Sarah4444

I would also love to see pictures! I have been noticing all kinds of different "freckles" appearing on my skin and wondering about them. The dermatologist I saw said they are freckles, but I am not convinced. I have gotten used to my skin over the decades and for the last few years it has been changing. I have wondered about "Diffuse Cutaneous Mastocytosis" - I can't find any pictures of it but it's described at its worst as being like orange peel, but in milder forms as a "freckle-like rash". How do you tell the difference between that and all the old freckles? My skin biopsy indicated some mast cells but the pathologist said they "did not appear to be increased in numbers" and he recommended another biopsy, which the dermatologist didn't follow through on. I have a feeling that might be pathologist code for "I have no standard of comparison having never seen this before and am just making a guess here". I would love to see some photos of "freckles" that have turned out to be masto, and of some confirmed Diffuse Cutaneous Mastocytosis, if anyone can direct me to them. And don't worry even if you are in denial Jared, I sort of am too. But we don't have a choice about whatever we have, and it's better to be on the right track. I think I need a second opinion from a dermatologist, but I am already seeing the ONE in Canada who sees CM. Wondering about a trip to Florida...we did have a snowstorm here yesterday

Aack! You'd better tell a doctor about this, reduce your dose...something. At least you know it works for you. Keep us posted.

Hi Jared - I just wanted to add that I am sorry to hear you have this, but very glad you are on your way to having a better understanding of what is going on. Julie gives great advice. I would only add that mast cell stabilizers have been very helpful to me as well. Hopefully as more doctors see patients like you, they will realize that POTS can be a presenting form of mastocytosis. Hang in there, and please keep us posted as you learn. Sarah

I also have a lot of trouble with smells - particularly fragrances in laundry products, but others as well. My reactions tend to be more the headache/dizziness/feel awful kind. Victoria, it might be worth seeing if you can try mast cell stabilizing meds like Nalcrom/Gastrocrom. It helps me a lot with GI stuff I think. It's an old med that's considered very safe and it's what Dr. Castells recommended I increase since I still have some gastro issues (she also added Ketotifen which I just started - another anti-histamine/mast cell stabilizer). With regard to whether these things run in families and how POTS and MCAD interact, check out the POTS thread on MSC - it appears to be complicated, but Sue's post of the NIH study looking into hereditary mast cell issues suggests at least some people are thinking this way: http://clinicalstudies.info.nih.gov/detail/A_2009-I-0086.html

This is really interesting. I also have vitiligo on my leg - in the reseach trial in the first post they mention depigmentation of skin and hair as being related to mast cell disorder. With regard to finding a doctor for this, I don't even have an allergist or immunologist in my community but from what I have read on the forums they often don't know much about mast cell disorders, MCAD in particular. On the POTS thread on the Mastocytosis Canada forum there are names of some of the mast cell experts, and you could also ask at a US forum. It's still a poorly defined and controversial area.

I used to have lots of trouble falling asleep and feeling wired, only to wake at around 3:30 every AM with falling BP/a terrible sense of malaise/diarrhea/tachycardia/shortness of breath (I think now these were the MCAD anaphylactoid episodes). This went on for so long I thought I was going to go nuts from lack of sleep. I got immediate and significant improvement after starting doxepin 20 mg before bed. I still have some episodes of insomnia, but nothing like before.

Chaos - That is a really interesting observation. My JHS diagnosis was made by Dr. Clair Francomano, but I am not "true" EDS, instead belong to a group of people she sees whose HDCT isn't named yet but has features of EDS (joint hypermobility but not in extremities like fingers and toes, and very soft skin) and Stickler Syndrome. It's so much fun trying to explain this to doctors. It would be simpler for me to just say EDS, but I'm interested to know if there might be others out there like me. Also, what is the profile for low flow POTS? How don't you fit it? Do you have MCAD symptoms? I keep wondering if maybe the docs are looking at the same problems and calling it different things.

Just to make things more complicated, as I am learning about MCAD I have met several people with various forms of mastocytosis/MCAD who feel that gluten is one of their "triggers" for mast cell degranulation/anaphylactic-type symptoms.

You just have to go here and the first topic tells you who to email to register and the details: http://cmsadmin.proboards.com/index.cgi

C- I was in a car accident in 1987 and sustained a spinal injury. Many of my odd health problems since then were blamed on chronic pain, even by me, and the rest I just ignored as best I could. I also got mono a few months after the car accident and I think it kicked my illness up a notch or two, but again this got all mixed up with the injuries from the accident. A couple of years ago I realized that something else/bad was going on along with the pain (and mixed up with it too). It has been an utterly exhausting journey and incredibly stressful at times, but having an idea what is going on really helps psychologically at least. With regard to flying, I would strongly recommend compression stockings. I have tried them to help with POTS and I don't find them very helpful, but when I have flown over the last couple of years (before I knew this was POTS) I have used the drugstore variety and found them helpful. I would hope the prescription/full leg ones would help even more. And stay hydrated.

Sarah, Can you post the site, so we can look at it? This video and also what you posted on the Mastocytosis site? Hi issie - Here is the video: With regard to my post it's super long and has an attachment - you don't have to pay to join MSC, just sign up (see explanation in my other post). Let me know if you'd like me to email it to you.

How frustrating for you with the mast cell triggers. I will definitely check out the summary you posted on the Mastocyosis Canada site. It's probably time I looked into this as a possible mechanism causing my POTS. It's just taken me so long to even find a doctor willing to treat my POTS, let alone find anyone willing to look into possible underlying mechanisms. All the best with the meds and I hope they continue to have a positive effect and you continue to improve. Thanks so much for the kind thoughts. I do find I do much better with midodrine and MCAD meds that either alone, and am seeing slow, steady improvement (interspersed with anaphyactic episodes!). Definitely look into the whole MCAD thing - if it seems to fit with your experience you can do a trial of basic over the counter meds and then wait for your doctors to catch up! Let me know if I can help in any way.

Hi issie - It's really long and has an even longer attachment. I can't figure out how to add attachments on this site, or even if that's possible (I'm far from a computer genius). But if you go to the Mastocytosis Society Canada forum site, there is a link for people with POTS, Connective Tissue disorders, etc. and it's there. If you have trouble, just PM me and I could email it to you.

Hi there, and welcome. It's my impression that many people do actually get better, while some others don't - I think most of us are in between, getting better to some degree and learning to manage and adapt to our symptoms. From what I have read, the sooner POTS is diagnosed the better in terms of prognosis. In my case I think I have had POTS at least since mid to late adolescence (I'm now 41 and was diagnosed last year). By last summer I was completely bedridden/crawling to the bathroom and back. I am definitely better than that now, but still nowhere near normal. I think the best we can hope for for now is to get well enough to exercise (and some really unlucky folks can't even get there, no matter how hard they try or how desperately they want to), either with fluid loading/salt and if necessary with medications and then see how well we can do if we can gradually regain muscle tone and stamina. I have gone from bedridden with chronic anaphylaxis, to now being able to manage 20 min a day of light swimming and walking. I still can't stand still for long (or even sit still for long some times) and my symptoms are still exacerbated by eating, stress, exercising too vigorously, etc. but at least I am better than I was. I still spend most of my time reclining, sitting on my couch with my legs pulled up, and lying down. My problem now is that it's so variable - one day I'm back to barely being able to get out of bed (and terrified this will last), and the next day I can manage a short dinner out with my family.

Can someone tell me if it is abnormal to have ANY antibodies to gluten? I had the bloodwork done for Celiac disease (anti-gliadin and anti-something else I forget) and I had some antibodies to both even though I had been avoiding gluten for several months by then. But the results showed that the anti-bodies were in the reference range so it said "negative" for both. Are there some people out there with no gluten antibodies? Would the fact that I had some even when trying to avoid gluten suggest that I might have abnormally high levels when eating gluten? I haven't had the energy to look into this whole area yet, am just avoiding gluten - any info would be appreciated.

Welcome, and as Jana says, it looks like you have come to the right place. I just wanted to warn you that you might still be in for some challenging times depending on how lucky you get with you doctors, but at least now you can start to explain your symptoms to yourself and not be so frustrated. I can't describe the relief I felt when I first came here and as I learned more about what was happening to me, but I think I then expected doctors to just understand too. POTS and all it's accompanying disorders are still poorly understood by most doctors so read a lot and be prepared to be patient and to explain a lot. I find it sometimes helps to bring studies in for doctors to look at. Some won't take the time, but for those who do, it starts to built their ability to help you. While anyone can relate to things like pain and fatigue, I think we don't realize sometimes how different we are and how hard it is for healthy people to conceptualize what it feels like to have many of the symptoms arising from chronic orthostatic intolerance.

Thank you so much for that info, Chaos and rama. Very interesting. I'd bet at this point that perhaps both progesterone and angiotensin II might be factors. PGD2 levels are often abnormally high in mast cell disorders too. My pain levels as well as POTS cycle with my menstrual phases.

Hi lotusflower- No, I haven't tried Florinef again yet. I am trying to get the MCAD stuff under control to see how much relief I get from POTS, especially as I can exercise more without going into anaphylaxis each time. I'm on 7 meds right now (7 more than I am comfortable with) but slowly I seem to be improving. I have severe variability in my symptoms though, one day thinking I'm doing better then suddenly way worse again - never sure why (I think it's these stupid mast cell triggers and I haven't got them figured out yet.) Hope you are managing ok - you might want to check out the summary I have posted on the Mastocytosis Canada site - I am trying to politely badger a few doctors into learning what is going on with us and I wrote an overview of who and how it's going. I know you are far away but they are very kind and willing to talk to docs in other countries - it's finding the docs in our countries that is the biggest challenge I think.

Hi Victoria- Julie has been at this longer and is better at answering questions I find, but I'm happy to give my two cents. While my othostatic intolerance is obviously worse when I am in a vertical position, like you, I find that my anaphylactic-type symptoms seem to worsen when I am lying down. When my catecholamines were measured at my TTT, my NE and epinephrine/adrenaline levels are low/normal when supine and sky high after 10 min tilted. This makes me think that when vertical, they high adrenaline works kind of like a natural epi-pen, blocking some of the anaphylactic symptoms. However, if the Shibao et al article is correct, the high catecholamine levels/sympathetic over-activity when standing may also be causing mast cell degranulation, making the ongoing anaphylaxis worse (adding to the trigger "bucket" that fills throughout the day for me) and then when I lie down, my NE/epinephrine go down, allowing the anaphylaxis to "break through" at least until my body somehow adapts (I often have to take extra Benedryl to help it out). I have no idea if this is what is actually happening, it just kind of makes sense to me. I also find it intreresting that Cholnergic Urticaria is thought maybe to be caused by a similar mechanism - there is an article about that on MSC. I think the fatigue has many sources, but chronic cerebral hypoxia can't help, nor can being in constant or frequent anaphylaxis. Also, in one of Dr. Grubb's articles he explains that it takes something like three times more energy for us than normal people just to stand up, so there are a lot of things sapping our enegy. My fatigue has improved with proper treatment of my hypothyroidism. I wanted to mention that on the Mastocytosis Society Canada forum, we have started a thread particularly for people with mast cell disorder, POTS and some kind of HDCT like EDS. It's not the same as here where there are many people to ask things on a daily basis, but more a place where we post what's going on for us and keep track of each other and our medical discoveries, particularly as they relate to the three overlapping diagnoses. You can set it so you are notified if someone replies to that particular thread, and just check in at the site from time to time. Anyhow, you (or anyone else with this MCAD version of POTS) can find it under "Mastocytosis and Mast Cell Disorders" - I think the thread is called "POTS, Connective Tissue Disoders, Nervous System..." or something close to that. I just mention it because over the past couple of weeks I have been in contact with several MCAD, EDS and POTS docs and I was going to post a summary of what I have learned there, if you are interested in reading it.

When I took Florinef it seemed to worsen my MCAD/anaphylactoid episodes. I also ended up in the ER, with tachycardia, swallowing problems, shortness of breath, itching - I may have been flushing but didn't know about MCAD yet and didn't know to look for it. I also developed a frustrating repetitive dry cough at that time. There is a video from a British TV program on the Mastocytosis Society Canada website of a girl having an anaphylactic event due to Mastocytosis and when I watched it, I instantly remembered the cough I had had on Florinef. (I had the cough all the time then, but the Florinef seemed to make it worse for some reason.)

Sensitivity to fragrances is commonly discussed on mast cell disorder forums. I have it as well, not as bad as yours. I have to avoid any scented detergents, fabric softeners, etc. It is tied into dysautonomic stuff somehow for me.

Naomi - I don't have cutaneous mastocytosis (I don't think). Usually CM refers to Urticaria Pigmentosa or something called TMEP and these people sometimes just have skin involvement, but often have systemic symptoms as well. I have dermatographism and Cholinergic Urticaria (if you write on my skin the writing turns into hives, and I get flushing from heat, stress, emotions). Apparently these are often seen in people with various mast cell disorders. With regard to how it presents, as Julie says it is extremely variable - some people seem to be fine or have relatively minor symptoms and then have acute anaphylactoid episodes when they encounter a trigger (like a bee sting), others have chronic miserable symptoms and still others seem to have both. songcanary - if you are trying www.mastocytosis.ca the website is still under construction, but google "mastocytosis Canada" and you should be able to find the forum, which is currently active. You have to sign up but payment is voluntary, so you can look around for a while and only join if you will be staying on in the future and you want to contribute/can afford it. I find there is a nice bunch of people there.

This is such an important question and I am tackling it myself right now. If you have high tryptase you are likely to have more luck with "regular" mast cell doctors - usually immunologists/allergists, hematologists, and dermatologists for those with skin involvement. However you have to see a doctor who is really familiar with mastocytosis, not one who just thinks s/he is. If your tryptase is low, you really need one of the mastocytosis experts, like Dr. Castells or Dr. Afrin. They understand MCAD (aka MCAS)and can order and perform the testing properly. For POTS/MCAD the group at Vanderbilt perform a four hour collection of urine after a severe flushing episode to look at methylhistamine levels (see the article called "Hyperadrenergic POTS in Mast Cell Activation Disorder"). Dr. Castells recommended a 24 hour methlyhistamine and PGD2 urine test. But the tests, along with any skin biopsies all have to be performed in particular ways or they will easily produce false negative results. The urine has to be chilled and handled according to a particular protocol, and for the skin and bone marrow biopsies for mast cell disease there are particular staining techniques - you also need a pathologist who knows what s/he is looking for, even if all the other procedures are done right. I have a dermatologist up here who is familiar with Cutaneous Mastocytosis but not MCAD. It's almost worse to have a doctor who is close but not right on, because they will do preliminary tests for tryptase but then if it's low, conclude inaccurately that you don't have MCAD. Then you have to bring in all kinds of research to prove otherwise, and doctors just LOVE that. Luckily my doc is nice and willing to put up with me, but I spoke with Dr. Castells this week and she said I really need to be seen at a specialized centre like hers in Boston, so we are going to try to make that work - that will be a fun health insurance adventure.

songcanary - I have had two tryptase tests and both were low. Because of that, I was also told masto was not the problem, but since then in personal communications with Dr. Afrin and Dr. Castells I have been told that low or normal tryptase is common in MCAD, not an indicator of its absense. Go to the Mastocytosis Society of Canada and take a look at the articles about diagnosing and treating MCAD that were published in 2010 and 2011 - both say high tryptase is not a necessary diagnostic criterion. The problem is that most doctors don't know this yet.

Thank goodness for Julie. After reading your post I remembered that I also have had the extreme sleepiness and uncontrollable shaking with episodes. But what I keep forgetting are the feelings - I also get the impending doom during one of the 9.9 kinds of episodes, and when they are less severe but ongoing, it's more like extreme malaise or panic. I just had several days of the chronic uneasiness/anxiety and I have to keep telling myself that nothing has really changed, that this will pass...and today I got really fed up, took some extra Atarax and ibuprofen, and I feel like a different person. Same problems, but much less worry about them. This is so weird. And I am so interested in the bee stings. I was stung by a bee in 2001 (and I stepped in a wasp nest as a child and had countless stings). After the 2001 episode the stinger was imbedded in my leg until about 2005. It was during that time that I started to have episodes of extreme vomiting/D - I thought I kept getting food poisoning, but now suspect it was anaphylaxis. It's like the sting kicked things up a notch. Also, I love the Canadian Mastocytosis forum for accessing really good info and studies, but there aren't too many regular users - can you recommend any other forums where questions might be answered more quickly? I have joined the wallack one, but wondered if there are others. And I love the duck.