continue to share information on it despite that fact, and I hope people will continue to.

@Jeff_w needs to be the one to respond to the post as these are the surgeons he personally endorses. There is a wider universe of US surgeons who do CCI/AAI surgery in EDS patients. You can ask for recommendations if you join the Beyond the Measurement FB group or other CCI/AAI groups on FB.

What I do know is that a sub-specialization has emerged among neurosurgeons in treating patients with EDS and other connective tissue disorders. One reason for this is that many doctors won’t operate on patients with connective tissue disorders b/c of the post-surgical complications and wound healing issues unique to this population. Some neurosurgeons still don’t believe non-traumatic CCI exists, even though the majority of the patients these surgeons operate on will have imaging that meets their standards. I suspect many EDS patients w/ CCI are rarely referred to non-specialist neurosurgeons by neurologists and some were rather diagnosed with conversion disorder like I was. Furthermore, these neurosurgeons generally understand MCAS, which adds a whole other layer of complexity to surgery and which many CCI patients will have. Lastly, many EDS patients will have multiple neurosurgical issues, e.g, not just CCI but “complex Chiari” (Chiari + CCI) or tethered cord or Tarlov cysts or spinal fluid leaks or any of a number of complications. You have to treat or at least take everything into account and missing something can result in poor outcomes. And so even where non-sub-specialist neurosurgeons can do the surgery and would agree with the diagnosis, they might miss *a lot* in terms of additional diagnoses and in management of complications unique to this population. This comes in part with experience and volume.

However, there are more surgeons in this sub-specialty than the four Jeff recommends. He will need to tell you why those are the four he recommends.

I need to think more re: inappropriate treatment and diagnosis. I don’t know how best to address this. We have no idea if the surgeons are appropriately or inappropriately diagnosing or how that differs by surgeon. What I know is there are many, many happy “customers” and some who had poor outcomes. Aggregate data is hard to come by (the Henderson paper @Michiel Tack posted indicating mostly satisfactory long-term outcomes was helpful). What is appropriate is so specific to each person’s case. I disagree with the general anxiety that people are being taken advantage of by predatory surgeons and know that my own experience was one of surgery is actually really hard to get! However, I agree that caution is warranted and the importance of going in prepared and with both eyes open.

 

[vsou]

I have been following this thread with interest. What I don’t understand and hope someone here might be able to provide information about is whether it is accurate that CCI can only be accurately diagnosed by a handful of doctors worldwide? And, if that is accurate, why that is the case if diagnosis and treatment of CCI has been established for some time? (And if it is not accurate, why is that what is being reported?)

I am writing as a severely ill patient diagnosed with both ME/CFS and EDS; as someone without a strong science background and with severe cognitive symptoms, who is nonetheless trying to keep up with what is going on well enough to make wise medical choices.

I appreciate the work Jennifer Brea has done to advance ME/CFS advocacy, and am happy that her health has improved, and that others have had improved health outcomes also.

However I have concern about the possibility of CCI surgery being performed in cases in which it should not be. I have read that some think that no surgeon would perform that surgery unless it was clearly medically necessary. I am genuinely very happy to hear that some people have had such positive experiences with medical professionals that they believe that.

Unfortunately, I have had very different experiences, despite the fact that I have been ‘fortunate’ enough to see expert doctors and specialists at very well regarded medical institutions. I have had multiple doctors offer me treatments, including but not limited to rituxan, that I do not believe were appropriate for me, without caution/full disclosure/appropriate consideration of my complete health condition.

Of further concern are the numerous lawsuits against at least some of the doctors who perform the CCI surgeries which allege exactly that issue: that inappropriate unnecessary surgeries were performed.

The idea that I have to trust the a handful of surgeons, said to be the only ones who have the expertise to interpret the necessary imaging, to tell me whether I need irreversible surgery; when those same surgeons stand to financially gain from that surgery and have numerous lawsuits against them saying that they performed unnecessary surgeries, is unacceptable to me.

For what it’s worth, I researched CCI surgeries years ago in relation to EDS, before there was any discussion connected to ME/CFS, and had serious concern at that time also.

I think that I am part of the population people on this thread are concerned about: so ill that I am desperate and have difficulty understanding all the scientific developments, with enough resources to possibly access medical treatments discussed. I appreciate that you are providing information here and are looking out for individuals who could be potentially harmed by inappropriate treatment.

I also appreciate everyone for sharing their experiences, including those who have had good outcomes from CCI surgery.

I know that this topic hasn’t been an easy one, but I think that it is extremely important to continue to share information on it despite that fact, and I hope people will continue to.

@Jonathan Edwards could you answer the questions from the first paragraph? If I understood your posts correctly, you are saying that CCI is a condition that all doctors are taught about (unlike ME, and actually from my experience in the US EDS either, though it may be different in the UK). So the idea that there are 4-5 doctors worldwide who can correctly interpret imaging to evaluate for CCI? What should I make of that? As a severely ill person, trying to research though I am often too ill to sit up or formulate ideas, that idea is definitely out there. Thanks for your time.

 

[Jeff_w]

@Jeff_w needs to be the one to respond to the post as these are the surgeons he personally endorses.

I responded to that just a few minutes ago. See my reply, above: Here.

Informed consent is really important. I definitely had that from my surgeon, in addition to the research I did on my own. But no, I can’t guarantee that all surgeons go through that same process with their patient.

Informed consent is another reason for carefully choosing a neurosurgeon.

If you choose to go to a neurosurgeon who does not fully inform you of the potential risks of surgery, then that should give you pause. I would not trust that neurosurgeon.

 

[Diluted-biscuit]

@Jeff_w needs to be the one to respond to the post as these are the surgeons he personally endorses. There is a wider universe of US surgeons who do CCI/AAI surgery in EDS patients. You can ask for recommendations if you join the Beyond the Measurement FB group or other CCI/AAI groups on FB.

I’m been debating posting this or not as I’ve not wanted to seem ‘hostile’ but I have to ask.

You said earlier in this thread that when you are asked questions you often direct people to Jeffs website for the answers. To me that is endorsing the information including the surgeons. Am I the only one that feels this way?

 

[JenB]

As I said before on here, in absence of a biomarker, all of us actually have *presumed* ME, no matter when we were diagnosed, or who we were diagnosed by. I have no doubt I have ME, nothing since 1982 makes me think I don't. But it is still presumed.

I guess I wonder if, since CCI is fixable, and ME is not, would you not now be happier if you *had* had a misdiagnosis of ME and a correct diagnosis of CCI?

Misdiagnosis happens. Am sure it happened in 1980s too, but probably less frequently than before Oxford criteria. And now we have a multiplicity of criteria. I know that you met ICC-ME and have confirmed diagnosis from top specialists. But you were still presumed ME, like all of us. And now you have made a remarkable and unusual recovery.

But most pwME, even those v severely ill for years, simply don’t have these particular alarming new symptoms you and Jeff developed after your respective surgeries. With all I am hearing about CCI, and other new cases, would there now perhaps not be a push on making CCI a rare differential diagnosis? Then people like you and Jeff would get access to surgery faster.

Or maybe that is too simplistic. And CCI is in fact holding some key to complexity of classic ME. (EDIT - to add last sentence)

No I would not, for all the reasons I have previous explained: https://link.medium.com/b3DcenVE7W

Yes, I do think CCI holds a key to our understanding of this disease.

 

[Jeff_w]

@Jeff_w needs to be the one to respond to the post as these are the surgeons he personally endorses. There is a wider universe of US surgeons who do CCI/AAI surgery in EDS patients. You can ask for recommendations if you join the Beyond the Measurement FB group or other CCI/AAI groups on FB.

I would encourage everyone to do their own research. My standards for choosing a neurosurgeon are quite high. Other people's standards may differ.

The "wider universe" of neurosurgeons are not ones who I can personally recommend, having done extensive research. I have seen them make too many errors in diagnosis, treatment, etc.

 

[Guest 102]

No I would not, for all the reasons I have previous explained: https://link.medium.com/b3DcenVE7W

Yes, I do think CCI holds a key to our understanding of this disease.

Sorry, Jen, but can’t read the new Medium posts and links at moment, I can’t absorb lots of detail, easily. I find the threads on here hard to follow. I did read your Medium post when you first got CCI diagnosis, before fusion surgery, last year and thought it was v well written - and I feel I have enough background on your situation. I guess I will wait for research to unfold these CCI connections, such as they are, but I honestly remain sceptical that it will help ‘Ramsay-MErs’ like myself from 1980s.

The chances of my having non-traumatic CCI (just read a post of Jeff’s) that is in any significant way contributing to my ME since 1982 are probably zero. I just don’t think you can extrapolate your own experience to others so determinedly as you have been doing in all these posts. There is no evidence, just anecdote. But none of us know the mechanism of ME, so never say never. (Edited)

 

[vsou]

Thank you @JenB for taking the time to respond. @Jeff_w would you be able answer my questions about why there would be only 4 doctors who can correctly interpret CCI imaging if this is a condition long established and treated?

@JenB I can not seem to locate where I thought I read who your surgeon was to see if my failing memory is correct so apologies in advance if this isn't correct; but I thought I read that Dr. Bolognese was your surgeon. If that is correct can you share whether you got multiple opinions prior to having surgery and how you evaluate the lawsuits by former patients of of Dr. Bolognese which allege that he has performed unnecessary or inappropriate surgeries?

There are a lot of extra concerns that arise with EDS and surgery of any kind, which actually makes me more concerned for patients who have EDS and ME (or EDS alone) and are considering CCI surgery. However, for me, going to one of the sub-specialist neurosurgeons really doesn't help those concerns since the lawsuits have been filed by people with connective tissue disorders.

It was several years ago (prior to Jeff posting about his research and surgery) that I researched this issue and I am too sick now to go back and do it again. However, if anyone is interested the information is out there, and like the story of the young woman who died in Oregon, the lawsuits include very compelling, concerning, sometimes heartbreaking stories.

I understand that your situation was different @JenB in that you had symptoms that clearly required surgery, which I would imagine made making the decision to have it easier if no less scary. But for those who are not in your situation, but are desperate to get better, I hope they will take the time to consider all these issues.

Every time I hear another one of the recovery stories, if I am honest, I have an immediate initial feeling of panic that I better get to on of these specialists fast before their practices fill up and I lose all hope of any recovery. Then my reason returns, and the reality that I am too sick now to travel to the East Coast even if I wanted too. However at another point in my illness I might have been desperate enough to take impulsive action without full understanding of all the issues. As I mentioned, I am in favor of everyone having the choice of what they are willing to risk to get better, but want everyone to have adequate information to make an informed choice.

 

[Guest 102]

Karen says she has ME. It is very possible to have both ME and hEDS. Many people in our community do. Several clinicians have written about this.

I am not sure why embedding something from a public platform is against the rules but will defer to the mods on that.

I am not sure *many* do.

 

[JenB]

Hi folks,

I am pretty sure I will get moderated as we are not supposed to talk about moderation but I realize that the level and nature of moderation on this board is just not something I am comfortable with. I have read this and other threads under the assumption that I am seeing a true and accurate reflection of everyone’s POV (who chooses to participate here) and that is not the case. I am disturbed by the number of people who have been quietly put on moderation or had their posts modified or deleted.

I would like to be able to stay, answer questions, correct inaccuracies, and contribute to the conversation, but I can see this is not the place to do that.

If this post does see the light of day, you can ask me further questions/ping me at:

https://twitter.com/jenbrea
jennifer@meaction.net

No hard feelings to any of the mods—your house, your rules. Just not my cup of tea.

Thanks!

Jen