Psalm 23

A number of years ago I tried low dose naltrexone as was recommended by my then Rheumatologist. He said it might help sleep ( which is no longer an issue), pain and fatigue. I gave it 6 months. Three months of 3mg then three months of 4.5mg. It did absolutely nothing for me. I'm glad some people are receiving some benefits from it. A few years after that he recommended I try Nuvigil. It caused severe insomnia and greatly increased my already significant flu-like feelings so that med only worsened everything. A number of years later after being diagnosed with mod-severe OSA my Pulmonologist recommended I try Provigil ( modafinil ). I told him about my bad experience with Nuvigil but he said he didn't think I would experience those problems with Provigil so I decided to gave it a go. Unfortunately I did suffer the same side effects. Only to a lesser degree. I later read something somewhere that people with mast cell issues generally had problems with meds like Nuvigil and Provigil. I then asked my Pulmonologist about trying Adderall which I had read some sleep apnea and pots patients were being prescribed. He was agreeable to that. So at this point how do I manage my fatigue; I take 5 mg of Adderall maybe two to three times a week. I never take more then the 5mg, I rarely take it past lunch time and I rarely take it two days in a row because while it does allow me to do more I have to expect I will require a lot more rest the day after. I suppose it's a bit of a push/crash effect. It's not a perfect approach but I'm desperate and I feel I need to do something to gain a greater degree of function at least some of the time. For me doing this is a bit of a risk because of the labile b/p. My Neurologist tells me to be very careful with taking this med and I'm afraid to tell my Cardiologist. On a side note have you ever had a sleep study done ? In thinking back to how long I complained of severe fatigue among other things I still can't believe how long it took for someone to finally recommend a sleep study. I was really surprised at the results. Consistent use of my CPAP has helped my fatigue maybe 10 percent. Not what I had hoped for but I'll take what I can get. Some people I have spoken with say using a CPAP changed their life. I'm still trying to be hopeful that at some point the Imuran/IVIG combination therapy will improve my fatigue. I know how isolating having a chronic debilitating condition can be. I'm sorry you're dealing with this situation as well.

Actually bruising easily is one of innumerable mast cell activation syndrome/disorder symptoms. When mast cells degranulate one of the many chemicals released is heparin which is a blood thinner. Many people with dysautonomia have a co-existing mast cell disorder. Definitely something worth being evaluated for as much of the treatment is OTC and for some can potentially eliminate or reduce the symptom severity. http://www.mastattack.org/2014/10/mcas-blood-bone-marrow-and-clotting/ This is basically a condensed down version of Dr. Afrins more comprehensive article. He has written a few books on the subject as well. If you google mast cell activation syndrome or disorder you'll find a lot of information on the subject.

Hello, Is your 2100 NE level the standing result from a supine /standing catacholamine test ? My results were 1236 supine and 2922 standing. The results were not influenced by any meds I was taking. I was told I had a hyperadrenergic response to standing. My b/p is high on ttt but labile as a general rule. Do you have a significant tremor when upright ? My initial diagnosis was neuropathic pots with a hyperadrenergic component. As time went on that diagnosis morphed into AAN. In my case the elevated b/p and NE level are compensatory mechanisms so salt ( 8 grams ) and 2.5 liters of fluid were recommended in addition to Clonidine and Mestinon. I have also benefited from an abdominal binder and compression hose which sadly I had to stop wearing as they resulted in burning skin. Because we are all so different with regard to diagnoses, possible reasons for abnormal test results and treatment recommendations your own doc should be the one interpreting your test results and making treatment recommendations. I hope you find the answers you need.

That's great that taking Imodium allows you to tolerate your Plaquenil. Bare in mind that if you were to take Imuran there is a lot of lab work required. One has to first have a Thiopurine methyltransferase test to determine whether or not you are even a candidate for taking Imuran. After starting the med there are weekly then every other week labs which eventually become monthly for the duration. Basically CBC with diff and liver function studies. What I call my low sensory overload threshold actually improved prior to starting the IVIG. It began to improve after starting on Clonidine and Mestinon and then improved more after starting on a mast cell cocktail. I think over time what has also helped has been my growing ability to block out noxious stimuli. I am still overly sensitive to motion and noise but in addition to med assistance my tolerance level is greater. Basically the passage of time has helped and also learning how to control my environment when possible. I'm sorry the stimulation sensitivity issue is so disabling for you. I know how horrible it is. Interestingly one med I was on that greatly exacerbated the problem was Lyrica. It took me being on it for a while before I realized what havoc it was creating for me. I'm not familiar with the use of transdermal scopolamine for stimulation sensitivity. Sounds like it's certainly worth a try. Like you fatigue is my most disabling resistant to treatment issue. I'm running out of treatment options. What have you tried for fatigue ? Yes. It is. So true. You're welcome. I wish I had something useful to offer up.

Hello p8d, I'm sorry my last post was not very clear. So I edited it. With regard to my b/p it is actually very labile with the exception of my ttt pressures which are very high so beyond the 10 minute standing time my b/p is all over the place. It does sound like we are very similar in that regard after all. I was mistakenly diagnosed with lupus by two different Rheumatologists and put on Plaquenil for 6 years which temporarily helped a number of different non-lupus symptoms which never made any sense until recently. Interestingly Plaquenil is now being used for POTS patients as well as for a number of other thought to be autoimmune conditions. If I don't realize any improvement from the Imuran I may go back to trying Plaquenil again. I actually had a choice of trying Imuran or retrying Plaquenil in conjunction with the IVIG. I chose the Imuran because I wanted a more aggressive approach. I'm sorry you are also dealing with debilitating fatigue.

Yes. It does help. Primarily with the ability to remain standing for much longer periods of time without developing jello legs, shaking, nausea, blurred vision, tachycardia, air sucked out of your lungs feeling and ultimately dizziness. Because I have an intact adrenergic function my b/p doesn't drop with a 10 minute ttt. Rather I have a hyperadrenergic response to standing so in my case we are hoping for a correction to my extremely high standing b/p and rest of the time very labile b/p which can range from 80s/40s to 200/116. I am sorry to say that thus far the IVIG has not helped my fatigue which has always been one of my worst issues. I will say my tolerance for external stimulation has improved over time. In addition to IVIG I am on Azathioprine ( Imuran ) in hopes of achieving greater symptom improvement. It's still too early to tell where this is all going to end up. I've heard others bring up Celltrend. It's peaked my curiosity. I have only had the Mayo panel done which repeatedly shows an elevated n-type calcium channel antibody. I think the general thought these days is that most POTS patients have an autoimmunity issue. I hope your Celltrend results help shed some light on your condition and provide you with some treatment direction.

Hello, I am currently receiving IVIG and am on Medicare but because I am receiving it for a Non - FDA approved diagnosis ( AAN ) Medicare will not cover it however my Aetna part D drug plan will. Go figure. It did of course have to go through an authorization process and it has to be done as a home infusion. The way it worked for me was my Neurologist sent IVIG orders to NuFactor ( specialty pharmacy ) who then submitted paperwork to Aetna who then approved it. NuFactor then contacted a local infusion company they had a contract with who then set up an infusion schedule with me. NuFactor ships all of the supplies and the Privigen to my home once a month which covers me for two infusions. I receive the IVIG every other week. There are only a small number of FDA approved diagnoses for which IVIG is supposed to be given so having it covered through Medicare unless you have one of the few diagnoses is virtually impossible. I really don't understand why the Medicare part D drug plan would cover it if Medicare itself won't but I don't question it I'm just very thankful it does. Best of wishes to you in your pursuit !

Hello and thank you Chaos. It's good to hear from you. Yes. It is very disappointing when a treatment either doesn't help or is not tolerated. How wonderful that your IVIG / Rituxan combination therapy is helping you. That must be pretty exciting to feel better then you have in a decade. Hopefully it's a result of the treatment and not related to a normal improved disease fluctuation state. I do realize it's a difficult balance between optimism and realism as one doesn't want to set ones self up for disappointment but yet is hopeful for improvement. I do hope as you continue on this combination therapy path your symptoms keep improving. At this point I am still tolerating the Imuran but am not seeing any further symptom improvement. It was really after 3 months or so of the IVIG that I realized significant symptom improvement in some areas but I haven't been able yet to move on from there. It's still early in the game though so I am hopeful. Please keep us posted on how you're doing ! Hello Sammy Jo and welcome to the forum. In answer to your question you would want to see a Neurologist that specializes in Dysautonomia / dysfunction of the autonomic nervous system. You would not want to see a Neurologist that is only familiar with the central nervous system which I dare say most of them are. This forum has a physician referral list that can guide you. With regard to any relevant labs the Neurologist would be the one to order them as well as all the other relevant diagnostic tests such as an autonomic reflex screen etc. Some lab work such as a paraneoplastic panel may not be available locally but the blood can be sent to a Mayo Clinic lab for processing. These are things to be discussed with the Neurologist you decide to see. Hope this info helps.

Hi Rich, That may not always be the rule though. I think it really depends upon the individual patient, the risk factors of adding in an additional potentially damaging medication and what experiences the Neurologist has had with combination therapies. The article you make mention of is from 2009 and involves a very small pool of patients. I should think that while more recent studies may not be published Mayo Neurologists would share relevant beneficial data with one another. I am a Mayo patient actually. Hi Kim, After three months of taking the Cellcept it had to be discontinued because of unacceptable side effects. Primarily persistent GI issues but I also had lesser issues with insomnia, headaches and swollen fingers. I'm sorry you had such a horrible experience with it. How frightening. Where you also taking it for an autoimmunity issue ? Last week I started taking Imuran. So far so good but that's what I said about the first week of taking the Cellcept. Any other AAN patients out there on an IVIG / Imuran combination therapy ?

Hi ANCY, I just wanted to to tell you how sorry I am at all you've been through and are continuing to go through. What a frightful situation to be sure. The only additional thing I can think to suggest trying are the Lidocaine 5 % prescription patches which I find immensely helpful for my back pain. I'll be praying for you my friend.

Hello ares22, Thank you so much for sharing ! I'm so sorry to hear that your symptoms have become so debilitating and your IVIG is not as effective as it had previously been. How disappointing. I've read about others experiencing the same thing which has caused me to wonder how long mine will continue to be helpful. I receive my IVIG every other week. I do notice it working less the farther away I am from the day of infusion. I can certainly understand your hesitation with taking Cellcept. It is by far the scariest medication I have ever been on. Thankfully thus far I seem to be tolerating it quite well but of course I have only just begun. The Cellcept starts out at 500mg twice a day for two weeks which will then increase to 1000mg twice a day so hopefully I will be able to tolerate the higher dose as well. Of course the lab results will weigh in heavily with regard to tolerance. Wednesday of next week will begin four weeks of weekly lab draws which then reduces to monthly. I don't believe this is meant to be a long term therapy. I am to return for reevaluation in June. It would seem with this condition that one can't think too far ahead about different treatments implemented and responses although some of the recorded IVIG or PLEX/Immunosuppressant combination therapy results are encouraging. Thank you. I sure hope you have success with the steroid therapy and don't have to go down the Cellcept path. Thank you for your support and being there !

Thank you corina !

With the exception of a one month hold which was required for ARS retesting I have been receiving IVIG for approximately one year. While I have realized significant symptom improvement in some areas in other areas there has been no improvement at all so combination therapy was put out there by my Neurologist as a treatment option. As of today I have started this more aggressive form of treatment and wonder if any other of you with AAN/AAG have gone this route. If so what was your experience like ? Did your condition improve ? Did you deal with any side effect issues from the Mycophenolate ? I have been very fortunate in that I have experienced minimal IVIG side effects so that part of the treatment has not been an issue for me. Thank you for any responses !

Hello, I'm sorry you've had such an issue with IVIG side effects. For some they can be so problematic. As was pointed out if your insurance approves it changing to a different brand can possibly help. I'm on Privigen. The rate starts at 30-45/hr and is not to exceed 75/hr but can be run less then the 50/hr if there is a tolerance issue. It did have to be stopped and restarted and the rate reduced a few times because of a transient hypertension issue. Per the Neurologist order I premedicate with Tylenol and Betamethasone. Normally Benadryl would also be given but because I am already on a large amount of antihistamines it's left out. When I first started the infusions I dealt with significant flu-like symptoms, lower back pain, chills, tachycardia etc. I can't say I have experienced difficulty breathing or chest. neck or head pressure though. I have found that Lidocaine 5% patches helps the lower back pain and Zofran helps the nausea although now I experience pretty insignificant side effects such as a stuffy nose, body vibration, slight tachycardia and sometimes my feet get a little red and swollen. I guess the way in which the IVIG is given depends largely on what it's being given for and your Neurologists preference. Mine began as once weekly for 6 weeks and then every other week. After about three/four months worth of infusions I began to notice significant symptom improvement. I'm receiving IVIG for autoimmune autonomic neuropathy. I do hope there is a solution. Best of wishes to you !

Thank you !!!