mkoven

Since I react so strongly to everything, and couldn't go off antihistamines without a major flare, they just did rast allergy testing-- it's actually a blood test. It was a little helpful for helping me know what to avoid. But the upshot is I just take antihistamines. I did shots as a kid and they never helped. I'm allergic to pollens, mold, dust, and any creature with fur.

In the US, the best bet is a geneticist. They (should) have the most thorough understanding of all the different systems a genetic defect in collagen can impact. But not all geneticists are up on the latest research. You might want to go to www.ednf.org for a list of doctors knowledgeable. If you have a really good pcp, and express your concern, he could probably send you for an echocardiogram to rule out any dilation of your aorta. But geneticists are the specialists here. Some have seen rheumatologists, but I haven't had good experience.

They are watching me closely because I keep track of what my aorta measures year to year and was struck by the change. The cardiologist herself did not say anything until I said, that's 8mm bigger than last year. So if you have a suspected connective tissue disorder, you should be getting echocardiograms. You should also know your aortic root measurement and be ready to ask for followup if there is any change. "Normal" is under 3.7, but there are adjustments for body size, age, and gender. In someone without connective tissue problems, surgery is usually warranted when you get to 5.5. In someone with EDS or Marfan's, the question of surgery is often raised at 4.5. My aortic root went from 2.8 to 3.6 in a year. I got it remeasured at 3.3. That doc is now comparing all three films to see how much of the difference is measurement error, so we can really know where I stand and how much it's grown, if at all. 3.3 isn't a bad a number in itself, though a little big for a short 42 year old woman. 3.6 is at the upper limit. But the big issue is the rate of change. I"ve heard that as many as a third of classical and hypermobile edsers have somewhat enlarged aortic roots, but that typically don't tend to progress. But tracking them over time is important, to be sure. If you do go to the ER with chest pain and an eds diagnosis, aortic dissection is usually the first thing they want to rule out. this is a reason to get a diagnosis in itself. just telling the doc you're flexible or hypermobile will probably not set the right studies in motion. so i'm being closely followed because I reacted to the change in number. because it was still technically "normal," I don't think anything would have happened without my asking more questions and pushing. In other words, we do have to be proactive and informed.

What terrible news! I'll echo Maxine's urging that those with any type of eds get regular echocardiograms. I"ve heard once a year is standard, unless it seems to be growing, and then it may be every 3-6 months. I've just had a bit of a scare, as mine was measured as having grown quite a bit in one year. I'm now having one of the top cardiologists who specializes in connective tissue disorders remeasure to see if it has, or was mismeasured. The tricky thing will be, if it has grown, as it will impact my ans treatments. BP and pulse have to be kept quite low and stable (ha!) if you have aortic enlargement. Apparently many edsers have mild enlargment, but that is stable. this should also be followed.

I have a hard time with benzos as they make me so tired and foggy. I can only take them when I don't need to be alert.

I have bad discs and can trace much of the nerve pain to specific discs. Big toe is L4-L5, top of foot, outside of shin-- I get that too. Other toes, sole, and ball of foot are L5-S1. Mine is usually triggered by too much sitting, bending, and other spinally risky activities.

I'm not a pharmacist, but I think beta blockers are contra-indicated with prinzmetal's as they inhibit dilation, and you need to dilate to counteract a spasm/constriction. I"ve been told no bb's.

The doctor had me take it before bed, so that the peak efffect would be when I need it most and its bp-lowering effect would be when I was lying down. I did feel woozy at first. but the cardio encouraged me to see if I could get used to it, and I did.

I wish I had more specifics. Knock on wood, things have been relatively quiet recently on the chest pain front. For the me the first thing is treating the dysautonomia aggressively. the swings in hr and bp are a big trigger for me. Getting up in the middle of the night is a trigger for me, so I wear compression to bed. and have to transition in and out of bed very gradually. The norvasc has been okay, but I'm on a TINY dose. I couldn't tolerate 5mgs, only 2.5. It doesn't aggravate my heart rate at all. At 2.5, all I notice is a little swelling in my hands and feet, but it did take me about a week to get used to it. I didn't feel so good initially. I believe it has one of the better side effect profiles of the calcium channel blockers. You do need to find a cardiologist who will listen to you and work with you-- who gets the dysautonomia and the possibility of spasm. Once I found her, she told me to call if I ever wondered whether to go to the ER. She also wrote a letter to take with me to the ER, advising them what to do, and giving legitimacy to my being there. We more or less decided since I haven't sustained damage from previous episodes, I don't need to go in if a give episode is no worse than previous ones. If it goes on and on or is much worse, I do need to go in. Nitro does not help me-- does it help you?

Hi Daisy, I second what others have said. Anxiety might cause an elevated heart rate, but I don't think anxiety alone causes an orthostatic increase in heart rate. A tilt table would tease that out.

I think swallowing is a brainstem-controlled thing. If you have compression from cci, chiari, or something else, it could be affected. Mine is worse if my neck is not perfectly positioned and when I'm tired. I also have a worse time with liquids, like the back of my throat doesn't know to close. Just had a swallow study, but it was earlier in the day when I'm better. They did see a little liquid penetrate, but not enough to be a danger. I was told to mix this product called "thick-it" to turn liquids to a more pudding like consistency to facilitate swallowing. It does help--just a little gross. It's something we're following.

I know how frustrating this all is. It does sound like a partial victory. Definitely follow up with the other guy. Of course the problem concretely becomes what to do about the instability, if he is indeed right that the risks are too great. And maybe you will need to be seen by one of the half dozen people in the country who are experienced with this type of problem in eds. Interesting about the gag reflex. Do you have trouble swallowing? That's been one of my recent issues.

I find a doctor's threat of dropping you if you don't do what he says disturbing. It is one thing if he says that he can't treat you anymore as he has run out of ideas, and wants you to be seen by someone who can help you better. But as an ultimatum?

count me in. some docs think i'm nuts to have so many bad reactions and allergies. i really want to avoid painkillers and antibiotics in case there ever is an emergency when i have to have them. i'm allergic to so many of each. it would be great to have a good explanation. but I do worry if I ever needed surgery or something else lifesaving.

Good luck! As you know, not many docs recognize or treat this. It seems to be a small handful of neurosurgeons, who, trained as surgeons, are less able to talk about conservative management. I don't know what to make of my own cci and what to do about it. I would be very reluctant to undergo a surgery that not that many are versed in and is considered controversial. They do this routinely at TCI, but as you know, there is a lot of controversy swirling around them. For now, I'm doing what I can to avoid getting worse. I have to keep reminding myself about proper head position, and using my mid-back muscles to keep my head erect and back, while keeping my shoulders down and neck back. I've been various types of scapular stabilization and pectoral release exercises for this. I've also learned how often I let my thoracic spine flex, rather than work on mild extension, with chest lifted and shoulders back. It does help. Besides that, there is only the brace. and then surgery. and the surgery does seem pretty extreme. And I really wonder what fusion will do to the rest of an eds spine. I was told that at this point my situation is not life-threatening/emergent, by Francomano and the neurosurgeon I saw in DC who does treat this. Right now it does seem to me that this is something one has to "live with" as long as possible--like lots of other eds unpleasantness. So I guess I would probably make sure I knew what my expectations were going in, what you will do if they are not met, and whether there will be a better place. But just like I don't like taking drugs that have just been released, I'm not enthused about trying out new surgical procedures.

I find his recommendations to be problematic--esp the bit about trauma???? I have a presumptive diagnosis of prinzmetal triggered by dysautonomia. I take 2.5 or norvasc, along with midodrine and florinef, but I don't have hypertension. I'd also heard betas were contraindicated-- and I can't take them as they they trigger some latent asthma. I think you need a different ans doc. It's not one size fits all.

I did pilates for three years with an instructor. I would recommend having at least one or two sessions with someone who can watch you--it's all about form and control, and most of it is not intuitive. I stopped going when my joint issues got too weird, but the basic principles of which muscles to engage have stayed with me. It can be expensive, but if you can afford one lesson, maybe someone could wathc your breathing. If you don't breathe properly, you could end up in a valsalva situation which I imagine could trigger a pots attack. and it's hard to know when to breathe when you're tensing your stomach.

I'd wait several days, and then try again with only two or three repetitions! When I took classes, we actually usually only did three reps of each exercise-- just very slowly with close attention to form.

just to follow up. I"ve since bought a mackenzie cervical roll that I put at the bottom of my pillow to go between shoulders and head, and I think it helps. (I can't get over how much my neck plays a role in so many of my symptoms. Not sure if it's THE smoking gun, but definitely one of them. Since my neck doesn't really hurt, I didn't make the connection. Butpoor neck/head posture brings on so many ans symptoms for me.)

apnea is usually worse in rem, as your muscles are immobilized so that you don't act out your dreams. My apnea is mild overall, but severe in rem.

Thanks, you guys, for the support! This forum is wonderful. I know that not many people can appreciate what a big deal this is and what a victory it will feel like. I have no illusions of speed--there will be no jogging/running. Hopefully just slow, steady, one-foot-in-front-of-the-other. And if I start to feel bad or injured (during my prep or during the event), I'll reconsider.

Hi, As I've posted elsewhere, in the past year I've been lucky enough to find the right meds and rehab to be able to exercise with my ans issues and bad joints. I'm now able to walk two miles, or about forty minutes. Overall, I feel better jointwise and autonomically when I exercise than when I don't. I think I'd like to register for my first 5k-- the Chicago conquer chiari walk on September 26. My goal is not speed, but injury-free completion. If it takes me 90 minutes, that's fine with me. It will mean slowly adding another mile to my walks over the next 6-7 weeks, keeping on top of cranky joints with braces/pt exercises/self myofascial release. If I can do this, I'll be thrilled. It would be nice to have a "normal" goal like this to look forward to. I figure it will also let me meet other people affected by chiari in person. Even if I get hurt, I'd still like to go, and bring the wheelchair. I've been thinking of this for a little bit. Then, this past weekend my husband and I went to cheer my sister-in-law on in the Chicago half-marathon. That was pretty cool, but looking through the crown, most participants were healthy looking people in their 20s and 30s. For me, a slow 5k walk may be just as much of an accomplishment!

I now have an aspen vista, which is padded, adjustable, and pretty comfortable. I had a Thomson collar before, which was quite uncomfortable, but also effective. The nsg said no traction, as he feared it would stretch out my ligaments further. The first nsg was also opposed. So I'm just trying to be meticulous about posture (head over shoulders, chest out, shoulders down, move arms from shoulder blades), and if that's not enough, on goes the brace. It does help. I now have another pt rx for my neck to work more on posture and stabilization. Just have to find a pt who won't stretch me.

I'm so sorry to hear so much is going on. I can totally relate to the frustration and well as many of the actual health issues. I haven't given up, but know that the majority of doctors aren't going to "get" it, let alone know how to treat it. When I saw Heffez, he was very cautious about attributing causality-- I have multiple abnormalities--some chiari, some stenosis, a tethered cord. he didn't see the upper cranial instability, but another nsg saw it. anyway, any of these issues alone can cause problems, and it's hard to determine which ones (alone or together) are the culprit. so if i had surgery, what should be addressed, and in what order, to have the best chance of making me better? I don't think I'd want to have someone just cut anything cuttable. I know I'm in no hurry to get cut, as I could end up worse off. But I would like someone knowledgeable following me. And sadly, with our combo of issues, there aren't many out there. I was also given the name of Arnold Menezes in Iowa City and Oro in Colorado. I don't know whether to try to see them, if it will only create more chaos. Plus, it seems like surgeons don't necessarily want to manage or "watch" people who are trying to avoid surgery. I will be putting in a call to my eds geneticist to help sort things out myself. In the meantime, I'm bracing when I feel most symptomatic, trying to maintain perfect posture while asleep and awake, and trying to keep my muscles doing their job to help compensate for the weak ligaments. I'm thinking of trying alexander technique which i hear is all about learning to move without compressing the spine. it does seem like a lot of the people describing their experience with cranial fusion, tethered cord surgery, chiari surgery end up having a lot of other troubles after surgery, and needing a lot of repeat procedures. in terms of the cranial-cervical instability, Dr. Heffez said that he thought that the fusion that some are proposing is far too extreme except in the worst cases. I don't know what to think.

you would first need to have the cause of the nausea determined. after umpteen years of intermittent nausea, my geneticist who knows I have a connective tissue disorder (ehlers-danlos), referred me to a neurosurgeon who sees a lot of folks with tissue weakness. It was discovered that I have a somewaht unstable head, neck, and that my cerebellar tonsils are sagging a little bit into the base of my skull (small chiari malformation). He tried me out in a hard neck brace to see if it relieves my symptoms, and it has really helped. my nausea has been almost gone since bracing. the thought is that my instability is causing me to compress nerves coming off my brainstem. a neck brace would only help nausea if instability is a factor for you. my guess is that most doctors are not well versed in these matters. I am lucky that my geneticist could see how different pieces of the puzzle fit together. I'd been to the gastroenterologist several times, had an endoscopy, and this really didn't show much. My stomach itself is not really the problem. with bracing, I've only had to take anti-nausea meds about once a week, as opposed to multiple times a day.