Mast Cell Activation Syndrome Q&A
What is recommended for frequent anaphylaxis if someone has Kounis syndrome and the person is having difficulty becoming stable?
It is not presently possible to predict which medications will be most likely to help which symptoms in which mast cell patients. Antihistamines, steroids, and benzodiazepines can be helpful in some flares of mast cell activation. Epinephrine has helped some mast cell patients with Kounis syndrome (and also some with severe hypertension), but the risks are apparent. Beyond use of emergency medications, the kind of patient described in this question needs to work diligently with his/her doctor in trying the many other medications found helpful in various MCAS patients to see what will gain sufficient control over the disease to reduce or hopefully even eliminate such life-threatening episodes.
I have MCAS, POTS, hypermobility, and probable mitochondrial myopathy, and I also have now developed inflammatory arthritis. Can MCAS cause an inflammatory arthritis? Are the high inflammatory markers just from mast cell involvement? My rheumatologist has started me on Simponi, and I previously was on Humira as my joints were swollen. I was diagnosed with seronegative rheumatoid arthritis. These are all serious drugs, and I'd rather not be on them.
MCAS fundamentally is a chronic multisystem disease of general themes of inflammation +/- allergic-type phenomena +/- abnormal growth/development in potentially any tissue. Also, "arthritis," by definition, is an inflammatory disease (the "itis" on the end of "arthritis" means "inflammation," and the “arthr” at the beginning of the word means “joints”). There is no non-inflammatory arthritis as distinguished from inflammatory arthritis. All arthritis is inflammatory, by definition. (There do exist non-inflammatory joint diseases, but the general word to refer to those conditions, since they don’t involve inflammation, would be arthropathy, not arthritis.) Therefore, yes, MCAS can be at the root of some cases of arthritis. Some MCAS patients manifest elevated "inflammatory markers" (for example, an erythrocyte sedimentation rate (ESR) or a C-reactive protein (CRP)), while other, equally symptomatic/inflamed MCAS patients show completely normal levels of certain inflammatory markers. We do not yet understand why certain markers are elevated in some patients but are normal in other patients despite seemingly similar types and degrees of symptoms. Finally, as previously noted, it is not presently possible to predict which medications will be most likely to help which symptoms in which mast cell patients. All that can be done presently to try to address any of the symptoms afflicting an MCAS patient (whether arthritis or any other symptom) is to identify and avoid triggers and then to patiently, persistently, and methodically try each of the many drugs already known to be helpful in various MCAS patients. Once a mast-cell-targeted drug which is effective for a given mast-cell-activation-driven symptom is found, it is possible that other drugs which previously were more “superficially” treating that symptom may not be needed any longer, but patients should always take care to consult with their prescribing physicians before stopping, or even adjusting the doses, of any medications already in their regimens.
Does taking antihistamine drugs daily put you at a greater risk of memory loss as you age?
Not that we know of. Long term use of H1 blockers has been *associated* with increased risk of developing dementia, but that is a long, long way from saying that long term use of H1 blockers *causes* dementia. Perhaps it's "simply" the case that MCAS can cause a wide variety of neurologic problems, including dementia, and who makes long term use of H1 blockers? MCAS patients do -- therefore demonstrating an "association" between H1 blockers and dementia without saying *anything* about whether it's the H1 blockers, more so than the disease, which is *causing* the dementia seen in *some* of those patients. Again, it's a very complex disease, and we still have a very long way to go (as in, many *decades* of research to be done) before we understand it completely.
My daughter has Monoclonal Mast Cell Activation Syndrome (MMCAS). Her platelets go up and down like a merry-go-round. She also is anemic. Why are platelets affected in only some patients, and how does this happen?
Unfortunately, we don't know the answers to these questions. Most MCAS patients ("monoclonal" or not) have normal blood cell counts most of the time, but blood cell counts can be abnormal on occasion, or even often, in some MCAS patients. Some have high red blood cell counts. Some have low red cell counts. Some have high white cell counts, some have low white cell counts. And some have high platelet counts, while others have low platelet counts. And in some of these patients with various blood count abnormalities, the counts often vary a good bit from one determination to the next -- all for reasons we just don't understand yet. I must caution that having a mast cell disease (or any other disease, for that matter) does not make the patient immune to developing any other disease, so before one goes assuming that any problem in an MCAS patient -- whether an abnormal blood cell count or any other problem -- is necessarily due to the mast cell disease, it is critical that the patient undergo appropriate evaluation for that problem, and in an appropriately timely fashion. Who knows? Perhaps the anemia is due to iron deficiency due to heavy menstrual bleeding -- a problem which can, in some patients, be ultimately attributable to MCAS, but still, if iron deficiency is found, then iron deficiency needs to be treated with iron supplementation if at all possible.
For those of us with neuropsychiatric symptoms, what do we do? What is the best treatment for mast cells degranulating in the brain? Many of us are at the end of our ropes with anxiety, depression, brain fog, etc. I’ve also seen quite a few mast cell patients, who, like me, experience ‘derealization,’ which is a terrifying feeling. What is the best treatment for insomnia which does not respond to benzodiazepines or hypnotics?
Those patients definitively diagnosed with MCAS who have neuropsychiatric symptoms should do what MCAS patients with other types of symptoms should do: first make sure (as much as is reasonably achievable, anyway) that those symptoms are not due to some other specific nervous system disorder, and then keep trying various mast-cell-targeted therapies until the therapeutic goal is reached: feeling significantly better than the pre-treatment baseline the majority of the time. Presently it’s not possible to predict which drugs or other interventions are most likely to help which symptoms in which patients. All that can be done is (1) keep trying various therapies until the goal is reached, and (2) keep conducting research into what makes this disease tick until such trial-and-error is no longer needed.
What if doctors don't understand about the benzos and they are pressured to not prescribe them? What then? I'm in a location where medical practice seems less than state-of-the-art.
If a doctor chooses not to be helpful to an MCAS patient (such as not prescribing certain drugs for a disease rooted in a cell bearing inhibitory receptors for those drugs), then the MCAS patient should politely find a different, more helpful doctor. I understand that's often not easy, but I don't know a better solution. There's no point in getting upset with the doctor who chooses not to help; getting upset won't help the situation in the slightest. It must be remembered that awareness of the existence of MCAS is so new that it remains the case at present, a decade after the first case reports of the disease were published, that virtually no physicians are yet aware of it, much less truly have a good understanding of it, and if *you* bore *responsibility* for treating a disease in which you had received no training, then you, too, would probably have qualms about it. It's not in any medical school textbooks yet, it's not in any medical school curricula yet, and even today there are virtually no doctors coming out of any post-graduate training programs who have even heard of it (much less have actual experience with it). Virtually all of the few doctors who are aware of MCAS have learned about it on their own (i.e., were not *taught* about it) and -- quite understandably -- have a tenuous understanding of how to even recognize the *possibility* that the patient might have this very complex and highly variable disease, much less how to actually diagnose or treat it. This poor state of awareness and education will of course improve in the coming decades -- the disease is so prevalent that it will have to improve in the coming decades -- but for now it has to be accepted by most patients that while they absolutely need a local physician to be their principal partner for dealing with the disease, they should not *expect* to find a local physician who is *already familiar* with it, and therefore what they truly need is a local physician who is willing to learn about it and willing to at least try to help the patient deal with it. Willing to learn, willing to try. That's what the typical MCAS patient should be looking for in the local doctor who's going to be his/her principal partner for dealing with this. Again, I know it's often not easy finding such a physician, but in my experience, such physicians do exist in most communities. They may be few and far between, but they do exist. And it will be tremendously helpful for MCAS patients to politely, respectfully help in the education of those physicians, for it has been my experience that each health care professional who comes to recognize the disease in his/her first one or two patients diagnosed with it inevitably soon begins recognizing it in many others. It's a good thing, of course, when a health care professional can help one patient with a disease, but it's obviously a far greater thing when the professional can help many patients with a disease.
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