photchkiss

My Heart Rate change is variable. Sometimes, the poor man's tilt table easily exceeds the 30 BPM threshold, but other times it does not. I believe my POTS is due to vessel dilation due to a connective tissue disorder. So, variables like temperature, time of day, hydration, etc. have a major impact on my POTS. Interestingly, I can have awful POTS symptoms even when my HR doesn't jump dramatically on standing. I am very skeptical about hard and fast clinical guidelines where at one point and time doctors rely so heavily on the TT test. My TTT showed an increase 30-35 beats per minute the entire time I was upright. However, if they would have done that test later in the day and in a warmer room, it could easily have been 40-50. I guess my point is that in my case, the HR reaction to being upright is dynamic and does not alwasy meet the >30BPM threshold. Joe

Thanks for the note. Marfan is looking unlikely in my case, I've been examined at a top clinic for this--most likely, its Classical EDS Type II (milder form) or some overlap disorder. You should have an echocardiagram if your mother has a dilated AR. Any doctor would quickly authorize this with a family history. EDS is more associated with vessel laxity (contributing to or causing orthostatic intolerance) than Marfan. In fact, in Marfan the aorta often becomes too stiff as it progressively dilates. There are many unknowns around these disorders, I will be participating in the NIH study in two weeks lead by Dr. Nazli McDonnell on heritable connective tissue disorders. Sometimes a mildly enlarged AR poses little risk of dissection, but other times the risk is high--they key is trying to figure out what the underlying cause is and this is no small task. I have been on a painful journey to try and figure this out. Since many of my symptoms are "borderline", it makes a concerete diagnosis more difficult. But I'm quite certain it's a mild variant of EDS. Hopefully, after an upcoming battery of genetic tests, we'll know more. Joe

Hi. My conundrum is that I have a dilated aortic root due to a yet to be diagnosed genetic connective tissue disorder (most likely EDS Type II), and the standard practice is to prescribe beta blockers to slow any progression and reduce risk of dissection, but I also have POTS which benefits from vasoconstriction, high salt intake, and other things that raise BP. I have also battled anxiety over the years and I've heard concerns linking beta blockers to anxiety. My cardiologist at a top clinic told me he has never seen this combination and he suggests low dose beta blockers to start. (20 mg daily) Has anyone else been in this situation--proverbial rock and a hard place? I have not started the medication yet, but the prescription has been filled. Your perspectives/experiences would really help. Thanks, Joe

Sorry you're feeling so poorly. Which type of EDS do you have? My experience with doctors regarding EDS related dysautonomia is that they are clueless. I've only met one physician who gets it--despite all the research that shows a meaningful link. Anyway, my autonomic tests showed the elevated heart rate on tilt (30-35 BPM increase the entire time I was upright) yet the neurologist wrote in his report--a remote possibility of POTS. It never ceases to amaze me that despite my description of dysautonomia, doctors are extremely reluctant to call it dysautonomia or POTS. Anything borderline seems to be left in no man's land. And of course whenever I'm in their nice air conditioned offices, sitting down for 30 minutes waiting for them to enter the room, my heart rate is less volatile and only jumps 10-20 beats when they do a casual exam. It's so frustrating. Yet I can leave that room, go outside where it's warm, my vessels dilate, and then my heart rate can jump 30+ beats just like that. All I've learned is to keep reading, researching and advocating for your health. See doctors as resources to help you, not as authority figures to paternally solve your ills. It's frustrating, but it's reality. We're all here for you, I hope you start feeling better, Joe

Hi. My cardiologist just prescribed low dose beta blockers, 20 mg per day, for me because I have a mildly enlarged aortic root. He seems very well versed in POTS--the first Dr. I've met that I can say this about, and he feels that this is the right first step for me. I am currently being examined for a hereditary connective tissue disorder which is the likely cause of my orthostatic intolerance. Before I take the first pill, I would appreciate any perspectives on beta blockers, particulary from those who have tried low dose and are dealing with a connective tissue disorder. Thanks, Joe

Hi. Could you please post the link to the site for the below quote. thanks!

Hi. I saw my internal medicine doctor last Friday. Here's what he dx'd after listening to my heart with a stethoscope: "pathologic heart murmurs with marfanoid features" I'm going in for an echocardiagram tomorrow. He wanted me to go Friday afternoon, but they were booked. Question: have any of you been told that you had a pathologic murmur? My doctor is clearly concerned about Marfan's Syndrome, which can be associated with POTS. I would appreciate any thoughts/experiences you've had. In particular, if there is anyone with Marfan's in this forum I would really appreciate your perspective. Thanks. Joe

Hi. Have any of you had your serum copper and/or ceruloplasmin levels checked? (mine are both low) If you have, and if you've had **low** copper or Cp levels, please respond to this post and describe your values and any medical advise/experience you've had with this rare finding? Thanks, Joe

Hello to all: Well, I saw a rheumatologist yesterday, and she said that my concerns regarding ehlers-danlos syndrome (EDS) are well founded. She said she has only seen 2 borderline cases in her career--and she used to be on staff at Mayo. But that my case was the clearest she has seen. She did not know much about the disorder and she had never heard of POTS. So, I'm pretty convinced now that I have EDS and that it's the cause of my POTS. Again, it's thanks to the posts on this forum that I explored the EDS world. Two questions: Can those of you with diagnosed EDS suggest the clinic and/or doctor that is the recognized national expert in EDS? Do you think I should see someone like Dr. Grubb for the POTS and another doctor for the EDS--or is there one doctor that can handle both? Thanks, Joe

Hello everyone: I joined DINET less than 2 weeks ago. Your support and feedback have been greatly appreciated. Looking at your signatures on posts, and seeing the clustering of commonly shared disorders may have gotten me closer to a diagnosis. Before DINET, I hadn't heard of EDS. Despite the fact that joint hypermobility, instability etc. have been plaguing me for years, I didn't know the cause. Like many of you, I've been on a multi-year journey from one doctor to the next. I've been tested for ALS, MS, YOPD, Wilson's Disease, etc. etc. Once I realized that I had POTS, it lead me to all of you. And once I began to listen to your stories, and read your signatures, it lead me to EDS. I'm a 37 year old guy. I'm an accomplished professional. But for the past several years, I've felt as though I was swimming up stream. And in the past year, my health began to rapidly deteriorate. Once I learned I had POTS--I went into life changing action mode. Treating the OI, has allowed me to go from functionality of a 3 on a scale of 1-10 to functionality of a 6 or 7. I have an appointment scheduled with a rheumatologist in 2 weeks to check me for EDS. In the past, I had many symptoms of Wilson's Disease, a very rare, genetic copper storage disease. I requested that my neurologist check for this, and when the lab tests came back for a protein called ceruloplasmin (Cp) and for copper serum levels both were low. My Cp level was 16 mg/dl--the normal range is 20-45 or so, with most people having Cp in the mid-thirities. My serum copper levels were also low. Through subsequent testing, Wilson's was ruled out--definitively. But now, ceruloplasmin and copper have come back on the radar in the world of EDS. As I dug into the various types of EDS, I noticed that some sites suggest that running Cp and copper serum tests are common in the test battery. Apparently type IX, otherwise known as Occiptal-Horn Syndrome, a milder variant of the child killing Menke's disease, is a type of EDS marked by low Cp and low serum copper levels. I checked my lab values (tested on 3 different occassions), and they are exactly within the range associated with EDS type IX, or OHS. My sense is I don't have this, since it is sometimes associated with mild cognitive deficits and even mild mental retardation, but the literature does say that intelligence can be normal. What scared me yesterday was a theme that emerged, that people with Occipital-Horn Syndrome usually live to mid-life. Do any of you have experience with EDS type IX or know of someone who learned that they had this at a similar stage in life to myself? --is it possible for a person with my background, at my age, to have something like this? And do you think that I'm doing the right thing going to a rhematologist (formerly she was with Mayo and now in private practice) as a next step? As I learn about EDS, I'm connecting the dots back to my childhood. I'm quite certain I have some type of EDS, but my intuition also tells me that something more is going on that has autonomic and neurologic dimensions. Your persepecitves, suggested links, or any other insights you can share are most appreciated. Thanks, Joe

I'm curious, have any of you every tried taking human growth hormone? It's very expensive, not covered by insurance, but I'm wondering if it could help strengthen our systems to deal with POTS. I have not tried this myself, but I plan on asking a doctor if it's worth a shot. For example, HGH allows you to put on more muscle--and perhaps this additional muscle would improve overall vascular resistance which, like the often cited "muscle pump", could help with venuous return and thereby diminsh the tachycardia. Joe

I'm an amateur classical pianist. So far, POTS has not gotten in the way of my piano playing in terms of dizziness, nausea, etc. However, singing is a different story. When I take my 3 year old son to music class, parents are expected to dance, stand up and down and sing with their children. I do my best to give my son the same experience as the other kids, but signing definately makes me feel sick. For me, having a very good artist bench for my piano helps--I can adjust the height just right, and the quality and firmness of the cushion helps. The artist bench I use is a Steinway bench, I think they are around $400. Not cheap, but it's a beautiful piece of furniture that may help. Joe

Dr. Grubb has written that POTS can be a stop on the way to MSA (multiple systems atrophy). He didn't cite the percentage likelihood of this happening, and I assume it's very low. But I'm curious, do any of you know of members that joined the forum diagnosed with POTS who later found that they had MSA? Despite lots of research to look for data on this, I can't find any studies that have tracked this type of progression. Thanks, Joe

Hi Amy: I don't want to worry you, but you may want to ask your doctor about MSA--multiple systems atrophy. Sometimes, people with MSA show brain atrophy in an MRI--only 20% or so of cases show this. I worried about this for a while, but my autonomic tests indicated against MSA since I don't have hypotentsion, urinary incontinence or retention, and other markers of MSA. I am not in anyway suggesting I think you have this, but it may be worth putting on the radar when you speak with your doctor. Warmest Regards, Joe

That is a beautiful and inspiring photo--we're behind you and know that you will proudly recieve your degree soon.