Tracking CCI / AAI MRI & Treatment outcomes

[valentinelynx]

At this time, I can do more than most with ME/CFS, but much less than a healthy person. I currently struggle most with cognitive and sensory issues on a chronic basis, while my fatigue waxes and wanes.

@Diwi9 : I wonder if you could be more specific about what you can do more of than "most with ME/CFS". I'm waiting for my consultation with Dr. B, and worry I'll be deemed not sick enough for surgery, despite having been diagnosed as CCI from my MRI by Dr. B, and having ME/CFS, constant widespread pain, MCAS, probable POTS, autoimmune issues (floridly positive CellTrend, GAD65 +, positive Sjögrens), immunodeficiency, receiving IVIG, gut issues with probable gastroparesis. And then there's the complete sleep reversal that has me still awake at dawn... Home traction feels like a good pain drug for a short period (less than an hour), while my PT's traction made me feel high.

 

[Mendo Zebra]

Home traction feels like a good pain drug for a short period (less than an hour), while my PT's traction made me feel high.

Dr B seems to be very focused on positive responses to traction. Yours sounds good. He dismissed me and said to get back to him, only if and when I have a "very positive response" to traction. My narrative is earlier on this thread. Good Luck.

 

[Diwi9]

@Diwi9 : I wonder if you could be more specific about what you can do more of than "most with ME/CFS".

. It's ironic to read my old post on a day when I'm doing poorly, but what I meant in that post is that I am able to do some physical exercise and can be upright for many hours. I generally do not have issues walking. The later it gets in the day, the better I feel. You mentioned the sleep reversal issue, that is a huge problem for me at the moment. I really hate sleeping half the day away and being up all night.

 

[valentinelynx]

I really hate sleeping half the day away and being up all night.

That's me, for sure. If I have an appointment or something in the day, I just get up and do it, although I may have had 0-3 hours of sleep, having gone to bed after sunrise. My husband hates it more than I do; he says he never sees me.

When you say that you feel better later in the day, do you mean after you've been awake for awhile, or that you are able to get out of bed more readily later in the day?

I am able to do some physical exercise and can be upright for many hours. I generally do not have issues walking.

My ability to do some exercise varies. A couple of months ago, I was doing a few squats and some wall push-ups. Now I don't feel up to it. I can be upright if necessary, but it doesn't feel good. No one, including me, understands how I could do 10 hour work days before my workplace went out of business. I could do it for 3-4 days max, but felt really bad at the end of the day and worse each successive day. I refused to work more than 4 days in a row. The best explanation I've heard is that I was "running on adrenaline". That's what it felt like; when activated by the need to "perform", I don't feel the pain as much or the pain of the fatigue. It was good for my ego, but bad for my body. I want to be able to enjoy working again.

 

[valentinelynx]

And I wanted to share a few details about Dr Bolognese’s initial consultation.

Questions he asked:
“When do you feel the worst?”
“When do you feel the best, most comfortable?”
“What has helped you the most?”

I had written up a detailed list for him ahead of my appointment but these questions caught me off guard a bit and I am still not sure I represented myself accurately.

@Mendo Zebra I wanted to thank you for posting that. I often lose my words and train of thought speaking with doctors (yes, I'm a doctor myself, but if anything that makes me more afraid of being judged by doctors...). Having these questions in mind ahead of time may help when I have a chance to talk with Dr. B.

Here, I'll try to answer now:
“When do you feel the worst?”

When I've been up and active for a few hours. After something has made me anxious or required physical exertion. After sitting and working on a laptop for a couple of hours.​

“When do you feel the best, most comfortable?”

After I've had a couple of hours of good sleep, especially REM sleep. Best if I fall asleep while using buccal ketamine (special lozenges from a compounding pharmacy) so I wake up with almost no pain. Sitting in a hot bath. And, I've now discovered, after a traction session! (but that only lasts a few minutes)​

“What has helped you the most?”

Medications. Different ones over the years. Tramadol was once a wonder drug years ago, but no more. Pain medication now makes life tolerable. The ketamine helps a lot, as well. And massage, specifically Thai massage, is very helpful for reducing pain from muscles, joints and connective tissue.​

​

Anyone else want to play?

 

[Diwi9]

When you say that you feel better later in the day, do you mean after you've been awake for awhile, or that you are able to get out of bed more readily later in the day?

. Both. I need a few hours after I wake up to get out of bed. But, sometimes late at night I can start to feel pretty good. It never lasts; everyday is Groundhog Day.

 

[rel8ted]

I am going to say something here that I think needs to be heard.
I was talking to another person who has had a cervical fusion the other day, we both are hypermobile. The discussion resulted in the following conclusions: you might not get the stellar results from surgery you hope for (likely), improvement is slow, results may vary with different surgeons, life is never going to be normal again. and you must take precautions to protect your spine because the fusion may cause instability at other levels later (confirmed by DPT who treats EDS patients). I had a compressed spinal cord and was rapidly deteriorating, surgery was the only way to change that (and improvement at 2 months is minimal, at best). I am in no way claiming to be a doc or condemning people researching CCI/AAI surgery, I am just saying it is a really, really big deal and should be a last resort. As a result of my fusion, it is now quite apparent that I have tethered cord. I had suspected it prior, but everything is amplified now. Facing the possibility of a second surgery is a huge thing. Knowing the possible outcomes is not making it any better. I will say that I would not have found my instability if not for seeing an EDS literate neurosurgeon, but I am also eternally thankful for his ultra-conservative approach.

 

[mattie]

1 year post-op update

Exactly 1 year ago I had C0-C2 fusion surgery in Barcelona.
I have written in detail about post-op experiences in a private thread on this forum.
After 1 year things have stabilized and I feel it is time to write a public statement.

I have ME and POTS since the beginning of 2016.
It started with POTS and moderate ME in 2016 and progressed into severe ME.
I was mostly bedbound in 2018.

Diagnosed with CCI (Craniocervical instability) and AAI (Atlantoaxial instability) by Dr. Gilete in Barcelona after getting the required scans from Medserena in Hannover. (01-2019)
My measurements showed translational BAI and translational BDI and obvious AAI.

With the help of my wife, a wheelchair, a cervical collar and some Klonopin I barely managed to drag myself to Barcelona for a consultation with Dr. Gilete.

Dr. Gilete recommended and I decided on posterior fusion surgery (C0-C2 fusion) after carefully weighing the possibility of improvement of symptoms and quality of life over the risks of surgery and the debilitating nature of a cervical fusion. That was the toughest decision I ever made.

The surgery was only 1 week later.
After the trip in Barcelona I was obviously in really bad shape and I feared that this major surgery would wipe me out completely. That never happened..

The first week post-op was really rough but doable. Pain management and hospital care were excellent.
Full recovery from this surgery takes about 6-18 months.

After my C0-C2 fusion operation in 02-2019 I immediately went from severe ME to moderate ME. I was no longer bed bound. Up to this day I have managed to stay out of the bedroom prison.

Three months after surgery I was interviewed by Jeff_w. You can read about my improvements in detail on his blog.

Most improvements came about directly post-op and in the following 6 months.
At 6 months I kind of plateaued. I have not been able to further increase my activity levels. I still have ME and POTS. I am still sick and very limited. I still get PEM when I overdo things.

So why only a partial improvement of symptoms?
One would expect that if CCI/AAI were “the one true cause” of my illness that improvements would have been more substantial. Obviously ME and dysautonomia are more complex than that.
Dr. Gilete himself mentioned that CCI may very well come secondary to ME.
Indeed after my surgery I recovered to a level similar to where I was at the start of my illness in the beginning of 2016.
Dr. Naviaux recently mentioned that:

the ‘fatigue factor’ in the blood of ME patients triggers collagen remodeling over time that can cause acquired forms of Ehlers Danlos-like syndromes

Article here: http://naviauxlab.ucsd.edu/wp-content/uploads/2019/12/Naviaux-Lab-Newsletter-Winter-2019_v10_sm.pdf

Disclaimer:
I have definitely improved and I am grateful.
Moderate ME is still no joke but it is liveable. Severe ME for me was not.
Obviously I will never recommend this surgery to anyone. I did it because I felt I had (almost) nothing to lose. For others a much more conservative approach may be advisable.

My case proves that having ME and a diagnosis of CCI/AAI does not mean your problems will be resolved with fusion surgery. I was willing to try it and some other desperate patients will try it too, but make sure you are well aware of the short and long-term risks and side effects. Also limit your expectations and make sure you can handle an outcome with limited improvements or even no improvements at all. You will be a guinea pig. You may even get worse.

The relationship between CCI and ME remains unclear; we only have a few anecdotal success stories.
More research is definitely needed.

What is also needed are updates on the outcome of other ME patients who have had this surgery. @Julia_S , @StarChild56 and I know there are more…)
At the time of writing this, 82 patients on this forum have been tested for CCI and/or AAI. Over 75% of them tested positive.
I can imagine they are eager to read as much as possible on the treatment outcome of others.
Facebook seems to be a much more active place on this topic. I am not on Facebook. I value my privacy.

Will keep you updated,
Take care,
M.

 

[valentinelynx]

@mattie
Since you bring up this:
"Dr. Naviaux recently mentioned that:
the ‘fatigue factor’ in the blood of ME patients triggers collagen remodeling over time that can cause acquired forms of Ehlers Danlos-like syndromes"
I'm wondering if you had signs of EDS prior to falling ill with ME/CFS?

 

[mattie]

@mattieI'm wondering if you had signs of EDS prior to falling ill with ME/CFS?

Nope, no signs of EDS.

 

[sb4]

Nope, no signs of EDS.

Did you have signs of EDS after coming ill? Aside from the CCI (?) ?

 

[Rufous McKinney]

"Dr. Naviaux recently mentioned that:
the ‘fatigue factor’ in the blood of ME patients triggers collagen remodeling over time that can cause acquired forms of Ehlers Danlos-like syndromes"
I'm wondering if you had signs of EDS prior to falling ill with ME/CFS?

In my view, this is likely a key factor involved in our ME. I've been ill for over 55 years...but mostly it all got much worse...in the last few years. My fingerprints are gone, my neck ligaments and muscles seem barely able to hold my vertebrae together. I've not been evaluated but I am personally convinced that the LONGER this goes on the WORSE this collagen problem can get and is highly likely contributing to my current problem.

Yet I doubt I am a surgery candidate. I'm heterozygous for hEDS.....per Ancestry DNA. Maybe that- somehow is able to express or indicates more vulnerability to collagen breakdown.

I used to admire my flexibility (not EDS level- but just more than most people).

 

[mattie]

Did you have signs of EDS after coming ill? Aside from the CCI (?) ?

No I did not.

 

[Mendo Zebra]

I had signs of EDS as a child but my EDS phenotype didn't "bloom" until after a few whiplashes in my 40's. With EDS came POTS and MCAS. And filling criteria for ME/CFS. And probable CCI.. ? It seems there are a few different paths to these... This article is very interesting: "Brain injury unmasking Ehlers-Danlos syndromes after trauma: the fiber print." I have the pdf but can't get it to upload, "
"Conclusions
We herein showed that patients with a hypermobility type of EDS were very frequently found to have specific brain lesions involving white matter tracts. Moreover, a significant proportion of our patients reported that a physical trauma had unmasked or worsened their EDS...."

the abstract: https://www.ncbi.nlm.nih.gov/pubmed/27102338

 

[valentinelynx]

I have the pdf but can't get it to upload, "

Here you go...

 

[Mendo Zebra]

Here you go...

Thanks!

 

[Rufous McKinney]

Here you go...

Just now: is the first time I have ever considered- that I was 18 months old in a car accident. The car spun out on ice and rolled over....I am tumbling upside down...there were no child car seats, no seat belts. The memory of spinning, and upside down and my baby blanket..and a person reaches thru the car window and pulls me out.

I was: ok. Checked by doctors and called Ok.

but maybe- something else ALSO happened that day..something that contributed to: all this. Add to list. stirr.

 

[hezza]

Thanks @mattie for your updates!

I have been sick w ME for 20+ yrs, at least 10 of those w severe ME. I always thought it was dengue fever (viral) induced. But a whiplash preceded dengue by 6 mos & brought on sleep problems & a measure of fatigue.

In retrospect I have had obvious signs of hEDS my entire life but was only recently diagnosed by a geneticist. Several rounds of Cipro for UTIs after already falling ill from whiplash/post-viral dengue surely didn’t help matters.

In 9/19 had Bolognese remote consult based on a supine MRI from 2014. Nothing indicated horizontal CCI on the scans but he strongly suspected vertical instability based on my positive traction response. He also suspected tethered cord. He ordered a cervical ct, renal ultrasound, & urodynamic study. Along w core strengthening & postural work w a pt, wearing a collar 3-4 hrs daily & another traction trial.

I was to alert him if I got worse- I did indeed get worse but how could I not when I have severe ME & now have all these appts to fulfill? Traction continued to be a rescue for me but it was temporary & not enough to overcome the repeated overextension of my energy envelope which thrust me into a downward spiral. Unfortunately when I let him know this he took it to mean the 2nd traction trial was negative. I tried to respectfully correct this but he no longer responds to my emails.

Meanwhile I was lining up geneticist appt, upright MRIs & a Dr Patel consult. So on 1/31/20 & 2/21/20 respectively these resulted in a diagnosis of hEDS & CCI + mid cervical instability. Patel referred me to his go-to pt who herself has EDS and it even caused a stroke but she’s used her magic body mechanics to pull up to very high functioning- it was amazing to meet her. She & Dr Patel both say that pt that is not EDS aware does more harm than good. I’ve lived that truth. Patel says if EDS aware pt fails for me he recommends C0-C2 fusion.

The dilemma is I prefer Dr Bolognese’s surgical technique. I also like the fact that he does ICT as a screening & will not operate without good measurements & positive clinical response. So guess I’ll keep bugging him until he listens

That’s the story up till now...

 

[StarChild56]

What is also needed are updates on the outcome of other ME patients who have had this surgery. @Julia_S , @StarChild56 and I know there are more…)

I have been spending my on line time on a FB group and I have not been here often.

Thank you for your update, I really appreciate it as I am sure many do.

I have been considering how/when to update here...I have some major medical challenges (not caused by surgery & still glad I had it). I guess I will just go over to my post that I believe is titled something with Update and CCI - and do my best to update.

Take care and wishing you the best.

 

[winterschlaf]

I have been spending my on line time on a FB group and I have not been here often.

Thank you for your update, I really appreciate it as I am sure many do.

I have been considering how/when to update here...I have some major medical challenges (not caused by surgery & still glad I had it). I guess I will just go over to my post that I believe is titled something with Update and CCI - and do my best to update.

Take care and wishing you the best.

Been hoping to hear from you, @StarChild56 . Looking forward to your update!