atlantoaxial subluxation C1-C2, stenos./myelop. C5-C7/EDS surgery? stats

Hello,

Just wondering how many EDSers out there with or without overlapping features of varying types have been dx'ed with the above cervical issues, are struggling with loss of neuro. function, and are considering or have had fusion surgery in the cervical area either to preserve function and/or to prevent paralysis? Also for those who haven't done the fusion and have AAS, have you managed to avoid the worsening through other means, including diet, exercises (isometrics) etc. etc.? The only support group I can find online for AAS is for DOGS, and this is makes things a bit difficult!

After my dx's from Dr. Henderson to have three surgeries, I am trying to choose the right path with the goal being that of the best chance of longevity and quality of life (49 years old- sort of Type III with manifestations and symptoms of IV and I, including early family deaths from CVAs etc., general weakness in thoracic/blood vessels, bleeding problems in past and present). We are from MA, and since there are no real studies or stats on this, I've been scouring studies on RA, which show patterns of survival as better with C1-C2 fusion and/or with chemo etc. but with the few studies on EDS showing poor outcomes over time; trying to weigh options. Would love to talk to EDSers from both camps, surgical and non-surgical. I am also very interested in talking to EDSers about the presence of auto-immune symptoms, particularly issues with overreactions in histamines, as there are studies in RA that show that chemo, which dampens immune response, can sometimes reverse AAS (but I feel so strongly that EDSers need to see some studies out there re: prognoses with and without C1-C2 fusion surgery!)
We're not sure if I will be even cleared for surgery (most docs are running the other way), but I do want to learn as much as possible.

Thanks so much to all.

Sincerely,

Diana Cleaveland

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9 replies. Join the discussion

Hi ---

Not sure if I qualify to answer - I don't think I have anything but regular old bad neck arthritis - I think my issues are worst at C4-C5 if I remember right - but also severe foraminal stenosis at higher and lower levels as well.... some central cord compression - but thus far not too severe - meaning I think mildly pushing in on the cord. I've got the miserable debate as to whether my experience of weak legs in spite of a fairly normal neuro exam experience (save some poor reflexes and loss of vibration sense, small fiber neuropathy too) is due to the radiographically evident cervical myelopathy events - or perhaps just weakness exacerbated after several other surgeries and in general poor conditioning since forever due to EDS....even as a kid not being able to do things in gym class etc...

So while I get neck pain - usually to either side - I haven't had a severe bout of arm issues in quite some time now. They do tingle - but I don't know if thats due to severe shoulder ortho issues, or do to my neck or due to carpal tunnel, or do to small fiber neuropathy. Tingling I can handle. Sometimes I get those bad headaches that start in my neck and then go up to the top of my head and then to my eyes - often this can be blood pressure related it seems - or maybe the blood pressure goes up d/t the pain - not sure.

One of my EDS doctors suggested that I might feel a bit better with the lift that a soft cervical neck collar can provide. Not to depend on it - but for sporadic use - to help elevate the neck head and chin upward - giving the neck some lift and support. I'll have to figure out how to get that as I forgot to get a script from him for one.

On days when I feel really lousy and weak - I wonder if it's my neck - but then I remember the other doctors saying that nothing shows it's my neck. All studies prove otherwise. In the meantime I'm caught in the middle - with the words in my head, 'you will become a quadraplegic if you don't have the surgery' 'you will soon be in a wheelchair' - Versus - 'I wouldn't let anyone do surgery on your neck as your neuro exam shows normal'.... Basically I just try not to think about it too much or I'll get all upset.

So again I don't have the AAS thing, I doubt I have tethered cord though it's been mentioned - I just have what one doc said was a swan neck deformity in my neck - and narrowing from disk and facet joint issues...

I do know that I had four major surgeries before ever knowing I had connective tissue issues - and while I'm not as strong and able bodied as I'd like to be - I had no major issues whatsoever - no infection, no problems with healing or terrible scars, no clots thrown or any PE issues - pretty much had a clean go of it each time. Yes I have issues and some now wonder if they weren't caused by the spinal anesthesia or positioning during surgeries etc... And one of the outcomes was not as good as I'd like - but that was surgical technique related.... All and all - I faired pretty well. But I keep telling myself I will refuse surgery for as long as I'm able to say NO - because enough is enough - and for me 4 big ones is enough. Deep down though I know if I MUST or lose my life - I'll probably switch and say, "well okay - one more" Now 2 years later - two of my big scars are getting pretty wide and papery - but that's minor really - as the wounds are totally healed.

Blessings to you on your path - I'll be praying for you - wisdom and confidence in what you choose to do - and that you find the studies and assurance you need to understand what may be the best way to go.

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Diana, did I give you this link? https://www.facebook.com/n​otes/lisa-j-markland/im-pa​ssing-along-the-docum ent-a​lready-on-this-page-inform​ation-for-those-with-ed/10​150193883297833 for a viewpoint to counterbalance Dr. Henderson's statement in his conference presentation that "Every patient surveyed, with the exception of one, said they would do the surgery again if they had the choice, and everyone said they would recommend it to a friend or family member."

I'm 54, been living with bad neck and spine problems for at least 5 years now, so they discovered my syrinx and other problems when I was your age. I've had a couple of surgeons examine me and tell me they refused to do surgery on me until and unless it was a life-threatening situation because I had no chance of successful rehabilitation. The problems I've had don't get better in my experience; but they also haven't got worse as much or quickly as feared back then. I have no idea whether the added disability from the surgeries would be any worse than I've gotten on my own, but I'm still not sure I could manage post-surgery at all well or do better long-term. But I'm an obstinate ******* who may not ever be able to manage rationality around this issue, even if faced with life-threatening. Ultimately you can only make your decision based on your own body and views, not what any of us say; and the more positive you believe the outcome to be, the more likely it will be (there's actually data on that!).

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Thank you. Thank you. Thank you Dessert1st, for your insightful perspectives, and EDSerMark, for your own objective lens, and an extraordinarily helpful link. I, like you both, am very independent (or @#$Y$#@), depending on the situation, so also very much into the idea of doing as much as I can by thinking in a way that is both logical and as non-invasive as possible, while also doing due diligence by getting many opinions. I've wondered so much why AAS shows up with RA and MS, Behcets and other autoimmune diseases, and EDSers often have either concomitant autoimmune issues and/or MS etc. etc., and also why it isn't obvious that there may be an autoimmune co-existing causal relationship and/or continuum between genetic collagen disease and autoimmune collagen disease (at least in a certain percentage of cases)! Not saying that surgery is unnecessary for all (and will do my own triple checking here), but dagnabbit, why don't these fancy doctors TRY out these other simple meds first, particularly if they will be administered post-fusion-surgery ANYWAY? I know, I know, I'm way too naive, and that has often gotten me into way too much trouble. But I will definitely not go gently into the long surgical night without first having tried every possible alternative! (By the way, I've noticed that they treat MS and RA atlantoaxial subluxation with chemo and anti-convulsants, along with a host of other stuff that EDSers often take)- wonder what would happen if they did a trial on those who had MS and/or RA (thus, with the proper funding and backing) and also tap into those w/ concomitant hypermobility/EDS- spinal issues, with chemo and/or anti-spasmodics, to see if these reversed the spinal issues? Now, THAT might be really interesting, particularly as there is one study that shows reversal of AAS with particular chemo treatments, in those with RA and others who don't have RA! I know that chemo is no cakewalk either, but I sure do find the idea of tamping down the immune system in a particular way very interesting in terms of some of the things that go with EDS.

Dessert1st- blessings on your path, too- I admire your choice and your spunk for taking one day at a time. Love and light to you both. EDSERMark, did you read the New Scientist article recently on the topic of positive-outcome-thinking? That's what your last sentence reminded me of. Thanks for that as well (maybe there's also a placebo for Bac*****; now that would have to be one really honking big pink pill (oh that's right- more like a drink so that we could more easily swallow it!) Sure beats metal prongs!

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I had the surgery. I don't think I'd do it again, if I had it to do over. A year later, my range of motion is so limited, I'm making modifications to my car. The neurological issues, which had disappeared, are back. And the debilitating headaches have returned. I've heard others say this indicated more surgery. I was unwilling to have multiple spinal surgeries before the first one was done. Thankfully, I saw a different neurosurgeon last week. He said there was no medical evidence that further surgeries would help.
Sorry, I don't know if that's what you wanted to hear, but it is my honest perspective 11 months following the surgery.
Best of luck to you. I hope you find your answers.

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Good morning Diana.

I have severe mixed type EDS (see profile) and, specifically, extreme instability in C1-2 (compared with severe instability in the rest) following accidents, surgeries (one EDS caused), and prolonged Tx.

I have discussed stabilization with my highly skilled and EDS-informed manual therapists, all of whom say what happens when one fuses C1-2 is that the cervicals below become hyper mobile and unstable in response.....SOMEthing's gotta give....

Micro exercise--isometrics etc daily and minute by minute practice of constant body awareness about how I turn, move, sit, sleep, have improved stability over several years (yes it takes infinite patience and stubborn optimism). I accept that I go through cycles of instability but i also know that I can cycle up, not just head down. If I quietly treat my neck vulnerabilities as serious, paradoxically I can avoid their becoming serious; if i treat them lightly, they become serious. I work full time and have an active life, during which I sometimes go through months of daily migraines, numb arms (at night), referred pain etc. I still wouldn't exchange that for irreversible surgery that, in my and other opinions, just passes the buck to another area, which, in turn, becomes troublesome.

I don't like irreversible procedures done on folk who have diagnoses not fully studied or understood.

I am not familiar with the use of chemo for any of this but I confess to a bias: I am on longterm preventive oral chemo for cancer and find that this particular chemo compromises the integrity of the soft tissue and just makes it even harder to maintain maximum stability in the cervicals and other areas.

You might want to look at prolotherapy but try it on another part of the body first to see how your body reacts. Personally I have had it recommended by those same practitioners I mentioned above and trust but my instincts say don't do it and my instincts have saved my life three times so I trust them.

Cold laser can also help stimulate the collagen production and general blood supply to the area. My manual practitioner uses this. It also reduces pain and inflammation.

Hope this helps to add to your due diligence.

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Dear Povi,

Thanks again, for your ideas- they're all very illuminating- what do you all think of the neck brace thing? Povi, do you wear a neck brace, all or some of the time? Just wondering if it's something that over time will worsen or improve the subluxion- instinct says that anything that keeps your neck totally unable to move 24/7 will over time weaken all the things that we need to hold our heads up!

Never heard of cold laser therapy, but it does sound interesting!

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I'm so glad to see this thread, as I have always questioned the conceptual basis of the stabilization surgeries in our case. I'm currently experiencing breathing and swallowing difficulties that could easily be traced back to C1-C2 instability. But I am trying to find out more about exercises that might help. We are always advised to use our muscles to stabilize our joints. OUr necks are chock-full of muscles. I feel as though this is a good place to start before strapping on the c-collar and getting surgery. Any advice as to where to begin to research this would be greatly appreciated!!

And, yes, Povi, the minute-to-minute awareness is the only way. I call EDS my ticket to enforced mindfulness. (But it is exhausting!)

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Hi there EllyMae,

Thanks for responding to the thread- I do hope your breathing and swallowing issues aren't too challenging; I have them, too- only eat once a day because things are so hard to digest, and digesting makes the tach. run faster. When things get stuck in the throat, I have to pound on my chest really hard (barely any gag reflex left), and with the hiccups that follow, I often remedy that by leaning down and swallowing a drink upside down, VERY carefully, but it usually works to fool the peristalsis into working right. (Makes for some very interesting dates with hubby!) I am going to look into Baclofen for neurogenic bladder; a little birdie out there said that it can help with spasticity, and that maybe it might even help with the AAS.

It's so strange that you talk about safe neck exercises, because that's exactly what my husband and I were discussing today. Do you ever get laryngitis and very weak voice in speaking or singing? The neurological changes for me sometimes result in sudden loss of range (vocally); ironically, I was a professional musician (singer/violinist/pianist/composer/teacher). Not about to let that "go down," today we went to an orthotist who fit Bauerfeind braces from the ankles, knees, back, up to elbows, and wrists, with lateral supports and orthotics so that I can start to walk more straight and actually walk and exercise- hopefully! (I've started to lose a lot of neuro. function, and tend to fall sometimes, so I have had to use a cane and lean a lot on my poor husband). But the BEST brace I got today is incredible; it has both stretchy and stiff backing, and it protects the lumbar area all the way up! This morning, I could barely croak out a word, but this afternoon, with this new brace that propped my innards totally in, propped my shoulders back, found a new, taller neck, now naturally pinioned to a somewhat large head, (kind of like a big basketball on a golf tee), but with a lot less pain! So I went home and started singing- in spite of laryngitis, I ripped that neck brace off, the Bauerfeind midsection brace pushing my lax innards together, forcing me to use all those old diaphragmatic muscles, AND, my neck muscles- it's a bit like a corset, but with support to hold stuff together! At first, it was very challenging, and the tachycardia got pretty bad, but by the end of 30 minutes, I was singing about 8 higher notes, with more strength than I had mustered for months (too busy worrying about all this doctor/thinking about potential death and paralysis business). It was then I realized, "SINGING is what I've always done my whole life, and it has kept my neck strong (up until the whole C1-C2 C5-C7/neck surgery/brace fiasco)- so singing and playing the piano/composing again is what I will start to do again, even if it's for 20 minutes a day!" It also helped my morale to hear the band Renaissance in NH the other night, (as I used to play/sing with the pianist for the band in gigs years ago); it felt so normal to be a real person again, not a patient, to be invited to hang out after the concert, and to have my musician friend treat me as if I'd never been sick, as if I hadn't lost the capacity to be that former "somebody!" Also, like you and Povi, I think mindfulness is so important, along with allowing for both bad and good days, seeking both old and new validation, cherishing interdependence and friendships, while not giving up all the things we like to do simply because docs think it's "too dangerous." It sure is scary to see things change over time, and to feel the neurological symptoms get worse, and to be aware that it might all suddenly come to a halt suddenly, but it's sure worth giving it one helluva try on the journey. (At least that's what I felt today- tomorrow could be the opposite, but that's OK too). After the rest of the braces arrive, I'm hoping that the lateral and hinged support will help straighten out walking, too. At any rate, tonight my husband felt my neck while I was singing, and he was amazed to feel how much movement was being used in this area, along with this massive improvement (with the help of the lumbar/thoracic brace) of my just being able to sit up straight more effortlessly, and to start using all the muscles I used to take for granted again. So singing is what I am going to get back into as much as possible in order to try to turn this neck thing around, because one has to use everything from diaphragm to head and back in order to sing, and the oxygen that goes to the brain is great, too. Even breathing deeply into the neck while meditating might help, and/or doing reading aloud or even some cold acting readings/poetry?

At any rate, I do wish you the best on your path, and to be able to follow your own deepest instincts. With our new mindfulness, every time the docs look at the scans and the results, my husband and I are actually starting to find it interesting and sometimes humorous to stand outside of the situation and simply observe. In that observational state, I can see that doctors' often fearful responses or lack of answers or guidance no longer have to result in my feeling lost or afraid. Docs are human, so they may be projecting their own fears onto us, and/or have their own agendas or ideas based on their own lives; as much as they care, they truly often can't know who we are as people, haven't seen our successes and joys and work, hopes, dreams. It took awhile (and some pretty down days) for me to appreciate that dreams don't have to just disappear, whether due to aging or illness. So, what I'm learning from all the folks on this great EDS Inspire site is that we always have the choice of when it is time to wear a brace, to take it off, to take a nap, go on a joyride, to have surgery, or not; after all, nobody knows much about this C1-C2 subluxation stuff. All we have to know is to trust what's inside- that place unique to each of us that makes us get up and want to live, laugh and love, for just another day.

Breathislife, thanks for your honest perspective in your courageous path, too; it's all so hard to deal with, and we're all in this little boat, making the best decisions we can in every moment. Love, healing and light energy sent to you as well, and to everyone who has taken the time to respond. Thanks to you all!

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I sang all through high school and college (though that was long ago). I sang at weddings, at church, in a cappella choirs, and all by myself at home. Singing was one of my great joys. I had a whiplash injury at 23 or so (51, now), and I think that was the beginning of serious problems for me. I'm down to about a 4-note range, now--G-C to be exact.

I have swelling in my larynx, and my vocal cords have been damaged by reflux, really severe reflux, for 10 years before I got surgery, which helped, but did not cure. I just pulled an article from the ear, nose & throat journal which speculates that our vocal cords deteriorate because of the high level of collagen. That said, I intend to sing however I can.

I agree that you cannot let the doctor's anxiety become yours!! Listen to them, but recognize that they have their own viewpoint.

I have some evidence of craniocervical instability, but I have not had the full workup, as I am not willing to go through surgery. My trouble breathing is obstruction at the level of the larynx coupled with laryngospasms from mechanical irritation, which I trace to a malfunctioning upper esophageal sphincter which delivers reflux to my larynx + bad posture that sends post-nasal drip onto my vocal cords instead of down into my stomach. The UES is innervated by C1-C2, just where I have trouble. Swallowing has been uncoordinated for at least a decade. Not sure how to fix that.

But, I really think that strengthening the muscles that control the UES + working on the muscles that stabilize C1-C2 would go a long way to helping me. Just not sure how to do it, and am trying to come up with a plan myself until I can find someone to help!

Keep at it Oriablue! Forge your own path.

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