Hi. I have MCAD. I have recently been also dx with chronic non-alcoholic pancreatitis. My GI doc questions whether mast cell disease and its subsequent inflammatory state is the culprit. Any thoughts?

Due to the fact that the broad themes of the effects of the more than 200 mediators produced by the mast cell are inflammatory, allergic, and tissue growth/development effects, and given that mast cells are present in every organ/tissue/system in the body, it’s not surprising that mast cell activation syndrome (MCAS) is a disease of chronic multisystem unwellness of general themes of inflammation, allergic-type phenomena, and abnormalities (often, but not always, subtle) in growth and development in potentially any tissue(s). And I should emphasize the “multi” in “multisystem,” so certainly the gastrointestinal system can be involved (indeed, the GI system is very commonly involved, in one fashion or another, in mast cell disease), and though the pancreas is less commonly involved in mast cell disease than other parts of the GI system, it’s certainly not magically immune from being involved.

The medical suffix “-itis” means “inflammation,” so your pancreatitis indeed means you have inflammation of the pancreas, and nobody should be surprised if a patient with MCAS were to be found to have inflammation of the pancreas or, for that matter, any other organ/tissue/system in the body. To be sure, pancreatitis (fortunately) is one of the less commonly found problems in MCAS, but it’s certainly a possible consequence of MCAS. Also to be sure, there’s nothing about having MCAS that makes it impossible to develop any other disease, and there are many diseases known which can cause inflammation in the pancreas, so if a patient is found to have pancreatitis, it’s important for the gastroenterologist to carefully evaluate the patient to look for any other causes of that disease – but it’s probably important for gastroenterologists to learn that mast cell disease, too, can be one such cause. If a patient presents with pancreatitis and has other symptoms and findings consistent with improper mast cell activation, and if diagnostic evidence of MCAS can be found and no other cause of pancreatitis can be found, then it’s quite reasonable, at least in my opinion, to ascribe the pancreatitis to the MCAS.

What actions should be taken when I have been diagnosed with Mast Cell Activation Disorder through urine and blood labs with rashes, hives, etc and my doctors are refusing to input the diagnosis into my medical chart under the list of diagnoses?

This question is one of the more distressingly common questions I receive, and I regret I have not been able to figure out any pat answer. I have observed there are some doctors who are willing to learn about diseases other than the diseases they were taught in their training, and there are some doctors who are not willing to do that – or at least are not willing to learn about diseases which they perceive as not having, or being unlikely to have, any impact in their particular areas of interest. And then there are the (fewer) doctors who seem more willing to “expand their horizons,” so to speak. I think it’s hard for any MCAS patient to make significant progress toward effectively controlling the disease without establishing a formal doctor-patient relationship with a relatively local physician (or other healthcare professional with prescribing authority) who is willing to learn at least a little bit about mast cell disease and willing to at least try to help the patient with it by prescribing trials of at least some of many different medications found helpful in various mast cell patients.

Fundamentally, the old proverb, “You can drag a horse to water, but you can’t make him drink” comes to mind. You can try to provide your less-than-ideally-helpful doctors with review articles from the medical literature which demonstrate the existence of the disease, its behavior, and the approaches for diagnosing and treating it, but most doctors are ridiculously busy and may not be able to easily find time to read (especially an article about a disease which they think has no intersection with any of the diseases they routinely diagnose and treat; keep in mind the only mast cell disease the medical profession knew about up until just recently, and the only mast cell disease doctors are taught in their training, is the rare disease of mastocytosis, which they’re taught has just allergic-type manifestations and which is the province of allergists and dermatologists and hematologists/oncologists because of its confinement (so they’ve been taught) to the skin and bone marrow) – though I’ll also acknowledge that it basically comes down to just how interested the doctor truly is in the matter, because if he’s interested “enough,” then somehow he will find time, even in spite of a busy schedule, to read the articles and learn about the disease and realize that your symptoms and your laboratory findings are consistent with the disease and satisfy present published diagnostic criteria, at which point it’s kind of nonsensical to not enter the diagnosis in your problem list, and nonsensical to not either try treating you for it or at least assist you with finding other professionals who will be willing to at least try treating you for it.

I’ll point out, too, that there’s just nothing to be gained by getting upset with any doctor who demonstrates that he’s not going to be as cooperative as you might like. Again, horses and water. You’re just not going to be able to force any doctor to help you manage a disease he doesn’t want to get involved with and doesn’t even want to learn about. Be polite at the initial encounter, and there’s nothing wrong with providing an educational resource or two (but certainly don’t hand him/her a big stack of literature, as that’s even less likely to be read than a single article), but if you get the sense that he/she is one of those doctors who just is not going to be willing to learn and willing to try, then politely take your leave and move on to try the next professional you can find who you have reason to think might be willing to learn and willing to try. It has been my experience that in most communities (at least in the U.S.), most patients with MCAS indeed are able to eventually identify some local physician who is willing to learn and willing to try, and I haven’t found that it’s doctors of any particular stripe (i.e., any particular specialty) who fit that bill better than any other stripe. It just comes down to how curious the doctor is and how willing he/she is to invest the (usually uncompensated, or at least grossly undercompensated) effort needed to learn.

I think there’s little risk – after all, there’s a reason these medications are available without prescription – in trying, at reasonable doses, the histamine H1 and H2 receptor blockers in patients who are suspected of having MCAS but who haven’t yet been “officially diagnosed” with this, but it’s my own style – and I’m well aware that different doctors have different styles and different approaches to this matter, and I’m certainly in no place to say any one approach is more or less “right” or “appropriate” than any other – to not have my patients with suspected MCAS pursue empiric therapeutic trials beyond the antihistamines until it becomes a definite diagnosis of MCAS.

I am on 10mgs of Loratadine 3 times a day, 150mgs of Ranitidine twice a day and 50mgs of Doxepin at night time for MCAS. I have excruciating Bone and Muscle pain 24/7 and I feel cold all the time even when the weather is hot outside. I was given Paracetamol for pain relief but it does nothing to ease it. I can’t take NSAID’s as I react to them too. I use heat packs to help ease the pain while at home, but if I am out I can’t take it with me. I am following Dr. Janice Joneja’s Low Histamine Diet and am really careful with Body products (I don’t wear makeup at all.) My home is a Scent-free home as I react to Perfumes and Scents as well as foods. What can you recommend for me to ask my Immunologist to ease the pain? Childbirth was a breeze compared to the pain I am living with every day.

I’m sorry to hear of your difficulties, but I hope you can appreciate that I can’t (responsibly/ethically/legally) provide personalized medical recommendations for patients with whom I’ve not established a formal doctor-patient relationship. For all I know, it might not even be MCAS that’s at the root of your pain and feeling cold; yes, MCAS can cause these problems, but there are many other conditions, too, which can cause the same problems. And even presuming that it truly is MCAS that’s at the root of your symptoms (i.e., that a thorough evaluation for other problems has been unrevealing and that appropriate laboratory diagnostic studies have proven you to have MCAS), the fact is that it’s (fortunately!) a very wide variety of drugs which have been found useful for various symptoms in various MCAS patients. For example, there are some MCAS patients for whom simple non-steroidal anti-inflammatory drugs (NSAIDs, even as simple as plain aspirin) can be very helpful for pain, while in other MCAS patients NSAIDs don’t help at all (indeed, in some MCAS patients NSAIDS even can be triggers) and instead it comes to be of any of a wide variety of other drugs which results in improvement in pain. Even if we presume MCAS is at the root of your pain and feeling cold, and even though it sounds like you’ve already got a pretty robust antihistamine regimen, that still leaves a very wide array of other drugs to be tried to gain better control over the disease and thus improve your symptoms, and I’m optimistic that you, as I see in most of my MCAS patients, can eventually find some MCAS-targeted “cocktail” which will get you to the goal of feeling significantly better than your pre-treatment baseline the majority of the time. That said, it’s one of my biggest regrets at present that we still don’t have any way to predict which of the many drugs found helpful in various MCAS patients is most likely help any one specific MCAS patient. Heck, we can’t even reliably predict which symptoms are most likely to be benefited from a particular drug across all MCAS patients. It’s easily the case that a particular drug may help symptom A and do nothing for symptom B in MCAS patient 1, and then in MCAS patient 2 the same drug will do nothing for symptom A and yet greatly help symptom B. The differences between the two patients aren’t “random” or “magic.” There obviously have to be real biological differences between the MCAS patient 1 has vs. the MCAS patient 2 has that makes for the difference in response to various treatments, but we remain terribly far from understanding the biology of this disease to the level of detail needed to figure out what those differences are – and to leverage that knowledge to predict effective treatment in the individual.

At present, lacking any studies to better inform my thinking, my recommendations as to which drugs ought to be tried for which symptoms in which patients often are based on my experience and on a wide variety of subtleties in the patient’s history. All I can offer you is to note that there are some articles beginning to emerge in the literature about MCAS and pain (e.g., https://www.ncbi.nlm.nih.gov/pubmed/28934791) which your immunologist ought to be able to easily find and read to get some ideas about how to treat you, and I’m also happy to offer that if your immunologist would like to discuss the details of your situation with me, I’d be happy to take his e-mail or call at our office. I can’t responsibly provide personalized medical recommendations directly to a patient I’ve never met, but any physician is free to informally consult with another physician regarding a patient he/she has questions about since it obviously remains entirely the questioning physician’s responsibility as to what he/she ultimately chooses to do to/for his/her patient. Thank you for your understanding, and I do sincerely wish you the best.

I’ve been trying to establish causality for a worker’s comp claim. The notion of primary MCAS would destroy my case, making my body responsible for my current condition rather than the prolonged malaria infection I acquired on my work trip to Uganda. I reached out to Dr. Heyman and he commented: “I think Afrin is only partially right in his work since some patients may have a primary MCAS, but others have had an immunologic trigger and the leaky mast cells are the epiphenomen. We can see this distinction in our transcriptomic assays.” That being shared, could you help me understand why you’re inclined to believe in a primary MCAS for everybody? (Or would I fall into a completely different category, given it’s likely CIRS is behind my MCAS, POTS, and CFS?)

First of all, to correct a misconception you’ve stated in your question, I certainly don’t “believe in a primary MCAS for everybody.” What I suspect – based on the most convincing preliminary research data on the subject I’ve yet found, from multiple peer-reviewed published studies conducted by Professor Gerhard Molderings and his colleagues at the University of Bonn’s Institute for Human Genetics – is that the great majority of patients who have MCAS per currently published diagnostic criteria likely have acquired mutations in one or more of the genes and other control elements in their mast cells which wind up causing those cells to both constitutively (i.e., at baseline) and reactively produce and release a bevy of mediators they wouldn’t ordinarily produce and release. In most (not all, but most) of the many MCAS patients I’ve seen, a careful history reveals an extent of problems which fit far better with a primary (i.e., mutation-rooted) MCAS than a secondary (i.e., purely reactive), let alone an “idiopathic” MCAS – but I’ll also be the first to admit that the genetic testing performed in the research lab by Dr. Molderings and his team to show that most MCAS patients have any of a wide variety of mutations in their mast cells is not available at present in any clinical laboratories and likely will not become available for at least several years to come, thus making it impossible at present (except in rare cases of so-called “monoclonal MCAS” featuring either the KIT-D816V mutation and/or flow cytometric findings of CD117 co-expression with either CD25 and/or CD2) to prove that any given case of MCAS is “primary” as opposed to “idiopathic.”

That all said, there’s no question that the natural behavior of MCAS is to demonstrate occasional marked escalations in baseline misbehavior of the dysfunctional mast cells, and when these escalations emerge, they’re usually permanent (which, I suspect, reflects development of additional mutations) and tend to shortly follow (by anywhere from a few days to at most a few months) a major stressor, whether it be a major physical stressor (e.g., surgery, trauma, puberty, pregnancy, childbirth, or a major immune system stressor such as a major infection or even – very occasionally – a vaccination) or a major psychological/emotional stressor. Furthermore, the inciting stressor may well resolve (whether spontaneously or with treatment), but if it provoked emergence of (or, more likely, provoked escalation of a pre-existing) MCAS, then the symptoms of that emerged/escalated MCAS are not going to similarly resolve (likely because mutations which develop and persist to the point of becoming clinically apparent rarely then spontaneously vanish).

As such, I don’t see a conflict with the situation you’re presenting. Yes, it could easily be the case that you had a pre-existing MCAS, which, as you put it, is “your body’s responsibility,” but if you then developed, in an occupationally related fashion, an infection (or other major stressor) which, even though subsequently cured/resolved, nevertheless permanently aggravated a (likely) pre-existing condition, then the permanent sequelae of that aggravation/escalation would seem to me to be an occupationally related issue. (To be clear, though, I certainly have no legal training or expertise and would never be confused with a disability or administrative law judge. I can only offer, with respect to the sort of situation you pose, what I think is common sense, and I’m well aware there’s often little relationship between common sense vs. laws and regulations.)

Finally, I’ll just note that if you’ve also been diagnosed with a “chronic inflammatory response syndrome” (CIRS), the question would remain what the source is of the elevated levels of inflammatory mediators which presumably have been found in you to support a diagnosis of CIRS, and since misbehaving mast cells are eminently capable of producing such mediators, and since mast cells also can produce mediators leading to vasodilation (and thus orthostasis and tachycardia) and fatigue, I would more suspect MCAS is at the root of your CIRS (and your postural orthostatic tachycardia syndrome (POTS) and your chronic fatigue syndrome (CFS) rather than the other way around.

Hopefully the technologies and techniques needed to more completely probe for primary MCAS (compared to the extremely limited means presently available) will become available in clinical laboratories “sooner rather than later,” at which point it ought to become much clearer who has primary MCAS (in which the mast cells and their mediators should be the primary therapeutic targets) vs. non-primary MCAS (in which other root cause(s) of the patient’s illness ought to be the primary therapeutic targets), but frankly, I’ll be (quite pleasantly!) surprised if that happens within the next decade.

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