dawson20

Like POTSgirl I was very lucky - from the get go my symptoms were so severe as to be unignorable - no amount of 'anxiety' could make a persons blood pressure drop to undetectable levels within minutes of a TTT. I lucked out and within a few weeks of my symptoms starting I saw a neuro in the hospital who knew his stuff and had me on a tilt table in a matter of weeks. From there out treatment was underway. POTS was actually my 'misdiagnosis'... but it was all that fit at first. I have an awesome cardiologist who has been willing to turn over every stone - Most of the doctors I have seen over the years have either been open and honest about not having an answer, or have been willing to try treatments without solid answers. I am reminded from threads like this to be thankful for my team of docs.

Very interesting! My symptoms started immidiately after delivery of my first son - my second pregnancy was awesome - my symptoms diminished, I was able to go down on my florinef dose, and I felt fantastic through most of it. The only 'adverse' effect was fetal growth issues and a very small placenta due likely to the beta blocker (metoprolol) I was on. Neither had a major negative impact on my son though and he was born healthy (if somewhat small) at 42 weeks. Unfortunately my symptoms came back with a vengance afterwards - but my underlying condition is not POTS, but an autoimmune condition - and this pattern is common with autoimmune disease (improvement with pregnancy, worsening afterwards). But the 'lull' in symptoms sure was nice!

Interesting - I'd never heard the term 'pandysautonomia' but when i googled it the symptoms are identical to AAG (as is the treatment)! Clear like mud right? my story is not posted - It's long and convoluted, and confused by several different diagnosis' along the way before everything lined up and I was dx'd with AAG. Basically it started after I had my first child with severe OH and tachycardia issues. Dx'd initially with POTS, the NCS. Mayo Clinic in '03 - major workup - dx'd with 'autoimmune endochrinopathy' though they couldnt define any specific antibodies, or endochrine abnormalities that weren't attributed to the meds I was on. I pretty much 'gave up' on an answer/treatment for several years. In '05 my son was diagnosed with a Mitochondrial Disorder (Complex 1 Deficiency) which threw everything off and for a few years I was believed to have Mito (what are the odds it was something else right?). Things gradually worsened... pupil issues, dysmotility, UTI's constantly, Orthostatic Intolerance just got worse and worse in spite of increasingly high doses of meds. This past winter my 'sitting down' blood pressure was 70/40 on a good day.. standing up it was undetectable. Anyways last summer I saw a mito specialist after having had a muscle biopsy to test for mito come back negative. The specialist confirmed I do NOT have mito, but instead he said I seemed to fit the clinical profile for AAG perfectly - his colleague (a neuro at the autonomic clinic in the same hospital) consulted and agreed I fit the profile well and my pupils apparently impressed them. They ordered the antibody titres at that point and suggested IVIG if the antibodies were positive. They were negative. that was earlier this year. My cardio still felt IVIG was worth a try as it has been reported to help in many seronegative cases of AAG. While waiting to get into see the hemotologist that was going to consider trialling the IVIG, my motility hit an all time low and I wound up inpatient with severe malnutrition/hypoglycemia. They were discussing TPN and/or tube feeding at that point and everyone agreed it made sense to try the IVIG first in case it would work. basically we had nothing to lose. I had my first round (a little over 2g/kg spread over 5 days) in April/May, then followed up with 450mg/kg every 3 weeks. 7 days after hte start of the first round my blood pressure and heart rate completely stabalized. My motility picked up and my pupils started doing what they were supposed to. It was phenomenal. By my 2nd follow up dose (last week) it was clearly not working any longer - my heart rates and blood pressure readings were worsening fast, and my appetite was gone. So they have just finished up another 3 day cylce of high dose IVIG (was supposed to be 4 but i got really sick (fever, headache, aches, etc) yesterday so they called off dose 4) and will resume at a more frequent and higher dose now. The hope is that things will continue to improve over the next 6 months with the IVIG. If things do not continue to improve at this point the next step is Plasma Exchange. We will know in the next week or two which route we will be going... I will be getting a port put in likely towards the end of the month once we know whether I'll be doing IVIG or Plasma Exchange long term (they use different types of ports depending on which treatment I'll be doing). That is the short version... *sigh* what is your story? Is it posted anywhere? I'm very curious to hear more from someone in the same boat!!

My tilt table did not involve any meds at all - it was just continuous monitoring while upright - that said it may be because my reaction was so violent just to being upright (within 4 minutes my BP was undetectable and my heartrated was 170+), that there was no need to use any meds to 'trigger' a reaction of any sort? I also had the same issue with being able to keep moving, but as soon as I stood still my BP tanked and my heart rate shot through the roof.... My cardio also explained it was like a blood pooling issue - your veins in your legs operate on a pressure system, the smooth muscles around them are stimulated by movement, and its that movement that contracts the veins and sends the blood to the top half of your body - when there is no movement, the blood can end up pooling (my feet would actually turn purple if I stood still). At least this was the cliffs notes version the cardio that did my tilt table gave me. It sounds like you really need a tilt table... it will be hard solid 'proof' that your doctors cannot chalk up to anxiety (in the eraly days I had a few docs play that card - It's so frustrating to know something is wrong and at the same time wonder if their right and youre really 'fine' and just have to get over it). As far as tests go it is not that invasive - there is an IV involved, but at this point, if you really want to make some headway on determining what is going on and how to treat it, you may need to psych yourself up and go through with it. Once I had my tilt table EVERYTHING changed - suddenly there was a cardiologist saying "something is very very wrong" and everyone else just had to agree... no more 'your stressed' speculation. it was a major turning point for me. It didnt diagnose anything but it got the attention of the right doctors and got me on a path where I got treatment and the right meds to get my life back until the rest of the cards fell into place for an actual diagnosis.

Off meds my heart rate would go from 70's to 170's within 30 seconds of standing (from any position). On meds it wasn't as shocking (the beta blockers made a big difference) and it would go from 50's and 60's to 90's and 100's - again within 30 seconds. laying down my rhythm would return to baseline within the minute as well.

It was the Anticholinergic Autoantibodies - part of the Mayo clinics paraneoplastic profile - there was another name for it but i cant think of it offhand... It is a specific antibody that is tested when AAG is suspected.

Just throwing my experience out there... I was diagnosed with POTS initially in '03, then NCS, then 'progressive dysautonomia' and later 'progressive severe autonomic failure'.... all basically a description and not a true dx... all they could do was say I was broken, and getting worse. Last summer I was dx'd in Hamilton ON with Autoimmune Autonomic Ganglionopathy (clincally - by last summer I had all the hallmarks - severe orthostatic intolerace in spite of massive doses of Florinef, midodrine, NaCL, and beta blockers, severe global gut dysmotility (very nearly wound up on TPN), hypohydrosis (didnt sweat), and pupil/optic nerve dysfunction) - when my antibody titres came back negative (which they do 50% of the time I'm told) I was dx'd with 'seronegative AAG'. That was March of this year. At that point I was waiting to see the Hemo department for assesment as far as a trial of IVIG therapy as the handful of case studies out there suggest that seronegative AAG patients who fit the clinical profile well (dysmotility, OH, pupillary dysfunction, hypohydrosis) do indeed respond to IVIG and immunotherapy. Come April the motility stuff hit a crisis point and I was admitted for 3 weeks for malnutrition/hypoglycemia and they started talking about TPN and small bowel feeding tubes etc. Everyone agreed we should trial the high dose IVIG first on the off chance it did actually work. It worked. within 10 days my blood pressure did not drop AT ALL when standing (this was mind blowing - prior to this, for the past 7 years, I couldnt stand without it dropping significantly, and for the past year, it has consistently always dropped to 60's and 70's systolic... by 10 days post IVIG my systolic BP was RISING when I stood....) My motility picked up, my pupils started responding appropriately, and my heart rate stopped spiking when I stood. I even ended up dropping the dosage on my florinef and beta blocker as my BP was getting a bit too HIGH... It was phenomenal. The improvement continued over the following month. every week I was a bit stronger, my numbers were a bit better, my medication doses were a bit lower. Once I finished the first round of high dose daily therapy for 5 days, they dropped me to the 'maitenance' dose every 3 weeks and unfortunately as of this week I officially am 'relapsing' as my pressures are not as good, heart rate is higher, and other symptoms are returning - they have started me on a high dose daily regimen again this week, and I will be put on a higher/more frequent maitenance dose from here out. The plan for now is to continue this maitenance until October and reasses then. IVIG is not necessarily approved for but is used frequently off-lable for many autoimmune neuropathies - some very obscure and unheard of (like AAG)... Immune therapies of this nature (immunosupressants, plasmaphoresis, IVIG) have been reccomended for me various times by various doctors over the past decade - this is the first time we have actually taken that step and trialled one though. I am in Canada however so I know the process of getting approval/coverage is different and easier. Just my experience...

Anyone else out there? Just looking for experience!

It's less embarrasing than passing out in a public place believe me... You owe nobody an explanation. it's a hard place to get, but you will get there. in the end none of those people are going to remember you or hte fact that you took that seat up 2 days from now, so why should you remember?

While this isnt 'common' - its not 'uncommon' either, even in healthy people. I get it all the time - i can count my pulse without ever 'feeling' for it, I can just 'hear' it and feel it, my Sister in law talks about, and my nefew (who is totally healthy) gets it. It can be related to low blood pressure, but it can also just be a variant of normal.

Its been a good 7 months since Ive been here... Not sure if anyone remembers me but hey Ill update you anyways... Last fall I had my PFO repair (heart defect that for whatever reason was shunting blood and causing my O2 to drop - I was O2 dependant most of last year because of it) and it was a roaring success - at least as far as the O2 stuff goes - Ive been off O2 since I rolled out of the OR! Very happy with not lugging oxygen around!! Everythign else has kind of fallen apart though. After the heart repair my cardio and I decided to attempt a verrry slow wean off the florinef - just to see if I could get off it. 2 weeks ago my blood pressure in clinic was 80/50 standing. (thats a drop from 115/70 sitting) last week it was still 80/50 standing and we bumped my florinef dose up again. today it was 77/50. yea not going well. I feel like crap, obviously. ive started on salt tabs (6000mg a day) and midodrine as well as pulled out the compression stockings for the time being. What has us particularily concerned is that in the past my drops were always related to a crazy high heart rate (why i was classified POTS). Lately my heart rate isnt shooting up, which takes it out of the POTS category and puts it squarely in the 'autonomic neuropathy' category... and its officially progressing. So not terribly happy with that. Im off to see the mito specialist for a repeat biopsy and a consult - were all pretty much certain he will confirm I have mito. my son has confirmed mito, and it appears they have isolated the gene responsible for it in him and I (maternally inherited MtDNA defect), but my understanding is more testing will have to be done before they can make that call. Its really weird trying to switch from 'mom mode' to 'me mode' as far as doctors go. the past several years I have really let my own stuff fall to the wayside as we dealt with our sons issues (which are unfortunately progressing too fast for our liking). Its hard to step back and actually deal with my own stuff. anyways - you will likely see more of me here over the next while... im sure im in for a rough year as they want to figure out where things are nerve wise - its been years since ive had any neuro testing done, and things have clearly progressed in that department.

It always takes about 2 weeks for me to adjust to a new dose and totally see how it will affect me... Ive been on Florinef (currently at 0.1mg, but ive been as low as 0.05 and as high as 0.5mg a day over the years) and the dose is changed often depending on what is going on with my potassium levels, BP, etc... even when im brutally sick it can take up to 2 weeks to start feeling benifits (and when i first started it, it took closer to 6 weeks to realise the full benefit). hormones always affect my BP's and how well the meds control them - my symptoms fluctuate with my cycle but Ive gotten good and predicting which days will be 'slow days' so to speak. Give it some time... depending on your metabolism, and your body, it could take a while.

http://www.netwellness.org/question.cfm/58443.htm Definately worth followup - sounds like if it was there in the past xrays and hasnt changed over time it could just be scarring form old infections or some such, but if its new it could be pretty serious... Squeaky wheel - hound them till the tell you whats up!

I am currently being investigated for this - will be seeing the Canadian specialist next month for a repeat muscle biopsy (had an inconclusive biopsy in '05 - pathology was abnormal but 'resp chain' testing was normal). My 6 year old son has confirmed mitochondrial encephalomyopathy - complex 1 deficiency (diagnosed through a muscle biopsy)- so we have been immersed in the 'mitoworld' for some time now. I actually have had issues my whole life but it wasnt until brandons diagnosis that we finally got direction to what might be the source of my issues. I stepped back from finding answers to my own health stuff for quite a while and just 'maintained' as best I could while we were dealing with my sons stuff but things have progressed (motility, orthostatic symptoms, further neuropathy signs etc) and its time to buckle down and get some solid answers for me. I have heard of (and actually met briefly at a mito conference) Dr. Cohen but i hadnt heard the comment about muscle biopsies likely being negative in dysautonomia cases - that is interesting as my biopsy was negative but it looks like we may (emphasis on the 'may' - the significance is yet to be determined) have found the MtDNA gene defect responsible for my sons (and theoretically my) mito. As far as dysautonomia/mito and diagnosis - my son has autonomic 'issues' (heat intolerance, temp regulation stuff, sweating stuff, motility stuff) but I wouldtn classify him as 'dysautonomic' at this point - his issues are hugely neurological and movement related (he essentially has the brain of a parkinsons patient). his muscle biopsy was crystal clear positive. I on the other hand have a negative (for mito) muscle biopsy and progressive neuropathy with multisystem issues (heart rate stuff, kidney stuff, gut stuff, etc etc) and honestly the odds of me having some other exceedingly rare condition (while still sharing the gene defect my son has) are astronomical.

Ive had atrial tachycardia for as long as I can remember... it took a long time to figure out what it was, but Ive always had 'flutters' and skipped beats and just odd sensations as far as my heart goes. the atrial tachycardia has mostly gone away with metoprolol but i still get runs of it when my potassium drops.

Before meds my baseline was usually 70/30 now (with meds) my baseline is usually 110/50 - Ive only been off meds a handful of times since starting and every time my baseline is actually lower (60/palp or even unreadable) though, so my unmedicated baseline still stinks.

Have you had a urinalysis done lately? foaming urine can be cause by excess protein in the urine... along with the other stuff (edema etc) this can all be signs of kidney damage... if you havent had a urinalysis done lately I would suggest that you request one... Ive never heard of foaming uring being related to high salt intake... hope you figure it out soon!

We keep protien bars in our backpack (power bar etc) all the time... their a great quick source of protein that keeps well in my bag... same with any kind of nut bar, packs of peanuts, beef jerky and even peanut butter packs (I have been known to give Tyler strait peanut butter off a spoon in the past!) Your right though - no easy solution, just lots of precautions...

have you seen an allergist/immunologist? This would be your first step - ask for a referral to one at a major centre, they may be able to figure out why your body is on such high alert. Certain autoimmune diseases can cause this type of scenario (antibodys to everything), and an allergist/immunologits would be the one to weed that out. Im sorry to hear about it, I know how frustrating allergies are and Im not nearly as sensitive as you to stuff - My allergies seem to be 'everday' things more than meds - I cant even take a shower because the water splashing on my face causes hives... sunscreen, makeup, cleanser, lotion, it all leaves welts on my skin. EKG leads and medical tape actually leave what I can only describe as burns on my skin. I do alright with most meds though. I hope an immunologist is able to shed some light on it all.

Im one of those people who responded beautifully to beta blockers as well... I take metoprolol (hefty doses, 50-100mg twice a day depending on where things are at) and it has given me my life back. We werent really sure what was working (I was on florinef and metoprolol) and honestly it seems to be a combination of the two because any attempt to mess with either usually results in disaster. BUT the florinef is less drastic... if I lower my florinef dose I feel rotten but my numbers are still reasonable, BP is a little lower and HR is a bit up but not crazy... if I reduce my metoprolol dose even a fraction (which we have done in the past) its a nightmare - my blood pressure is unreadable when im sitting (seirously - they record it as 0) and if Im lucky it will register around 60/0 when Im lying, not to mention my heart rate is through the roof - we dont know for sure if the blood pressure is falling because the heart rate is so high, or if the heart rate is so high because the blood pressure is so low... but we do know Im a complete train wreck. A pharmacist friend of mine had some thoughts - she said metoprolol is typically use to lower blood pressure, and lower heart rate (as most beta blockers are), it also strengthens the contraction of the heart (which is why its used in heart failure?). So she was thinking my improvement must be related to that action of the drug - strengthened contraction would = better BP readings... That said we have no clue at this point... the only way to find out for sure is to actually take me off it compeltely and see what were left with... We plan on doing this in the fall - their admitting me and im going cold turkey off everything to see if we cant iron some of this stuff out.. right now we dont know whats working and what is just 'extra' meds. I am curious too though - several docs Ive talked to have been baffled by the fact that beta blockers INCREASE my blood pressure.

www.silvereagleoutfitters.com is where we got our cooling stuff - my son has severe heat intolerance from his mito and the vests (50$ for childrens 65$ for adults) seriously changed his life. They have hats, bandanas, neck wraps, even hard hat liners... they act by evaporation - you soak it for 10 minutes, then ring it out (its dry to the touch and does not make your clothes wet when you ring it out properly) and put it on and they can last 4-6 hours in 90 degree heat (longer in cooler temps).... We love them!

I have taken midodrine off and on during bad spells but really I find the side effects outweigh the benefit for me... my numbers are better, BP is higher, the orthostatic drop (while still there) is less shocking, and I dont feel *quite* as rotten as usual, but the side effects for me are unpleasant (the bugs on teh skin sensation, crawling scalp, pounding heart). Ive got POTS and NCS (for lack of a better description... when I stand up my HR soars, and BP tanks) and for me the biggest benefit was the betablockers. Sorry teh side effects are problematic... its hard to find something that works so well, and doesnt work so well at the same time.

Wierd as this sounds I find the best solution for me is Sopporo (ichiban or ramen) noodles... the dehydrated kind with the flavor pack... TOTALLY unhealthy but packs one heck of a salt punch when you add a bit more salt to it... I find it loads me up on salt, and makes me thirsty, so it works well for rehydrated in a hurry... If I miss a florinef dose the same thing happens to me, I lose all my fluids very fast... If I follow up with my noodle snack quickly Im usually able to curb the fluid loss... I guess its really just salt loading... I just make a meal out of it... Hope your on the mend soon!!

I have occasional lows but for the most part I handle fasting alright (my lows are usually reactive to overly sugary foods with little or no protein). My youngest son however has severe ketotic hypoglycemia - he cannot fast at all for more than 3-4 hours during the day and requires a massive dose of corn starch at bedtime to stabalize his blood sugars overnight. Any medical procedure requiring fasting requires that he be admitted ahead of time for IV D10 (saline solution with 10% Dextrose). IV D10 is usually more than enough to stabalize a persons blood sugar while fasting when their prone to hypoglycemia, provided it is run continuously... as soon as its stopped the blood sugar will tank as it is akin to drinking nothing but sugar water steady... theres no protien to stabalize it at all. as long as its running though, blood sugar will stay steady. IV D5 (5% dextrose) is used sometimes for my son, when he is not sick, but more often than not they opt for the D10. We find with Tyler that sugary liquids (apple juice for example) are good for bringing his sugar up in a hurry if hes low, but they stink for keeping it there - if we dont follow up with some protein fast his sugar will tank even lower than when we started. we only ever give him sugary stuff when his sugar is low and we need to get it up fast, otherwise sugars are just bad news for him. Fruit will do this too - he needs to balance fruit with protiens or we end up in teh same place. Corn starch (raw, mixed with milk) will keep his sugar up for longer periods of time (Its a very complex carbohydrate and takes alot longer to break down than other stuff, so it acts as a 'slow release' carb to keep the sugar up longer) which is why we give it to him at bedtime and occasionally during the day if he is sick and his intake is down. This is generally only reccomended on the advice of a doc though because the amount of raw cornstarch needed to keep sugars up is alot and that can cause problems of its own (we deal with absorption issues, diarrhea and high sugars with Tyler because of this... fair trade given that without it he wakes up with a blood sugar around 40 (2.4) most mornings. Anyways, weve been immersed in hypoglycemia control over here... sounds like you have already figured out hte biggest triggers (too much sugar, not enough protein)... but yes the IV solution that they run to deal with hypoglycemia is essentially just sugar... its the constant flow that keeps it up.

A 3 month wait for semi-urgent - are you in Canada? (I am and that sort of 'hurry up and wait' scenario seems all too familiar). Im so sorry to hear about your daugthers struggles - I went through similar challenges as a teenager and I know first hand how hard it is to try and achieve 'normal' in the middle of all that junk. Sounds like you are doing the right things... I would suggest talking to her doctor about hte fact that the receptionist isnt taking you seriously when you call (completely none of her business to make judgements like that) - let her know how you feel... if they arent willing to be more receptive you may want to consider a new doc... our relationship with our primary care doc is paramount to ongoing care. Does she have a pediatrician? You may want to consider this - often pediatricians are able to get kids into clinic and specialists alot faster than regular family docs... especially if you find one affiliated with a major childrens hospital (if your lucky enough to live near one). We tried to stay with our family doc for my son when he first got sick mainly because I didnt want to start over with a new doc, but once things got complicated we needed a pediatrician... the waits were simply too long through our family doc.