E Soskis

Well...once neuropathy starts, there is not much we can do to stop it - worrying about it might actually make it worse as chronic stress, anxiety, and worry cause the release of substances that increase inflammation throughout the body thereby increasing symptoms. I think that the more information we are able to obtain about our disease, the better we understand where our symptoms are coming from - this reduces anxiety and helps us to keep on keeping on!

I'm not sure why your physician does not want to have IV Benadryl ordered as a "prn" drug. It works in a totally different way than Solumedrol. Benadryl is an antihistamine and blocks the histamine release from allergic responses - it is a front-line drug for allergic responses. Solumedrol is a steroid that suppresses the immune response. They both work together in totally different ways to help block any untoward reaction you might have. I'm just glad you were able to obtain the IVIG - it is a difficult drug to get due to the shortages. I wish you all the best and sincerely hope this will help you.

Local - I won't go anywhere else anymore unless absolutely forced to....I just can't tolerate travel- flying is a nightmare and driving is too hard on me. I am quite fortunate to have local MD's who go out of their way to assist me so, I'm definitely staying local.

Oh yeah - down I go - fully awake and aware that my blood pressure is quite low. During my tilt table test, my BP dropped to 40 systolic and my heart rate was 20 and I was still conscious - barely but, I remember everything! I know I can still walk around with a systolic BP of 60 - I think we develop a tolerance to the low BP more so than people who never have low BP then drop with it suddenly: they wilt and we just get fuzzy-brained!

I am told that it is the neuropathy associated with dysautonomia. My very first symptoms some 25 years ago began with a tingling feeling in the left side of my face. I have strange sensations of tingling arms, legs, and feeling like water is dripping down my leg or arm. Lately, my feet feel like "ants" are crawling all over them. I frequently attempt to "brush off" a bug that I think is on my arm only, there's nothing there!

I was on IVIG for 2 years. I also started with 3 days of infusions followed by weekly infusions that went on for 2 years - I could not tolerate the "normal" infusion rate. The infusions had to go in very slowly consequently, what should have taken less than two days for the loading dose took about 3 full days. The weekly infusions took 8 hours a week. Since IVIG is a protein, your body eventually makes antibodies against it. The IV solumedrol should help suppress this response. I would also ask your MD about IV Benadryl as that also assists with allergic response to the foreign proteins. I stopped the infusions when I could no longer tolerate the adverse symptoms - I had developed a full-blown allergy to it. Once this happens, you can never go back to IVIG. I have to say, however, that during the 2 years it worked, it worked quite well. It was the first time I ever had any relief from the dysautonomia symptoms and allowed me to go back to work full-time - even if it was a desk job. I truly think without the IVIG, I would have died. I had reached my end-point: extremely low BP, 70mg of midodrine a day, flourinef, mestinon, beta-blockers, and others no longer were very effective - I was actually planning my funeral when everything turned around with IVIG. I hope it works great for you and lasts a long, long time. Remember, everyone is different - you may not have the same adverse reactions I had - I know people that have tolerated it quite well with minimal symptoms. One more thing, be sure your MD has ordered you something for pain just in case you have any type of headache with the loading dose.

Ice cold legs, ice cold arms - peripheral neuropathy and Raynaud's - especially with a flare, they get even colder. I can relate to the hot flash - for some reason, my whole body will get very hot then very cold.....don't you just love the ever-changing nature of dysautonomia? - never a dull moment.......

Could it be because you sit for a prolonged time with your feet hanging down and your posterior compressed by the shape of the seat? - that cuts off circulation and nerve supply and of course, dangling your legs down is problematic for autonomic dysfunction....

Yes - autoimmune diseases can be quite difficult to diagnose. My personal feeling is that there are a whole bunch of autoimmune diseases out there that we know nothing at all about. When I began my quest to find out what was causing my symptoms, it was about 20 years + before I was diagnosed with AAG. My primary physician at the beginning told me that he knew in his heart something was wrong and it was probably autoimmune but, he had no way of testing for it. "We'll just wait and see" - something would turn up positive eventually. It is always far better to treat the patient and symptoms than to treat (or not treat) because of a lab value printed on a piece of paper.

When nerve pathways are altered, so are pain pathways - we all interpret pain differently - it depends upon which set of nerves are messing up at the time. My pain pathways are so screwed-up that I often don't recognize pain. I may be restless, agitated, and grumpy only later to find out I was in pain. Any type of narcotic has an adverse effect on patients with autonomic failure - particularly the GI system. I loathe taking narcotic pain medication and usually only do so when the nurses at hospital insist (because I don't recognize pain well). I have found that the autonomic failure, particularly from autoimmune origins, causes a generalized inflammation throughout the body - I have found for me that ibuprofen is a real help - it is an anti-inflammatory and almost always improves my disposition greatly -

I agree with all of the above - also sounds like you could have Raynaud's from autonomic neuropathy - dangling legs down is a definite "no-no" - I keep a little stool under my desk at work - I sit at a computer all day and I can still "elevate" my legs a little bit - my legs also turn splotchy, purplish, and feel like a gazillion needles are being stuck in my feet - always worse when standing or hanging legs down

Good grief! This explains why my head feels like it is going to explode at times.....who knew?

So sorry you are having all this progression of symptoms - I have not progressed as far as you have yet - have your MD's thought about IVIG or Plasma Exchanges? - I would be dead today without having had these treatments. I still take plasma exchanges about once every 2-3 weeks and have done so for 3 years. I know things are still progressing but, it has slowed down the progression dramatically.

I am functional but, with many limitations. I have had dysautonomia many years but, in 2006 things began to worsen. So, it is a decline but, seems to be a rather slow decline in the scheme of things. I think we probably have periods of rapid decline, then stabilization, then more decline. I still work full-time but, at a desk job. I don't do much housework - have a housekeeper for most. No heavy lifting, no exercise, occasional walk around block leaves me exhausted, not digesting food very well anymore - I expect to continue to decline over the next few years but, I have come to terms with my disease and try to accept whatever is thrown my way. It would be very easy to become depressed but, that is a dangerous door to open - try to stay positive - enjoy your kids - find the fun in life you can participate in and do it! Don't dwell on the "what if's" and "what might be" - just deal with everything on a day-to-day basis.

Yes - my blood count dropped while in the hospital but, it is on the way back up. I've had 5 surgeries on my arm to try and create a fistula but, unsuccessful due to my chronically low blood pressure. I'm very wary of this "necklace" procedure - it connects blood vessels from subclavian right to left and tunnels under the chest - I just don't know much about it and there is very little out there on the internet except for the procedure itself. I really would like to know the long-term implications and what type of life style changes I will have to make.

I'm just home after 5 days in the hospital with a blood stream infection from my permcath and infusaport - unfortunately, they had to remove all of my hardware and I don't have anymore vascular access. The vascular surgeon is talking about performing a "necklace" procedure on me to give me vascular access for plasma exchanges. I've read about this and it sounds rather barbaric. Does anyone have any experience with this procedure - advice - tips - hints, etc...???

With any medication, you have to weigh the risks vs the benefits - the good vs the bad - if the negative side effects outweigh the positive benefits then, it may not be the medication for you. I do agree you should talk to your MD about it. I have been on midodrine for many years and have become used to the side effects. I also get chill bumps and a cold feeling but, if you think about it, midodrine causes all your peripheral circulation to clamp down and shunt blood toward vital internal organs and blood vessels to help increase your BP and central circulation. This would be why your arms and legs feel "cold". I also have the tingling scalp - but, it really is of no consequence. I just couldn't make it without midodrine - I can tell exactly when my blood pressure begins to improve because I feel better overall and begin to think more clearly. I'm sure I can tolerate a higher dose than most since I have been on the medication for over 7 years - I can take 20 mg at a time and not become too hypertensive.

I think you have to discuss with your MD about the rate of infusions. I used to take saline very frequently - several times a week. Since I am a nurse, I was able to administer them to myself and I judged the rate by how bad I felt. If I needed volume in a hurry, I ran it in quickly (1 hour or less) - if I just needed a boost, I would take 1-2 hours to receive the liter. My physician left it up to me because he knew I would do what was right. I have not been on infusions regularly for a couple of years but, am about to start again. Now, I have a central port and a perm cath with double lumen access. If I use my port, it only takes infusions at 250cc/hr max. If I use the perm cath with the large double lumen access, I could dump in a liter in less than 15 minutes. My experience has been that if you take it too rapidly, it simply runs through your system via the kidneys without much benefit. If the infusion is long and drawn out, it does not give the volume necessary to "plump up" the blood vessels. Once you start, you will quickly learn what works best for you.

I have AAG and IVIG was the first treatment I received after my diagnosis. I received the IVIG every week for over a year and when it helped, it worked quite well. After about a year or so, it quit helping - my symptoms gradually increased again. I have been on plasma exchanges now for going into 3 years and it too is beginning to not be as effective as it was at first. My physician said that when I was ready, he would try the IVIG again - I'm not at that point yet. Although the IVIG worked well for a time, I had a lot of side effects that were bothersome. I developed an allergy to it that required me to be pre-medicated with Benadryl, solumedrol, and other compounds that left me feeling loopy and disconnected. I was so sensitive to the IVIG that it could only be infused extremely slowly - it took over 8 hours to get in a very small dose. This is why I am reluctant to go back on it. I know people though that have had it and had no reactions - you will just have to see how it does for you. If you find that it is helping and you plan to be on it long-term, I recommend you get a port placed. I still have mine in my chest and it is accessed quite frequently for IV fluids and other treatments. Your peripheral veins will not be able to handle long-term IVIG as it is hard on them and causes them to be stiff and difficult to access. I wish you all the best and hope this is an effective treatment for you. IVIG is very difficult to obtain - it may take some time to get started but, once you do, the pharmacy will see that you have the regular infusions you need.

Unfortunately, I am quite familiar with burning hands and feet - the first symptoms of Raynaud's DZ began with my feet and lower legs turning a lovely shade of purple at odd and random times - then my fingers began to blanche white with a dead feeling which was/is exacerbated by cold weather - now, I have "bugs" crawling on my lower legs, feet, and hands pretty much all the time - if I get too tired, too hot, or do too much physical activity, my hands and feet feel like I am being stabbed with needles over and over - it is a hot, prickly feeling - sometimes, feels like they are on fire. I am told by my neurologist it is just the progression of the Raynaud's and not much can be done - he shrugs it off so, I try to also....pretty annoying......and painful......

I had mine done a few years ago and at that time, there were only 2 labs in the US that performed this specialized test plus a few others related. Mayo is one of the labs and if you ever think you may need to go to Mayo for treatment, you best have the test performed through them. They do not "recognize" the other lab that performs the test - and will make you repeat it through their lab. Vanderbilt also uses the lab through Mayo. I don't know the name of the other lab - it's been too long ago and my memory is ka-putz.

I've been advised by my cardiologist and internist to take in at least 6 grams of salt every day - when I slack off, I notice a difference. When I consume too much, I get puffy all over - I don't measure or count grams, I just use my body to tell me if I am on target or not.....

Actually, it was when my heart rate began to fail that my cardiologist recognized I had some type of autonomic failure.(in 2006) The blood test performed was after my neurologist remembered reading about AAG and he ordered the antibody test to see if I had it (in 2010) - the test was so positive, it was off the charts. So, yes, there is a blood test HOWEVER...a negative blood test does not rule out AAG - I have read that there are patients who test negative for the antibodies but still have the disease. I have been battling symptoms of dysautonomia for at least 14 years.

I have to agree with you - how can we be OK when we feel so yucky? -It doesn't make any sense! I'm sorry you feel so bad - it is definitely not fun to be sick all the time - for me, I had to quit dwelling on the "why's" and move on to the "how", as in, how do I make this better? Maybe it is a little easier for me because I have worked in medicine for so many years. I have seen the very real limitations on true medical knowledge and treatment of uncommon illnesses - in some ways, medicine is quite advanced in knowledge and understanding but, there is still a long, long way to go. The more common a disease is, the more is known about it since that is what gets studied and researched the most. I mean, when you think about it, how many illnesses and medical conditions are truly "cured"? We treat the symptoms of heart disease but, it is not cured. Some cancers are cured - until they recur somewhere else in the body. We can't cure a common cold. So...we're pretty good at managing symptoms but, that's about all!

no - different type of dysautonomia than POTS - autoimmune autonomic ganglionopathy - still a type of autonomic failure but, is autoimmune in origin