Sarah4444

I was surprised by the lack of sensitivity when I read that for the first time as well. I also read a review of the article that Dr. Raj wrote, in which he diplomatically pointed out that the suggestion of the name Grinch Syndrome could be hurtful to some patients as it could be perceived as trivializing our illness. Has anyone out there met Dr. Raj or had him as their doc? From his writing about POTS he seems to be a very compassionate and thoughtful person. I suspect that sue is right, and that since POTS seems to have multiple causes, exercise will be more completely helpful to some patients than others. These articles always make me feel like I am being blamed for having this illness, while I know in reality I struggled against it for a long time, finally succumbing and becoming deconditioned. But the illness definitely preceded the deconditioning. I worry sometimes that docs who know nothing about the complexity of POTS could be mislead by this kind of thinking.

I have also had the feeling like I have a ball wedged in my throat. I have wondered if this might be related to esophageal inflammation, which apparently is common in mast cell disorders. It has improved since I began the meds listed below, but is still annoying when I lie on my back.

There is a lot of info about the connection at the Ehlers Danlos National Foundation (www.ednf.org). Julie and I have been meeting more and more people who have HDCTs like EDS, hyperadrenergic POTS and a mast cell disorder. I don't think it's a simple as EDS causes POTS, although there are some theories about hypermobility leading to mechanical problems that could in theory cause autonomic dysfunction. At this point I'd say that EDS is frequently associated with POTS, but the causes haven't been worked out yet. I suspect that they'll find out mast cells play a role somehow.

Hi there, and welcome. I just wanted to mention that my feet seem to be falling too, according to the HDCT/EDS specialist I saw, Dr. Francomano. And apparently there are over 200 different hereditary disorders of connective tissue, with EDS being only one of them - she suspects many people have collagen defects/HDCTs that haven't been diagnosed or even named yet. Also, after not having an infection for as long as I can remember, I too got my first cold sore and another infection at the same time this month, right after my period. There's something weird going on with my immune system, but don't know what yet. Hope you continue to improve.

Welcome, and I hope posting here helps you and your daughter. We all know how scary this can get - it must be so awful to be worrying about your child with this.

Talk about a teaser. My cousin is a librarian, I'll ask him if he can find it for me without breaking any laws.

It is quite an amazing feeling, isn't it, to read that article and realize finally there are words for what we are going through. I was diagnosed with hyper POTS through tilt table test with catecholamine levels, up here in Canada. But even my autonomic doc was pretty unfamiliar with the MCAD concept - I was lucky enough to find a dermatologist who was willing to try the meds with me (which helped a lot), but I am still looking for someone who can help me put the pieces together. I can't remember if I suggested this before, but try to watch the "Mast Cell Activation" videos on youtube made by Dr. Castells and her colleagues. You might be able to get the meds though your family doc and get some symptoms relief while you wait to see Dr. Gribb. Also I find the Mastocytosis Society Canada a great place for support and info (you don't have to be Canadian). I wish you well, and let us know what happens. Sorry I can't be more helpful.

I also have odd salivary gland behaviour, but more like Naomi's. If I am hungry or think about food I have noticed that saliva sometimes actually squirts out of my mouth if I open it. But I also have very dry skin and hair.

Issie, what paper are you referring to, about the way we are treated by doctors? Who wouldn't publish it?

I wish I could tell you I have found how to make doctors understand how severe and distressing this is, but I haven't. My mother is a physician and she and my husband came with me to see some specialists; it helped with one doc who knew her, but another treated me with a level of contempt that astonished her. I think there are many problems, the main being that they have no idea what we're talking about so it sounds like we are exaggerating our symptoms. It's a catch 22 - if you tell them how bad it really is they think you're hysterical, but if you minimize the symptoms to be socially acceptable they don't get how serious it is. I have had the most luck with female physicians and with geneticits but that may just be my situation. All I can say is keep trying. I have kind of built a treatment plan for myself that has at least alleviated the worst of the suffering for now, and I am lucky at least that my family doc listens to what I have learned and is willing to let me try meds and to make referrals. I still wish he would actually read things I bring him and try to understand, but the genetic counselor I see is doing that so at least I am able to talk things over with someone. I think the level of indifference and unkindness that many of us have encountered in the medical system is incredibly unfair, counterproductive, dangerous, and borders on malpractice. On the Mastocytosis Society Canada website there's an article about the preferential treatment of male masto patients (and the terrible discrimination faced by female ones). I think this applies to POTS too, with its high female preponderance. It's sad.

Thanks for the info, Julie, and I am so glad to hear you are doing better. It does sort of feel like salt loading should be at most a short term fix while they look for safer and more sustainable ways to help symptoms over the long term. It's kind of depressing being a senior citizen in the POTS world at 41, but I am glad that others like you are figuring some of this out for us. I wonder if it would be worth letting Dr. Grubb and those at Vanderbilt know your experience - since POTS is such a new diagnosis they probably don't know what to expect for us as we age, and maybe telling them what we experience can help them sort this out and start looking into the issue?

You don't sound dense at all, Victoria. I had never heard of MCAD either until Julie from this site told me about it since my symptoms were similar to hers. MCAD is Mast Cell Activation Disorder, and it's a confusing disorder that is only being identified and sorted out. Some doctors seem to think it's actually undiagnosed Indolent Systemic Mastocytosis, while others think it's a disorder of mast cells that degranulate too easily. Start by Googling the article "Hyperadrenergic POTS in Mast Cell Activation Disorder" (Shibao et al, 2005) and if any of that seems to match your symptoms, take a look at www.tmsforacure.org to learn more about MCAD. There is also a good youtube video called "Mast Cell Activation", made by several mast cell doctors. I have joined the Mastocytosis Society of Canada and there is alot of good info there too (and a thread for those of us with overlapping POTS and other disorders). Just let me know if you need more info - for me, a combination of POTS and MCAD treatments seems to be the best I have come up with so far (but still working on it!).

Hi Victoria- I just want to welcome you and tell you how sorry I am to hear how poorly you have been treated on this difficult path. It's one thing to not diagnose and help patients appropriately, but so much worse to tell them it's all in their head or suggest they are making things up. Have you looked into the hyperadrenergic POTS/MCAD angle? I am still trying to figure things out, but know that I also felt critically ill a lot of the time before starting the MCAD meds. I still have ongoing, frustrating trouble with orthostatic intolerance, but at least I don't feel like I am dying all the time. I am Canadian so I can't help you with a medical referral, but hope you can find someone to help you soon. Please let us know what you find out, and I hope you start to feel at least a bit better soon.

They do not recommend beta blockers if you have mast cell activation, as apparently they can make things worse. That may be what is happening with you. I took metoprolol briefly (just a tiny dose) and didn't feel much better or worse. From what I can gather, blood pressure can be all over the place with this version of POTS. I am doing better now that I take MCAD meds, but am still frustrated by my level of disability. I have gone from being bedridden, only being able to huddle to the bathroom and back, to being able to drive short distances, walk around the house and do short activities. But I still can't stand long enough to cook an elaborate meal, tidy my house up, that kind of thing. I may just have to accept this, but would love more improvement of course. If you follow these threads you'll see how others are doing - at this point it seems like we are trying to learn together and get healthier and more functional.

Kellie- So glad you found us. It sure sounds to me like you have POTS. Since you are also having all the flushing and high blood pressure, you may have hyperadrenergic POTS. There is an article you should read and give your doctor called "Hyperadrenergic POTS in Mast Cell Activation Disorder". Try googling it, and if you can't find it let me know and I can try to send it to you (for some reason links don't always work when I post them here). This is what I have, but I don't have the high blood pressure, mine tends to be too low most of the time. Be prepared though, for the fact that many doctors don't know much about POTS (which you already found out, it seems). Try not to get too frustrated but keep pressing for help. I hope that your current doctors look after you well, and that you start to feel better soon- Sarah

It's so interesting to see that study. Before starting doxepin at night, I would wake every day at 4 AM with this awful falling sensation, major tachydardia, flushing, shortness of breath, awful malaise... As sue says, it would be nice to know why this might be happening, but it's somewhat comforting to know that it happens to others too.

Hope- I am sorry that your son and you are going through this. I also have lots of pain as part of my medical situation, and started having difficulty in my teens. However, I did go through a kind of partial remission period in my mid 20s-mid 30s. It is hard to predict what is going to happen, but I have hope that now that doctors are beginning to label and study these disorders, children your son's age should have a bright future. It seems that mast cell activation plays a significant role in this for me (if it isn't the root cause) and wondered if your son had any symptoms of it? Treating it with medications has helped reduce my suffering, and avoiding gluten seems to help my with my cognitive difficulties. I really hope things get better soon.

I don't know the mechanism, but I had a lot of heart pain when I was extremely sick last summer, and it is one thing that is much better now. I have read reports of masto patients having chest pain, so I am not sure what is happening in us. Hopefully yours will get better with treatment.

Hi Marie- From what I can learn so far, MCAD is still being defined by doctors and is difficult to diagnose. Some doctors seem to think it is actually indolent systemic mastocytosis that isn't being picked up because testing is inadequate at this point. Others think it is different from mastocytosis, in that it involves mast cells that degranulate too easily instead of over-proliferating. There isn't an immunologist where I live, so my learning and diagnostic processes have been slow. I have seen a dermatologist but didn't have positive skin biopsy or tryptase. The next step would be urine methylhistamine or PGD2 testing, but these aren't available where I live. They are now looking into whether I can get approval from my health care system to see Dr. Castells in Boston. Doctors seem to know even less about MCAD than they do about POTS. Take a look at tmsforacure.org and the Canadian Mastocytosis Society and see if you think you fit, and then start trying to find a doctor who will help you figure this out. Good luck.

Sami, I am so sorry you are going through this. I did not know it at the time, but I also had POTS while in university. I know exactly what you mean about trying until you destroy yourself; I changed my academic and career paths many times, went part-time for two years, ended up doing a master's degree while lying on a lawn chair and have only been able to work a few years part time. I was so worn out by school that I had to choose between working and having kids - I didn't know why, but knew my body was going downhill. I have two great kids and am so glad I made the choice I did, but really miss having a career (and an income!!). When I finished school and wasn't working, I did better as I was able to rest when I needed and could manage my time with more flexibility. I can't tell you what to do or even what I would have done differently, but I know what you're going through. Try to reduce your load and simplify as much as possible, but if you can't manage, maybe taking a semester to try to recover a bit might help. If your body is telling you that it's had all it can take, no matter how much it ***** to do it, you may just have to change plans. Can you take any courses online or anything? Just be careful - I pushed through so much that I don't think I'll ever bounce back. You're having to make such difficult decisions at this stage of your life. I really hope that with the fast pace of research and medical discovery, they'll be able to help you (and all of us) soon. Good luck.

Twenty five or so years, and I think I am up to 46 doctors. Part of my problem is that symptoms developed over time, would come and go, and I learned to ignore and accomodate for them until I literally couldn't anymore. The whole situation was also confused and obscured by chronic pain. When I had tried to explain my symptoms to doctors in the past I got nowhere, so I kind of gave up. I finally started to figure it out for myself, thanks to this site, and went on to get officially diagnosed. Still a work in progress, though.

Dr. Francomano told me that people with EDS and other collagen defects seem, in her opinion, to be more likely to get autoimmune diseases.

Tia- Sorry to hear you are having such a miserable time. I get hives in response to heat and stress, and I flush after I eat. The severity of these reactions seems much worse when my POTS is bad (or vice versa?), and everything gets worse for me pre-menstrually. I wish I understood how this all connects (I am currently trying to arrange to see Dr. Castells). In the meantime, my severe itching and other symptoms (diarrhea attacks, weird sleep episodes, etc.) are much improved on the masto meds she recommends on her youtube video - but POTS is still a big problem. Hope you find some relief soon.

I have wondered about this too, as my parents have some similar symptoms to mine. Some things, like joint pain that runs in the family, can be explained by the hereditary connective tissue disorder. Dr. Francomano's theory about how autoimmune illnesses seem far more common in people with HDCTs, perhaps because of leaky maternal/fetal barriers in pregnancy seems to suggest a mechanism by which autoimmune antibodies could also be passed from mother to child. However, I wonder if something like getting a particular strain of a virus could explain clusters of illness?

Thank you so much Julie. The minute I start to think I am understanding this a bit, I just get more confused again.