Dr. Erin Nance has completed a series for the month of October where each day she covers a diagnosis that disproportionately affects women and is often missed or misdiagnosed as something else.
On day 21, Dr. Nance covered MCAS - Mast Cell Activation Syndrome and mentioned its nasty sidekicks it is often with; Ehlers-Danlos Syndrome and POTS/Dsyautonomia.
You can find more information about MCAS at GARD - Genetic and Rare Disease Information Center, along with NORD - the National Organization of Rare Diseases. Feel free to look up Ehlers-Danlos while on the sites as it is also classified as rare at this point.
We're on YouTube too: https://www.youtube.com/@taleoftwozebras
Amberlyn aka Mrs. Labeezy's Amazon Must Haves includes some of Ann's loved items too:
https://www.amazon.com/shop/mrs.labeezy/list/1U6RAYC0SJK4T?ref_=cm_sw_r_apin_aipsflist_aipsfmrs.labeezy_NQXZZ7SVJ2PSF3EZ700Y&language=en_US
Zebras are the rare type of spoonies often affiliated with the Ehlers-Danlos patients. Please check out the website: https://www.ehlers-danlos.com
If you'd like to be a guest, please reach out to Ann and Mrs. Labeezy at TaleofTwoZebras@gmail.com
Giving credit where credit is due: Christine Miserandino revolutionized the chronic illness community by sharing her Spoon Theory with the world on her blog: https://butyoudontlooksick.com Because of her contribution, those suffering with chronic illness are often referred to as "Spoonies" and we often refer to energy stored and expended in units of spoons.
Dr. Erin Nance has completed a series for the month of October where each day she covers a diagnosis that disproportionately affects women and is often missed or misdiagnosed as something else.
On day 21, Dr. Nance covered MCAS - Mast Cell Activation Syndrome and mentioned its nasty sidekicks it is often with; Ehlers-Danlos Syndrome and POTS/Dsyautonomia.
You can find more information about MCAS at GARD - Genetic and Rare Disease Information Center, along with NORD - the National Organization of Rare Diseases. Feel free to look up Ehlers-Danlos while on the sites as it is also classified as rare at this point.
We're on YouTube too: https://www.youtube.com/@taleoftwozebras
Amberlyn aka Mrs. Labeezy's Amazon Must Haves includes some of Ann's loved items too:
https://www.amazon.com/shop/mrs.labeezy/list/1U6RAYC0SJK4T?ref_=cm_sw_r_apin_aipsflist_aipsfmrs.labeezy_NQXZZ7SVJ2PSF3EZ700Y&language=en_US
Zebras are the rare type of spoonies often affiliated with the Ehlers-Danlos patients. Please check out the website: https://www.ehlers-danlos.com
If you'd like to be a guest, please reach out to Ann and Mrs. Labeezy at TaleofTwoZebras@gmail.com
Giving credit where credit is due: Christine Miserandino revolutionized the chronic illness community by sharing her Spoon Theory with the world on her blog: https://butyoudontlooksick.com Because of her contribution, those suffering with chronic illness are often referred to as "Spoonies" and we often refer to energy stored and expended in units of spoons.
Hello, and welcome. Got any spoons. I'm your host Ann. I'm not a doctor, I don't give medical advice. I do share my personal experiences, tips, tricks and life hacks of dealing with chronic illness, disability and pain. If you've been listening for any time at all, you'll know that I'm a tic tock fan. And this month, one of the content creators is Dr. Aaron Nance. And she did a 31 for 31. She covered the 31 most misdiagnosed illnesses that affect women. And you can guess that I have a few on her list. Day 21 happened to be mast cell activation syndrome. And she acknowledged that it comes with a few nasty buddies and she termed it the unholy trinity. And I thought Yep, Dr. Nance gets this. The Unholy Trinity would be Ehlers Danlos Syndrome, mast cell activation syndrome, and Pots/dysautonomia. And why do I put a slash dysautonomia? Well, because I'm a rule follower, and POTS is on the Dysautonomia spectrum. It is, when you have a tilt table test, two things have to happen. Your blood pressure drops a significant amount, and your heart rate goes up by at least 30 beats a minute. So yes, I passed out. The heart rate for me doesn't go up. I have a really hard time regulating my temperature and other things that are the autonomic nervous system like my GI tract is paralyzed. So I am definitely on the board with dysautonomia, but not specifically POTS - Post Orthostatic Tachycardia Syndrome, in case you're wondering. Now, Dr. Nance's series is very informative, and real. She has gotten stories from the spoonies firsthand, she has seen this, she has her own story of what happened recently, in the wake of her getting COVID and the complications that she experienced and the the real world experience as a patient and being misdiagnosed. So I think that's what spurred her interest in this in this series. And I just can't say thank you enough. After each little informative video, she does suggest that if you have this disorder, if you have this diagnosis, share your story, share your symptoms, share what you do to help every day live with the this illness. And I think that's basically what my podcast is that I want you to know you're not alone, and that you are loved. And I try to give my experiences, my tips, my tricks, my my life hacks, right. And as of late, it has been trying to help others accommodate us spoonies in what might be helpful, because I know there's another side. And part of that is selfish, because it does get overwhelming to focus on one's health. And I definitely want to try to stay positive. And it's been harder lately with the October slide. And I did have another experience this week that kind of proved that point. Maybe in the opposite. I used our county health system. My palliative doctor suggested that. And it was a two hour intake appointment. Now they had set aside two hours. I wasn't like, over filling my time. Let's just put it in fact, I ended 15 minutes early. Thank you very much. But the the social worker, psychologist not exactly sure what her title was, you know, she asked a lot of questions, and kind of got to start from the beginning with my mental health and how my physical health impacts that. And after the hour and 45 minutes, I actually felt a little bit of relief, because I felt heard. And she wasn't listening just to hear me vent. She was listening to do the intake and then coordinate resources, resources that I haven't had access to prior. And I'm not exactly sure what she's going to recommend. But I knew she was on my side. And that was a relief. There wasn't any fighting. There wasn't any. When I say fighting, you know that that hey, over explaining, like, this is me, this is real, this is what happens. And so that was nice to be heard, to be validated. And to say yeah, you qualify for services, you need help, and wasn't made to feel bad about that. So if you're struggling with chronic illness and all that, if you have a counselor or even if you think maybe You could use another counselor maybe for different area, there are some that specify in trauma, there are some that specify in relationships. And, you know, there are some that focus on weight and body image and that type of thing. Maybe you need more than one therapist, I don't know. And yes, it does get expensive. And that's another reason why going through a county mental health service, they have sliding scales, they can accommodate people's budgets. And so I think that's a really great resource that I want to make sure that I share. If you haven't checked it out, it is not as I thought, only for people without insurance, they do take insurance, and they have a sliding scale. So that's a beautiful Win win in my world. But to get back to the unholy trinity, and to talk about how I was diagnosed, and who diagnosed me in and how do I exist with these illnesses. First of all, I was a very late diagnosis at 48. And I was diagnosed the day after this chiefs won the Super Bowl, and being from the Midwest and a huge cheese fan. How ironic. It came on the heels of being diagnosed with global dysmotility. I was at the time, overweight, I still am. I'd been to the GI doctor numerous times knowing there was something wrong. I was walking with my dogs three hours a day, I had walked so much. In fact, that seeming my primary, she did X rays, and I had developed bone spurs. And she had put me on a steps limit that I was not to do more than 3000 a day. Well, three hours of walking 3000 steps is about a little over a mile. So I was doing way more than that, more like 12 to 15,000 steps, if not more. And we had this lovely off leash dog park. It was easy, I could do it. And it wasn't eating much at all. In fact, eating was not a friend. It hurt. And that was not new to me either. throughout my lifetime, I I wouldn't say food and I were friends, I didn't have an eating disorder. I always wanted to eat. I never restricted my calories for really anything. I just being an athlete in high school, I guess I always went to the Okay, I need to work out more, maybe change things up. But I hadn't really been challenged with my weight. Or at least if I had, I knew exactly what I needed to do to remedy it. And this was the first time that nothing was remedying this quite excessive weight gain and the walking and then not eating much. But then when I did eat, I was really full really quickly. And it was just a huge struggle. So I ended up having an acute pain attack went in and was diagnosed with acute pancreatitis. Now, I already had the diagnosis of chronic pancreatitis. And that can happen even though you don't drink. So I had had a pancreatic stent at one point, which they put in, and then they removed and I had my gallbladder out already. So I was in the hospital. The GI department that I was followed by happened to be at this hospital and they happen to have a new doctor. And this new doctor looked at me looked over my case and said, I think you might have something called gastroparesis. Like really? Wow. Okay, because that was in my world for different reasons. And I listened to him, we had an appointment, and we set up the smart pill. So we did the smart pill, any way to do a smart pill normal people have a digestion process of 24 hours, it shouldn't be in your stomach more than four hours. It shouldn't be in your small intestine. I don't know the specifics right now. And then your large intestine but from start to finish 24 hours. So they have you go in on a day. They you eat a very specific meal, you swallow the very big, smart pill, and then you have a harness with a monitor on it. And what the smart pill sends to that monitor is the pH of the its atmosphere, which is one way they can tell where it's at in the system. And then it measures the number of contractions Peristaltic contractions you're having and the strength of those contractions. So I have this harness that I have to wear and then I have a diary to keep of what I eat when and then when I have bowel movements. And the idea is that you will likely pass this and then the signal will be lost, they don't expect to recover that pill cam. It's not a pill cam, I'm sorry, a smart pill. So at factor five days, and I'm still getting the signal, and I'm still there, I did have to go back to my doctor, and they did an x ray to make sure it wasn't lodged, it wasn't stuck, and it wasn't. So they gave it another four days. And finally, that passed, and I turned in the harness and the, all the data. So it wasn't much longer. And I was diagnosed with global dysmotility, we worked on the diet modifications, and using medications that were available for gastroparesis. And per someone who has the diagnoses that I didn't know I had at the time, I do tend to get the most rare of rare side effects. So for me, there's a medication called Domperidone that you can take. And I was able to procure that it is not in the States, but you can get it from Canada. That was my doctor making that decision. And my decision I'm not suggesting or making that making that diagnosis or medication recommendation for anyone. My experience with it was that I had the elongated Qt I was passing out and not knowing what was going on. And it happened while I was in an appointment with my therapist of all people, of course, and I ended up going in and we figured that out. So that was why that wasn't a very good solution. And we looked at a J tube, which goes to your jejunum. And we did that procedure in September, I was diagnosed in June of 2017. So in September of 2017, I had my first J tube placed and there were complications. There were likely those complications again, because I had not yet been diagnosed with Ehlers Danlos. And they didn't know that my skin would react in a in a way that would be dangerous. They didn't know. So that happened. And the stoma was not healing, and I had complication after complication. But as you know, with medical, you always have to do the step therapy and you have to fail the therapies. First you have to do medications and you have to do old medications that are cheaper before you go to the premium stuff. And then you have to try all these things before they allow any type of surgical intervention. And then for me, it was a year, it was a hellish year of getting the tube replaced every two to three weeks, I had an infection. I was in the hospital for 42 days, I nearly died from a bowel perforation that happened during the surgery and that I had ventral hernias, we replaced the stoma, and it just got bigger. It was not helpful. I was on a ton of medication. That wasn't really working. I also didn't know why at the time, but I could have told you throughout my entire life, these meds don't work. I don't know. Either. They're not working for me. Or I'd get the super rare side effects. So all of that, that finally, in 2018, I was given a port, and I started TPN total peritoneal nutrition. And then hydration. I think I started hydration first before I did the TPN. And it was still a very difficult existence, I was in a lot of pain. And there's always seems to be something wrong. Why why is that there was always something wrong. So and at that time, I was part of a large international gastroparesis support group on Facebook, as well as a more local group. And I was the moderator of a tubie group. now that's T-U-B-I-E because I had a J tube. And I'm talking with these people and lamenting, and I've talked about the groups before, typically the most vocal in the groups are the sickest, they need help. They're searching for help. And it may be a question that's asked every single day because there's 1000s of people worldwide that are affected. But today, it's their issue today. It's their question, and it might have been answered yesterday. And you're kinda like just use a search feature, but they need somebody to listen to them and recognize them. And so I asked if you're in those groups and that you give a little bit of grace, because someone gave you grace when you first joined the group. I can guarantee it. You were asking the same questions that they were. And so as I'm lamenting about these things that are going wrong, there was several people saying, Hey, have you maybe looked at this issue? Ehlers Danlos Syndrome. And I'm like, I have not kind of started looking into it. But I'm of the generation and working with a therapist that I don't want to google myself into fear. But I went to a reputable site, the Ehlers Danlos. Society website, I saw the Beighton scoring, I saw the 13 different subtypes. And everything was clicking everything. And I'm like, This is me. They're right. This is me. And so I went through the system. And there was not a single geneticist in my metro area in the Midwest that was willing to see anyone to diagnose Ehlers Danlos, unless you had an abnormal EKG. And then they would be looking at the vascular type. Now, the most common type, the hyper mobile is the one of 13, that is not able to be detected on genetic testing, the other 12 you're golden. But this is the most common, and that's why they have the scale the Beighton scale, it does not take a geneticist to diagnose it. But doctors just don't want to go there. And often they'll ask why do you even want this diagnosis? Why are you looking at this diagnosis, it doesn't have any treatment, there's no cure. It's because I need sanity. Because it makes sense. It's who I am. Instead of saying, I have idiopathic gastroparesis, it's a lot more like, logical that something has been wrong with me, genetically, my genes are messed up. That makes sense. For 50 years, I have been messed up, knowing in my heart of hearts that something was wrong. And you can only take so much, you know, you ignore a lot. You pass it off as Oh, you just have a sensitive stomach. Oh, you must not, you know, there's too much acid or there's, you know, whatever this that all the rationale in the world, you're clumsy, you aren't good at sports, because it hurts. That's why I was a swimmer. Every time I tried a sport, I did do softball, I was a catcher. Like before puberty, and that was probably my blessing. But, but I couldn't do that forever. But swimming I could. And that was because that was helping me. Unbeknownst to me, it was very beneficial that I was a swimmer. So knowing how difficult getting the diagnosis can be knowing that every single geneticist in my metro area had declined me as a referral because I didn't have an abnormal EKG. I did some searching. And I found a doctor who has a very nice pedigree, all the right degrees from all the right places, and in fact, had headed the Mayo Clinic genetic department and had then since gone out on his own noticing that there was this to bring up anything on my list. Because this doctor in his interviewing was hitting all the points from childhood was this. need for people with Ehlers Danlos Syndrome. He had a niche Is this your situation? Did you find this? Yes, I bla bla bla that was that needed to be addressed. And he decided to do that. And in order to do that his practice is not one that's based on insurance. He is a self pay guy, he does telehealth, and he has this very niched specialty. So I was able to get in with him rather quickly after I found him. And I had a bunch of notes, all of the reasons why I hit the mark on the EDS spectrum on the diagnosis and all of these issues. I mean, we had 48 years guys, there's a lot of issues that started when I was a kid. answered. So with that. Did you XYZ Well, yes, Doc. Absolutely. And we just progressed in he is 100%. You have EDS. And then doing the family history. There are some outliers that are concerning. In my family history. I don't have a ton of information. Because my mother and father divorced when I was young, barely a year old and that side of the family. I don't have a lot of information. Other than weird stuff happens like a cousin passing away from an aneurysm in their 30s and another pair Another cousin died from heart attack in their 30s. That wasn't right. So with that information, the doctor chose to do the further genetic testing to see if I had any other forms. And I didn't. But when we got together to discuss that result, I said, Thank you, you know, this has been so enlightening. For me, it's really put a lot of things in place. And I'm really still struggling with a few things, and I wanted your take on it. And that's when we discussed mast cell activation. I didn't know that diagnosis, I was not specifically looking for that diagnosis at all, whatsoever, I thought these rather new symptoms, were just another part and parcel of the EDS gig. And maybe this doctor would have a treatment suggestion because he had been so helpful in giving best practices like, you know, putting stitches closer together or explaining to my family that my body is constantly working to stabilize itself. So for me, walking a block can be equivalent to them walking a mile, you're not really walking a mile in my shoes, you only have to walk a block. Now, I don't know what the actual ratio is. But the point is that with a connective tissue disorder, your body is working in overdrive, to stabilize, it can overcompensate you can have a bunch of other things that start not being nice. And, and that's what really drove me to talk about this with Dr. Nance is thing with mast cell activation. So I know I've talked a lot, I'm gonna take a break, you're gonna get to listen to the lovely bumper music, Mr. Mr. Made for me, and I'll come back and then we'll focus on mast cell activation syndrome. Welcome back. So mast cell activation syndrome, what I was experiencing was being having what would be perceived as allergic reactions. But to weird things at weird times. I always knew I was more sensitive to poison ivy, like, I don't have to touch it, just knowing that it's pollinating season. And when it's a windy day, I have to cover up completely because my, those spores that are pollinating can come in contact with my skin, and I will have a massive reaction. And there was the weight gain. There was trouble breathing. There's the flush and the itching. And I just seem to be getting worse, more things I was reacting to. And I figured it must be my sensitive skin. My connective tissue. This is why my GI system is you know, debunk and that type of thing. But when I talked to my geneticist, and explained how life was pretty dang difficult, I wasn't sure how to navigate. And I was concerned I might need to carry an EpiPen like I was in the mall one day, and walked by a new restaurant that had seafood. I could not get past it soon enough, I could feel my esophagus closing, I was like, This is bad. This was really bad, what is going on. And then I also at the time had a port. And I still do. But I was allergic to the tape. And then they gave me a different one. And I became allergic or at least allergic like reaction where there's a rash and there's itchy and it swollen. And it would actually, you know, hurt the skin where it would like, deteriorate it so I'd be I'd be bleeding. And none of that made sense. And so he said and informed me that of the unholy trinity is like here's here's some questions and again, like the first time I didn't have to have a bunch of bullet points. He was like, Does this happen? I'm like, Yeah, does this happen? Yeah. Okay. And specifically, what was going on? Was bone pain. I was having episodes of debilitating pain it felt Like a vise grip on my bones, it usually affected my tibia, you know, below the knee. And it wasn't like my whole leg was in a vise grip. Nope, the muscles were not contracting, the muscles were not affected. It was not an outside, it was the inside, bone level bone crushing pain that had me screaming and going to the ER, for relief level 10 All the way. And the doctor explained to me in my case that that was a mast cell reaction that the mast cells were like to hide out in the bones in the marrow and that it was flooding the system. And I was like, oh, okay, what can I do about this, and especially having a port, and he recommended that I attempt to use Benadryl. So I talked with my doctors, and that's one of the biggest things in my toolbox is IV, Benadryl, that I'm able to infuse myself through my port, and it has given me my life back, I feel, I was to a point where I could not function at all, I never knew when these pains were going to hit, or when I was going to not be able to breathe so well. And if you go to an allergist, you're going to test negative on the allergies, it's not that it's this mast cell, it's been mass produced. So that caused this it can cause rapid weight gain, it can cause the bone pain, it can cause diarrhea, it can cause flushing, it can cause headaches, I mean, a whole host of things. In the scary part. It's never the same thing. Or it doesn't. Like once you react to something, it's not like you're going to react to that the same way all the time. Dr. Nance's example of eating chicken nuggets that you've eaten for 20 years. And then one day when you're eating it, you really can't breathe all that well. And you're like, what's going on. And so you tend to start isolating in that's absolutely true. Because you can't function the when I was hit with that type of pain, I needed a wheelchair, if I was outside of my home, and I probably would be calling other people would be calling an ambulance like this is not okay. And I knew that. You always want to try to go home though when you're constantly sick. So that is the crazy making that today, it's random. It might be a food, it might be a flower, it might be your animals. It really can be anything present in your world. And even as recently as last week, I was prescribed a magnesium cream for my muscles that are really tight. And I went to put them on and my hands started burning. I was like, oh, and I got it off. And I washed up real good. And I and I thought I was okay. And I actually laid down only to wake up a short time later, in a major coughing fit. That was not normal. And I go to the bathroom and I look and I've got the rash on my chest. And it is a wheezing that I'm experiencing. So yep, I grabbed the Benadryl and I started getting that into my system ASAP. And that's probably the most severe reaction that I've had in a long time, severe enough that me myself and I will be talking to my doctor about the possibility of getting an epi pen. It was that scary. But then again, maybe I tried to use it next week and nothing happens. So that's I cannot stress how crazy mast cell activation is in the randomness, let alone EDS and it's basic randomness. You know, your shoulder goes out today. Your knee goes out tomorrow, but you're just pushing things back in, because that's what you live with all the time. Your muscles can contract and they're overcompensating for the connective tissue. So you've got Charley horses going on, but all over your body that are excruciating. You know what a charley horse feels like? Just keep that going every day for several hours, until you can do enough soft tissue, muscle massage possibly to release it or you can't and you might need to get extra assistance. I know that's what happens with me. There's might be some medication. And in fact, I know there's medication that you can take my unique situation makes a lot of medicines unavailable, because I don't absorb them. So that's the challenge. And if if it's not scary enough, just hearing about it, I know that if you're living with it, you might also be breathing a sigh of relief, because you're not alone. You are not crazy, there is something wrong, it is random as all get out. And I can encourage you to seek help and support groups, maybe go to the National Organization of rare diseases, I'll put that in the in the description, and do a little bit of research. I mean, there's nobody more vested in your health than you, right. And so while some doctors take offense that you've, you've Googled your symptoms, others in in better doctors know that they can't know it all. And that you are motivated. I would probably, I know that being well informed patient can be intimidating. I've experienced that. So there's a balance to be had when you're having an appointment. And you're asking questions. And what I have found most helpful is to say, Yes, I'm having these symptoms. And I started to look at it, I just want to know your thoughts. What do you think? Because I have this crushing bone pain. And I don't know if it's a mast cell response. Because that was there. Have you heard of mast cell activation? What do you think about that? Or what are your thoughts on this bone crushing pain, so that you are bringing forth the knowledge that you have, and also letting them be part of the conversation because you don't know it all? And neither do they. And that's called teamwork, you check on something, you inform yourself, they give feedback, hopefully, they're informing themselves and checking into it. And we can work on a plan of how to how to handle this. So for me, it is the Benadryl, I may need an epi pen. And just being very mindful and aware of new stuff, I tend to be very brand loyal and brand specific, with shampoos, lotions, clothing, because clothing can be treated in for stains, or permanent press, or things like that. And it can have a very serious effect on me. And that's not fun. So one way I handle it, is being brand loyal. Is that the end all be all? No, I just found that I have fewer reactions that way. And that's what it's all about is just trying to have fewer reactions, and not letting the disease scare you into becoming a hermit. Because there are treatments, and there are precautions one can take. And then educating yourself and your loved ones. And speaking of loved ones, these diseases, disorders, syndromes, whatever your favorite word to use is, we're more proper, I don't know that I'm the most proper person in the world. Absolutely, I am not. It can be crazy making for them too. And the thing is, we just have to accept, accept that your loved one knows their body accept that this is the situation and that it's going to get better it will pass it will not be this episode will not last forever. That's really helpful to repeat to yourself. And for your loved ones to tell you while you're in it. And for you to remember, like I don't know, if you need to have a sign, we probably should like this too shall pass. It's doesn't mean it's gonna never come back. But it's not going to be there for ever. So you know your body, you know, when you need extra help, and when you should go in and when he needs to talk to the doctor. And yeah, you will be evaluating on a regular basis. And something like what happened with me this past week might happen to you, where I've been pretty good for a while now and things have gone a little haywire on a couple of things. So I need to go in we need to discuss further treatment options. And that's where I'm at. So check out Dr. Nance that she does have the 31 for 31. So I've talked about just the three the unholy trinity, but there's a lot more and you're not alone. This is, unfortunately very common. The response that most women get is you need to lose weight. And or if you're skinny, you have an eating disorder. Because there's nobody can actually eat healthy, apparently. And oh, yeah. That so lose weight or gain weight, something, fix your weight. And it's all in your head. If you'll just reduce your stress, take an anti anxiety, go for a walk, do some meditation, yoga, you know, that type of thing, try some melatonin. That'll be great. that'll that'll fix everything. Because the average woman who is misdiagnosed spends between six and 10 years, looking and being and having the wrong diagnosis or misdiagnosed, like not at all diagnosed. So it's a thing. And Dr. Nance is trying to fix that she's trying to educate people. She encourages people to tell their story. And that's what we're here for. If you'd like to share your story, and don't have a platform, you can always email me at GotAnySpoons@gmail.com I would love to talk with you or share your story if that's what you want me to do and give your permission. And we'll keep going we'll keep talking about the stuff because we need to we need to scream it from the mountaintops. There are too many people suffering that don't need to suffer. And that's what we're here for. You are not alone. And you are loved. I will see you next week.