Have EDS or HSD? Why MCAS May Worsen Your Symptoms | Dr. Clair & Dr. Harris

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[00:00:00]70% or more of patients with EDS may have mast cell disorder, mast cell activation syndrome. So, I really think that it's the low-hanging fruit, so to speak, because it's so much easier to treat than the all the joint issues with EDS. That's a long process, but this is something that really should be aggressively treated because so many people have it and the likelihood of responding is so good that it's really worth pursuing it.

[00:00:27]>> [music] >> Okay. Hello, everybody, and welcome to the Doctors Making Videos podcast.

[00:00:33]Today, we have Dr. Claire Francomano and Dr. Jim Harris. Let me introduce both of them. Dr. Claire is the head of the Ehlers-Danlos and Hypermobility Spectrum Disorder clinic at IU Health, Indiana Indiana University School of Medicine, and she focuses on the care of patients with hypermobile Ehlers-Danlos Syndrome, EDS and HSD, which is a hereditary genetic condition that affects connective tissue in the body. Okay. So, today in this podcast, what we're going to talk about, we're going to talk about how to recognize MCAS, what symptoms does it cause, and especially how it relates to patients with EDS and HSD. And, of course, we're going to talk a little bit about how to treat it, how to get management, what you can do about it. So, to discuss these and other questions, our guest today is Dr. Jim Harris. He's a board-certified allergy and immunology specialist. Allergy is the specialty that focuses on conditions like hives, allergic reactions, and uh more on mast cell activation syndrome or MCAS. So, that's MCAS.

[00:01:36]He's also currently the medical director of the EDS clinic, which is a telehealth company. So, they see everybody virtually. They see patients who have been diagnosed with or are suspected to have Ehlers-Danlos Syndrome. He leads a team of providers who see patients in over 20 states. They just started a couple of years ago, but are already one of the busiest, most successful EDS clinics in the country. Dr. Jim Harris, Dr. Claire Francomano, welcome to the podcast.

[00:02:01]>> Thank you. It's great to be here.

[00:02:02]>> Great to be here, Charles. Thank you so much.

[00:02:06]Today, we're talking about mast cell activation syndrome, and this is a condition that affects so many of our patients, right? We see so many people with hypermobile EDS and hypermobility spectrum disorder who live with the symptoms of MCAS. The scientific literature has estimates in it that range from 30% of the people with hEDS and HSD to some people think it might be as high as 78 to 80%. So, it's really a very, very important issue for our patients to know about. And for some reason, it seems to affect women more than men. We don't really know exactly why that is, but so many women have it. The flushing, itching, rashes, hives. Even women who don't have EDS struggle with these symptoms. So, it's really, really an important issue for us to discuss. I thought it'd be a good idea for us to start with describing the mast cell. If you could just tell us a little bit about what it is and what it does, and then we'll jump into some questions about the symptoms and how we recognize mast cell activation syndrome.

[00:03:18]>> Well, thanks, Dr. Claire. That's a That's a good place to start. So, first of all, the mast cell is one of the cells in the body like the red blood cells or the white blood cells like lymphocytes or neutrophils.

[00:03:27]And probably the best way to describe it is one of the immune cells. It helps defend us against invaders like bacteria, viruses, uh foreign bodies, a variety of different things.

[00:03:38]And so, it's meant to help us. It helps with the healing process. It helps us in a variety of different ways, but can also harm us when it's overactivated. It It can release various mediators that cause a variety of symptoms that you're describing that we're going to talk more about today as well.

[00:03:52]>> Alrighty, thank you for that introduction. So, could you talk a little bit about the history of diagnosing and treating mast cell conditions?

[00:04:02]>> Well, we've known about mast cells for a long time. In fact, we've known it caused diseases as many as 50 years ago.

[00:04:08]Well, at first we knew that it caused a systemic problem, systemic mastocytosis, which is much like a cancer. It's a very severe disease, can even be fatal.

[00:04:18]But, it's been more recently that we've recognized it to cause a less severe problem, the MCAS that Charles was describing. In fact, the first criteria were only defined in 2010. And, since then we've been debating the issue back and forth. The initial criteria were were threefold. The first was that you had to have the symptoms uh in at least two out of 10 systems that were consistent with mast cell activation syndrome. Secondly, you had to have lab data to support that. Typically, we've talked about tryptase levels. You can also measure breakdown products of uh from the mast cell in the urine, like leukotrienes, prostaglandins, and histamine. So, there are tests available to test for this.

[00:04:55]And, the the third thing is that they have to have response to therapy. So, everybody agrees on the symptoms. The most common of which is the skin, you started to describe some of that, flushing and the itching, the heat intolerance. These folks have trouble taking a hot shower. They can't tolerate heat in the summertime, which is a big problem for a lot of our patients living in Florida, Texas, and people that deal with a lot of heat outdoors all the time. Often housebound. Um but, the controversy comes in in in actually make the diagnosis. Do you really need the lab tests or not to confirm that?

[00:05:25]And, I would say in many of our patients, we end up treating them for mast cell problems before we have lab tests back and find that many of the tests are normal, and yet they've responded well to therapy. So, the debate goes on and on. Do we really need to have the lab data to confirm that and support it? Certainly, when you have positive tests, it does confirm it. But, how about when the tests are not are normal or negative? That's where the debate rages.

[00:05:47]>> So, there's kind of two camps in the allergy and immunology world, right?

[00:05:52]There are those who absolutely insist that you have to have the labs, and others who feel that if there are signs and symptoms in two or more organ systems, and the people are responsive to the medications, then we can say that they have mast cell activation.

[00:06:11]>> Yeah, it's it's interesting because I think there's merit to both sides of the argument. In fact, there are two consensus papers out there. One's called consensus one, the other one called consensus two, that uh debate the issue back and forth. But, I think in in in our minds, I think that what we see in our patients is that so many of these EDS patients respond well to antihistamines, like it it seems it's interesting to me that the debate continues. Frankly, we have I'm an allergist. I'm a board-certified allergist. And so, for years, I've treated uh urticaria, hives, and we treated them without having confirmation of the lab test. In fact, we used to tell people, "We do all these tests, and 90% of the time it's going to be negative. 10% of the time we'll find something that will help us to understand what caused the hives, and we treat them anyway, regardless of what those tests show." And so, it's I find it comparable to what we see with the mast cell situation.

[00:07:04]>> Yeah.

[00:07:05]And we talked about the sensitivity and specificity of laboratory tests.

[00:07:11]And my impression is that the tests for the mast cell inflammatory molecules, they're very specific, because if we find them, it really tells us that the mast cells are activated and they're releasing all these inflammatory molecules into the tissue.

[00:07:29]But, they're not that sensitive. So, we get a lot of false negatives, right? And for that reason, sometimes it's just not that helpful. Honestly, I don't usually send the tests for my patients if they have the symptoms and they respond to the therapy, I'm happy. They're happy. We don't need the information from the test.

[00:07:53]>> Yeah, well, you I think we agree on that point for sure. You know, the tests are are helpful at times and certainly I I usually do get a tryptase level, but very often with the patients we see by telehealth, the results we get may be delayed by days or weeks or sometimes months before the patient actually goes and gets the tryptase test.

[00:08:10]And then the other criteria that is interesting is that uh the further confirmation is that you have to have documentation of a tryptase level uh during a flare-up, which usually describes anaphylaxis. And that there's a formula for that. It has to be an increase from baseline of 20% plus two. It's a formula.

[00:08:30]And the hard part about that is it's very difficult to capture one of those flare-ups. Many of our patients don't really describe flare-ups. And so uh we end up chasing that number for a long time before we actually get something to show for it. So, it makes it very difficult to use the criteria to diagnose the uh condition.

[00:08:48]>> I think when people aren't feeling well, the last thing they want to be doing is going and get a lab test done, right?

[00:08:54]When they're in one of those flares, it's very, very hard.

[00:08:57]>> You're right. They want to have a plan to get better is what they want to do.

[00:09:00]They just want to get >> Yeah, exactly. [laughter] Exactly. Exactly. Yeah.

[00:09:04]>> Yeah. So, I want to jump in here. I've got a question for that. So, if somebody is suspicious that they might have MCAS or they have mast cell involvement, what might be their first step? Like, can they assume if they have certain symptoms, is that going to lead them to think, "Okay, like I probably have some mast cell involvement?" Or is it very important for them to get a definitive diagnosis?

[00:09:26]Can they get their primary care to check it? What does that initial process look like?

[00:09:29]>> Well, first of all, patients with hEDS have so many symptoms and so many issues that often times the issue of flushing, itching is almost secondary for them.

[00:09:39]And so, they often don't bring that to their doctors to tell them about it.

[00:09:42]Secondly, if they do, then they have to be aware that many doctors and providers are just not that familiar with MCAS or even with HEDS. And so, it's a challenge. These patients face a lot of hurdles. I mean, most of them are diagnosed for months or years before they actually get the correct diagnosis and treatment. So, there's several barriers. One is that the patients often don't or they may not see it as severe.

[00:10:07]Secondly, if they go to the provider, they may not be that familiar with it.

[00:10:10]And if they are, they may not be familiar with the test that could be done like the tryptase and urine test and so on and go from there. So, a lot of challenges in getting to the correct diagnosis and treatment.

[00:10:21]>> And I would say it's just really important for anybody who lives with HEDS or HSD to have MCAS on their radar.

[00:10:31]Because the mast cells can contribute to so many of the symptoms that people with HEDS and HSD live with. And that leads really into the next thing that I was going to ask Dr. Harris about, which is everybody knows about these skin issues, the itching, rashing, hives, but I think many people are less familiar with the issues that mast cells can cause in a whole host of other organ systems in the body.

[00:11:03]So, could you take us down that list and just sort of go through the many, many things that mast cells can do and when they're causing trouble.

[00:11:13]>> Well, that's a great question. And the short answer is it can affect pretty much everything in the body. And so, I'll just kind of go through a laundry list. I think the first and probably best known is associated with HEDS and mast cell is POTS. And we often call that the trifecta. So, that's a cardiac condition where the heart races especially with standing up. They're fluid depleted. They're often very dry.

[00:11:36]They have to drink a lot of fluid to keep up. They get dizzy spells. They can pass out. And the definition of POTS is that their heart rate goes up by 30 to 40 beats per minute while their blood pressure stays the same. And that helps us a lot with treatment. There are ways to treat that effectively. But that's part of those common condition that we deal with that people are familiar with.

[00:11:53]But beyond that, they can have uh standard allergy problems like nasal allergies and asthma. Those are treatable things. They often have migraines. They have chronic pain. They have chronic GI problems, nausea, cramping, and bloating, constipation, diarrhea, abdominal pain.

[00:12:10]Uh women tend to have lot gynecological issues. Difficult, heavy periods are very common. And of course, the bone and joint issues that we deal with with HEDS and HSD that also affected by uh the mast cells. Mast cells release histamine. Histamine causes inflammation. Inflammation causes pain.

[00:12:29]And so, uh a lot of the pain issues, whether it relates to the migraines or to the bone and joint issues, are related to the mast cells and can improve with treatment.

[00:12:38]>> I think a lot of women are very surprised to hear that some of their gynecologic issues could be related to mast cells. Dysmenorrhea and also premenstrual even symptoms, you know, that people have, sometimes really respond amazingly well to treatment for mast cell activation. And this can be very surprising to people.

[00:13:00]>> Yeah, it it it it it almost seems too simple in some ways. A a condition called uh PMDD or premenstrual dysphoric disorder is very common. And so, not only do they have difficult periods, heavy periods, but also their problems start about mid-cycle from ovulation on.

[00:13:16]They're often more depressed. They're more moody. They're more irritable.

[00:13:20]Their more fatigued. Their EDS symptoms get worse.

[00:13:24]And uh another interesting thing about the mast cells is that it releases not only histamine but also heparin, which is an anticoagulant. And so that contributes a lot to the heavier periods that they deal with as well. Mast cells aren't the whole answer to that, but it's certainly a big part of that. And they often do get a lot better with treatment just from that alone.

[00:13:41]>> So interesting. And the other really interesting thing is that interaction with the autonomic nervous system.

[00:13:49]Because I think many people are surprised at the degree to which the mast cells can be contributing to those POTS type symptoms. People think about POTS as a separate entity.

[00:14:02]But it seems like there's this vicious cycle where the mast cells make the dysautonomia worse and then the dysautonomia makes the mast cells worse and they go down this spiral that we have to interrupt by getting the mast cells under better control.

[00:14:20]>> Yeah, it's kind of a chicken and egg thing. You know, we certainly see a lot of people with hypermobility that are relatively symptom-free and many people that have mast cell disorders as well that aren't hypermobile. So you can have them as separate entities and yet it's clear that they feed on each other. You know, again it's a chicken or egg, what came first, but but clearly you have to deal with both of them together to really get the patients under control and to really eliminate their symptoms.

[00:14:46]>> Some people are under the impression that you have to have anaphylaxis to make the diagnosis. What's your feeling about that?

[00:14:53]>> Well, clearly these patients are prone to have anaphylaxis. And I think those that could go back to the tryptase levels, those that have higher tryptase levels are much higher risk for that.

[00:15:04]And also if they have a condition called hereditary alpha tryptasemia, which also elevates the tryptase levels, increases more tryptase in system. But by far the great majority of our patients really don't describe symptoms that are consistent with anaphylaxis. Most of us think of anaphylaxis as a condition where you might go into shock, your blood pressure drops, your heart rate goes up, you may need epinephrine or resuscitation to survive.

[00:15:28]Rarely do I see that in the patients that we see, and you know, at our clinic, I've seen probably over 1,000 new patients in the last year or two with hEDS, and rarely do I find that condition. And yet, it we you always ask about that, and and if they do have that kind of a history, we certainly take it seriously, we provide them with epinephrine injectable epinephrine and EpiPen, but I do testing that would help us to sort out why that may have occurred. But But the great majority of people really don't seem to have symptoms that fit with anaphylaxis, so I think that makes it really difficult to make that part of the diagnosis.

[00:16:03]>> You mentioned the hereditary alpha tryptasemia. That's a very interesting condition that's just been described really in the last decade or so. That is one situation where I do order tryptase levels when I'm suspicious about the hereditary alpha tryptasemia, and that would be in cases where there's a really strong family history of these mast cell issues, and also they start from a really early age.

[00:16:31]So, for those people, I think it's really interesting to check the tryptase level and see if they are elevated. But the caveat to that is they don't have to have elevated tryptase levels. Like the studies from the laboratory that initially reported it found that the people can have tryptase levels within the normal range, but at the higher end of the normal range even with the presence of the hereditary alpha tryptasemia. And just to say a little bit about what the gene is on that, you know, it's the alpha tryptase gene, and there's a duplication of that gene. So, ordinarily, there's one alpha tryptase and one beta tryptase on each of the chromosomes that carry those genes.

[00:17:25]And with the hereditary alpha tryptasemia, you get two copies of the alpha tryptase gene. So, it's a really interesting genetic phenomenon. And actually, sometimes people can have two copies of the beta tryptase gene, too.

[00:17:41]So, that's just a little side note. It doesn't apply to most people with MCAS, but it is very interesting.

[00:17:49]>> But but it is something you really need to think about if you do get a high tryptase level. And you mentioned I have a question for you, and that is you mentioned some people will have that even with a low How can I say it? A high normal level. So, what kind of level are you thinking about where you might really suggest getting a a level of the tryptase?

[00:18:09]>> was about six. But it's about six. I think the normal range goes up to about 12. And there were people in the studies that showed that with the alpha tryptase duplications that had tryptase levels in the 8 to 10 range.

[00:18:26]>> Yeah, that's definitely your area of expertise. And I think it's as much as we sort of dismiss the importance of tryptase levels, there are times where that needs to be pursued. I would agree.

[00:18:37]>> Okay. So, let's get down to the root causes here, Dr. Harris. Like, what causes it? What gets these mast cells releasing all these inflammatory molecules into the tissues and causing all this havoc?

[00:18:51]>> Well, it's really interesting because some people seem to have very young age. I mean, I've treated uh family members as young as two or three years old that had flushing and itching. Put them on antihistamines, they get better. They tolerate summer heat better.

[00:19:04]They have fewer reactions to mosquito bites. I mean, a lot of things seem to benefit from the treatment in identifying these people at early age.

[00:19:11]And yet, most of them don't seem to have that kind of onset. It seems to come on much later. For girls in particular when they reach puberty, it seems really activate a lot of the symptoms. Many illnesses like COVID can activate the mast cell and the MCAS. And many of the patients that we have been diagnosed with long COVID actually have activated mast cell activation syndrome. And so, treating them for the MCAS often is very beneficial. Uh other infections can do it. I've seen it follow influenza.

[00:19:38]Simple bacterial infections like chronic sinus infection can do it. Sometimes medications will set it off. Uh I've seen it get triggered by surgery. A simple surgical procedure after which they never quite recovered. The mast cell activation syndrome kicked in. And so, it can be multifactorial. Can be environmental as well. Some people with heavy mold exposure will have it. I think many of the people that are diagnosed with Lyme disease and other conditions like that often have mast cell characteristics that fit with their condition as well. So, it's really multifactorial. Uh the things that can set it off >> Okay.

[00:20:11]Dr. Harris, do you find people with MCAS or mast cell issues, do you find patterns? Like, oh, okay, when you go into a certain area, you get triggered. Like, maybe that's the mold or if they're consume some certain type of food, do you find those patterns many times?

[00:20:28]>> Uh yes, you know, what what once you have the MCAS, there are a lot of things that can trigger it because your system is already on hyper mode in a sense. So, many times certain foods will set it off. Uh we'll talk more about diet, but uh diet, certain foods will set it off.

[00:20:42]Uh certain smells, sometimes perfumes or odors will trigger people. Uh light can set people off. And again, it's kind of the chicken and egg discussion again. Is that really what started it or is it really just a sign that they're already so activated that minimal stimuli will set them off. And so, we do try to identify those things, but it goes back to treating the underlying condition really uh see the improvement of those problems.

[00:21:06]>> Yeah, I imagine there's a range of some people they're kind of more mild symptoms and then obviously very extreme even up to anaphylaxis.

[00:21:14]>> You know, in in my experience in my clinic I I see that many many patients have a there's a point in time they can say when their clinical situation started to deteriorate.

[00:21:29]Like something happened. Either they had an infection, an accident, there was some catastrophic thing and it could even be an emotional thing, a divorce or something like that.

[00:21:41]Stress in their lives of a physical or emotional nature. Before that they were hypermobile but they were functioning well, they were doing well, everything was kind of going along.

[00:21:53]They might have had a little bit of pain but no no big deal, functioning well.

[00:21:58]And then this thing happened and then everything just deteriorated. Things kind of went downhill pretty rapidly.

[00:22:07]And it seems like it could be that what's happening is the mast cells are getting activated as a result of those stressors and really a lot of the pain, fatigue, GI symptoms, all kinds of things that people are living with consequently are a function of the mast cell activation.

[00:22:31]Do you think can we lay it all on them?

[00:22:33]>> My experience is same as you Clara in that people often can pick that point in time where things got worse and often times they don't have a lot of insight into it. They hadn't really thought about it. They just noticed they got worse. When you go back and really dig for that often times you can now to find a cause but also a way to help them get out of the mast cell activation problem because if you know the trigger you may understand the treatment better. Now whether it's avoidance or certain medications or whatever it might be but it is it's to out why did they get worse all of a sudden because it happens to so many people. One of the more common things was it was COVID, of course. And people might have been okay with the first COVID, they get a second or third time, and all of a sudden that's the one that really sets them back, and they don't seem to recover from that. So, yes, the trigger is really a key thing in this whole process.

[00:23:20]>> So, but the good news is that most people do really well if we can find the right treatment for them, right?

[00:23:28]So, can just lay out the strategies for treatment? Like I I have my mast cell 101. Like what I know as a general geneticist is antihistamines, mast cell stabilizers, and limiting the histamine in the food.

[00:23:46]But you have a armamentarium that's much, much bigger than that. So, can you give us the whole picture in terms of treating?

[00:23:54]>> Well, the first thing is that that really is good news because most most of these people really do get better with the treatment. And uh it's a combination of medication and diet and lifestyle. I think those are the big three things we kind of put together. Uh you mentioned the medications, which are are many. Uh over-the-counter antihistamines can be very effective for this condition, and many people see benefit right with the first couple doses of it. And uh over-the-counter antihistamines in higher doses than we're used to giving.

[00:24:23]You know, typically we give them once a day because that's what the package says, but they may need two, three, four pills a day to really get things under control.

[00:24:30]And another type of antihistamine that we usually use to treat GI problems like heartburn, like famotidine or Pepcid, can also be very effective in addition to the over-the-counter antihistamines that we all think about like Claritin, Zyrtec, and Allegra. Beyond that, there's a whole number of things that can be helpful. And part of my experience comes from treating hives over the years because they follow much the same game plan, frankly. But the leukotriene antagonists like Singulair can be helpful in many cases.

[00:24:57]Uh in some patients, proton pump inhibitors seem to like over-the-counter aspirin can be helpful. But there are more severe cases that require stronger antihistamines like hydroxyzine. Many times you have to treat the other problems to get their mast cells to behave. For GI problems for example, you have to treat that separately sometimes with cromolyn. I mean there is a whole range of things we can use. Like cromolyn for example is an oral supplement you might take with meals.

[00:25:21]Other drugs like ketotifen, quercetin, luteolin, they're all mast cell stabilizers that can be helpful. In some cases more severe cases, we often go into some biologics like Xolair which blocks IgE in the system which can be a player as well. So the mast cells go along with the allergy system pretty well. And so many of them also have pretty severe allergies with them. So Xolair is a biologic and sometimes Dupixent can be helpful as well.

[00:25:48]So there's there's a lot of things that are out there to treat these people. I guess the key is you have to make the diagnosis first of all, recognize it as a problem, and then move ahead with the treatment.

[00:25:58]>> Now the Dupixent is specifically marketed for treatment of hives, isn't it? But sometimes you it can be helpful even in the absence of hives.

[00:26:08]>> Well, you know it's interesting with the biologics because as we have our experience with them and deal with them more, the indications are expanding almost all these drugs. Look at the GLP-1 drugs and how that indication are expanding with those. Dupixent for example initially I believe was an asthma drug. And then it expanded to atopic dermatitis.

[00:26:28]And now it's used to treat eosinophilic esophagitis. And so it may be all these things are related.

[00:26:34]It has the same basic mechanism of action as it did before, but we're recognizing that it may help a lot of conditions that we hadn't really thought about before. So I think the biologics may play a big role in the more severe cases of mast cell activation syndrome.

[00:26:48]>> Yeah.

[00:26:49]>> I think this is a question for both of you. I'm kind of curious about the timeline people might expect when they start treatment, what they can expect resolution. I imagine there's a range, but Dr. Claire, I'd like to get both of your opinions on that.

[00:27:03]>> Well, when I start with my mast cell 101 and I I have people they start on two different kinds of antihistamines, the H1 and H2 blockers. So, usually I recommend Zyrtec and Pepcid. And then cromolyn sodium to stabilize the mast cells and a lower histamine diet. And I tell people to see how they do over a month with that regimen. Kind of go on full court press with the two antihistamines, the cromolyn sodium four times a day and limiting themselves to foods that are ranked zero and one on the histamine food list, which is available. It's a Swiss website that provides the histamine list and we can give the link to that in this recording. That's usually what I do is that month to see how that antihistamines, mast cell stabilization, and lower histamine foods how they do. And very often people are really remarkably better after a month on that regimen.

[00:28:15]But sometimes it's just not enough and that's when they need to go to an allergy immunologist such as Dr. Harris and and start looking at those different antihistamines or the biologics or >> All that whole bag of tricks that Dr. Harris just told us about.

[00:28:33]>> Yeah, my approach is really pretty similar to that, Claire, and I I and I appreciate how you approach it.

[00:28:39]I I usually give them a timeline. I tell them, "Give me four to six months to fix you because you You you have to go low and slow with all these drugs that you give people and uh you know, if they have a reaction, which they often do, you have to be able to identify what it was that set them off and so we go fairly slowly with all the things you talked about and I think that one thing you mentioned about diet and how important that is and I think the longer I deal with these patients, the more I realize that diet is so important. The low histamine diet really does have a huge impact on a lot of people and we use another one called the low FODMAP diet as well, which is basically a low carb, higher protein, higher fat diet that eliminates dairy and gluten and many people don't recognize the foods that set them off and and following one or both of those diets can make a big difference with a lot of these people and and sometimes I will refer to dietitians as well because they have expertise in these diets and also in the EDS and they can be very helpful in managing these people as well. There's it's it's a multi factorial treatment plan and you have to do all those things to make things work and I I like your low histamine diet. I like that approach. I think trying to be as objective as possible with these foods because you really do need to test them out one at a time.

[00:29:58]Many people come in and say, you know, I never understood why I can't eat tomatoes and that's the reason. I can't never understood why you know, an avocado makes me sick and it gives them insight into things they may have already kind of recognized but haven't pursued it. Plus frankly, most people need a little shove when it comes to changing their diet because it's not an easy thing to do.

[00:30:19]It's a lot easier to take a few pills to fix things.

[00:30:21]>> Right. Right. But I did I had one patient who just making the dietary changes took her 90% of the way back to where she was when before she got sick and that was really a quite a revelation to me that that those dietary changes could really make such a huge impact because she was such a happy camper when she came back. She didn't need her antihistamines anymore, wasn't using the cromolyn sodium. As long as she stayed with the lower histamine diet, she was good.

[00:30:58]>> Well, you know, that's really the ideal thing if we could get our patients to the point where they can manage it themselves and not require medications.

[00:31:03]That's really what we all hope for and what we all try for and and sometimes when [clears throat] you get these people under control for a few months, then you can start backing off of things. They get their body kind of settled down and then you can back off and it seems like over time they may need less and less medication because they gradually learn how to adjust their lifestyle or diet or whatever, but I just the fact that they've gotten the inflammation under control for a reasonable length of time allows them to back off from the treatment.

[00:31:30]>> Yeah. That's what we're looking for, for sure. So, what are your suggestions?

[00:31:35]Like how if people are thinking that this may apply to them and they they are concerned that maybe the mast cells are really contributing to a lot of their symptoms, what should their first steps be?

[00:31:47]>> Well, I I think it's interesting the advice that I might give about that because many people come now asking about MCAS and they may have not talked to their doctor about it or they talked to them about it and they said recognize the the issue. So, many people could try antihistamines at home and see how they do. Uh modify their diet on their own, I think. But that said, I think that there are many problems that really need to be treated by providers and physicians. So, I think going to an expert in HEDS, typically that would be the best person to see, someone like you to get them started on the right track because the problem with specialists like me, for example, as an allergist or a cardiologist is that often times you identify a problem, you go to see that specialist for that problem, they stay in their silo treating only that problem and they never get the big picture because these patients are complicated, they have long histories, they have multiple systems involved, and it's it's it's difficult to put it all together. And that's one of the things we try to do in our clinic, in our EDS clinic, is to look at patients comprehensively and not just treat one problem, but treat them all together. But so I think that the need help for individual problems is helpful, but at some point you need to have somebody who's got that broader picture to look at everything together, like an HEDS expert or someone like us. And there's many people around the country that do this that can provide that kind of comprehensive view.

[00:33:10]>> And I can suggest for people the Ehlers-Danlos Society website has a directory of medical professionals with expertise in the management of HEDS and HSD. That's a good place to start if you're looking for a provider who's local to your geographic area.

[00:33:29]>> Yeah, I would agree. And we we use that a lot, frankly, especially for physical therapists who are trying to find somebody help, which as you know is a really important part of HEDS management. Not so much the mast cell, but more the HEDS side of that hypermobility. But the the website can be very helpful.

[00:33:46]>> Yeah, there's a lot of great information there. Ehlers-Danlos.com.

[00:33:51]>> So, I have I have a couple community questions. These are questions that kind of surfaced with research on the internet. These are from patients. We can kind of rapid-fire a couple questions that are kind of common questions about MCAS and EDS.

[00:34:04]>> Sure.

[00:34:06]>> Okay. So, Dr. Harris, I think this one's going to be for you. My tryptase came back normal. Does that mean I don't have MCAS, sir?

[00:34:12]>> Yeah. Yeah, so a normal tryptase does not rule out MCAS. As we we talked briefly about that, but but certainly if it's elevated, yes, you're much more likely to have MCAS. But if it's normal, which is the case for most of our patients that we see, it does not rule it out. And so I I still think that it's worth treating at first of all identifying and treating the symptoms, seeing if they respond as you might expect. So, the flushing, the itching, the hives, a variety of other issues like the GI, headaches, migraines, all that to see if they respond to treatment whether it's diet or the medications we talked about like antihistamines. So, yes, a normal tryptase does not rule out MCAS and you still need to treat the symptoms to see if they respond.

[00:34:52]>> Got you. Okay, perfect. And Dr. Claire, this one's for you. So, I have EDS. How do I know if my GI symptoms are EDS or MCAS?

[00:35:00]>> Well, it's challenging.

[00:35:03]But some of the suggestions that there's things that might make you think that they're related to MCAS is if it's in response to a particular food or a particular set of foods. The typical GI symptoms of MCAS are abdominal pain, gas, bloating. Sometimes people have really unexpected diarrhea right after they eat a food triggering them. So, all of those things would suggest that it could possibly be a mass cell reaction in the GI tract.

[00:35:39]And one way to sort of start sorting that out is to look at the histamine food list and see whether the foods that are provoking those reactions are the higher histamine foods. That would give you another clue that it's it's likely to be the mass cells.

[00:35:58]>> They can kind of be a detective. I don't think Dr. Harris mentioned that you have to really test one at a time. Is that the case?

[00:36:04]>> Yes. So, when I when I suggest to my patients that they take a month or 3 weeks with the zeros and ones on that food list, then after that they add in one food each day and maybe have that food a couple of times during that day to see if it provokes a reaction. And then they can sit can tell like they they can deal with it well or it is a a trigger and then they move on to the next one and try another higher histamine food the next day.

[00:36:38]And some people think that you should do it for two or three days in a row not just a single day with a food to really test it out but you'd be a long time getting up to a really higher variety diet by doing that. So I I I suggest one day. I don't know what do you do Dr. Harris?

[00:36:57]>> No I I like your approach. I don't like to stretch it out forever because you can take months to get to where you need to be and frankly patients don't usually have the the patience to put up with it that long. You know I I tell them the low FODMAP or low histamine diet I say just go all out and try that for a week to 10 days at least get some sense of is it going to work or not and then you can have an idea to go forward with the rest of the dietary challenges. And so it all makes sense to challenge one food at a time to sort that out because even some of the foods that seem like they're high histamine don't bother certain people and yet other ones that may be low on the histamine list may actually be the worst ones but but the diet the low histamine diet gives you clues on where to go with it and I think the patients appreciate that guidance cuz typically they've not had that before.

[00:37:46]I will say one thing about the typical symptoms the the mast cell I tend to think of mast cell problems causing diarrhea whereas the EDS with the motility issues is more constipation.

[00:37:56]But with that said when you treat the mast cell either one of those might get better. So the diarrhea usually gets better but often times the constipation does too in the EDS patients. So it's it's worth a try to treat it and also pursue the diet.

[00:38:11]>> Awesome. I think we've got one last one and then we can do some final words and close out. Can I get better or is this just managing symptoms forever?

[00:38:18]>> Yes you can get better.

[00:38:20]>> I think >> Well yes as Dr. Harris was just saying, you know, oftentimes people's just overall inflammatory states does tend to calm down as we treat the symptoms. So, I've seen many, many patients who have really turned things around by treating their mast cell issues.

[00:38:40]>> Absolutely. You know, it when you think about the numbers you mentioned earlier on that 70% or more of patients with EDS may have mast cell disorder, mast cell activation syndrome. So, I really think that in it's it's low hanging fruit, so to speak, because it's so much easier to treat than the all the joint issues with EDS. That's a long process, but this is a something that really should be aggressively treated because so many people have it and the likelihood of responding is so good that it's really worth pursuing it.

[00:39:10]>> That's great to hear. Nice. Thank you both.

[00:39:13]So, do you guys have any last words? I do want to close out with where people can find you and next steps to work with you, Dr. Claire, and also work with you, Dr. Harris.

[00:39:23]Dr. Claire, you want to start with that?

[00:39:25]>> Well, I just think I I'm going to come back to something I said at the very beginning, which I think that anyone who's living with Ehlers-Danlos syndrome or hypermobility spectrum disorder should have mast cell activation syndrome on their radar because the mast cells can contribute to so many of the symptoms that our patients with EDS and HSD live with, including pain, fatigue, GI issues, you know, in addition to the POTS and some bladder issues. So, it's really, really important to be thinking that this may be a contributor to some of those symptoms because it is so treatable and can really help in the management of all of those other symptoms that are seemingly unrelated. I think I think the mast cells sometimes are like the the glue that holds the whole EDS complicated picture together.

[00:40:29]So, I would really encourage people to to be thinking about that if it's not on their radar. Unfortunately, there are so few of us who really specialize in the Ehlers-Danlos syndromes and hypermobility spectrum disorders that it can be hard to get an appointment. Like I know my waitlist is very, very long, several years now.

[00:40:50]So, I really encourage people to use that professional directory on the Ehlers-Danlos Syndrome Society website and to try to find a local practitioner and also to use all the resources that are there on the Ehlers-Danlos Society website and take advantage of the many, many educational opportunities that are out there to learn more about their condition.

[00:41:19]>> I also want to plug Dr. Claire's YouTube channel. She's putting out a lot of great resources right now, so go and check that out.

[00:41:24]>> Thanks, Jen.

[00:41:25]>> All right, Dr. Harris, what about you?

[00:41:26]>> Well, I agree with Dr. Claire on all those issues. It's really important to address this comprehensively with not just the EDS, but also recognize that mast cell plays a major role in a lot of the patients that we see and it's it's important to treat all those aspects.

[00:41:42]It's an important part of the trifecta, which again is hEDS, MCAS, and POTS. And often times the POTS gets better with treatment, and so often times it becomes the the premier symptom that gets people to see a physician because they may be passing out, you know, dizzy when they stand up, they can't function on a daily basis. But again, all those symptoms, pain, discomfort, fatigue can respond to treatment of the mast cells and I would encourage people to seek providers that are willing to treat all that together, not just one piece at a time. And I think EDS clinics like our clinic, one of the reasons we put our clinics together was to provide that kind of comprehensive care to treat all those issues together, not just one thing at a time. And so, I think identifying and treating those problems together is really an important part of the care of these patients. And over time, I think we become more and more important as we develop more providers who are better and better at taking care of these problems and recognizing them.

[00:42:35]So, I I'm optimistic about the future.

[00:42:37]I'd like to compliment Dr. Francomano on her efforts over the years, education, teaching all the rest of us how to practice and take care of these patients because they are a challenging group.

[00:42:48]Uh they have many problems, and it takes a lot to put it all together. So, uh if if patients want to see us at our clinic, we are the EDS.clinic. We have many providers to see patients on a regular basis in about 20 some states.

[00:43:02]So, we're happy to help if we can. But again, thank you so much for Dr. Francomano for her expertise and her knowledge and what she's done to lead the organization as well and moving us forward and take care of all these patients.

[00:43:15]>> Thanks so much, Dr. Harris.

[00:43:18]So, I mean, I think the what you're doing with the telemedicine >> [clears throat] >> is a really wonderful contribution to this patient population because people can get care, you know, from a distance. They don't have to actually go to the providers. And this is really, really helpful for so many people. So, thanks for what you're doing to put it all together.

[00:43:42]>> Thank you for that.

[00:43:42]>> Yeah, thank you both so much. This was amazing conversation.

[00:43:46]Thank you both. We will have all the links. So, if you all want to see the resources Dr. Claire and Dr. Harris both talked about, if you want to call in to Dr. Harris's clinic and can schedule an appointment, just check the links below in the video description. You can see those there. And thank you both again.

[00:44:04]>> Thanks, Charles, for bringing us together.

[00:44:06]>> Absolutely. My pleasure.