arizona girl

J, what meds are you currently on for this. I'm right in assuming your bp/hr are normal whilst supine. Interesting that the cleveland doc wants you off the meds when you already had a positive tilt on them. Usually the concern with being on meds is that you will get a normal tilt and miss the problem. Dr. Grubb is lovely, you could try to get in with his partner Bev earlier. Also, since you are so close I would call them on a regular basis for a cancellation. Mine appointment got moved up 6 months, by doing this. There is one really nice receptionist, that did this and another one who acts like fort knox to get in. So depends on who you get. Glad they were able to rule out pheo, these hyper symptoms do mirror them, and I also have an adrenal nodule, and was told it wasn't pheo. I do know this when I was on clonidine, I developed an addictive response to it requiring more and more of it to keep me low. I had to get off of it and that was awful. It needs to been done slowly and another bp med needs to be added in. I'm lucky I got through it, without a serious problem. Dr. grubb prescribed me labetalol, he also did not require I go through another ttt.

Hi, Jen I saw Grubb too. I liked him, just to far away to keep going. Just curious what did he diagnosis and treat you with so far? When I arrived on his door step, we already knew my bp went up not just my heart rate and I also had just come back with positive skin biopsy for small fiber neuropathy. My neuro wanted to go ahead and treat me with treatments targeting autoimmunity. I had also had a history of elevated ana and suspected autoimmune issues. He did agree with those findings and said that they may not be able to find the antibody cause yet as Mayo was continuing to find new ones. He ordered the supine/standing catecholamines which confirmed his diagnosis of hyperpots. He put me on labetalol as I also got hypo/brady when supine. It does help, but my other treatments for underlyers has also improved things as well

Oh Rich, I sooo know how you feel. I too knew my biopsy would be abnormal, it so crazy that we would be happy about that, but of course we are happy because now we can prove that it isn't crazy in the head. So the magic doors now open to further diagnostics, a new beginning to looking once again, I hope they find your underlyer so you can get it treated. So alli lu ya!

Hyperpots, I'm on ivig too, in my case for the cvid. What brand are you on and how are you handling your infusion. I'm getting 70 grms once a month. I'm so happy they found your cause. Wow that was some pretty high ne, mine went from 400 to 1000ish. I'm still swinging but not as wide. I also had an mthfr gene defect that we just started treating with methylfolate multi support. I had hashimoto too and have had improvements with that after adding in T3 to synthroid. I'm so glad for you.

Hi, Before I give you my opinion on this. Would you mind sharing how you were found to be hyper pots? I went through some pretty specific testing and have some pretty specific symptoms that lead to my diagnosis. Having said that the answer to the question is why or what may be causing those symptoms and labs that define hype pots. In my case, mine is most likely caused by autoimmune small fiber autonomic neuropathy, my neuropathy is most likely a result of a combination of an immune deficiency/cvid and autoimmune disease. In my case at this time there probably is no cure yet, however there are treatments to manage my underlying causes and with that there have been improvements in my symptoms and labs, but it will require ongoing managment. I am not able yet to feel functional enough to go back to paid job, but I am able to contribute to the world in other ways. Thank goodness for the interenet and dinet, so that there is a way to purpose. I don't know who told you such a bleak prognosis, it is far to soon in the research and understanding of this subset of pots to come to that conclusion. I also believe that this subset is much more common then currently documented, as my neuro suggested "it's not rare, just rarely diagnosed". It is important however to rule out potential underlying causes as improvements can happen once those are treated properly. It will take work, but I believe there is hope to have improvement in symptoms with the right treatment plan for you.

Actually, now that you mention that, when I saw Dr. Grubb, that is exactly what he said. That mayo already new about 5 and that they would continue to find more.

There is a big difference between muscle spasm and muscle weakness. EDS to the hypermobility as you know does cause muscle spasms and pain. Muscle weakness doesn't always cause pain but it would make you to weak to walk or raise your arms above your head. Basically those are your motor nerves and involve the large fiber nerves. The small fiber nerves are associated with the autonomic issues. It requires a skin biopsy and/or a qsart test, a ttt will also uncover it. So you already know that there is autonomic issues, then you have the skin issues and now if you do fit the description of muscle weakness that takes you into a different directions when large and small fiber nerves are found together, that looks more like an autoimmune neuromuscular disease. Which does require more indepth diagnostics, including the antibodies you mentioned. Perhaps you could do a post asking for neuromuscular neurologists in the uk. There are also some dysautonmia websites in the uk you might look at. I understand your insurance procedures are different in the uk, but there has got to be a way to get around it. Perhaps if you had the name of a doctor that can do the type of diagnostics, you'd have more luck, maybe even be able to ring them directly. So, sorry you've had to wait so long. Not that it will make you feel any better but it took me decades to find the right doctors.

A rheumatologist would not most likely test for these conditions. Sometimes a dermatologist will catch it, but most doctors that treat and test for them are neurologist who specialize in autoimmune neuromuscular disease. You may actually need a skin or muscle biopsy to prove or disprove it. With muscle weakness that is neuro not eds, then you add the skin issues, If I were you I'd look further and see if you can find a neuro that specializes in those conditions I mentioned. Also if you google them you can read about them yourself and see if they fit you. Do you know what kind of eds you have? Also just because you have one diagnosis doesn't mean you can't also have another.

I can't advise you on meds, but I was wondering have the doctors ruled out Dermatomyositis or polymystosis. There are also other autoimmune neruomuscular disorders like polyneuropathies. There are treatment plans for these that are different then the way you would treat your standard dysautonomias.

Most pheresis is done in a hospital setting I'm in the phoenix area. It's a big city and it still was done in the hospital. The machines are rather large and require skilled staff to run them. A catheter not a port was required for me I was on it for 3 months prior to the switch to ivig. There were several other patients who had a port put into their underside fore arm, that had been getting it for years. Ivig had not worked for them. My understanding is that the antibodies build back up in your blood so you will repeatedly need to strain them out of the blood, which is basically what pheresis does. If you have those antibodies they will always come back. Many are able to switch to ivig, which goes after the antibodies in a different way. I don't think the ivig worked as fast as pheresis, but it is a treatment that is much easier to stay on long term. Pheresis because of the ports/catheters will always put you at higher risk for infection. As I said before this was my experience, perhaps they do it different else where.

I can only speak from my experience. I was lucky enough to land in the hands of a neurologist who specializes in "autoimmune neuromuscular disease". After proof of a failed TTT along with a positive skin biopsy for small fiber autonomic neuropathy. He ordered pheresis for me. Just so you know they have to put a catherter in your chest area and you have tubes hanging out your chest. Even though it was a treatment, the pheresis was also part test to see if I would improve. I actually did improve and rather quickly, however I got anemia along with an infection, which is a risk with pheresis. Though they can do things to prevent that. At that point is when we found I had an immune deficieny and I was switched to ivig. I would suggest you try to find a neuro in your area who treats neuromuscular disease and who utilizes pheresis and ivig. They will be in a better position to evaluate and treat you for pandy. I believe I have a form of it as well and I also was negative for the antibodies.

Hi Angela, I see his partner Levine, who I was referred to after a failed TTT. That referral wouldn't have happened had I not asked the magic words after a normal emg, "Well, what about SFN". Levine at the first visit orderd sfn, sleep study and other labs to rule out carcinoid and such. My skin biopsy and sleep study were abnormal the rest thank god were normal. He started me on pheresis, on which I improved until I got an infection and anemia. At that point he ran a Total Quantitative immunoglobulin" and we found out I had hypogamaglobulinia/cvid. I then qualified for ivig. The first ivig product was awful for me carimune. I'm on privigen now and the chronic low grade fevers are gone and infection rate has improved. I do get rocephine shots when I get one now. I still have autonomic issues, but they are better. So how do you think this plays in with what your finding with the new protazoa doc? Did saperstein offer you any treatment plan? They do such a good job with skin biopsies there, it be a shame not to have them do one. It was a relief for me to know that I had diagnostic proof of the autonomic dysfunction. How'd your move go. I'm going up to scottsdale this week to f/up with a natropath that works with my rheumy.

I found the post by just putting arizona girl in the search box above. It was interesting rereading where I was back then compared to where I am now. The tests are in bold/italics below. Eventually when I had another infection on board we found my total igg and igm were very low and that is why I get monthly ivig infusions. Also one of the things I did when I first joined dinet, was to go to dinet's home page and read everything on it, especially all the links in mechanism and causes. I was able to rule out many potential causes by doing that. The info on small fiber neuropathy fit me and were the magic words that sent me to my current neuro. Primary immune disease however was still not on my radar, it was however on my neuro's. Hope this helps. "Posted 17 August 2009 - 04:34 PM I got really lucky on the testing. I knew all these tests needed to be done after reading up on this website, but not where to go to get them. I questioned my 1st neuro about the small fiber testing when the large fiber emg was normal. If I hadn't been so well informed by this web site, he would have sent me on my way. So the request for the small fiber testing, not the dysautonomia was the only reason I got referred to phoenix neuro. How refreshing to me that Dr. Levine knew about dysautonomia and new that we had to test for a bunch of stuff to figure it out. My cardio vascular workup really could only look at this from their end. Cardio testing included the positive TTT, which really changed everything for me in getting heard anywhere else. This has been the most struggle free quickly executed doctor's encounter I have ever had. The tests the neuro ordered where: Neuronal Nuclear w/reflex, Angiotensin converting enzyme, immunofixation, immunoglobulins quantitative, sed rate, sjogren's antibodies, amphiphysin antibody test, ganglionic AChR Antibody test, VGKC. The skin biopsy and sleep study. I don't have a copy of any of the labs yet, waiting on them. He only addressed the biopsy and sleep study findings and said you have neuropathy, but we don't know why. Not knowing why didn't seem to matter to him, the positive biopsy, was all he seemed to need to know, to decide treatment. If you google these different tests you can see what they are for. Also if you go to Phoenix Neurological Associates web site, it talks about the many different disorders that IVIG can be used to treat. He also ordered new testing for methylmalonic acid and homosysteine, to see if I have an underlying B-12 problem. My sister has this. So, I think the two immuno tests above did check my IG's, I just don't know what they are yet. The only time I get red, flushing is in association with heat or pressure, like crossing your legs, red marks left. I have been trying for 20 years to get someone to figure out what's wrong. It almost feels unnatural that since april, all of this has wrapped, so quickly. I'm like no really, really we finally know whats wrong. Not sure all the why's have been answered, but does that really matter, when there is enough now known to go ahead and treat. I have been waiting for the treatment phase for evvvveeeerrrrr!!! I want to see what Dr. Grubb take is first. As far as the IVIG, I googled it, and you will see it is now an approved treatment for a lot of different conditions. One thing to note is that there are several different IVIG products on the market, made with different support ingredients. So you want the one most likely to not interfer with your particular condition. Like me with hyperinsulin, wouldn't want a product that has glucose as a base. I did also read that if IGA is low that is harder to treat and you can have an allergic reaction from the product. GEEZ 500 is already below the range what are they waiting for!!!

Actually, some may not even know what pots is. Though infection can be a cause of a high heart rate. It may be on some rheumatologist radar due to the fact autoimmune disease can cause small fiber neuropathy which triggers the autonomic dysfunction. My doctors who treat my hyper pots is my cardiologist who did most of the orthostatic testing and my neurologist who is in a subspecialty of neurology dealing with autoimmune neuromuscular disease. He ran all the crazy some scary tests on me looking for underlying cause. He diagnosed the small fiber neuropathy with skin biopsy and the immune deficiency/cvid with blood work. If you want to see the tests he ran on me I posted them a while back, you can search my posts for Dr. Todd Levine. If you see a neurologist it is best to ask if they treat neuromuscular disease. Many don't

It couldn't hurt, just be prepared to explain to them clearly the symptoms your struggling with and what you need from them. You'll have a better experience that way. Depending on where you are, most infectious disease docs are mainly in the hospital setting. It can be hard to find one with an outpatient practice. Wish you the best.

Yes, they are very different. One looks for infectious causes of illness, like tb and lyme for example and the other looks at autoimmune diseases of the muscles and joints. I saw infectious disease due to my infection rate and was cleared of issues in that area. I am still seeing a rheumatologist.

Pots dad, also I'd make a new post with your question. You will get more responses, as your post is sort of buried in this old one. I agree with corina, the doc needs to be involved on this one. It could be something as simple as she is no longer requiring as much florinef because she is improving and it is lowering her to much. I've had those low readings at times as well and that is when I feel at my worst. That is hypotension, not pots and for me can trigger syncope or near syncope, which will happen to me under certain situations. Mine happens rapidly however. I called them my episodes, they caused dramatic symptoms and collapse, nausea, clamminess, cotton ears, loss of vision and melting to the floor. I would recover after my body reset on the ground. Staying down till recovery is a must. Have they ruled out other underlying causes for your daughters autonomic dysfunction? I have several potentional causes. A skin biopsy diagnosed small fiber autonomic autoimmune neuropathy. By treating my underlyers I've had some improvements. I know she is of the age group that your probably being told she will grow out of it. I hope so! There are many of us for which that is not true. I'm now in my 50's and had symptoms as a child. Only in the last five years was I properly diagnosed and now improving with proper treatment. It is so wonderful that she has you as her advocate. It is hard I know, but that is what she needs from you. You keep fighting for your girl! We should all be so lucky to have a father that cares so much. If you can get to the bottom of this while she is still covered by insurance, that is really important. It took me years to get diagnosed partly because I had no health insurance.

I've had the pee dreams before, but thankfully never peed in my sleep. That is something I would bring up to my doctor though as involuntary loss of bladder/bowel function is often caused by something wrong neurologically.

I'm hyper diagnosised by Dr. Grubb in Toledo. He prescibed me labetalol the short acting form, I am on baby dosages of it and have the flexibility of increasing the baby dose on bad days. Labetalol is an alpha beta. I was on clonidine prior to seeing him and it was a terrible drug for me. I was first put on the patch form and developed a skin allergy to the adhesive. It also never completely controlled the hypertension. Of course at the time I had no idea I was swinging posturallly. When we switched over to the pills is when it got really scarey, it was almost like my body developed an addictive response, requiring more and more of it to keep my pressure down. Labetalol has worked fairly well, at low doses it keeps me down most of the time, however I still do get spikes. I can also not take it at all with no serious rebound. We are however treating my underlying causes, so I have had some improvement from those treatments. I swing from low to high as a result of autoimmune small fiber autonomic neuropathy which was probably caused by autoimmune disease/immune deficiency, accompanied by hypermobility and an mthfr gene defect. Take your pick which ones the underlyer.

Tb stands for tuburculosis. While it is on the rise in certain segments of the population, it isn't common in others. However, if you think you may have been around someone that had it or have been around the sub population that have it, it can't hurt to get tested. It is a simple skin test called a ppd. Mine is actually positive but I have no detectable disease. So that can happen too. A CRP c reactive protein, actually is being used more and more in the adutl population, especially in cardiology and in patients who are immune compromised, as I am. It is often a more effective marker for infection and inflammation then a cbc. I know this as I have also had abnormally elevated crp and hscrp. It however is not a test that most primary doctors go to first. You will probably have to ask for it. Hope the rheumy appointment goes well and you feel better soon. Glad to hear that the fever is only in the evenings now. If you are at all interested in reading up on immune deficiencies you can go to primaryimmune.org and read there.

So glad you went in. I hope it recovers and there are no lasting effects. When I saw Dr. Grubb, he said something similar to me. Something like, "patients get fixated on pots and attribute everything to it, this concerns me as I fear they will overlook a serious illness like cancer. Many things can cause pots and everything that is wrong with us is not just pots, pots is usually being caused by a secondary issue. If it hadn't been for grubb and two other doctors telling me I needed to figure out what was wrong, I probably wouldn't know what I actually have and wouldn't be getting the much needed treatments I'm now getting.

I'm not sure what is up with the fever, this is new and not normal for you right? Have they also checked your crp? It is another marker for infection. If you had a workup to see if your immune system is working properly? CRP along with a total quantitative immunoglobulin, sub igg panel, followed by a vaccine challenge would prove or disprove it. If your immune system is compromised in some way you are not able to put up a normal fight. A fever though is a least a positive sign your body is doing something. If this feve doesn't go away I'd go to urgent care and see if they come up with something different then your primary. Normal White Blood Cell Count Does Not Rule Out Bacteremia Daniel J. Pallin, MD, MPH Journalroduction Abstract Of 289 patients with bacteremia, 52% had normal WBC count and 17% had neither WBC elevation nor fever. Introduction Despite multiple studies showing that a normal white blood cell (WBC) count does not exclude serious disease, physicians in all specialties continue to behave as if it did. To assess whether a normal WBC count or absence of fever reliably excludes bacteremia in patients with suspected infection, investigators conducted a secondary analysis of data from a prospective study of 3563 adults who had blood cultures at a single emergency department. Among 289 patients (8%) with positive blood cultures, 77% had fever and 48% had elevated WBC count on initial measurement. Neither fever nor an elevated WBC count was noted in 17% of bacteremic patients. Comment The fact that leukocytosis is associated with infection does not mean that the white blood cell count is a good test for infection, because many patients with infection have no leukocytosis, and many patients with leukocytosis have no infection. Temperature is also not foolproof, but this investigation was limited by its reliance on initial temperature only, and some patients may have been found to be febrile later in the visit. The WBC count is the right test for neutropenia and malignancies of the white blood cell, but it is not a discriminatory test for infection. When evaluating a patient for possible infection, WBC counts should be used only as part of validated multivariable decision rules that have adequate predictive value for medical decision making, such as the Bacterial Meningitis Score (JW Pediatr Adolesc Med Jan 31 2007). Journal Watch © 2012 Massachusetts Medical Society

Hi emma, I'm going to bump your post, so our mast cell members might see it again. I can't answer your questions as I'm not up on this issue. I though am going to ask to get tested for dairy too though.

Chaos, I'm wondering about the eds thing too. When I saw the genetic doc about the mthfr she noted I had mild hypermobility and made mention of high dose vitamin c. However she also got the mthfr treatment wrong and gave me folic acid script instead of methylfolate. She is also a professor at the UofA in az. Of note I can do the thumb almost, I can do all my fingers at the first joint 90 degrees. I can bend just the the tips of my fingers down. I can put touch my hands behind my back vertically. My right ankle rolls and I fall off of it. However the areas that cause me the most discomfort are pain and spasms of my right si, hip, glut and hip flexor and my upper and mid back and where the first back joint meets the neck. When those are out I have a lot of pain. I also have gi issues which I assume are due to the sfn, I have trouble with dry eyes nose and mouth. High norepi on standing and then I have the immune stuff on top of that. With your experience with eds does these seem connected to you? My treatments for the different things are improving things but I still have'nt cleared many of the symptoms completely and I wonder if I'm missing something? I don't think I have the mast stuff as I dont really flush or have obvious allergy symptoms and I'm so dry I don't like taking antihistamines as they further dry things out. I have to take them for my infusion. If you have any insight I'd be grateful.

I feel like I have to go a lot too, mine also is often clear. I also never drink as much fluids as I should and still feel the bladder pressure and I don't like the idea that if I drink more I'd never leave the bathroom. I seem to get rid of my fluids rather quickly. I don't salt load because I'm hyper pots. I too also drink smartwater pre, during and post infusion, which I get once a month for cvid. If you have a costco near you they have the liter size for less then a $1 a bottle, in multipack. I'm going to have to see if I too, actually pee less when I'm drinking it.