arizona girl

Thanks raisin! That is helpful to know. Anyone else have a good through experience with their tilt table test?

Well as good as it can be considering, for most of us, the test can feel like a small form of torture.

Hi raisin,

Just needed to clarify a few things. Was the high igm part of the Total quantitative immunoglobulins (TQI), which includes igg, iga, igm, igd and ige; or elevated for a specific disease test like the arthritis panel? Titers can be run just for specific diseases and can means something completely different then checking the total immunoglobulins. Another factor at play can be your sex as well as your age range.

If it was part of the TQI panel, then it helps to also know the range that was used and where all your immunoglobulins fell in those ranges. If this was the test that was done and only the igm was out of range then your doctor needs to further work that high igm up, probably by a hematologist. It may be some kind of monoclonal gammopathy. It can also point to infection, do you know if you have a normal cbc w/differential panel?

Here is a link to mayo's lab test definitions on this lab test: http://www.mayomedicallaboratories.com/test-catalog/Clinical+and+Interpretive/8158

Also here is a link to primaryimmune.org and their definition of hyper igm, it can't hurt to explore the other areas of this website as well: http://primaryimmune.org/about-primary-immunodeficiencies/specific-disease-types/hyper-igm-syndromes/

Hope this helps further your research, so you can ask your doctor informed questions.

A lot of us have had one done. Who do you think did the most through job in testing you? Meaning did they test your blood volume and catecholamines, who long, etc, or anything else that I might have missed that can be done during the test.

I need to refer someone and want to send them to who does the best testing as experienced by my fellow members.

This was my experience with Dr. Grubb too! It was very helpful though and sent information to my local doctor.

Yes, this is my understanding too. After I had a spontaneous colon rupture along with the fact that I have family members who are very flexible, I had the genetic test done for vascular, it came back negative thankfully. I do though continue to stay aware of the possibilities. Dr. Grubb said I was mildly hypermobile, but I was not told I have EDS.

I think the best eds doc out there right now is Brad Tinkle in the chicago area. Here are some links about him, including something he calls a bookmark.

Here are the links: http://drbradtinkle.com/#home http://leftpawpress.com/books/medical

This is from a blog on last years edf conference: http://edser.weebly.com/notes-from-2013-conference

dg,

There are some canadian physicians have you looked into any of them. Canada may have more flexibility in getting treatment options covered then in the USA. There have been several members that were treated for aag up there.

Spin,

If you give the other members the general part of the country you are from, you will get more suggestions on who to see. Have you tried searching the dinet physicians list to see if there is someone closer to you?

I think there is an eds doc near chicago, dr. Tinks that has been mentioned in the forums, another dysautonomia doc in that area is Dr. Barboi.

I saw Dr. Grubb too and had a similar experience. It takes a long time to get in with him.

What were the results of your qsart, cat and sweat testing? If those were positive that can point to a small fiber neuropathy and that often can be autoimmune in nature. Vandebilt is also looking into autoimmune causes now as well. They also have a research program you may qualify for.

It is odd that your pots and eds docs aren't relating to each other. It is very common that EDS's patients have a dysautonomia due to the floppy nature of their veins, thus the EDS is causing the autonomic symptoms.

Happy to help, but remember my diagnostics and presentation are different from ty's, though there are some similarities. I also do not have low bp, but the opposite. I'm not in a wheelchair. I was going very high on standing. I had catecholamine testing done supine then to standing and on standing my norepi went from 400's to 1100's. That is called a hyperandrenergic response and is compensatory. I did have some near syncopes with added stressors, which we now know was caused by my body responding to the high bp/hr. Syncopes force you down and the body can then re-regulate. They were pretty dramatic, but not frequent and had been happening since I was a child. I am grateful I don't have the low blood pressure. I think that low bp is much harder to work around then a hyper response. I feel for tyler if he has any of the symptoms I get when I'm going into syncope, they are awful. To have that every day, thats tough.

Have Tyler's Cats been checked yet? Has he had a skin biopsy to see if there is small fiber nerve damage yet? Also did he pass an emg which tests for large fiber damage (this one looks at your motor skills and strength)? A neuro is who looks into these.

I now know to use an increase in my pain levels, as a sign my body is fighting something, my spikes in bp/hr are also often associated with me fighting something. As you know with low immunity, you don't always run a robust fever with infection, because your body can't mount a proper defense.

With strep on board I imagine that alone is increasing his pain. Here is the thing about trying to exercise with an active infection, it depletes energy and therefore takes away from the bodies ability to fight the infection. Isn't that why they recommend rest and fluids when you have a bug! I think it is wise to go after first things first. That infection is first priority, mainly because infections that aren't bought under control can kill us and as you know do the things to ty's body that are happening - tremors, pain and headaches. I am going to start pt now because we have addressed some other things first. My last three attempts at PT were a fail and actually made me worse.

My pre ivig headaches were almost always associated with me not being able to move my gi track. The only real headaches I get now are just after my infusion and/or dry nasal passages.

As for going out in public. I never stopped. My infections were never upper respiratory, except for tonsilitis and a few ear infections. I'm guessing because my IGA was still normal. It was my igg and igm that were low. I was careful though when going to the hospital. I also never touched things and kept my hands off my face. I carry the alcohol wipes and am still careful about what I touch. Strep is a different bird though. If I were him I'd wear a mask for the airborn stuff. Doing the ivig in the hospital has it's risks too! The sooner he can get those in the home or at a nonhospital infusion center the better. Car rides are never fun when you have low bp. I still hate being a passenger and getting jostled around. It is better if I'm the driver.

I'm sad to hear that your doc is still fussing around with the carimune, trying to make easier to tolerate. That is exactly what happened with me and after four months we finally had to switch and only then did I start to improve. I lost four months messing around with it.

Did I cover everything! Look forward to seeing what the doctor says when the culture comes back.

Hi Jade,

Does sound a bit like you aren't getting all you could from his doc. Have you actually seen the results of his tilt table? If you have not ask for a copy of all your son's medical records including doctor notes and all tests that were done. You have a right to those. I have a hard time believing that with obvious and acknowledged hypotension that there were not some findings on his tilt table test. Does your son always have low BP whether he is lying down or standing? Or does it only occur when he stands. If it only occurs when he stands then that is the definition of orthostatic hypotension and is just one of the forms of dysautonomia. POTS and syncope are some of the other forms. You can also have more than one form at the same time.

Seems you need some more info. There are a few pediatric doctors that might be good to see. It depends on what part of the country you are in. One of Dinet's medical advisors Julian Stewart is one, the other is a Dr. Boris who also specializes in eds in philly. EDS docs are hard to find and you may have to travel to fine one. Floppy veins in EDS as you know contributes to the low blood pressure. Have they done the eds iv genetic test on him yet?

The other members have given you some good tips and from what you've posted it doesn't appear he has been given all the treatment options available. Orthostatic hypotension causes many symptoms that are hard to overcome. To say just go live your life and if you'd only do some excercise you'll be fine, I feel is almost cruel. Exercise of the legs will strengthen the muscle pumps and help pump the blood back to the head. Your son is not making this up, he does feel as bad as he says he does, which really does make exercise hard to do.

As a patient myself being validated that what I'm dealing with is real, is very helpful to hear. Sounds like you are a good parent and advocate for your child. Also be his cheerleader and acknowledge that you know what he is dealing with is real. So many of us get treated like we are not of right mind, it is a relief when some acknowledges that things are real and we don't keep having to prove to someone like a doctor that it is. He needs some hope that things will improve, and it doesn't sound like he is getting that from this particular doctor. Many of us here have had to move on and find doctors better suited to our needs. It is okay to do that.

Hey Rachel,

Sorry for sounding like debby downer yesterday. I just wish there was a magic wand that poof could make it all go away. Tyler is young and they are making so much progress with the research, including stem cells which may even pose a cure. He has more time for them to figure it out. I'm already on the downer side of 50. I lost so much time with not being heard or diagnosed in a timely manner. The illness has caused huge losses in my life. The mthfr along with pcos caused infertility, so I have no children of my own. I do have many godkids and nieces and nephews who love me. So god does make a way. I need to remember that I have much to be grateful for, including my current medical team and a treatment plan that has stopped the progression and is keeping me stable.

I'm not sure if you're asking about post infusion symptoms or improvements of symptoms over the long term. I will answer both. Once we switched to privigen the improvement of the post infusion flu and headache was significant. I felt I got worse with each Carimune infusion. It has a lot of sucrose and I was already prediabetic because of the pcos, so it was just a bad drug for me.

Since switching I still get the headache and flu aches but they are much milder and some times don't clear until the 5 day window is over. I've been managing them with pain meds, diazepam, icepacks and rest. After talking with my doc yesterday, it is probably because I still have low blood volume from the nerve damage, and drinking fluids isn't enough to rehydrate me, I get rid of my fluids rapidly. Right now even with the mild post infusion symptoms, you'd have to fight me to take my ivig away.

The symptoms that have been improving with continued treatment, are my all over body pain. The peripheral neuropathy type,except I had it full body. I used to wake up digging my fingers into my hand, with a rigid body, biting my bottom lip. I still have pain, just rarely that type. My very low grade afternoon fevers, which also spike my bp/hr are now gone, along with that my wbc is no longer elevated chronically. Yesterday it felt like I was getting a sore throat and swollen glands. Today gone. So I appear to be fighting things off now, except of course for the yeast. My heart rate rarely goes above100 now. Though I start low on waking, so 96 bpm still feels like I'm exercising. My bp swings are closer together, though I still get my spikes. I was one of those that are hyperandrenergic with a surge in norepi on standing. I now get a burst of energy and can do more for about two weeks following infusion week, before I start slowing down again.

I never had tremors. My guess would be for Ty until the antibiotics along with the ivig really knock that strep out, he is going to continue to battle this. He may need the broad spectrum antibiotics for an extended time. Of course be on the watch out for cdiff, if they do that. A peny shot three weeks apart, seems to me an opportunity for him to build up a resistance to it. I also had trouble with the levequin/cipro class of antibiotics, as they can further nerve damage. I tolerate rocephine very well. My infections were cleared by days of iv antibiotics, granted around a small colon rupture, but also another four days a year later after an elective colon resection. The day of the surgery my wbc was way up again. Since then I get the rocephine shots if my wbc elevates. I have not had to have any of these type of treatments in over a year. So something is working. Not perfectly, but enough to have a better quality of life, even though I still have fatigue and aches and pains here and there. I'm pretty weak muscle wise and need to start rebuilding my strength with exercise and am going to start PT again.

Your poor guy! He sure is a trooper. I really hope this time next year or sooner, we see him in a much better place and much improved health wise. Let us know what happens.

I just talked to my neuro about trying to tweak my treatment plan to see if we can get anymore improvement. We are going to add some iv saline with my infusion to help with the headaches and post ivig flu. Fingers crossed it works and I recover more quickly from the infusion. He said there isn't much more we can do to suppress the autoimmune end of things. My only other options are the chemo level immune suppression drugs, which really are probably to strong for me to take with the hypogamma on board. Plus I can't even take methotrexate because it becomes toxic when you are MTHFR. He put me on cellcept before I knew I had hypogamma, and I got really sick.

We also discussed that this was why I did so good with the pheresis, as we were removing the antibodies from my blood. Which kept them from attacking me. I was doing that up to 3 times a week in the beginning, but then I got an infection and anemia. For me the ivig is easier to do then the pheresis, even though it was just as effective. However, he said the ivig doesn't stop the body from making the antibodies it just neutralizes them, or another word for it, modulates the immune system. Kind of like the clown in a bullring saying no over here not there. I also suspect that some of the small fiber autonomic nerve damage, may not be reversible. Some SFN can be reversed with treatment. I just have had it for far to long before it was diagnosed to believe it is totally reversible.

So, I guess no treatments knock things back to normal, as much as I wish they did. We know I still have the autoimmunity attacking me, because my recent thyroid antibodies while lower are still elevated and my lichen has yet to clear and I once again have yeast. I suppose that is better to deal with than tyler's strep and everything else he has going on.

I've had 3 appointments this week already, with two to go, and another two next week, then ivig again. I am so tired of my social life being found getting medical services. I'm sure tyler knows how that feels. I keep going though because I do think there is still room for some small improvement. I know at some point I will have to accept that this is good as it is going to get. Thanks all for letting me vent.

Hi Rachel,

I'm assuming that when you asked katy about the rocephine shot you meant me. I have had my shots at both urgentcare and my primary care's office. It depended on whether the elevated wbc's came in high during office hours and if I could get in with my primary care in a timely manner. When I can't I go to a local urgent care, who knows my history and gives them to me. I believe infusion nurses or home health nurses can give them, if they have a script from your doctor and is ordered ahead of time.

The medication is a bit thick so the shots hurt a little, also depends on how good the shot giver is. I prefer the shot to weeks of the oral antibiotics. However when I have an infection I may require up to 3 shots fairly close together, usually within a ten day period. We monitor my wbc and stop when it returns to normal and symptoms are gone.

It really sounds like with the bixan not stopping the strep and now peni, your doctor should be getting that antibiotic resistant testing done, so he doesn't build up further resistance.

Glad to hear your doc is open minded and willing to make the necessary adjustments. This hasn't been an area of medicine the cardiologist are up on, as they stay in their lane. Now that many of these types of patients present with cardio symptoms first, I am glad to see some of the antibody/viral research now involving cardiologists like Raj at vanderbilt. Sounds like you are in good hands.

Rachel,

IVIG helps replace the immune system and modulate it. Even patients that have CVID do still get infections. The ivig helps reduce the number of infections it does not get rid of them completely. You will find these type of discussions on the cvid support group over on the "gentle strength" forums.

Over time the infection rate should improve. I had several more infections the first two years after starting. Prior to that I had a chronically elevated wbc often with elevated neutrophils. I did have an improvement in my rate of infections, after starting. Even though I never run robust fevers including with pertonitis after a colon rupture, i was running very low grade afternoon fevers prior to starting ivig.

In the last year I have not run those fevers and my wbc has been normal every time I thought I was coming down with something. As I said before the one exception to my ivig treatment was when I was on carimune. I got worse while on that and improved within six months of starting privigen. It did though take me six months to see any improvement once the switch was made.

IVIG as it is only IGG can not boost immunity to all types of infection, for example it does not stop yeast infections which are fungal. So it really depends on the antigen the body is fighting. It is known to help with most bacterial infections, which strep is.

Glad you called your doctor right away. Often antibiotics must be prescribed along with ivig. Another thought is perhaps Tyler has built up a resistance to the penicillin. I believe there are test out there that can check for antibiotic resistance and will point one to an antibiotic that will be more effective. It may be time to see if you doctor will prescribe a more board spectrum antibiotic. I like rocephine shots, as they have worked well for me and knocked down my infections and lowered my wbc quickly. Sometimes I did not know what the infection was, we assumed they were GI related due to my symptoms. With out getting an antibiotic on board though, my wbc would continue to rise.

Trying to avoid antibiotic resistance is one of the main reasons ivig is prescribed. You all are still really early in the game in treating this. Do you think he may have been exposed to the strep during his recent hospital infusion? Hospital infections are very common. My mother has had several that almost killed her. It looks like that is who I got my hypogamma from.

Hope this helped. Please let us know what your doctor says.

Thanks for the update. How's he doing now that he is past the 5 day window for post infusion flu and headache. He getting any energy boost or feeling better? That headache post infusion is just so much fun!! Sometimes I don't get it until the 4th day sometimes on day one. I haven't figured out yet why that happens.

Hmm I'm having trouble with poor sleep too! I'm homozygous mthfr, i wonder if the b6 might help me. The juicing is a good idea, you have a recipe that doesn't use mangos? I'm not a fan of those. Do you think just taking the active form of the b6 as a supplement might work just as good? I'm suppose to watch my sugar load. I am already taking methylfolate and methyl b12 for mthfr, but honestly the homozygous mthfr is the only test they did, so I don't know if I have other issues with the methylation process or not.

I do very much crave fruits and veggies often, I sometimes wonder if that is my bodies way of compensating for what it can't convert itself.

Wishing you both a good month.

I'm sorry to hear this, it sounds sort of scary. Have you had an mri before this last one? We have a friends daughter her dealt with seizures and saw a great doctor here in az. They were able to get it under control and she has been seizure free long enough to drive again.

I'm glad you are seeing a specialist, that is the right course, especially now that the last one caused a head injury. Did you sense this last one coming on before it happened. That would be my biggest concern until you see the doc, is making sure a new seizure doesn't cause another injury. Sure I'd think if you had something going on in your brain it would probably cause symptoms including brain fog.

Often symptoms that we attribute to dysautonomia are the result of an underlying undiagnosed medical condition. I wouldn't be embarrassed at all those care providers witnessed it, as their witness statements ensure that you have the evidence to show something is wrong and it needs to looked into.

Don't give up on pursuing this until you feel like you have the answers that make sense to you. Trust yourself and your instincts.

Hi christine,

If you are one us who's bp/hr swing with change in posture, it is sometimes hard to get it right after you stand, as it is in the process of changing rapidly and the cuffs can't catch it. If you can stand still or lean slightly against something like a high dresser with your cuff arm level with your heart and resting on the top with the machine next to your arm on top as well, it may be easier to get it. You might also have to wait a few minutes for your bp/hr to stabilize before you can get a reading. Of course if you feel like syncope is coming on then lay down as quickly as you can.

Do you know yet if your bp/hr go up or down on standing? If you're dropping you may have to have your doctor document it in a safe place like his office or during a tilt table test. As you can't risk a syncope unsupervised.

I recently ponied up the money and bought the omron bp791lT off of Amazon. The one that has the software on it so you can download the info to your computer. It also has an A & B option so more than one person can use it. I use the A for my supine readings and the B for my standing readings.

When you're in the ac room also lay down with your legs propped up, so you can reset your body before getting up again. I do this all the time when trying to get things done and it really helps, even if things take longer.

Oh also I heard coconut water is full of natural electrolytes, but look that up and double check.

Your welcome. A few more thoughts come to mind after reading your last post. Small fiber neuropathy is often patchy, which is why a neuro who is up on it will do more than one test. You can have a one be neg and the other positive. In my case the skin biopsy was enough to show I had it. While they take a skin punch at both the lower leg and the thigh, small fiber is diagnosed by the lack of density of the nerves on the lower leg. If there is also damage at the thigh level it means a different type of neuropathy.

A borderline diagnosis then would be made by the density not just the location. Do you know what you're density was and the range that was used? A year later without any real treatment on board yet, my density had reduced further. So it is a test worth doing again. Personally I think the skin biopsy is the gold standard and better than qsart. If you have issues with heat regulation and sweating then the sweat test they do where they coat you with purple dust and put you in a hot box, will really show where that nerve damage is when it comes to sweating.

Immunologist are also allergists. Did they do allergy testing on you? I can't imagine what else sixty pages of immunological labs would consist of. Hopefully you read all of these yourself.

An immunologist trained in treating primary immune diseases, (most are not) can also test to see if your global immune system is working correctly. That test is call a Total quantitative immunoglobulin and consist of your IGA, IGM, IGG, there is a sub IGG panel, igd an ige (ige shows allergic reactions) Then a vaccine challenge test if any of those come back off.

Do you have diabetes or been screened for other autoimmune, endocrine, rheumy or hematological disorders? When you say your TTT test was highly positive what does that mean? Tilts can point to a variety of dysautonomias. What are your symptoms that cause you the most problems? The reason I ask these questions is depending on what your responses or diagnostic finding are it will point you in better direction to manage it.

Sometimes a cause of the neuropathy can't be found yet, it is then consider idiopathic and the only options are to manage it by treating symptoms. Eventually though if new symptoms occur one may find the cause, so it doesn't hurt to periodically revisit certain testing.

Hi Goshi,

The correct term for neuropathy that can effect the autonomic nervous system is "Small fiber neuropathy". The large fiber nerves are the ones that control your motor skills like moving your arms and legs. The small fiber nerves control all the functions that your body does without you knowing they are working if they are working right. When they aren't working right, you can feel it. There are several types of small fiber nerves, some control sensory or pain functions others control all your other autonomic functions like heart rate/BP, temperature regulation, digestion, the ability to sweat, etc.

There is very legitimate testing for small fiber neuropathy today, skin biopsy and qsart, along with testing the autonomic functions with tests like the Tilt Table, can prove it exists. The causes though for this type of neuropathy are "endless" as my neurologist told me. The most common cause is diabetic neuropathy. However, to name a few, autoimmune diseases, small cell cancers, toxic exposures and injuries can also cause it. That is why it is important that one looks at all their global symptoms even if they seem unrelated to each other. Defining your symptoms along with your diagnostic testing can point towards a proper diagnoses if your dysautonomia appears to be do to a primary cause.

In my case after much testing including everything I mentioned above it was proven. We then went on to find I also had 3 autoimmune disease that like to party with a primary immune deficiency called hypogammaglobulinemia or cvid.

I have stopped declining and have had improvements once we started treating appropriately. We are still tweaking with hopes of further improvement. Autoimmune diseases are difficult to treat, so I still do struggle, but I am grateful we can at least manage it.

I hope that clears up the confusion. I can't say I agree with the comments your neuros expressed, as mine feels exactly the opposite. However my doctor does specializes in autoimmune neuromuscular diseases, which is a very specialized area of neurology, that many mainstream neuros are not as educated in. I always listen to my gut, when evaluating a physician's opinion. We are all human and can have biases, that is even true amongst the centers that are doing research.

Hi LM,

Glad it hasn't been too severe and they handled the reaction. I know when I have an elevated wbc at the same time as infusion, the infusion is rougher then usual. I think because the immunoglobulins go after the infection. Glad to hear the infusion rate is that low, probably safer for him and also why it is taking longer. You will still need to watch for post infusion reactions for the next 5 days. Hopefully the doctor will send you home with post meds to help keep that from happening. Don't forget the hydration, it is really important.

Do you know what brand of ivig they gave him? Please keep us updated.

Hi LM,

I should also mention the headache or post infusion flu as I call it can happen up to five days post infusion. So keep an eye out for that. I can feel great the day after and then get it on day 3 or 4. Also since he is going on oral pred, keep an eye out for yeast infections from that. Those of us with low immunity can get it easier from both steriods and antibiotics. Diflucan has become my friend. My antigen arm test showed I have no immunity to it, didn't react at all. Do ask them about his kidney function labs as well after the infusion and keep an eye out for those kind of symptoms.

In some ways PEX might also be really good for tyler. It basically is like filtering the bad stuff out of your blood. They use albumin as the replacement blood now. So that is also safer. I was on twice a week for several months. I did improve until I got an infection and then anemia. They also have to surgically implant a catheter, which can also be the source of infection. Taking showers was a pain, cause I had to keep my chest toggles from getting wet.

PEX can also be used in conjuction with ivig, it just has to be spaced till after the ivig leaves the system in two-four weeks. That is so you don't filter out the good immunos they just gave you. That is also why I have to have monthly infusion or those on the subq form have to do it weekly. To be honest I had a good early response of symptoms to PEX. It took much longer for my symptoms to improve once I switched to ivig. PEX is still an option to add back in for me, but I don't think I would need to at this point, as I am stable on the ivig. I can tell when it is wearing off and I do get a real energy boost once past the post infusion flu/headache.

Your a good mom so I know you will read everything you can. I will keep praying that Tyler is one of the patients that just sails through his infusion with zero side effects. That does happen with some patients.

Rachel, I'm so sorry to hear this, but glad your doctor caught it. I know you were trying to avoid the ivig. I will pray it helps Tyler as it has helped me. My infection rate has dropped. The first infusion though can be rough and give a really bad migraine. From my experience it is important to know which brand of ivig your cardio is going to give him. There are ones that have almost no iga in it, which will help keep the reaction down in his case. Also the ones that have no sugar, are easier on the kidneys. See if they will give him iv saline before to boost his blood volume, as ivig can thicken the blood. I now take a baby aspirin to counter that. If he can't get the iv saline, he needs to really hydrate before during and after the infusion for a couple of days.

The most important thing that keeps side effects down is the infusion rate. It should start very slow and slowly tirate up and to be safe not exceed 100 on the first go. I am only up to 150 after being on it 3 years. It takes 5.5 hours to infuse 70grams in me. 70 grams is also the most anyone should ever receive at one time. Children may be limited to an even lower dose. Often the induction dose is given over a couple of days starting with 30-40 grams.

Premeds are very important to keep reaction down. I take benedryl and tylenol and used to be given iv decadron in the beginning. I can no longer take the steriods because I have hyperinsulinemia and they push me towards diabetes and cause weight gain. I also have diazepam (muscle relaxer) and hydromorphone (stronger pain med), to knock down any break through migraines. My head and neck muscles get really tight before the headache. I never taken them at the same time though, and leave at least 4 hours between them.

Hang in there, you are such a good mom! I will be keeping you and tyler in my thoughts and prayers.

Did you test positive for aag? I'm going to have to look up the poly gam, that might have another treatment course. Seems like your up on stuff so you probably already did that. I know the healthcare systems in our countries are different, there are some uk's on the site, maybe see if they can recommend someone to see in your area. Primary immune I believe is international so you could see if they are working with someone in your area.

I see why your caught in the middle between EDS and autoimmune causes, as they are treated very differently. I too have mild hypermobility, so I'm proof you can have autoimmune and EDS at the same time. The famous Dr. Grubb diagnosed me with it and hyperandrengeric pots/syncope. He actually put me on labetalol as it is a alpha/beta blocker, that is short acting form, so I can take it as needed. It has worked well. I'm not swinging so much now though because we are treating the other stuff, so there are days I don't need it.

I can also see why your neuro would like to try to find cause for the small fiber, as treatments vary depending on cause. I had the skin biopsy and that along with my failed tilt and symptoms proved mine. My neuro said the causes for it can be endless, and he tested me for a bunch of scary causes which were negative. It was after that and my positive response to the pheresis that we found the hypogamma and then the autoantibodies for hashi and skin biopsies for uctd and lichen then proved the autoimmune component and probably primary cause. I had very high to low ana's on and off for years. You are right that only certain conditions are approved for ivig, I do believe AAG has been added to the list in the USA as FDA approved. My neuro started with pheresis since he could get that through unchallenged, my positive response to pheresis and the plaquinel also prove the autoimmunity. My ivig was approved through insurance though because of the hypogamma/cvid, not the small fiber. He has me as polyneuropathy and they wouldn't approve it for that.

I know eds is considered a genetic defect but it does still effect the connective tissues as do some of the autoimmune disease. So I can see treatment being tricky for you. Even if you get the autoimmunity under control there is still the floppy vascular system that can cause the same type of symptoms, so you might still be symptomatic. The genetic doc I saw said that they are now treating eds with high dose vitamin c to help the connective tissue improve.

Well I hope that helps. I do feel for you and how difficult it is to get everyone on board and fight for what you think is right. Don't give up and take a mental vacation from it here and there, if it gets too stressful dealing with your healthcare system. Then go put your warrior hat back on and try again.

Hi elizabeth,

What country are you in? I can only tell you my experience from a usa perspective, I know things can be different in other countries.

First though, your doctors have provided you with incorrect information about ivig and pheresis. I had pheresis and now get ivig going on 3 years now. I also have a similar diagnostic profile as you. My neuro who treats autoimmune neuromuscular disease first prescibed pheresis. Blood products are no longer used with pheresis in the usa they use albumin where the blood product used to be used to do the exchange. As for ivig it is safer then a blood transfusion as it goes through many levels of filtering and screening today. There is a small chance that some unknown antigen could be in it that they don't screen for, but the scrubbing process would usually kill anything like that as it does for the known antigens they test for. From what I understand though if you do have aag, ivig is the main treatment plan. So it is confusing what your doctors are saying. Often because of the expense involved some doctors don't like to prescribe it.

Also there are screening tests that must be done before ivig is put on board. If you ever get to that point let me know and I will get you the list of tests. Not everyone is a good candidate for it. As looneymom mentioned and posted in the past her child's tests showed it might not be a good choice for him and could harm him.

An excellent resource about ivig products is http://primaryimmune.org there is also a magazine called ig living. Also primary immune has a doctor referral resource to help you find a doctor in your area.

Your symptoms do sound like an autoimmune neuromuscular process, similar to mine.The treatments you've described sound only like they are trying to give you relief from symptoms not treating the cause of those symptoms. From my experience those treatment will not stop the progression of the disease process and things can get worse. I also am on plaquinel to address the uctd and it has bought my inflammatory markers down. For me it is a good drug as I can't take stronger immune suppressants because of my immuno deficincy and a mthfr gene defect. The ivig boosts my immune system and helps modulate the attack on my small fiber nerves. I also take thyroid meds for hashimoto and glumetza for hyperinsulinemia/pcos. I still don't feel great but I have slowly had improvements in my labs and some of my symptoms.

Do as much research as you can, in cases like ours information and documentation is power.

Yes, I don't know how common it is but it does cause dysautonomia symptoms. My neurologist actually did a blood test on me for it, along with a bunch of other rarer causes. Thankfully it and carcinoid came back normal. My skin biopsy for small fiber though came back abnormal and I was diagnosed with idiopathic polyneuropathy. Was treated with plasmapheresis and had improvement, then got an infection and anemia. At that point my immune system was tested and I had hypogamma and then qualified for ivig. BTW I never ran proper fevers even when it was obvious I was sick. So, my infections were going untreated.

Like an onion the layers kept coming off and we soon discovered I had several companion autoimmune diseases that like to party with hypogamma. Hashimoto, uctd and lichen. I also have pcos and metabolic syndrome. My 3 different skin biopsies for the sfn, uctd and lichen proved more diagnostic then my blood work. Often antibodies don't always show up when you have hypogamma. I had obvious symptoms of thyroid and uctd for years before blood work finally showed elevated antibodies. It took forever to get diagnosed.

So all that to say it can't hurt to rule things out. The doctor that diagnosed me specializes in autoimmune neuromuscular disease, which is a small sub specialty of neurology. When I first saw him, he said to me "There are endless causes for pots, lets rule out what we can."