mkoven

anyone know how common the machines are or the technology is? I imagine my local mri facility can't do it, for example.

sounds like you're getting some good information! I'm sure you know to get copies of everything to bring with you. If you'd like, we can then swap notes about good docs in central Illinois and chicago. I',m going back in ten days. Michele

Two questions- -where does one get cine mri's done? --to Maxine, and anyone else with cranial instability, I've noticed I have trouble lying on my side--it seems to push my head over, make me dizzy and give me a headache. How do you sleep? (Back? side? what type of pillow(s)?)

florinef has helped me tons in conjunction with midodrine. I take 3/4 of a pill a day. I was afraid of gaining weight and if anything have lost, because it lets me be more active. I hesitated until I was beyond desperate. I recommend doing a search on it. I'm also very med sensitive-- but as they say, you gotta play to win. You can start a quarter pill per day. At first it made me a little jumpy, but not anymore.

Interesting. Where did you have the cine mri done? Maxine, I'll empty my mailbox! Thanks!!

thanks for your replies. curious, maxine about your wanting to avoid tci. you can pm me if you'd prefer not to get into it on the forum. this guy does have experience with eds and chiari, but sometimes i wonder if in talking to a surgeon, you get to hear about surgery. I'd like to hear more about the "natural history" of these issues without surgery, and examples of good and bad experiences with surgery. i haven't healed well during prior surgeries. and i;m also concerned that a fusion would mess up the rest of my neck, where most of the discs are already bulging or herniated. wouldn't that put more stress on them?

I just had an appointment with the neurosurgeon recommended by the eds geneticist. I'm frankly overwhelmed and not sure how to sort through everything he told me. He says I do have a 5mm chiari malformation, an abnormally low clivo-axial angle of 135 (normal is 165), and Harris and Grabb-okes angles showing cranial-cervical instability. With these together, he thinks my brainstem is getting pinched and this is causing the dysautonomia. He also told me I have ossification of the stylar hyoid ligaments bilaterally that could be putting pressure on the carotids.He did an mra with my head turned both ways to look at that. He also said the spinal cord shows some change in signal, showing stress on it. I think these were the highlights. It came at lightening speed without a lot of elaboration about the significance of each of these findings. I explained that we were leaving town on 7/6 for Illinois (I've been on sabbatical in DC). For now he has me in a hard cervical brace till I return on Monday. He then said, depending on my response, he would consider a halo vest , which as I understand involves screws to the skull. He then talked about thepossibility of a suboccipital decompression-fusion. He is of the opinion that the dysautonomia is a result of some combination of all this. I don't have a sense of how emergent/urgent this is. If it is not emergent, I don't think I'm ready for anything invasive, to include the halo vest, till I understand better, gather more information, and get back to the midwest. (He seemed to think I could continue to travel to DC to see him from Champaign, which frankly is not realistic). I really don't want surgery, or even screws placed into my skull unless it's absolutely necessary. But I don't want to do myself any harm either. Feels like a whole can of worms just got opened. What do you guys think? ? He came highly recommended

Once I finally got on the right med combo, did some cardiac rehab, and learned how much to compress and drink, I am able to exercise and find it helpful. I walk on a treadmill on an incline. Oddly, I get lightheaded on a bike, but not really while walking anymore. I think I must compress vessels in my abdomen when I sit, and when I walk, I get better blood return. I also have a strengthening regimen for ans and bad joints. There are days around my period when things are harder, but I generally feel better when I work out. I'm very careful not to push or increase intensity or duration too fast. In general, I feel best when I keep moving. My joints hurt more and my ans is wackier when I don't exercise. But spring and summer 08, I couldn't exercise without bottoming out my bp and scaring the $%^# of myself and anyone looking at my vitals. A bp that drops from 110/50 to 70/30 WHILE exercising is not tenable! I no longer check my bp, but I wear a hr monitor, and drink loads afterwards. I wait till my heart rate is under 100 before I leave the gym. I also make sure not to overheat. I sweat buckets on the treadmill, and need to wear a shirt that lets the sweat evaporate and cool me down.

An experienced geneticist is probably the best to diagnose eds. I believe the top people are saying that "benign hypermobility syndrome" and eds are often the same thing, and there is nothing "benign" about it. Perhaps the word benign is used to distinguish it from vascular eds, which does have much greater risk of mortality? I recently saw Clair Francomano in Baltimore who was very thorough and has seen a lot of connective tissue disorders. She was able to tell me that there is something rather atypical in my tissue, as I tear before I stretch. She also noted all sorts of things that noone else had noticed earlier. I actually have a "marfanoid" set of body proportions-- (proportionally long arms and torso) which fits with a connective tissue disorder, which no one else had noticed, as I'm overall short and stout--but my wing span is four inches longer than my height and ribs sit almost right on top of my hips, because they are essentially "too big" for my lower body. All this just helps fit the pieces together. There aren't a lot of docs with enough experience to diagnose eds, esp the non-textbook presentations.

I wear open toe 30-40 most of the time, and 40-50 on bad days. They are hot, but worth it. I really don't like anything confining my toes, for some reason. At this point I no longer find them hard to put on, since I've been doing it for almost three years. I don't think the compression in the closed toe is meant to include the toes, so I don't think I'm losing anything. I can tell by their color, though, how I'm doing! If I've been on my feet they are often red; if I want to pass out they turn purple!

I've had allergic type reactions to excessive sun exposure going way back. It's usually only with intense expsoure, though. I went to the bahamas as a teenager and it was pretty bad. But I've had it happen on several occasions.

florinef actually improved my headaches. but that may just be me.

I felt a little better immediately, but really started feeling better when I went from .05 to .075--the full effect took a couple weeks. this drug really stabilized me and got me somewhat functional. I wish I didn't need it, but I'm so grateful I tolerate and benefit from it.

I"m sorry I don't know what those terms mean. Let me second the idea that you get fitted/titrated for cpap. I"ve been on it since 4/08 and it really helps so many things--most obviously fatigue, pain, and mood. And untreated apnea will slowly wreck anyone's cardiovascular system and brain tissue--without pots or eds. Every episode of apnea is like a suffocation, sending your ans into a tailspin, even if you don't remember those events when you wake up. I know you know this, so I'll get off my soapbox. It took a couple tries to get the right mask, and to get used to it, but now I wouldn't dream of going to bed without it. When I unintentionally fall asleep in front of the tv or in the car, I can really tell the difference. Your body needs good-quality sleep to heal the best it can, and apnea can impede that. I believe a number of hormonal processes involved int issue repair occur most in the deeper stages of sleep --the stages that tend to get disrupted and shortened if you have apnea. I've found that www.apneasupport.org is a very helpful forum for info, support, etc. Cpap didn't cure my ans or eds issues, but as the doc who got me on cpap said, sleep apnea will make every health problem worse and harder to deal with.

I think I was given daily blood tests and somewhat elevated glucose came up-- nothing scary-- I think the highest was 145 fasting, but not normal. Even though I have a family history of diabetes, I'm usually normal (75-95 fasting) and my tolerance test was fine. But on steroids, I"ms tarving, and feel like the only thing that will hold me is straight protein. blurry vision can be a sign of high blood sugar--but not necessarily. My fasting blood sugar remained a little elevated for several days. the steroids also temporarily did something to my white blood cell count. everything returned to normal, but steroids are really drugs I try to avoid. Fortunately for me, I don't react badly to florinef.

I was given 3 80mg doses of solumedrol in a 24 hour period before my cath. It makes me less nutty than prednisone, but it still feels like a whole lotta adrenaline coursing through your veins. I remember when the nurse was pushing it in the iv, I felt like I was literally having panic injected into me. Pretty awful stuff. I crashed the next day, and took another couple days till I was back to normal. It played with my blood sugar and gave me blurry vision for a couple days and even made some old scars start to open up--with its tissue weakening properties. Muscle aches and tachycardic too. But it did pass. Sorry for the ignorant doctors. It's infuriating.

I think it is worth getting a workup and treatment plan from an ans specialist. I didn't really improve until I went to Cleveland, even though my meds are pretty unremarkable--midodrine and florinef. Because my local docs had minimal experience treating this and rxing these drugs, and it seems SO WRONG to them to increase anyone's bp (esp in a middle-aged, over weight woman), I was stuck, and getting worse and worse. CC wasn't perfect, and there were areas of ans they weren't interested in helping address, without their diagnostics and suggestions, I can't say where I'd be.

I've heard that the test isn't standardized--it's done and interpreted differently in different centers. I'm glad that my one and only ttt was at cleveland. I also don't faint/haven't fainted. My ttt showed "late tachycardia"--meaning that my increase was over 40bpm but didn't kick in immediately, and hypotension. I also was still on meds, so probably would have been more pronounced if they'd had me discontinue those. I felt bad, but didn't faint. I think if I'd done it in my home town, they would have called it negative, as they're fixated on the faint. In my daily life, my response to standing is variable. Sometimes I am more potsy, sometimes I actually drop my hr and bp. My current pcp, who does know about dysautonomia, prefers to just call it orthostatic intolerance. In other words, regardless of whether it's hr or bp, I don't stand still well without my vitals doing odd things. I also have a hard time standing because of my joints. I don't want to repeat it, as it really takes a toll on my hips and pelvis to stand for so long without moving.

Interesting to hear how common this is. csf is cerebral spinal fluid. The brain floats in it. most docs think of orthostatic headaches as caused by a leak, even small. But I guess fluctuating bp would also affect csf pressures.

So frustrating. Sometimes I can turn things around with lots of salt/fluids, extra compression, and a dose of midodrine.

anyone have headaches brought on just by being upright? My docs say this is usually a sign of a csf leak. could it just be from dysautonomia?

I've been wearing 30-40 waist high for nearly three years. They no longer feel tight. I have to say though that on bad days (during my period), I wear 40-50.

I've got it. DEfinitely get a sleep study. CPAP take some getting used to, but it is very effective. I also recommend searching under apnea. There have been many posts about it, and a number of us have it. It's underdiagnosed in the general population and in women. If you have eds, it's quite common, because of lax tissues, and the high arched palate a lot of edsers have. Untreated apnea is damaging for the healthiest body.

As someone who's had chest pain, I'd argue for a stress test-- that way they can see if there is at least a rhythm disturbance that could be dangerous. You could also ask for a holter monitor , wear it to work out, and press the button if you have symptoms. I've heard that the holter isn't as good as a regular ekg, as it only has 2-3 leads, but would show something flagrant. You could also bring your bp cuff to the gym. In other words, you want to capture the rhythm and your vitals when you're having an epsiode. I have at least three different types of chest pain-- one from my bp not rising adequately during exercise; one triggered by autonomic swings that might be causing vasospasm; one from my ribs/pecs. I can't always distinguish them. The first one improved a lot with a combo of midodrine, florinef , and compression. Florinef really helped me to turn the corner. But generally to get the docs to respond, they want empirical evidence of the problem, so the more you can document, the better.

I should have mentioned this in my other post. The first day, they will have you do a tilt table test. Other testing will be scheduled, based on that. If it's other ans testing, since it's in the same department, they're pretty good about squeezing you in fast. I had to be seen in another department, and that was somewhat more complicated. It's a fairly tightly run ship, and she's very good at diagnostic testing. I had the ttt one day, and then came back soon after for hemodynamic testing and another ans tests, because of the results of my ttt.