Ask the Mast Cell Activation Syndrome Doctors: MCAS Questions Answered

Do you have any low salicylate suggestions?

Salicylates are a (sub)class of non-steroidal anti-inflammatory drugs (NSAIDs), and NSAIDs, though helpful in some MCAS patients, actually trigger activation of dysfunctional mast cells in other MCAS patients. Salicylates are available not only as drugs and supplements but also are commonly incorporated into foods and household products. Just as with “low-histamine diets,” there are a number of suggestions freely available from different sources as to “low-salicylate diets,” but I am unaware (as of this writing in April 2018) of any peer-reviewed, published studies demonstrating significant benefits specifically in MCAS patients from any particular low-salicylate diet, so the best I feel I can responsibly recommend at this time is that if the patient is interested in trying a particular low-salicylate diet, then it’s probably okay to try it (barring any other unusual aspect of the diet which the patient’s providers may recognize as risky for that patient) as long as that dietary change (1) is tried in isolation from any other changes in the patient’s regimen and (2) is abandoned if, after a month’s trial, the patient can’t honestly say to himself/herself that there’s obviously been a significant improvement from that change. I have found the website http://www.salicylatesensitivity.com to be a useful site for informing patients (in the Food Guide and Product Guide under the sites Information tab) as to the wide range of foods and household and personal hygiene products containing salicylates.

How is it possible to have a KIT mutation in the blood work but not in the bone marrow?

It indeed would be quite unusual to have a KIT mutation detected in peripheral blood but not in bone marrow, and anytime discrepancies arise between test results which one would expect to be consistent, the question arises whether one result or the other is in error (laboratory testing in 2018 is good but is far from perfect, and no test is immune from generating an erroneous result at least on occasion) or whether there are differences in the *methods* used to obtain the discrepant results. Certain methods for detecting KIT mutations are far more sensitive than others, so if a given patient had a low level of a KIT mutation, and if different methods for detecting that mutation were to be applied to blood and marrow samples, then it could easily be the case that one sample yields a positive result while the other sample confusingly yields a negative result. Furthermore, beyond the issue of a tests sensitivity for a *given* mutation, there is the fact that certain methods for detecting KIT mutations test for *different* KIT mutations than other methods, so it is easily possible that one method might be able to identify a particular mutation which another method simply cannot identify. The devil in explaining laboratory testing discrepancies such as posed in your question almost always lies in the details of the methods used to generate the discrepant results in the first place.

Does MCAD cause intracranial pressure? Would this be a possible explanation for constant headaches and ringing in ears? My hematologist diagnosed me with MCAD. What are treatments for intracranial pressure?

As of the time of this writing in April 2018, I am unaware of any peer-reviewed, published studies looking specifically at this phenomenon, but in my experience, yes, I have seen a number of cases of MCAD (much more often MCAS than mastocytosis, as one would expect given the suspected far greater prevalence of MCAS than mastocytosis) in whom, among their great many other problems consequential to the disease, there also was increased intracranial pressure leading to its own array of symptoms and findings, which can include headaches and ringing in the ears (though certainly there can be many other causes, too, of those symptoms, and it would be important for somebody suffering those symptoms to undergo a thorough evaluation for other causes). There are a variety of treatments for increased intracranial pressure ranging from medications such as acetazolamide and steroids to surgical placement of shunts for draining excessive cerebrospinal fluid. Treatment is highly individualized for the serious condition of increased intracranial pressure, and a specific treatment plan for this condition usually is crafted after careful consultation with specialists such as neurologists and neurosurgeons.

How does MCAS impact pregnancy and breastfeeding?

As of this writing in April 2018, there is very little information available in peer-reviewed, published studies regarding the impact of MCAS on pregnancy, let alone breastfeeding. In my experience, there seem to be higher rates of certain common complications in pregnancies in female MCAS patients than in women not known to have MCAS, including hyperemesis gravidarum (excessive nausea/vomiting), miscarriage (usually in the first trimester), pre-eclampsia, and premature labor/delivery, and though it has been my experience that more female MCAS patients are negatively affected by pregnancy than positively affected, I would be remiss in not mentioning that I also have seen occasional female MCAS patients who report one or more of their pregnancies to have been the one point in their lifetimes of chronic illness in which they felt remarkably well, with remissions in most or all of their symptoms. The biology of why different female MCAS patients respond differently to pregnancy (some affected negatively, some affected positively, and others seemingly not affected at all) has undergone virtually no research and simply is not understood at present. It should be recognized, too, that, pregnancy and delivery are major physical stressors, and regardless of how well (or not) any given pregnancy and delivery may proceed, it is possible for such major stressors to trigger a permanent escalation in baseline dysfunction of a female MCAS patient’s dysfunctional mast cells, leading to an overall worsened state of disease either during or soon after the pregnancy. Finally – and though, again, there have not yet been any peer-reviewed, published studies on this specific matter – in my experience there does seem to be a somewhat higher rate of breastfeeding complications in female MCAS patients compared to women not known to have MCAS, including insufficient milk production and mastitis (inflammation of the breast).

Do you find swelling around the eyes with hives (specifically lacrimal system swelling) common with MCAS? Glasses are even causing pressure, so I’m blind! I can’t find much on it anywhere. If so, what treatments would you recommend?

Inflammation in various structures in and around the eyes (including the lacrimal (tear) ducts) is common in MCAS. Most MCAS patients report “irritation” of one sort or another (often described as “dryness,” “sandiness,” “grittiness”) in their eyes. This irritation is more commonly felt than is so bad as to also be visually apparent to others (as with, for example, a “bloodshot” appearance in the sclerae (the whites of the eyes). Aside from “irritation,” actual swelling around the eyes to a degree noticeable by others is less common but certainly not rare; when swelling happens, it more commonly is episodic (sometimes shortly following distinctly identifiable triggers) than continuous. Many MCAS patients, too, report an “eye” problem of blepharospasm (spasms of the eyelids), which may be more of a neurologic problem than an actual eye problem. Although many MCAS patients also report occasional, usually brief episodes of deterioration of vision (typically a problem focusing vision), fairly few MCAS patients (in my experience) develop significant sustained problems with their vision. Although any “systemic” MCAS-targeted interventions (most commonly, orally ingested drugs which then are absorbed from the gastrointestinal tract and circulate throughout the body) have potential to help eye-related symptoms, certain mast-cell-targeted medications can be applied directly to the eyes, such as certain histamine H1 receptor blockers (for example, olopatadine and azelastine) and cromolyn and ketotifen. However, most commercially available formulations of medicated eyedrops (whether for mast cell disease or any other disease) are provided in multi-dose containers (bottles, vials, etc.) and thus must also contain preservatives to try to diminish contamination, once the container has been initially opened, by various microorganisms – and the dysfunctional mast cells in some MCAS patients are reactive to some of those preservatives (for example, benzalkonium chloride). Therefore, some MCAS patients sometimes need to go to extra efforts to obtain formulations of these eyedrops not containing additives to which they react. If eyeglasses are causing intolerable pressure on the surfaces of the nose, one wonders whether helpful solutions might include switching to less weighty lenses (many lenses these days can be made from much lighter plastic rather than heavier glass) or applying a bit of antihistamine (e.g., diphenhydramine) or cromolyn ointment to the affected surfaces of the nose – or perhaps even simply placing a soft barrier, such as a tiny piece of gauze, between the nose pads of the glasses and the skin. Contact lens (and/or the fluids associated with their use) provoke intolerable eye irritation in some MCAS patients and simply cannot be used in those patients.

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