Anne Maitland - Living with Mast Cell Activation Syndrome

okay good morning so I have the pleasure talking about a cell that has really been kind of like the Rodney Dangerfield of the body for like a hundred years and really has come to the forefront just within the past I would say ten years which means that the rest of the medical community will figure it out in another ten years but many of us are living with with massive mediated disorders it's just a question of whether or not it's mass all activation syndrome and so we're going to talk a little bit about what mass all activation syndrome is and then how you can get it under control so you had to ask yourself do you have MassHealth activation syndrome and this kind of tells you all the different types of symptoms that can happen because mast cells are actually living in every single organ whether it's your skin your gut your brain your spleen your joints and if they are inappropriately activated it's kind of like calling the police and the SWAT team into your neighborhood because you have a cat in the tree so there are lots of mass cell mediated disorders it's anticipated if you're living in an urban or industrialized country one out of two of us is experiencing some type of muscle activation issues whether or not it's nasal congestion and you have asthma or asthma-like symptoms IBS and actually neurocognitive impairment and and what's even more startling a report just about two years ago identified that at least one out of 50 of us have had an anaphylactic event so why are we seeing such a huge rise in these hypersensitivity issues a lot of it has to do with the fact that we have dramatically changed our environment within the past 20 years so we're gonna say you know life before plastic and life after plastic we spend more much more time indoors we have less control over the food we eat we haven't even less control over the air we breathe or the substances that land on our skin and this has really caused the genes that we've inherited from our ancestors to be completely confused borrowing this from the the multiple chemical system multiple chemical sensitivity practitioners basically you have individuals that are susceptible to insults from the environment whether you're talking infectious the most one that's governing attention right now is is Lyme and how that has actually opened the door to a chronic hypersensitivity disorder you might have seen the top the article in New York Times where they talked about meat allergy so these individuals when they get they develop Lyme and then they end up with a delayed hypersensitivity reactions so here you have an infection that opens the door to a chronic hypersensitivity disorder and then upon reintroduction of other different types of triggers basically your immune system just does not stand down so we've noted that in the allergy immunology community that anyone young or old and now we're starting to see adults and seniors or seasoned as I like to call myself inside of my AARP card are now susceptible to having mass or mediated inappropriate activation impacting different types of cell systems um and unfortunately a lot of these individuals end up having delayed diagnosis because internationally we have a shortage of allergy immunology specialists and just in this country alone we have seventy seven training programs out of the seventy-seven training programs only three do research on mast cells so we have a lot of individuals who are just not trained on how to recognize and then diagnose and treat these Massell mediated disorders and because there's such a delayed diagnosis partly because patients have a tendency to kind of focus on individual symptoms so you end up going to that particular specialist general practitioners are ill-prepared to appreciate immune mediated disorders because they got one lecture on mast cells twenty years ago and so a lot of these individuals end up you know the tests are normal or the test Renata ordered and so because it's not measured it doesn't exist so you have individuals that really end up being sick and then practitioners have a tendency to kind of be if you're lucky they just are dismissive and sometimes they actually can be quite harmful when dismissing and actually started doing treatments that are inappropriate and actually cause more damages art this is not unusual for a lot of individuals that have massive immediate disorders to have comorbid psychiatric illnesses partly from the the disease itself and also partly because of the lack of responses from the medical community so the best way to kind of start learning how to take care of math cell disorders is to ask yourself do you have a muscle activation syndrome and here are just some examples of patients that have come through our practice and typically you have to have at least two organ systems that are impacted that will suggest that mast cells are just regulated so you can have skin and gut involvement you can have nasal congestion and headache syndromes you know it's that combination that will say okay should we start looking at seeing whether or not mast cells and misbehaving or not and then there are three criteria that have been put out and we're still working on this but essentially three questions do you have signs of symptoms worsen form a cell's misbehaving do you have medications such as over-the-counter antihistamines benadryl claritin Allegra is there tech zantac taga med pepcid or prescription medications such as Chrome one or singulair and you show modest improvement of your symptoms that suggested that you might have a mass i'll activation syndrome and then what data do we have to support that your mast cells are misbehaving and I'll talk a little bit about the roadblocks for each one of these criteria but I want to just go into a little bit more detail about each one so it's gonna take a core des native team so you really need to identify a practitioner who's willing and able to work with you will not dismiss your symptoms won't say that this is you know somatic disorder conversion disorder or you know it's just it's just allergies and then coordinate this other specialists that are going to be involved in your care because more than likely you're gonna need gastroenterology and neurology psychiatry dermatology urology because again if mast cells are misbehaving they really can cause problems in all of those organ systems so the first question is do you have mast cell activation syndrome or god MCATs and then once you identify somebody I'm hoping that this talk will provide you with some questions that you can work with with your practitioner to try to navigate the very convoluted complicated maze of the health care system in order to find out whether you have mast cell and then figure out what medications or treatments can be helpful to you besides just avoidance which I have to tell you tolerance is not free so you really need in order to restore tolerance to what you eat what you breathe wood lands on your skin it's really important to kind of reduce the stress of your body so then you can then develop tolerance to these exposures even temperature so I kind of borrowed this from the asthma world where it's important to step up to your next checkup and basically you want to screen for symptoms or signs that are worse than for maskull's misbehaving see whatnot you have any risk factors it's not unusual I can't tell you how many parents bring their kids in and they're going like this or you know they telling me that they're bloating I had one mother actually take over the appointment of her daughter and the daughter pushed back this is my appointment mom but typically this will run in the generations as well and then you also have to kind of look at what kind of risk factors you have even in the environment that you grew up in then I'm going to go into some of the tests that are available unfortunately we have very few commercial tests available to identify mass cell dysregulation but even with those tests we might be able to figure out whether or not your mass cells are misbehaving or not well and then the next idea is to kind of just educate yourself about this condition and how it can interact with other organ systems so it's not unusual especially in the alleged aliens community that if you have massive dysregulation it's very possible that you're also going to have a connective tissue disorder and also a neuropathy so it's not unusual for patients that come for an evaluation through our practice that we do a referral to a neurologist who's knowledgeable about peripheral neuropathies a gastroenterologist who who's aware of the fact that mast cells are major contributor to irritable bowel syndrome and we also have input from otolaryngologist as well because getting that upper airway under control is key for sleep disorders headache disorders TMJ and also just reducing and also being able to obtain tissue to try to get the diagnosis of massal activation as well so it is a patient practitioner partnership where you want to get the best working diagnosis you want to try to maintain normal function as much as possible you want to optimize therapy whether you're talking about prescription medications over-the-counter medications adjuvant interventions such as chiropractors a functional neurologists acupuncture and I know I'm missing a wonderful lecture right now about medical cannabis but there's a role for all of these treatments depending on what's causing your mast cells to be overactive and then the goal is really if you'd have you know eat well sleep rest be able to exert yourself and maintain function as best as possible and then after all these things you need to ask yourself am i satisfied with the care that I have it's the relationship that I have with the practitioner helpful so here's the checklist do you have symptoms and signs worsen form a cells misbehaving release to organ systems do you respond to medications that target micel Massell mediators and then what test results do we have to support that math cells are just regulated so this is just another way of viewing the fact that these are the various organ systems and the manifestations of Masum dysregulation that can cause gastrointestinal symptoms neurocognitive impairment actually if you look at patients that have a much more common or well-known form of mass cell activation disorder which is mastocytosis these individuals will present to a practice either with skin symptoms or gastrointestinal symptoms but the third most common complaint is an anaphylaxis or Vern itis there actually is neurocognitive issues mood disorders difficulty concentrating difficulty thinking short-term memory remember see deficits anxiety depression and so it's really important to understand that and we need to kind of develop short you know short screens for general practitioners to try to evaluate whether individuals are having neurocognitive issues as well as opposed to just prescribing a SSRI or medication and ignoring the rest of the symptoms and getting an accurate diagnosis so to kind of give you an idea of all the different factors that are released by ourselves depending on what organ system they're in - necrosis factor platelet activating factor these are all things that can influence how your blood cells are working how your brain is working it can cause joint inflammation and gastrointestinal distress and a lot of the treatments are actually going after the factors that get elucidated or don't get elucidated when the mast cells are triggered so when it comes to measuring massal activation factors like I said there's very few commercial tests available essentially we have urine tests twenty-five-year in collections which are not that much fun to collect and then once you collect them you have to hope that the technician doesn't leave it on the table while they're getting a cup of coffee and then shipping it off properly to Mayo or interest Arab in California we have blood tests histamine as well strip taste many of you may have had the trip taste measured and they say that that's normal that's not the way to approach it you have to treat mass activation syndrome like an individual we check blood tests for individuals that are having heart attacks you want to get a baseline and then if you have an acute event you want to check the trip taste within four to six hours of a Qun event and if it goes up two points plus 20% that is diagnostic from a cell activation syndrome and then to be honest the best test is actually going into the tissue where the mass cells are potentially causing the problems so understand that mast cells are not blood-borne they are only found in the tissue so it's important to kind of get a biopsy so if you had an endoscopy or a colonoscopy to let the gastroenterologist know that the pathologist should be standing for the mast cells they should count them there to see whether or not they're round or spindle shaped and then they should also see whether or not they're clustered or hat or have up regulated activation markers saying that these cells are just not standing down and then as far as treatments the the most of the studies that have done have been performed have focused on histamine blockers but understand histamine is just one of the factors that gets released by these cells and at some time on some occasions history doesn't get released at all so we have histamine blockers of which we have the first that work on the first histamine receptor like benadryl and zyrtec we have those that work on the second histamine receptor which are classically known as heartburn medications such as pepcid and zantac we have medicines that are more known for asthma such as singulair and accolade and Zhai flow we have mass L stabilizers such as kota-kun crumblin as well as just went out of my head I'm Elizabeth and I'm Elizabeth was actually interesting it was the first biologic that was used to treat asthma back in 2003 it's now indicated for asthma and chronic hives I had one patient I wanted to prescribe this medication to you and she didn't have asthma she was mostly complaining of GI distress and headaches and I asked her if she inches and she said not it doesn't bother me I'm like that wasn't the question I just need to know whether you itch or not and once and I said if you itch more than you know a couple of times a week for the past few weeks that is actually indication to prescribe this medication and see whether or not it be helpful for their symptoms or not unfortunately when it comes to having different organ systems involved we have tendency to kind of go to various practitioners who have a tendency to focus on their a little corner of the sandbox and that's why another reason why individuals that have massive dysregulation end up having very delayed diagnosis the average time to diagnosis for some of these and many disorders can be up to a decade so one of the first roadblocks really is the fact that we have a tendency to have general practitioners who that have been poorly educated on the immune system and then we also have practitioners who really just kind of focus on their area and say this is IBS or this is this is anxiety or this is a sleep disorder and and because none of the no one's talking to each other no one's coordinating the care individuals end up having coping with these symptoms and and more than likely get progressively worse just because of the lack of intervention or recognition of those symptoms the second diagnosed second delayed diagnosis is a lot of practitioners just don't know how to use the tests that are available for identifying individuals that have massa activation disorder I'll share the story of a patient that I had been taken care of for about three years and she had chronic hives and her tryptase was two or three that I repeatedly checked and she had been dealing with IBS for twenty years and failed to tell me that until I saw that she was taking medication in linz s on the electronic medical record we went ahead and did a endoscopy what she hadn't had in 10 years because there's been no change of her symptoms and she had more than 90 mass cells per hide power field so it really is important to kind of revisit and try to go at a different angle to see whether or not you can identify whether or not someone has overactive mast cells or they have too many mast cells that may be contributing to their symptoms so the the the other thing I want to point out is individuals that normally have a normal trip taste it may be indicative that the sensor that is causing the mast cell to the overactive does not require trip days for instance we have individuals who have food induced anaphylaxis to peanut and I can check a trip taste all day long and it'll never be elevated doesn't mean that this person doesn't have anaphylaxis or food induced allergies it just means that there are other receptors that might because in the mast cells to contribute to that reaction and this is again emphasizing it's really important to kind of look at what what chemicals might be released that would be suggested that mass cells are overactive so this is just an illustration that if you have antibodies against pollen or mold or bacteria you may see trip tanks or histamine released but there are set of receptors that a lot of practitioners are not aware of called toll receptors and if you engage if the mast cell sees a bacteria or virus - the toll receptor they'll try to control that infection through other things other than histamine and tryptase so again it's really important to kind of look at the blood on different occasions look at the urine and different occasions and then biopsy the organ whether it's the gut the nose the skin to see whether or not the mast cells are overactive or not and then the last the last criteria whether or not you respond to medications or not really has to kind of focus on the quality of the medications and whether or not the mast cells are being called into action appropriately or not so this is the back of a benadryl bottle and you can see there are lots of different ingredients in there back in the 1970s the FDA approved even preservatives and fillers to be added to baby food so it can sit on the shelf for a year so it's not unusual for several the patients that I care for that they get their medications compounded in order to reduce exposure to soy and corn and milk that's added as a binder agent to a lot of these food medications that are either over-the-counter are prescribed and also to kind of just reduce their exposure to coloring as well I'm still shocked that benadryl is as violently pink well actually I like pink but if it's very pink and that could be a bit of an issue as well god bless you the other issue is the fact that mast cells actually have a job to do so before styrofoam and plastic and super stop and shop and highways being you know 500 yards within your roads their job was to protect us against infectious challenges toxic challenges they're essentially the original board Border Patrol and then after the insult was quelled their job was to help coordinate the healing process which i think is an interesting thought in individuals that have connective tissue issues understand that mast cells are really important for help coordinating wound repair and so if it's a question of individuals that have connective tissue disorders is the connective tissue disorder causing the massive dysregulation there's a possibility that the mast cell population is contributing to a diffuse connective tissue disorder so once you think you have a mass all disorder unfortunately a lot of my allergy immunology colleagues have a tendency to say you know your skin testing was negative and your and your your blood tests to pollen and food and medications that allergen specific IgE but thinking that I geez the only way that your muscles activating you know it's kind of like the thought of the police officer that's guarding your neighborhood has a single mugshot and that's the only way that they can tell that this person might be dangerous to you there's lots of different receptors on these cells and depending on how these cells are being tickled may may be an indication of why your mass cells are misbehaving as an allergy immunology specialist I've been taking care of individuals with chronic hives for twenty years and I'll stand up and say I'm also president of that Club because I've been dealing with cold induced hives for twenty years as well and I can tell you that you know if if my hives if I'm not having any hives that tells me that I'm actually copacetic but if I see a change in my hive pattern it's telling me that something is happening whether I might have a respiratory infection I might be in a hotel conference room that's really cold but there are lots of different ways that math cells can be tickled these are just some of the conditions that can be associated with increased mass L activity and dysregulation and I can tell you the top column when it's allergies or autoimmune attack area where which means literally another part of your immune system is decided to attack your mouth cells histamine blockers might work but for all these other conditions you have to have other interventions so once you get the diagnosis of Masel activation to sort it's really important to sort out what flavor of Massah activation disorder you have what is most well known by healthcare practitioners but is rare less than two hundred thousand cases worldwide our individuals that have mastocytosis or what's called mastocytosis junior monoclonal muscle activation syndrome then you have individuals and I think this is really important because this is the first time it has been identified that a enzyme that's only found in mass can cause a diffuse connective tissue disorder so individuals and there's a company here gene by gene that can do the evaluation individuals can inhabit inherit a duplication or triplication of this gene so every time that mass cell is triggered you're releasing two times or three times as much tryptase and individuals and this was an observation from Joshua Milner at National Institutes of Health they will end up with the trifecta of a connective tissue disorder worsened for a hypermobile ehlers-danlos syndrome they'll have dissident Omiya in the form of pots and they will also have hypersensitivity to allergic and non allergic triggers or muscle activation syndrome but the majority of us the mast cells would be inappropriately called in it's kind of like your police officer being called on 9-1-1 because your dog your dog is barking at your at your sister i don't know so but it's really important to understand what is tickling the mast cells to increase their activity and so when individuals once i try to identify whether or not mass cells are overactive not i try to figure out why and in this community the one thing that's extremely common might be what's called de mater graphism or de menthe agraphia we have the ability to write on your skin and it turns into a hive so that's that is a classic marker for individuals that have mass cell activation issues and then i'm i also label this i'm an idiot i can't figure it out but we still have individuals that have video Pathak anaphylaxis and there's more research to be done so again do you have massage vation syndrome what flavor do you have and so as an allergy immunology specialist i have to be grateful to the to the practitioners in this community I've learned how to do Buyten screens and and when I presented this to the American College of asthma allergy immunology the allergy the ten people that were in the room for that talk basically said what we have to start bending people to try to figure out whether or not they have a high perm you know they have a mass all to sort of my guess you do so these are the battery of tests that I have a tendency to order when patients come through to see whether or not they have a secondary Messala activation syndrome so allergy testing is important you don't necessarily need to go for another endoscopy or colonoscopy although I did just meet a patient I don't know how the gastroenterologist got this paid but she had nine colonoscopies in ten months I you definitely want to do a Rheumatology panel whether or not this is rheumatoid arthritis or move toward a flashlight where I think with the rheumatologist call zero negative rheumatoid arthritis you want to check for autoimmune antibodies against the thyroid I think the thing that's really important is to start checking for antibodies against the peripheral nerves and it's important to identify a neurologist that has expertise in looking for small fiber neuropathy definitely screening for errors Danlos syndrome where connective tissue disorders and I'm just going to briefly talk about some of the research that we've done looking at patients that have come through we've actually identified individuals that have immune deficiency and their symptoms are hypersensitivity reactions they have if they have a history of infections recurrent infections to see that severe infections its remote but this is where your history and you you know taking note of your journey is really important to try to identify not only that you have a masla activation issue but it's immune it's a deficiency in another component of the immune system that might be driving in so I don't know you may have seen these signs I've seen this once actually when I came down to the meeting in Houston is it just a cold so these are the ten warning signs for looking for primary immune deficiency you know recurrent infections to pneumonias I actually had taken care of a woman as a fellow who had 20 pneumonias in 25 years and the only reason she came to our attention is because her pulmonologist retired and turned out she had a form of immune-deficiency called common variable immunodeficiency and now has brought the Exorcist because she was improperly diagnosed so if you're having sporadic infections recurrent infections if you have autoimmune disorders interestingly enough you can actually present with autoimmune hair loss thyroid disorders diabetes this may be a sign of you having lacking a component in your immune system either in the cell or the proteins that might be causing that to be happening and then also some forms of malignancy are also associated with mean deficiency as well so in the past two years I've had the pleasure of a young lady who came up from Chile and we actually went through charts and we identified individuals that had mannose binding lectin deficiency idiopathic cd4 lymphocyte of P Nia and antibody deficiencies and their complaints again wasn't infections it was recurrent it was hypersensitivity disorders so basically there was a loss in the check and in the immune system and you know the analogy I commonly used with a lot of patients is you know if your house is on fire and the police show up would you be upset with that and you'll say no but the police officer doesn't necessarily know how to put out the fire and if they start to help they could potentially make the situation where it's or get themselves hurt as well so this is what the mast cells are doing they probably are compensating for the lack of a protein or cell that's much more effective in dealing with that infectious or toxic challenge and because we're an environment where we're being increasingly exposed to chemicals that are considered toxic whether it naturally made such as mold or artificial in the form of off-gassing from newly manufactured materials the mast cells are not standing down so in these individuals the treatment from mask law activations how to live better feel better is you need to identify whether you have a mass L disorder or not and then you need to figure out what's causing those mass cells would be dysregulated if it's allergies these are the recommendations avoidance measures but and especially with diet and a lot of patients have a tendency to focus on what they eat and my response to that is that is important you eat maybe four or five times well if you have to saw - Gnomeo six or seven times a day you breathe 18 times per minute and your skin is constantly exposed to the environment so it's really important to have a much more comprehensive approach regarding modifying your environment and understand when you continue to modify your environment you kind of start losing the ability to develop tolerance as well if you have susceptibility to infections whether it's sinus pneumonia or chronic Lyme or beryllium or Canada or herpes virus it's really important to try to understand whether or not you have an immune deficiency that's contributing to that and then you need to kind of focus on the treatments we've identified two individuals that actually had hepatitis B and treating the hepatitis actually got their hypersensitivity disorders under control and then also we have plenty of medications and more coming regarding treatment of classic autoimmune disorders whether you're talking about rheumatoid arthritis or psoriasis or Crohn's interestingly enough we have at least two patients where I've used for tuckson in order to get their amin hypersensitive ations under control as well so in many ways I'm going to encourage you to be like that mole and try to focus on if you're having little things to get that evaluated before it becomes a big thing we have a tendency to meet patients when they're completely debilitated from massagist regulation which is associated with you know not as commonly associated with having nerve involvement whether it's in the form of small fiber neuropathy or chronic immune demyelinating polyneuropathy as well you know and I love the ilk of it's better to forestall disaster than treat it I have many patients who come through can complain t of headaches and congestion issues I will screen for asthma if they or as well as gastrointestinal symptoms because understand a lot of the throat symptoms can be post nasal drip or it could be laryngeal 400 reflux this is just contents of the stomach making their way to the throat as well so when you start to meet up with with your practitioner and and try to do it in small bites because I have one patient who showed up with her history which was eight point back in front on five pages it's a lot to take in but I can appreciate that because you know I shared with you that I've had chronic urticaria but if it wasn't for my participation of being welcome to speak here I would have been able to diagnose my daughter who has this as well regarding the Ahlers davos at mass or dysfunction so it's really important to kind of if you're working with a practitioner who's not taking you seriously then you need to identify another practitioner who will and so I'm going to kind of finish off with this comment from polymath singer who has an incredible story so if you ever have time this is a woman who started as a cocktail waitress and scripts and now she has a major division of National Institutes of Health and the allergy immunology division she's the one who basically said that this is not a question about having positive antibodies I can go out right now and test 100 people for an antibody against peanut the interesting question there is twelve people well out of the hunter will have a positive test but only two out of the 12 will be symptomatic so to lost tolerance but ten maintain tolerance and so if we can figure out how the individuals lost their or more importantly how the ten retain their tolerance which definitely involves reduce of stress whether it's in dealing with infections changing climate what-have-you will be able to try to restore the balance that you're going to need regarding math self focusing on what they need to go after and ignoring changes in temperature airborne fresheners whether or not the food that was cooked the day before has broken down and now has chemicals in it that might be irritating you and with that these are the steps are feeling better and I'm going to say salud and I'm happy to take questions or shall I wait till later good fabulous well thank you for your attention I appreciate your efforts

Channel: The Ehlers-Danlos Society

Views: 34,884

Rating: 4.928968 out of 5

Keywords: hypermobility, mast cell, MCAD, MCAS, hypermobility spectrum disorder, hypermobility syndrome, Ehlers-Danlos syndrome, Ehlers-Danlos, allergy, allergies, immunology

Id: LddITjni4G0

Length: 36min 15sec (2175 seconds)

Published: Fri Oct 19 2018