Kaitlyn

Chiari & POTS

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 Two years ago I was told I had a small Chiari but that my symptoms were so violent the Dr. wasn't convinced it was the source of my problem.  I saw him again this week because I had seen an article that discussed loss of fine motor skills and Chiari and when my neuro psych eval was done in addition to cognitive impairment I had decreased fine motor skills (I wouldn't think this would typically be caused by Dysautonomia, chronic fatigue, lack f oxygen due to lie BP etc) and wanted to discuss pain in my neck and some stenosis that had been noted.

Anyway ... when he realized I have since been diagnosed with Dysautonomia and POTS he asked if they had an explanation for the POTS. Uhm ... no. Chiari can cause POTS and all of the symptoms you're having. Well I realized there was a connection but thought he had ruled the Chiari out. 

To make a long story short I am going to wear a vista collar for two weeks (again) to rule out any problem with my neck contributing to the dizzy/balance issue. He said if it isn't improved in two weeks the neck isn't the problem. Sounded to me like he was definitely putting decompression surgery on the table at that point. I'm feeling a bit shell shocked. So many questions are going through my head: could this clear up a lot my symptoms? What if it doesn't help at all? Is it worth the risk?

Just wondered if anyone here had Chiari as the cause of their POTS, had decompression surgery and how successful or what your experiences were with it?

Appreciate any advice. By the way, the Dr I am seeing is Dan Heffez of the WI Chiari Center and from the research I did two years ago he is one of the best in the country. 

Thanks in advance!

kaitlyn

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Hi Kaitlyn,

Glad your doctor is re-evaluating this for you.   I wasn't sure what a Vista collar was, so I looked it up.   I certainly believe that in my case at least, the problems in the neck are definitely contributing, so would be very interested to know how you make out.   I have on my own tried some cheaper foam neck collars just to see if this would help.  I have found that holding the neck immobile seemed to make the dizziness worse, but the Vista collar is certainly much better and hopefully may help clarify things for you.   I also have cervical stenosis issues, and, for example, yesterday I accidentually "jerked" my back and neck, and today the dizziness is very severe and just more POTsy.   Just curious as to when you had your first Chiari testing, was it an upright MRI?  I ruled negative for Chiari on MRI, but wonder if a regular lying MRI will show a more mild case?   I hope it turns out not to be Chiari for you, as I know the thought of decompression surgery is scary, but sometimes I wonder if it would be worth going through surgery to have relief of all our symptoms.  Please do let us know if you feel inclined, and good luck.  I know, I have read some on Dr. Heffez - wow, so glad you are able to see him!

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Hi Amy -

Thanks for your response. I guess with Chiari or Cervical Stenosis a person could have similar symptoms. I have to be soooo careful with my head and neck. Reaching for things, turning my head, rolling over in bed, any movement can set it off. 

I definitely have a Chiari 1Malformation. I noticed on the vista collar order it was one of the diagnoses he listed. It was found on a laying flat MRI. It is about 3 mm which doesn't meet the current clinical classification. However, I read a poster board presentation that Dr Heffez et al presented in 2012 where they divided patients into three groups: G 1 3mm or less, G 2 5mm (normally required to be classified Chiari) and G 3 >5mm. What they found was actually an inverse relationship between between the size and the amount of symptoms. In addition they didn't find one patient in group 3 who had a worse symptom than those in the other groups. What Dr Heffez told me is that even though I don't meet the 'typical' clinical classification Chiari is a funny thing. 

I think he was ready to let it go at the collar didn't help last time, it's not your neck. But I want to be 200% sure before considering surgery for the Chiari so I suggested giving the neck collar another try to be positive. I am more able to differentiate symptoms now 2 years later and know what is caused by BP drop versus POTS versus this 'other' thing that hasn't been fully defined yet.

The research I am seeing says 85-95% of patients see at least improvement. But he said I could have improvement but it isn't guaranteed. He also said we won't ever have more clarity than we do now. I have a Chiari. I have pots and it could be caused by it since there is no other explanation for the POTS or my symptoms. Chiari is one of those diagnosis by exclusion things.

Yes the vista collar is the only one he would recommend. I complained about it because it was so uncomfortable and I wasn't sure it helped it all. I actually think I remember it hurting more than helping but he didn't budge as far as a softer collar. He said it wouldn't do any good if it didn't completely immobilize my neck. Hope that bit of info can help you. :)

Thanks again

kaitlyn

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Thank you so much, Kaitlyn, for the further information.  Interesting that your Chiari was able to be seen on your "flat MRI."  I think sometimes radiologists could just miss it possibly, too, if it is small.   I sincerely believe that also, that you could have a small defect, but still be symptomatic from it.   Everybody is different,. and doctors may tend to generalize as to what are normal symptom patterns.   I always remember my mom's allergy doctor telling her that you can be mildly allergic and have bad symptoms, or be highly allergic and not have many symptoms at all.   Doesn't pertain to this, of course, but you get the idea.   But with you dealing with other conditions, that is the problem, not knowing what is causing what - it is so confusing, and I'm glad you are checking it out further.  Maybe it will give you a further much needed piece to the puzzle.   I can just imagine how hard the hard collar is to wear, as even the "soft" ones are hard to use.   I would be very interested to know if it ends up helping you.   I have the same problem on movement of the neck causing dizziness and faintness, and these are at times that I am not tachycardic, so you have to wonder if it is a totally separate thing.  I'm afraid, unfortunately, that we may never know for sure.   Can I ask you a question too?  (or any other forum members who could chime in!), whether you ever get up at night and immediately after on trying to walk, get very dizzy, weak and tachycardic?   I got this last night rather severely, even though I have had it many times before, just not that severe.    It is just my feeling, but I think holding the neck in one position while sleeping "sets things off."    

I just wonder, too, how many other forum members have neck issues and/or Chiari and have noticed dysautonomia "appear" later in life, being that POTS is usually diagnosed much younger.   If indeed these conditions can be causative, it would make sense that it can become more pronounced as we get older.  

I would have made a good polster, huh?  Sorry.   I'm sure everyone can relate, with the election calls upon us!  

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Yes it is interesting and sometimes I wonder if a 3 mm flat would be 5 mm standing up. Or if there is no correlation. But I have read discussions on a different forum where people talked about getting a standing MRI. 

I am on a Beta blocker now so I don't have as much tachycardia (though I still get the symptoms) but I am often very dizzy getting up after prolonged sitting or lying down. That is one of the many catch 22s we live with. Trying to balance chronic fatigue with enough movement to keep the blood flowing. I get up very slowly, sit on the side of the bed first to get my bearings and then slowly stand and begin to walk. It's a hassle but better than falling. I miss the days of shooting out of bed like a rocket :)

Not sure what meds you are on if any but one that has helped me some with the muscle weakness is Mestinon. An added benefit, well two actually: you can get a bit of a bump in BP and it also helps with bowel motility if that's a problem.

As far as head / neck movement causing dizziness. I agree it is a separate issue. I am beginning to be able to recognize the difference in symptoms finally. This dizzy / balance thing happens even when my vitals are fine.

No apologies necessary lol - great questions! I know I have had symptoms for many years but more severe progression over the last 5 years or so.

Hope you have a better night tonight!

kaitlyn

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Hi Kaitlyn. 

I have a similar story. Chiari, too small to really matter (I don't remember the size), flat MRI, POTS, Pure Autonomic Failure, pain in my neck at the base of my scull. It is bad enough this last week I can barely turn my head.

My dizziness happens if I turn my head too quickly, or go from upright to flat or the other way around. Bending over or leaning my head back to look up at the ceiling is also bad. There have been a few times that the dizziness has lasted a couple of days and it was miserable. It was like trying to walk drunk and when I finally made it to a stationary object to sit, it felt like I was on a carnival ride that I couldn't get off of. I was literally holding on to my bed so that I didn't fall off.

If I turn my head too far, I start to faint and as soon as I turn back even a little, I get instant relief.

Depending on which of my doctors you ask, I get a diagnosis from Minear's disease, some kind of middle ear stones, or that it is just part of PAF. Like you, I never understood how you could see if something was hanging down too much if you didn't look at it while it was hanging down, but I just let it go because I didn't really like the idea of even "minor" brain surgery. 

I am going back to the doctor in a few weeks and had already decided this is a topic that I want to bring up. The pain is just getting to be too much.

I'm very interested in what you learn from your doctor visit. I have a high school friend that had Chiari surgery and is doing great. Evidently, this is more common than I thought. 

If you don't mind, please keep us posted. I wish you luck in finding the answers.

Kim

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Thank you, Kaitlyn, for your corroboration.  I agree it is certainly a catch-22; you want to stay in bed due to feeling poorly, but then it causes more deconditioning, and then it is even harder to get up and start moving all over again.   I'm glad the beta-blocker is at least helping the tachycardia, and also that the Mestinon helps.  I totally agree that the dizziness can be separate from the dysautonomia; will be very interested in what, if anything, the collar does for you, and/or what your doctor says concerning the Chiari, if you don't mind updating us!    

Kim, so sorry you are experiencing this too to such a degree.   Your description, unfortunately, of dizziness on turning your head and in different positions, basically just upon movement trying to do any activity, sounds so familiar - I couldn't have explained it better.   That becomes the question, again, as to whether the dizziness and/or headache is part of the general dysautonomia/blood flow regulation problems or from Chiari, neck problems, or even inner ear problems.  Trying to decipher it all!  I would definitely mention it to your doctor, to maybe check if other problems are going on in addition to your PAF.   From what I have read, even vestibular problems are common with dysautonomia, so maybe they are just co-morbid conditions; thus, the reason for so much disabling dizziness even when we are not necessarily having dysautonomia symptoms.   I know myself, I can be not feeling POTsy, but then go into the bathroom to do something with my head tilted back, and will become extremely faint; in my case I have neck stenosis/arthritis, so believe that to be the primary problem.  Thus, since you are having similar trouble when turning your head, hope you can check this out further.   One has to wonder about general vestibular problems too when any movement seems to cause dizziness.   It seems so many here on the forum are dealing with multiple co-morbidities, which is why it is so hard to figure it all out.  Good luck in your search for further answers, and to all out there.

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Kim - what you describe is exactly what I woke up to in the middle of the night the day my illness progressed. I spent days and weeks in between rounds of Decadron feeling like I was in constant motion even when not moving. The slightest head movement was so awful. I am very sorry you experience these same symptoms. Mine are somewhat stabilized by low dose benzos which tell the brain it's not having the false sense of motion. What are you doing for treatment?

Kim & Amy - regarding above they were convinced it was an inner ear problem. I had every test possible which was so miserable because though every test was negative it made me feel even worse. My ears have been cleared by not only one but two of the top Drs in their field in my state. 

It is a full time job trying to figure all of this out. The dizziness you describe Kim and that I think we are all talking about is not the same as POTS dizziness. That much I know for myself for sure. Whether it ends up being my neck or the Chiari I don't know but so far I am 6 days in on the collar and I have not noticed any relief yet. Unfortunately. Just a lack of sleep as the Vista is not real conducive to sleep lol. 

I will definitely update you after I see the neurosurgeon again. I see him Friday. Appreciate the conversation and connection about this!

kaitlyn

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PS - Kim according to my neurosurgeon the normal clinical requirement of size for Chiari isn't necessarily relevant. There is information on his website for the wi Chiari center. If you're interested look particularly for the study / poster board presentation I mentioned above.

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Thanks, Kaitlin. I'm very interested in that info. I always go to my doctor with a list of questions and a pile of research. This is going on the top. I am back in his office on the 10th. 

I don't currently have a neurologist because I just haven't found one near me and wasn't really sure that I needed one since my cardiologist has been so good at handling this stuff, but I may need to be more proactive because my headaches are getting worse too. My parents only live 2 hours from Vandy, so I may try going back there again.

I'lI check back here to see what happened with you next weekend if I have time. I will be trying to clean my house for my parents to arrive. They go with me to Charleston, SC to my specialists. Cleaning at my pace will take 3 days - I'm sure you know how that goes.

I can't imagine keeping my neck immobilized as a solution. I hope there is a more comfortable plan going forward for you. I hope they find it.

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Sorry to hear, Kaitlyn, that the collar isn't helping, but good luck finding further insight at your appointment.  I admire you sticking with it - I didn't know you had to wear it while sleeping, wow!    I have found myself when trying to just wear a soft cervical collar for a short time, to see if it would help, it actually worsened things, so just don't know.   I think if one has to wear a collar after an injury, for example, where they don't want the neck to move, maybe it helps, but if you have chronic neck/headache problems, maybe not so much?    Also to you Kim in your travels and for finding some further answers.   I admire the determination both of you have in going the distance to seek help!   Take care.

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Kim - the pain in the back of the head sometimes into the the neck and pressure behind the eyes is one of the key Chiari symptoms confirmed by Dr Heffez again on Friday. I have heard of people coming to WI to see him from all over the country. 

Amy - yes it took a lot of determination to keep the collar on especially at night. It felt like I was being choked and made breathing difficult.

long story short: the collar didn't help so though there are some issues in my neck they are not the cause of my symptoms. The Chiari is extending into the foramen magnum and causing brain stem compression. My case is not as clear cut as some so he can't guarantee results. If we do decompression surgery we could get excellent results to no results and I would have to be prepared for that going in. Obviously he feels the potential benefits outweigh the risks or he wouldn't offer the surgery (or be able to get it by the insurance!). I have a big decision to make.

thanks for all your support! Hope you are both doing well!

kaitlyn

 

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i Kaitlyn - Thanks for updating us on your appointment.  I'm sure you were glad you had the opportunity to confer with Dr. Heffez..   Did he do an upright MRI then?   I'm sorry to hear that you do have confirmation on the Chiari, and now have the decision to make, especially since you would be taking the gamble as to whether it will help, as you said .   One would think that if you definitely have brainstem compression, it may be a big piece of the puzzle, and it certainly would make you lean toward the surgery if there was a good chance of feeling better.  That would be such a miracle for you.   Not an easy decision, though, I know.  I will certainly be thinking of you as you make a decision and, if you do, please do let us know.   Don't know if there is a Chiari and such forum, but maybe you could get some further info there as far as success stories!

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Thank you! No he didn't need an upright MRI to make the diagnosis. Yes there are Chiari support forums and I will start perusing there for more info now that I know all of this and am facing the decision of surgery. Thanks for the thoughts and kind words. Take care of your neck - wishing you the best as well!

5 hours ago, Amyschi said:

 

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Hi, Kaitlyn. Hope you are feeling better. Sorry the neck brace didn't work, but I can't say I am surprised. I was hoping for a different answer though.

So, I am back from my doctor in South Carolina and now have a referral to a neurologist for my next visit. They still don't think my problem is the Chiari but suspect a problem with my blood pressure in and around my brain. They are looking to run some tests with the new neuro. I could go back to the old one that ruled out the Chiari, but not sure I should. Speaking of him...

I forgot who at Mayo ruled out my Chiari back in 2008. It was Dr. William Cheshire. My doctor just got back from some autonomic conference a couple of weeks ago and ran in to him there. Evidently, he is also one that researches and publishes. I saw some recent articles since I last researched Chiari, so maybe they know more than they did when he saw me or maybe it really isn't the Chiari for me. I haven't had time to read them yet and probably won't until after the holidays. It looks like I was seen by a very good neuro back then. If I get a different opinion from the new guy because of new research, I may try to go back to Cheshire to see if he concurs. 

Keep me posted to as to what you decide for your treatment. I hope you find relief. I just wish it didn't have to be brain surgery.

Take care.

Kim

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Hi Kaitlyn.

I just saw the neurosurgeon's office today. I got a script for a standing MRI. Did you have one? If so, with POTS issues, how did you stand up long enough for the MRI? Don't MRIs take 30 - 45 minutes?

I'm now having problems with my DLCO on my pulmonary testing. I guess my lungs aren't doing the gas exchange effectively. Did this happen to you? My cardio has pulled all my records including outside records from the beginning and is starting over. I don't think he has an answer for the dizziness or this new lung development. 

I would love to know how you are doing. Hope you are doing better.

Kim

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This discussion couldn't have come at a more opportune time for my girls.  My oldest has gotten really bad within the last 8 months or so, and now we are trying to get her in to see Dr. Gerald Grant at Stanford.  Evidently he's the only one on the west coast that is capable of doing a Chiari/craniocervical instability surgery on someone with Ehler's Danlos.  She had an upright MRI a couple of months ago.  It wasn't standing, though, it was sitting.  Do you have to do an actual standing one?   My daughter would have passed out after the first couple of minutes.  You have to stand so still.  Even so with the seated one, they had to keep saying 'hold still'.  She wasn't moving, but her body has been spasming involuntarily for quite some time now.  The MRI is slightly blurry, but we are still going to get the CD to an expert even though the radiologist who read it said no Chiari.  They are often missed.  Have you looked into the videos and medical journal articles of Dr. Fraser Henderson?  He's like doctor royalty to me.  I think Dr. Grant was at one of the seminars that Dr. Henderson was at, so that gives me hope that, if we can get in to see him, he might be of the same mind set as Dr. Henderson.  There is a huge dysautonomia component in those with Chiari and/or craniocervical instability.  It certainly explains all of my daughter's symptoms.  There are just so few doctors who understand it.  The combination of Ehler's Danlos, dysautonomia, and Chiari/craniocervical instability is not something your general or specialized neurologist or neurosurgeon understands.  We've just exhausted our 13th neurologist.  Didn't spend a lot of time with him before he readily admitted that our local facility just didn't have enough specialized equipment to help her further, so instead of wasting our time with him, he would like to send us back to Stanford.  I appreciated his honesty.  You might do a little research on cervical medullary syndrome, also.  Explains a lot of dizziness and vertigo as well as a host of other symptoms.  Think you would be amazed, I was!  My daughter has an assortment of 5 different cervical collars that she has tried.  The one they are going to order for us next is the Miami J collar.  It's a bit lit the basic Aspen, but has broader thoracic support.  She tried the Minerva, but there was too much pressure put on the jaw.  With EDS, you can't put that much pressure on something that moves.  My daughter has a lot of trouble with TMJ and just subluxing of her jaw, so that wasn't a good fit for her.  Take care and let me know what you find out!!

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Quick update on my upright MRI and my Chiari. I saw the neurosurgeon a few weeks ago to get the results. I was stunned to find out that I don't have a Chiari like I was told. I have craniocervical instability. This didn't show on the flat MRI. It only shows on the upright MRI when they make you lean forward and then lean backward. Evidently, all of this dizziness is from my brain stem getting squished. He did say my brain stem drops a little which might be why the other guy called it a Chiari, but that is not the proper term for it. As I was warned, the neurologist said I do not need surgery (working from the flat MRI) and the neurosurgeon said I need surgery (upright MRI). To be honest, I expected to hear, yes, Chiari, no it isn't big, no problems here, next patient. So, when he said, "Oh, wow! You need surgery." I was completely flustered and don't remember details, just big picture stuff.

When I hesitated, he said we could try Physical Therapy and a neck brace first. He told me he didn't think it would work, but it was worth a shot if my insurance would pay for it. He said there was a better than even chance it would make things worse and to stop immediately if it did. He warned me not to wear the neck brace unless I had a headache because it would further weaken the muscles around my neck causing further instability. He spent a lot of time telling me how quickly his patients bounce back. When I asked their ages, he said they were 19. I pointed out that I am 47. I told him to gather data from his 47 year old patients with dysautonomia before we chat next time. All this, "she got back on her pointe shoes within 12 months" things just isn't going to apply to me. Fusing vertebrae and something about plates sounds like something that is a big deal for someone my age. I also have Rheumatoid Arthritis.

Has anyone gone through this at my age? If I do this it will be after Christmas. I talked to my cardio who knows both the neurologist and the neurosurgeon with divergent opinions and he said not to rush in to anything, but if I did need the surgery, the neurosurgeon he sent me to was the only one he would have do it. Of course, I live in Orlando, the neurosurgeon is in Charleston, South Carolina. I don't even know how the logistics of post op appointments are going to work, but I'm putting the cart before the horse.

Any stories or tips would be great, even horror stories. I want to make an informed decision.

Thanks.

Kim

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