RichGotsPots

Wow Alex! I just saw this post for the first time. I am so glad you finally got approved. i have been trying to for months. I have autonomic neuropathy and Sjogren's like Lauren Stiles, but I am beginning to think my Sjogren's is secondary to Churg Strauss. Once I get my Rheumy involved I think I will get approved too. The only thing is the protocol is more like Guillane Barre then AAG. They actually might alternate Plasmapheris and IVIG. Glad you made it threw your first infusions. High dose steroids are great for autoimmune too so that can't hurt. The only thing I would ask add to your pre load before infusion is IV Saline for extra hydration. If you are on facebook there is IVIG groups. Also Primary Immune org that arizona mentioned has a medical guide for IVIG infusions because that community gets it the most. I know a guy who has been getting it for 30 years every month and never even used a port. He is on one of the forums. It all depends how much /KG you get but most ppl I know take 6-8 hours. Also for diabtics there is a type without sugar in it. Also if anyone can't take it any more as mentioned above then they can always try out plasmapheris. That is your own blood being taken out and filtered of autoantibodies and then infused back in... Works very similiar to IVIG except it can only be done in the Hospital. Both AAG and CIDP ppl get this or both. Also I found a research paper that said you need to test for IgG level right before and after. They found that if your IgG levels didnt rise substantially from before levels then it need to be given at higher dose or else its not affective. I dont have the link to the article right now as its 5:15am lol but maybe another day I will post it here. Prayers it starts working soon. If I get it for Churg Strauss it is 2g/KG and I alternate with Plasma :-x

Those are from this http://www.ncbi.nlm.nih.gov/pmc/articles/PMC1221991/pdf/11463332.pdf

NOS INHIBITORS There are a bewildering array of NOS inhibitors described in the literature and in use as pharmacological tools. Of these the most widely used have been -NMMA, -NNA and its methyl ester prodrug (N G-nitro--arginine methyl ester, ‘ -NAME ’) and aminoguanidine. Table 4 shows the efficacy of some of these in inhibiting the three human NOS isoforms

NOS inhibitors may have very different effects on biological systems depending on whether they inhibit the syn- thesis of both NO and other species [e.g. NG-nitro--arginine (-NNA) on nNOS] or not (e.g. -NMMA on nNOS)

The peripheral selective α−agonist midodrine is the only agent approved by the FDA for the treatment of orthostatic hypotension.43 The pressor effect of midodrine is due to both arterial and venous constriction. The efficacy of this agent has been demonstrated in open−label and double−blind studies.43−45 Midodrine, the prodrug is activated to desglymidodrine the active α−adrenoreceptor agonist. Absorption as the prodrug may theoretically minimize direct vasoconstriction of the gastrointestinal tract. Midodrine is rapidly absorbed from the gastrointestinal tract. The peak plasma concentration of midodrine occurs in 20−40 minutes and the half−life is 30 minutes. The half−life of desglymidodrine is 4 hours. Patient sensitivity to this agent varies and the dose should be titrated from 2.5 mg to 10 mg t.i.d. The peak effect of this agent occurs 1 hour after ingestion. Potential side effects of this agent include pilomotor reactions, pruritus, supine hypertension, gastrointestinal complaints, and urinary retention. Central nervous system side effects occur infrequentl

The peripheral selective α−agonist midodrine is the only agent approved by the FDA for the treatment of orthostatic hypotension. The pressor effect of midodrine is due to both arterial and venous constriction. The efficacy of this agent has been demonstrated in open−label and double−blind studies. Midodrine, the prodrug is activated to desglymidodrine the active α−adrenoreceptor agonist. Absorption as the prodrug may theoretically minimize direct vasoconstriction of the gastrointestinal tract. Midodrine is rapidly absorbed from the gastrointestinal tract. The peak plasma concentration of midodrine occurs in 20−40 minutes and the half−life is 30 minutes. The half−life of desglymidodrine is 4 hours. Patient sensitivity to this agent varies and the dose should be titrated from 2.5 mg to 10 mg t.i.d. The peak effect of this agent occurs 1 hour after ingestion. Potential side effects of this agent include pilomotor reactions, pruritus, supine hypertension, gastrointestinal complaints, and urinary retention. Central nervous system side effects occur infrequently.

http://www.med.nyu.edu/content?ChunkIID=38207

If its gastroparesis then fiber is not a good idea so puree stuff will be bad, if its pooling then carbs are not good. That' what it is in my case and I switched to a 90% carb free paleo diet which is also good for autoimmune too. But most likely its the fiber and motility because that can cause vomiting. If it's acid reflux which can also cause vomiting the. Google some acid free diets. I have heard of all day apples the first day with intro foods the following days and some natural and otc meds..like a zantac and Prilosec combo

So happy I you posted on this older posting so I could find it. I have autonomic neuropathy which is actually what gangliopathy means in medical translation becaypuse the ganglia are the autonomic nerves so they got all fancy with calling it ganliopathy. So first off everyone who has AAG or autoimmune Pots needs to get a baseline with either a QSART or Skin Biopsy so that they can retest their nerves to see if a particular treatment is working. Next before and after every IVIG treatment or atleast the first few months. IgG levels need to be taken before and after via blood. Doesn't matter if they are low or high what matters is that after the IVIG treatment they go much much higher. If they don't go much higher then the amount of IVIG is not enough and a high dose is needed until there is a boost. This affects how well IVIG will work. IVIG is usually tried first and if it doesn't work well or if you can't handle the side effects they do Plasma. I have never heard of autonomic problems caused by Plasma and I know a few AAG ppl on Plasma doing well. With Plasma the danger is blood clots when they take the blood out but they mix in anticlotting agentqs to minimize that greatly. With IVIG the treatments need to be done very slowly from 5-8 hours, you need to take tylenol, get IV saline before and after and take an allergy pill to minimize side effect symptoms. Lastly, Mayo did a few case studies on AAG meds and said,"There is preliminary evidence that novel immunosuppressant agents such as Mycophenolate mofetil (MMF) and Rituximab may be effective in certain patients who are unresponsive to IVIG or PE." Also I read a cleveland clinic report for sarcoid which is autoimmune too that said once patience had autonomic neuropathy steroids stopped working and only IVIG helpped 66% of patients recover. So I believe that if you test with autonomic neuropathy then it gets to the point where it doesnt show up on a skin biopsy they these immunosuppressants can be affective. private message me if you have questions

There is a POTS couple who was visiting St Paul Minnesota for pancreas surgery for the girl. The guy had a terrible seizure and is now stuck in the hospital for days. So the girl is stuck alone and weak. Is there anyone out there who can help or knows anyone who can help out for a few days. This is really urgent. They have a dog and she is too weak to walk it even.. Thank you!

Awww, I have a wild rabbit that I have been feeding for 2 years outside my front door. He comes hoping up to me when I call to him now. Sill to shy to let me pet him. But he will eat a snack just 6 inches from my feet makes my day to feed him!

Pulmonary autonomic denervation study comparing Diabetics with and without Autonomic Neuropathy and a control group it's amazing what they found and it was 18 years ago http://0-jap.physiology.org.library.pcc.edu/content/81/5/1978.full

I figured out that people who get lung transplants are a good to compare us to. "During lung transplantation, vagal pulmonary afferent nerves are transected in order to remove the native diseased lungs.Lung transplantation may therefore be a human model of vagal pulmonary afferent denervation" ( https://journal.publications.chestnet.org/data/Journals/CHEST/21983/1192.pdf ) So basically I'm calling all my research friends out to look for studies focusing on this denervation and what symptoms it leads to and what if anything helps correct it...

I think it's really tricky to catch this and not sure if an EEG or wakeful apnea study would catch it.. With all the research I read it is so rare to see any studies on our Medulla and Hypothalamus. I will keep you posted. On my research of central hypopnea. I think they are common with illnesses like Parkinson's..

I strongly believe that pots breathing problems stem from ganglia small fiber autonomic neuropathy interferring with the central singals from the brain to maintain the proper breathing rhythm. But there may be a component that comes from the Medulla or Hypothalamus (central part of the autonomic nervous system). I know about 2 dozen potsies who have had sleep studies and about half were diagnosed and the other half were not. Which makes me think that not all of our apneas or hypopneas happen while we sleep and that a day time minitor would be helpful for us to catch it. Out of the ones diagnosed who had breathing issues almost everyone had better day time control over breathing. The nighttime cpap and bipaps are helping to retrain the automic system at night and it carries over the day. I also know a handful of ppl who use their device during the day a few hours... My guess is that Hypopnea (reduced breathing) is more common then a full on Apnea (a stop in breathing).

The skin biopsy measures the autonomic nerves in your sweat gland of your skin. Its a quick pretty painless nick of your skin.

I have a friend who also has this really awful breathing problem and they started taking IVIG and it got much better. Thats why I'm sure is nerve/neurologically related. Actually in order to get the IVIG her pulmonologist did a diaphragm fluoroscopy test called a Sniff test. Ask your doctors for it. It's non invasive, not even contrast. You just breath while laying then standing, which sound perfect for us. Her doc diagnosed Motor Focal Neuropathy because of it, which is a stretch but its a cover reason to get IVIG. I also have a theory breathing machines like BiPaP and CPAP would benefit us because our autonomic system regulates sleep and it's like those machines would be training our autonomic system while we sleep. So it's like subconscious training. I think the part where our system forgets to breath for us is like an awake apnea. I don't think we could call it anxiety, but it's like the nerves aren't sending signals to our brain to breath because another signal is blocking it. Sure anxiety is one type of signal, but I think any overriding over sensory signal will do the same thing. I also noticed my GF getting a weird breathing issue as me. Possibly our rhythms clocks are getting shared. Why can't I get the good one she had though :/

Thanks for the intro Foggy i have a pretty big poll on here for breathing problems, what helps and what makes it worse. You're not alone. It's my worst symptom. I'm on disability because of it. So far I learned I have Sjogren's syndrome which destroys my small fiber autonomic nerves and causes pots. So it stands to reason that those autonomic nerves are also destroyed in the lungs and are impairing breathing. Usually neurologist are focused on neuromuscular diaphragm large fiber nerves. There may also be some involvement with large fiber diaphragm nerves too but I thimk it's mainly the autonomic ones giving us the most grief. I still have to get an EMG, but most pots ppl I know test normal On their EMG. The other test I want is called a Sniff test and it supposed to test for Motor focal neuropathy in the lungs. i know someone who got IVIG after an abnormal sniff test. Private message me if you have other questions about breathing issues. Rich

Welcome! I used to wear 30-40 compression socks but switched a year ago to CEP quad sleeves. Same thing in my opinion and much easier to wear and less embarassing

Dr. chemalli is certainly one of our best pots doctors. He is really embrassing the skin biopies among other testing.

@issie- i have no Dopamine but high NE. @Anna- if what Freeman hypothesises were true then my BP wouldnt be so wacky.

Well having one of either of the two Sjogren's antibodies is the most definitive. If you are negative for them like I was, then they do three other test. They do a salivary scan, eye test and biopsies. There are 2 types of eye tests; one for tear production and the other for dry spots. They have two types of salivary scans. And usually they do both or either a lip biopsy or a parotid biopsy. Out of these 3 types of test the biopsy is the most definitive. So I would say many ppl classified as AAG actually are seronegative other autoimmune disease. In a study Mayo published about the outcome of 6 AAG patients half were seronegative AAG antibodies, yet the same therapy helped those patient who were negative and Mayo hypothesized that they probably have some other underlying autoimmune disease. What I don't understand is why other autoimmune cause neuropathies isn't FDA approved for the same treatments. Even AAG isnt approved for IG and Plasma yet..

Thnx Corina!

I have zero Dopamine, or at least it came up to low calculate. And 2 other ppl here told me they had the same. My NE was 634 after 10 min standing, bet it would be at least double or triple if I went 30 min.

I had dry mouth but I always chalked it up to pots because I never had it before my pots flared up bad. And My eyes aren't dry at all during except in the morning when I wake up. I still have to go for a salivary scan and eye testing to see how bad the damage is..