Mcad And Anaphylaxis + Ecg

[Victoria]

I've been trying to read about MCAD and am now wondering this: What are symptoms of anaphylaxis as it pertains to MCAD? Are symptoms different for everyone? Does a person always flush when experiencing anaphylaxis? Can anyone share their anaphylaxis symptoms with me?

I've had some frightening symptoms through the years, but anaphylaxis wasn't considered, so I'm not sure about this even though I respond to H-1 and H-2 blockers.

Can anyone clarify for me?

BTW, I saw the EP cardiologist Friday and he ordered an ECG. This time I had only borderline T Wave abnormalities with sinus rhythm (my T Waves are usually inverted). He said the ECG was unchanged from previous tracing, but this facility didn't even have a previous ECG to compare it to. Oh, well.

The cardio said he didn't see any signs of an autonomic problem (big sigh but no worries because he doesn't specialize in the ANS). Also, I just got done wearing my 48-hr. holter. At least this EP cardio deferred my care to Dr. Grubb.

[Sarah4444]

I can tell you what I think my anaphylactic symptoms are, but I get confused. I have so many symptoms, and there are so many things that overlap with people who have POTS but don't seems to have MCAD, so I'm not sure what's what.

When I was really bad, I had pretty much complete orthostatic intolerance, and my bp would collapse/heart race very quickly many times a day (even lying down) and every time I tried to stand. I think that this extreme version of POTS for me was what Julie calls "chronic low-grade anaphylaxis" (although it wasn't really low grade at that point, it was more chronic anaphylaxis), when my sympathetic nervous system just got so overloaded trying to keep my bp up. According to that Vanderbilt article, the excessive NE and NPY may cause mast cell degranulation, theoretically leading to this severe on-going vascular collapse.

Now that I am feeling better, I have times when I can be upright to some degree, but any standing still causes me difficulty - I can feel the blood drain out of my head, my legs get heavy, I can't think straight, etc. My guess is that the constant mast cell degranulation and release of things like histamine, over time causes such a strain on the sympathetic nervous system that it becomes POTS; what I think I mean is that chronic low-grade anaphylaxis eventually led my body to no longer be able to keep blood in my head (leading to all kinds of other symptoms due to cerebral hypoperfusion). The mast cell docs talk about syncope and shock, but I am not sure they are clear about how POTS may develop due to mast cell disease, and I think the chronic cerebral hypoperfusion is important for doctors to know about.

However, I now also get more clearly defined anaphylactic episodes. For instance, several days last week I felt well enough to walk briskly, and each night on those days I had episodes of dramatic flushing, tachycardia (supine), shortness of breath and difficulty swallowing - sometimes with itchiness and sudden diarrhea. It sort of feels like a dramatic and unpleasant amplification of what's going on in my body all the time, though. I have wondered if I tend to get these episodes at 11:30 PMish now because my midodrine has worn off and/or when I lie down, my NE levels go down. My catecholamine levels from my TTT showed that my NE and epinephrine/adrenaline levels are low when I am supine and incredibly high after 10 minutes tilted - it's almost like when I am standing I am getting a constant shot from an epi-pen, which dampens down any anaphylaxis that might be going on, but when I lie down and my NE and adrenaline decrease, the anaphylaxis "breaks though".

I have no idea if any of this is right, it's just my theory of what's happening. It sounds silly, but I think I am having some degree of anaphylaxis all the time. When I was very ill it was 7-8 out of 10 all day, with episodes of 9.9 at night (where I'd end up in the ER). Now it's more like 4-6 out of ten on and off during the day, with occasional 9/10 if I overdo it. Things that seem to trigger a worsening of my symptoms include standing too long (or even sitting too long), concentrating, heat/showering or rapid temp changes, eating, stress, alcohol, hormone changes (esp the first two days of my period) and exercise in a vertical position (and any combination of these). Some foods also may be contributing, especially gluten. And although the dramatic anaphylactic episodes tend to happen at night, the orthostatic intolerance is worst in the mornings.

Now this doesn't explain the fact that I also have a hereditary connective tissue disorder like EDS and that a large proportion of EDS people also have POTS. Either I have two sources of POTS, or it's all somehow the same thing for me. Also, I have things like pain, fatigue, brain fog, etc. that could be related to any or all of these issues.

I don't know if this makes sense, but hope it helps a bit.

[juliegee]

Great question! Sarah answered it valiantly. I think you'll find the most information from joining a mast cell disorder support group and asking that same question. I think you'll be amazed at the variability. some present with severe abdominal pain, "D" or vomiting. Others experience the throat closing/tightening without skin symptoms. Others get hot, break out in hives and get panicky. It varies. Rarely, does an MCAD patient have all of the symptoms at the exact same time.

Docs tend to think that you will break out in full body hives and/or flush and then your throat closes. That textbook presentation has NOT been the case for me- although I have chronic urticaria (that responds well to my MCAD meds) and I sometimes do break out in full body hives (in response to something I've eaten that I am NOT allergic to) and I do flush occasionally- (more before I was medicated.) Additionally, there are many times when my throat feels tight, like it is closing and my voice even gets hoarse....BUT the two (skin stuff & throat closing) don't necessarily happen at the same time. For me, the signs of full-blown anaphylaxis are a feeling of impending doom. I get a buzzing in my ears. My vision dims. My HR goes crazy, 180-200 BPM even though I am sitting down. My BP rises, then eventually drops. I usually have to run to the bathroom for "D." The whites of my eyes turn bright red .If I can control things with meds (antihistamines), I shake uncontrollably while I recover. I am freezing cold. My hands and feet turn dark blue/purple. Once I stabilize, I can do nothing but sleep at that point. Sometimes this is preceded by a coughing or sneezing attack, a feeling of numbness or tingling on my lips or throat.

I go weeks, months, even years with "low grade anaphylaxis" where my POTS and NMH symptoms are out of control. If I were to grocery shop, I would experience pre-syncope many times and lose my cart often (cognitive craziness!) My throat and chest would be intermittently tight. Sometimes breathing in hurts. (Atrovent helps with this.) Smells, stress, pistachios, bee stings could et this off. BTW, I am not allergic to pistachios or bees via skin & RAST testing. The trigger constantly changes- frustrating as you can't avoid it

It is important to note that there are NO reliable laboratory markers for anaphylaxis. A high serum trypase used to be the gold standard, but docs are realizing that trypase doesn't rise in all patients with anaphylaxis. High methylhistamine, serum tryptase, or prostaglandin D-2 are all indicative of anaphylaxis or MCAD. But, it is incredibly difficult to catch a high level as mast cells release and degranulate very quickly often leaving no known 100 % reliable laboratory markers. You HAVE a high test caught. That will speed your DX.

I have used my epi-pen several times and it was terrifying as I am never sure I am having anaphylaxis. The first time, my HR was so high and all of the counter maneuvers my doc had taught me, like bearing down (like you are about to defecate) or massaging my carotid artery were NOT working. I was about to lose consciousness and knew instinctively I was about to die. My husband was with me. He stuck the epi-pen in my thigh and only held it in for 5 seconds vs. the prescribed 10 as we wanted to gauge my response. It immediately stopped all of my symptoms....with no negative side effects. Keep in mind, an epi-pen is the WRONG treatment for tachycardia if it is NOT caused by anaphylaxis making this a very difficult treatment decision.

You have to get to know your body & recognize your signs. If you can reverse them with antihistamines, it's probably anaphylaxis. If you get more stable, less symptomatic with MCAD meds; the duck is walking If your symptoms reverse with an epi-pen; that duck is talking. I realize that this probably sound scary and was before I figured it out. But, with meds, I am amazingly stable. I take daily meds & keep extra antihistamines and en epi-pen with me at all times. At this point, I'm incredibly grateful to have figured out what's causing my symptoms and to have some control over them.

Keep researching & asking questions. The more you know, the more control you will have over this craziness.

Hugs-

Julie

[Sarah4444]

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.

[songcanary]

I couldn't agree more about Julie. I learn more from her at one sitting than with all my doctors put together!

I just got tested for tryptase and it is low normal. So my allergist says there is no support for mastocytosis. I still can't help but wonder about MCAD. I have some intermittent vague anaphylaxsis type symptoms, too. Oh well, back to the drawing board.

[Sarah4444]

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.

[HopeSprings]

OK, this is where my brain fog and cognitive issues get the best of me. I am trying to understand all that was just written and I can't seem to get it into my head... and I really want to because I suspect MCAD in myself.

Julie - how did you get the MCAD diagnosis and where?

Sarah - does the fact that you have cutaneous mastocytosis make you more likely to have systemic mastocytosis? How was the skin form diagnosed in you?

Thanks

[songcanary]

Sarah4,

Thank you for that! I tried to get into the site long ago as well as today, but apparently they are still having an upgrade? My allergist is always really on top of things but I want to research this as much as I can before driving him more nuts. You made me feel better. I was actually disappointed in the low tryptase level, it would have explained so much.

[HopeSprings]

One more question. Is MCAD episodic? Is it a set of symptoms that appear -very obviously- and only after a specific trigger?

[Sarah4444]

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.

[Victoria]

Thank you so much Julie and Sarah! You've given me a lot of great information and I sincerely appreciate it. I'd have come back sooner but my laptop keeps overheating and shutting down. I've learned to keep the poor baby on an ice pack while I'm using it.

I'm intimately familiar with all of the symptoms you mentioned, Julie. They don't crop up at the same time, but sometimes in twos or threes. I have watery diarrhea when I'm at my worst and have lost a lot of weight from it over and over again. I was put on creon for suspected pancreatitis (elevated pancreatic enzymes and pain), but the creon didn't help. The croaky voice is another symptom that comes on rapidly and can last for a week or longer. Blocked airway is another. Tachycardia in the 200s. Sense of impending doom. Flat-out panic. The tinnitis and buzzing in my ears can be so loud I feel like I have locusts living in my brain. I also get itchy, but only at rest. Another one (strange as it sounds) is a sense of something crawling on my skin, through my hair and in my ears. This only occurs when I'm very still or lying down. Is norepinephrine is high when I'm on my feet or when I'm lying down? What causes the symptoms when I'm at rest?

I saw an article about MCAD and it mentioned increased dopamine. This just can't be anything else since I'm responding well to the H-1 and H-2 blockers. Also, I have EDS (hypermobile type with blonde hair, blue eyes), a small heart, mitral valve prolapse, and those are all red flags.

The brain fog is killer. I had neuropsychiatric/IQ testing when I was having a good day and my IQ was in the 88th percentile — normal bright range. I had the same testing at Mayo and I could barely hold myself up in a sitting position that day. Anyway, my IQ at that time was only in the 9th percentile! I was considered profoundly intellectually impaired! I came back home and my IQ was immediately tested again and it was back up to the 88th percentile.

I'm wondering about my high eosinophils? I'm thinking they may be increased with mast cell activation? How about something that resembles hyperplasia? I had a vaginal hemorrhage at age 26 that required a D&C. The pathologist said if I was older he would definitely refer to it as hyperplasia, but because of my young age, didn't want to use that term. I've heard I have hyperplasia before, but I can't recall right now what it pertains to.

I can relate to your experience with the epi, Julie. I have an epi pen here, but I'm afraid to use it because of the tachy and anxiety that goes along with it. Gosh, what if I made it worse? Zoiks! I think my heart or brain would explode. Steroids do the same thing to me, but I sure don't understand it. My doc just prescribed Flonase and although it helps with my constant "allergic rhinitis," it makes my heart feel like it's working harder, I feel a tad bit more jittery and anxious, and I'm having more trouble sleeping at night.

And what's up with the overwhelming fatigue and lethargy? What is the hypothesis? Or do we not all feel that way?

You guys are awesome and I'm learning so much from you. I don't feel nearly as frightened now that I've read your replies.

[Sarah4444]

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.

[issie]

I'm interested in reading it Sarah. Please post it.

[Sarah4444]

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.

[Victoria]

Thank you for sharing, Sarah. What you said makes sense and seems to also apply to me. Dr. Grubb's info about taking three times as much energy as an unaffected person? My heart and body feel like they're in frequent state of marathon running or cardio exercise.

Cerebral hypoperfusion seems the only logical explanation for my brain fog and the huge drop in my IQ.

I joined the mastocytosis Canada forum. Thanks also for that info!

[juliegee]

Sarah's collection of information is ground breaking. She is also collecting people with this triad of disorders and MAKING researchers aware of us. You go, girl (after a nap ) I want to help all I can. Tell me what I can do.

Quack, quack-

Julie