Validate Yourself

image of a slightly bent almost bare evergreen tree from the perspective of the ground looking toward the sunny sky between two tall red rock faces

Bend without breaking

It’s March 20th, 2017, and the new extremely complex and greatly updated Ehlers-Danlos syndromes nosology and diagnostic criteria haven’t even been out for a week, and yet it feels like a month has already passed, my brain is so blown by so much new information and the very emotional response that has erupted since! Lordy!

Many people with very rare types are now wondering if they have the right rare type, but the current material for learning about it is so highly technical and complex, it takes a very geeky brain to parse any of it.

And I’m including our doctors, who are by definition smart to begin with: medical school selects for same. Most of us (and them) are just not so steeply versed in modern genetics much less the finer points of connective tissue disorders, much less these new, extremely similar forms of the Ehlers-Danlos syndromes, phew!

Even if they are willing and interested to learn some or even all of this new material, they just don’t have the time in many cases as they are overburdened (at least in the US) with our bloated insurance system in most cases, and struggling to keep up with all the other new rare diseases being found elsewhere. Sorry but we’re just a subset of a growing pool guys.

And we aren’t the only rare disease community after all! I’m starting to know how our democratic government representatives must feel – with millions of people lobbying for their particular individual self-interests, and some in extremely persistent fashion. (As well they should.)

But especially many of us diagnosed with the most common form of the Ehlers-Danlos syndromes, the hypermobility type, are feeling quite up in arms as we appear to be “losing” our diagnosis thanks to the newly tightened criteria for same. Yet we are being given a sort of “catch-all” bin called Hypermobility Spectrum Disorders to fall into so that we at least can be diagnosed with something valid now, instead of just sent away as “just depressed”, if anything at all. Or worse: a harmful misdiagnosis of Munchausen’s or hypochondria or conversion disorder as some have painfully experienced. (Ouch!)

This has been quite upsetting for some of us who very understandably feel like we’re losing our  very very hard fought diagnosis, even though we’re technically not. Myself included – I no longer “pass” the newly tightened Beighton score in Criteria 1 here even after answering the 5 pt sub-questionnaire despite being as bendy as Sofi Dossi as a child and suffering from myriad issues all my life. (I’m now a stiff 2 at best, more likely a 0 in the Beighton score now at 50.01 yo, so no dice as far as I can see.)

But technically our diagnosis is just being changed FROM hypermobile EDS TO the new category of HSD: Hypermobility Spectrum Disorders in cases like mine. (Emphasis on spectrum! (And mine.))

I think if it didn’t take us well over ten years on average to get diagnosed to begin with (25 in my case), we might not be so upset. Especially since, as my long time readers well know, I’m deeply convinced that what we now call the Hypermobility Spectrum Disorders, or what passes for them on the “milder” and less bendy side are not at all rare, just rarely diagnosed as I used to say about hEDS befrore. 

I honestly think most are currently getting diagnosed with fibromyalgia, but that is my unprofessional opinion only of course. Though I know of one doctor who agrees with me now and increasing numbers of patients are starting to make the connection with a CTD and comorbid MCAD of some kind (usually MCAS).

Further, I happen to know of some hEDS (formerly EDS III) patients, including a leading rheumatologist in Chile himself who have told me they not only aren’t bendy now, but they never were. But they suffer from all the same systemic issues that arise with weak connective tissues throughout the body including joint instability as I am now. (I just “break” now, I don’t bend almost at all anymore and oh is it painful! I long for the bendy days of my youth, heh.) So they may not even get picked up by this new category even at all!

So it would seem there is some tweaking and adjusting to do, which The Ehlers-Danlos Society are quite willingly undertaking. Furthermore, we will be revisiting the whole works in just TWO short years from now, not the twenty painful ones we just took to get even here!

And… as I’ve been trying to re-assure my followers as I have volunteered to dive into the HSD waters of my own accord, my condition is still very real, as is my suffering and ALL of my pain. (And how, ow!) I’m just not bendy anymore and unsure I will be at all soon.

But let me be very clear: we are not required to be re-assessed or re-diagnosed and re-classified unless a) we wish to be and request it, b) we are required to be assessed for some reason such as applying for disability, or c) we apply to participate in a research project some day involving the hypermobile form of EDS and it is required then.

So in truth, technically, nothing should have changed for me or anyone already diagnosed with hEDS (formerly EDS-HT, aka HMS and JHS and EDS III elsewhere and in the past). We are all just contemplating and seeing where we do fall according to the new classification scheme and criteria. Or at least I am, speaking for myself of course.

Leading me to urge everyone, diagnosed or not, suspecting or otherwise, to validate yourself.

Heck, even before this criteria change, as too many know (and some aren’t even aware yet), it was extremely hard to get doctors to recognize any but the grossest signs of the rarest types of the Ehlers-Danlos syndromes. Now they have to double check they have the right one, there’s a flock of new, extremely similar types in a plot to further confuse. And still rule out any other similar HCTDs.

This will continue to be the case, despite all of our best efforts to educate as many doctors as possible all over the world. Someone somewhere will continue to be dismissed, invalidated and disbelieved for any number of unfortunate reasons, sometime even AFTER being diagnosed, most of which we can’t control.

And some patients even with the grosser signs of a rare type will still struggle to figure out which one they have until molecular testing catches up, which I think will sooner than later. I’m dearly hoping so for all our sakes!

I am friends with the mother of a lucky young boy who has yet to receive a confirmed diagnosis after over 6 long painful and expensive years of trying, despite traveling to see some of the best specialists in the world for almost every heritable disorder of connective tissue, and being tested six ways to Sunday. He was so afflicted, that Make-a-Wish even granted him his dying wish 3 years ago!

And even he can’t get diagnosed properly yet, and not for lack of effort! So we’re not alone in this problem, even on the opposite end of the hypermobile “bendy” spectrum. Thankfully doctors DO recognize he has some form of CTD, and wisely do suspect a form of EDS and treat him accordingly, but not one has been able to finger a single known pathologic genetic defect in him to explain all of his troubles. They have diagnosed a growing handful of major comorbidities like epilepsy and POTS thankfully so he’s getting supportive treatment and a service dog thankfully. I keep telling his mom he has “Stryder’s Syndrome”; i.e, he may be the first or only one of his kind!

So just as I did for the last 30 years while being written off as “just depressed” and “just a woman” (ha), you really need to learn to validate yourself. No matter what anyone else says or thinks about you, ever. In all ways.

Heck, my dear black and LGBTQ friends and family have dealt with this their whole lives – experiencing invalidation, disrespect and dismissal at almost every turn by very closed-minded bigoted people all their lives. There is just some unfortunate trait that runs in humanity causing a small but very stubborn percentage of us to be extremely closed-minded about some things, and in some rare cases, about everything. It’s just really unfortunate when they are the very doctors we’ve been trained to turn to for help, and nay, often pay thousands of dollars for the privilege of seeing.

But in the end, even some diagnosed and clearly afflicted patients STILL get dismissed and invalidated, sad to say, just not as often of course. And sometimes in different ways. Again, it’s just human nature in some unfortunately to do this. I’m guessing it is anxiety-based, or just plain narcissism as was my dad’s case. If they don’t mind, it doesn’t matter, right? They can dish it, but they sure can’t take it, sigh.

But we can never change someone else’s mind. Only our own. In truth, we are lucky if we persuade anyone else of any of our views it turns out – it is natural human instinct (usually) to cling to the familiar and resist change for most of us. (Not all. Never all.)

So do yourself a favor, and give yourself – and everyone around you, and your hard-working doctors a break for a minute, and validate yourself.

That is, know deep inside that even if no one else has affirmed your experience, said they believe you or given you the help you no doubt need and deserve – it will be forthcoming in due time okay? “Believe it and you will see it” to quote the late great Dr. Wayne Dyer.

Nothing worth doing is ever easy. And good things really do come to those who wait and persist – I promise. I knew deep down all those years that there was something truly wrong with me even if no one truly or fully believed me, we just didn’t know what. And instead of wasting thousands of dollars and probably hundreds of hours trying to beat the medical system at the time, I just ignored my milder issues, triaged the worst as they arose and got on with life as best I could until it radically changed in 2012, leading to my diagnosis finally after all those long painful years.

And boy am I glad I did. Not only did I get to participate in the working world and enjoy several incredible hobbies including gardening, photography and folk dancing, I traveled to some incredible places (albeit in pain, like where the above photo was taken in Utah), and lived a pretty darn full life until I suddenly couldn’t. Or at least not nearly as easily anyway.

Not that my life is over now, it’s just different, and how. (Ow!) It’s a lot harder to enjoy all of those activities now, though I’m ever so slowly returning to a couple in small amounts. Not to mention have blossomed into a writer as you’ve seen here. And have settled into my new life as a spokeswoman for a so-called rare connective tissue disease that no one can quite agree on fully.

I’m still me, Jandroid, the creative android. I still matter. As do you, diagnosed or not. I believe you, even if no one else does, okay? And The Ehlers-Danlos Society really does too. And if you keep the faith, and believe in yourself, ultimately others will too, I promise. I just fear that our doctors often fall into the more stubborn than not category, so this gets to be a little harder than some other reasoned arguments we make.

Trust – and heal – your gut I like to say. One day we’ll look back and laugh at how ridiculously hard it was to diagnose this condition, I promise. Keep the faith! We’re stronger together!

Love and light,

Jandroid, the aging android

 

 

 

Posted in About EDS | 2 Comments

Handling Change

I’m writing this post ahead of what is likely to be a day of great change for many if not all in the greater Ehlers-Danlos community, if not the world (whether they all know it or not, smile).

It is Monday March 13, 2017 as I type this, and we are all waiting on tenterhooks for the new EDS noslogy, which is just a fancy way of saying new diagnostic criteria to come out this coming Wednesday March 15, 2017 in the American Journal of Medical Genetics which yes, is behind a paywall. (Please honor this copyright, thank you.) You can’t access the full articles there without paying though, so many are quite upset at what feels like another barrier to this super hot information we really want to see.

Since so many of us are being poodles on their pantlegs and out of a practical need to get the information out as far and widely as possible, the international scope Ehlers-Danlos Society  (aka “The EDS” get it?) has promised to post them FOR FREE as soon as humanly possible with the permission of the American  Journal of Medical Genetics.

However, they will not be out BEFORE March 15th, so if it is not March 15th, 2017, they are not out yet, okay? :) Yes, a couple of related articles have been published earlier behind the pay wall, but the actual full EDS diagnostic criteria (aka “nosology”) will NOT be out until Wednesday March 15th no matter how badly you want them, free or not.

I am being emphatic, because I’m finding our community to be one of the single most impatient and highly anxious I have ever ever encountered. (And I thought I was impatient!) Heh, my doctor has no idea how good he had it with me. Hey, I only waited five years to finally get some cromolyn sodium recently covered by my poverty care insurance while my colleagues had it covered the whole time – but no, I’m not patient, sigh.

I’ve watched many of you get and have access to much better care and doctors than I have all this time, trust me. I smile when some of you complain about traveling 2 hours to see some of the best doctors back east in the US. Most of us out here in Oregon have to travel across the country to see those same specialists. (We don’t have them out here you see.) Try 2 days and 2 thousand miles of travel and often $2000, okay? Then we’ll talk. But you don’t hear me complain now, do you? No, I just get on with making lemonade out of my lemons along with my colleagues.

But furthermore, I’ve watched while a small, but very vocal minority of the community literally lose their shit all over Lara Bloom and The EDS as they shared an FAQ ahead of the criteria’s publication two weeks ago to help ease their enormous workload answering questions from this incredibly anxious and demanding population (us). I for one apologized to her and the EDS on behalf of the rest of the community because we know that they are working their butts off doing their best to pave the way and deal with these high levels of anxiety in us.

Not everyone was given good tools for coping with change. Clearly some got more than others, and some could use a bit more help.

So I wanted to address dealing with change in general just a bit since it seems to be a challenge for our community in general – not just around this very real and valid medical update. This is a historic moment in EDS history, and my heart goes out to all who paved the way for me and others to be here posting about it as it is increasingly recognized, thank you.

But I’m finding that a good number of us tend to really struggle with change in general, myself included. We tend to be a little (or more) rigid in our thinking and behaviors – wishing for some things to happen at the same  time every day, or in the same order, or in the same way. And to fail to believe in our selves and have faith in the future despite hands extended to us in help.

E.g. I could set my watch by my dad in my childhood – he ALWAYS got home at the exact same time (or very nearly) with rare exception, and performed the exact same ritual upon arriving: Hang up his coat, slip on his comfy moccasins, set his coffee thermos on the kitchen counter, go pee, come back out to the kitchen, pour himself a cup of now cold left over Folgers coffee from the Corningware percolater I grew up on, grab a handful (probably the exact same number) of Planter’s peanuts from the container on the end of the book shelf, and sit down and read the paper on his end of the couch while smoking a Lucky Strike cigarette. (Or three. He chain smoked, so I didn’t keep count.)

No asking how my day was at school. No listening, even if I tried to tell him – all I got were grunts, until he was done reading the paper and then, and only then, maybe, I might get his attention for a fleeting five minutes before he was onto his next ritual: a nap on the couch for 30-60 minutes followed by the first evening martini. With a green olive in it. (My mother was either drunk or absent after she left when I was ten, so no help there.)

If life conspired to prevent the above ritual (such as one of us having an injury or illness), he would deal with it – but it was clear he was very unhappy. So we didn’t disrupt this routine if we could help it. I learned to put my needs second or more often, last, to be not only not heard, but often unseen as well. (Yes, it took years to find my voice, thanks to all who helped.)

But I now observe this type of behavior in my fellow patients, helping me realize we all share aspects to some degree or another of this trait of rigid behavior and thinking, some more than others.

No, NOT ALL. I did not say all, now, did I? A handful of you are quite the opposite however, relishing chaos – this post isn’t for you natch! (Stop being so black and white!)

This is where the problem lies – leaping to often if not always erroneous and hasty conclusions, and then sticking to them like glue and over-reacting to them long before the evidence is in that this is correct or even necessary. Leading to oodles of wasted energy and unnecessary drama from such misunderstandings and communication breakdowns.

I call this sticky thinking or “velcro-brain”. I know, I have it too, just a bit more mildly than my dad and many of you. I deal with you in the support groups after all, trust me, I’m getting used to it.  You’re all variations on a theme of my family minus the alcohol. (I’m finding it most common in those diagnosed with ADD for what it’s worth, but that’s just my unscientific observation.)

I have always craved routine, and security and stability – even long after I healed from the chaos of my alcoholic childhood home, i.e, having nothing to do with said chaos. It is my wiring, how I am. My neurology if you will. Whatever label you want to give it: Aspergers, OCD, ADD, I don’t care. (I think it’s a soupy combo of some of all of the above myself.) Bottom line:

I don’t like change.

And neither, I’m finding, do many of you. (Again, not all! I can’t say that enough, smile.)

But I realize this upcoming change, with its threat of potentially losing a very very hard fought and hard-won diagnosis represents more than the usual level of loss for some of you. Hey, I might be one of you who loses her diagnosis too, we don’t know yet – I’m waiting to see also. But after waiting for well over 25 years, and being dismissed as “just depressed” and my fav, “just a woman” (heh) to get my first proper diagnosis only after I very suddenly became a completely and totally shattered body in a wheelchair at 45 (five years ago), trust me, I don’t want to lose my diagnosis of hEDS and all the medical validation that brings finally either!

But… I will live, even if I do lose that label. First off, because I now believe in myself, even if some over-opinionated (likely rigid and mildly Aspie or OCD) doctor doesn’t also.

And also, because we won’t lose the medical validation and new-found knowledge we’ve uncovered all these years. Yes, our diagnosis may change FROM “hypermobile EDS” TO the new, very real, very medically valid category of “Hypermobility Spectrum Disorder” or HSD. But guess what? I haven’t changed a spot despite the label change. Heck, our condition has been written off as “just depression”,  hypochondria, somatoform and conversion disorders and Munchausen’s for years. I can handle HSD a whole heck of a lot better than the others TYVM!

And guess what else? The Ehlers-Danlos Society and myself will not stop advocating for you. 

Even if you don’t meet the new criteria of HSD for that matter. Trust me, the medical world is slowly waking up, and realizing we fall on more of a spectrum than they realized before, and that not only can the condition change from seemingly totally benign to life-altering as mine did, but it also varies within a family, even between identical twins! So obviously there are other environmental and epi-genetic factors at play we aren’t even fully aware of yet that lend to the expression of the hypermobile form of the condition at least.

It may be years before we fully uncover all the causes and drivers of what is currently called hypermobile type EDS and now HSD for which there is still no single known genetic mutation for the vast majority. (I don’t count the rare Tenascin-X subset – they’re truly rare, even more rare than vascular EDS cases okay, Miss Pendantic?) I’m talking about the vast majority, most of whom get diagnosed currently with fibromyalgia if anything at all besides “depression”. Or any of the other myriad common major comorbidities I’m personally finding comes with this package for as yet unknown reasons.

As I continue to assert, in my opinion, at least the hypermobile type of EDS is not rare, just rarely diagnosed. (Or at least, what used to be called hEDS.) Castori et al cited as high as .5-2% may have it in 2012 here! (That’s up to 1 in 50 people folks – far from rare! Others have cited at least 1 in 100, or 1 % elsewhere in 2013.) If it’s now easier for doctors to believe in the new category of Hypermobile Spectrum Disorders than hEDS, then so be it. At least they’re realizing something is going on that isn’t “normal” and can lend to easy injury and our myriad comorbidities.

And I can’t help adding that I’m finding the same rigid behaviors and B&W thinking I find so common in our EDS families to be quite common in our doctors. Hmm, fancy that! I almost think we’re likely all related. Gee, ever notice how many doctors and nurses we have in our families? Quite a few! I.e, we are literally all battling each other! Oh the irony, sigh.

So it’s not the message, but the delivery that needs work. Your feelings and concerns are valid, but don’t let your fear get the better of you and make you over react. Take a deep breath, step back, and try to have faith that I and thousands of others including The EDS have your back, literally in some cases. And we will not stop fighting for you to be seen, heard and believed, I promise. No matter what they want to call it, okay?

Meanwhile, I urge reading my post on Anxiety and EDS for some tips and insight into what might be driving some of this seemingly disproportionate anxiety. Again, the underlying concerns are valid. It’s just not the message, but the delivery you may wish to work on.

I’ll lastly close with the pro-tip that many of us are also battling narcissists, both in our families and in our doctors. (This disease seems to select for same.) Again, not all. But some. And if you find someone is completely not listening to you, or misunderstanding you or twisting your words, or totally ignoring or invalidating you as my late father used to do (bless his pickled soul), then walk away and save your spoons! Trust me, they are not worth your energy. Do not give them so much power over you, okay? Believe in yourself, know you’re right even if they won’t admit or recognize it, and pick your battles. 

Find another doctor as needed when able. Notice I didn’t say “if”. It’s always possible, even if it’s not always easy. I’ve seen patients with nothing scrape up the change to fly across the country or even the “pond” to see the best specialists in the world. The internet is a powerful tool. Remember, it took me 25 years to be properly “seen” and “heard” finally, and I was dismissed by almost all of my prior doctors that whole time, not just my PCP of 15 years at that point. In his defense, he’d been misinformed about EDS being so rare when my form probably isn’t right? Whence I’ve forgiven him – and brought him with me. (He was open to my diagnosis once he could “see” it finally thankfully.) NO, it shouldn’t take becoming a shattered body to be ‘seen” and heard. Why do you think I’m writing so much now?? 😉

And if it’s a friend, partner or even your own family member who refuses to believe and validate you, find someone else who DOES love and believe you to talk to okay? Save your spoons! (Energy.) We have precious few, and you need to conserve them for staying as well as you can despite this painful package. Just because you’re related doesn’t mean your family will automatically love you the way you probably deserve, okay? And when they don’t, we have to love ourselves instead. No, it’s not fair, but I promise, life is better when you stop trying to change them (cuz we can’t, actually), and just take care of yourself instead. (Ask any ACOA or AcON you can find.)

I believe you, okay? Hang in there! We will get full and proper recognition for ALL of us in time. Nothing worth doing is ever easy. And all good things come in time. Heck, neither of my now clearly symptomatic (in 20/20 hindsight) late parents got diagnosed right? No wonder they drank! They were self-medicating, as I bet a lot of our homeless population is. I probably would have too in their pre-internet shoes. Keep the faith, it’s getting better, even if it doesn’t feel like it yet.

With love and all the support I can muster for ALL of you, patient or not,

Jan March 13, 2017

 

Posted in About EDS | Tagged , , , , , , , , , , , , | 3 Comments

Wherefor art thou Dysautonomia?

image of a dessert spoon on wood

A precious spoon!

“Homeo(stasis)… oh homeo(stasis)… wherefor out thou dysautonomia?” to mangle a phrase with apologies to all you brilliant Shakespeare scholars. And mix some medical metaphors to boot.

Anyone who’s been exploring either Ehlers-Danlos Syndrome or the highly comorbid Mast Cell Activation Diseases (either masto or the new diagnosis of MCAS as of 2016) has likely stumbled on the term dysautonomia, if they haven’t been diagnosed with some form of it already.

A majority of EDS patients I know experience either mild or more severe Postural Orthostatic Tachycardia Syndrome (POTS), or some variation of this including Neurally Mediated Hypotension (NMH) and more. But likewise most MCAD patients I know end up exploring or being diagnosed with some variation on this theme also or find they should in time too.

But just what is dysautonomia? And didn’t POTS used to refer to telephone lines?? Ah, those were the good old days of IT support dear Jandroid, when we had Plain Old Telephone Systems! Oh how my worlds – and acronyms – have collided, lol!

I have given short shrift to all of these topics so far on this blog because I got off lucky with a much milder “case” than most that I realized I could manage myself, knock wood. So I never even pursued the diagnosis beyond an EKG with my doctor in 2012. I was just too busy trying to literally stay fed and watered in early 2012 during my hEDS onset “storm” that by the time I figured out what was going on, I had already started to remedy it with supplements. So I carried on with managing my hypermobile type EDS and my MCAS which was much more show stopping at least at the time. Yes, I consider myself very lucky! But that doesn’t’ mean I didn’t enjoy some trouble. And how…

Technically dysautonomia refers to dysfunction of your autonomic nervous system, which like it sounds, runs all the “automatic” operations we normally don’t have to think about, or shouldn’t. Things like breathing, digestion, heart rate, blood pressure and temperature regulation. Any or all of these can go out of whack, slow down or stop with dysautonomia, depending on the cause. (Hold that thought.)

At the height of my storm, I started experience varying bouts of tachycardia out of the blue. Thanks to my online support groups, I quickly realized what was going on and went to see my doctor about it. Of course, my heart behaved perfectly during my exam, just like how your car engine doesn’t make that funny noise for the mechanic right? So of course… I’m a crazy lady who just thinks she’s sick. Wrong!

I actually got stuck on my way out of the clinic, though I never told my doctor, because I realized it was going to be another wild goose chase and I just wanted to get home. I called a friend for help, and made it safely, ultimately, but I’ll spare you the details. I continued having mild tachycardia off and on until I had a friend help me fetch some Gatorade. I did this after remembering that they recommended this for my dad years before when he’d been passing out randomly and said he needed potassium. Sure enough: my tachycardia stopped instantly! Boom. I had one answer for myself anyway, along with just plain improved hydration.

I proceeded to make my own home-made “Gatorade” after that, and didn’t have any more tachycardia. I later found sufficient food sources that I could tolerate (I react to tomatoes and coconut, so yeah, those two easy solutions are out for me) in the form of carrot juice and molasses. (Not together!) Along with all the salt I can stand on my food and lots of filtered water otherwise, I’ve not had another bout since.

But again, I’m a mild lucky case. Some of you will have been so bad that you got your POTS diagnosed first via a tilt table or other testing done by either a cardiologist, or a neurologist, if not both along the way. And some of you will have needed to take something stronger than potassium to manage yours, including variuos things like fluorinef and midodrine. (Talk to your doctors!) I’m so grateful I dodged this bullet.

However, I did not manage to dodge some cranial-cervical settling that messed with my breathing before all was said and done. Apparently my brain case had settled just enough on my head stalk that I was mildly squishing my brain stem and/or my occipital lobe (or both), and I would literally forget to breathe – in the day time! Like, all the time. I had to consciously remember. to. breathe! Crazy!

Again, I was still just so busy just trying to stay fed and watered and shut down both of my businesses and start applying for disability at this time that I couldn’t even manage to get this diagnosed. I figured it out when my colleague was diagnosed with central nervous system apnea aka “CNS Apnea”. Apparently some people end up with a pacemaker to help them remember to breathe if it gets bad enough! So how did I remedy this one you well ask?

Well, you may recall that my geneticist had recommended both Vitamin C and magnesium supplementation during my diagnostic visit of Feb 14th, 2012 when I asked about “best remedies”.  (Not cures, mind you, I knew better.) Well, again, it took me so long to even begin to address the problem that it had started to clear up on its own by the time I was getting around to it. Literally, my body and neck especially had apparently restrengthened enough from my religious supplementation regime that I was no longer having trouble breathing by about March of 2013.

What a relief! It just ever so gradually continued to improve, and I now no longer even think about breathing – most of the time, knock wood. But this can certainly be more show stopping for some of you, as it was for my colleague. I think she did end up with a pace maker before all was said and done.

I’m also lucky in that I can tolerate cheap OTC Vitamin C and magnesium and take loads of the stuff now much to my benefit. I’ve only continued to strengthen accordingly and now maintain the ability to walk albeit still with one Loftstrand crutch yet. (I went from walking to wheelchair in 3 weeks 5 years ago January 2012.) Many don’t tolerate almost any form of these two supplements, and have poor digestion, so struggle to re-strengthen as well as I did. And others of you have worse settling or instability in your necks that continues to mess with your minds – literally.

I see your posts on Facebook, they’re heart-breaking as you literally struggle to keep your good heads on your shoulders, sigh.

But dysautonomia is so much more than just POTS and CNS apnea. It can mess with your heart rate, sending it shooting sky high in almost no time, and keeping it there for indefinite periods, leaving you feeling breathless and anxious, like a motor racing out of its mounts. Alternately some will have such sudden and harsh BP drops that you literally pass out (experience syncope), or begin to (pre-syncope). Get up slowly you guys!

And in all cases stay as calm as possible, this too shall pass, I promise, even if it doesn’t feel like it just now.

But here’s the thing: the handful of people I’ve met who think they “just have POTS” and nothing else (or my favorite, fibromyalgia) have unfailingly turned out to show signs of at least hypermobility if not also MCAD of some kind as well upon further inspection and inquiry. Think about it: with rare exception, I really think POTS and dys are the likely “love-child” of weak connective tissues all over the body lending to flaccid veins and weak valves which leads to blood pooling in our lower extremities. And comorbid MCAD lends to third-spacing and peeing out too much causing more hypovolemia (low blood volume), so between the two we’re just sort of doomed. Keep your fluids up everyone!

But plain water intake alone won’t do the trick. Most find the water just goes right through and doesn’t “stick” without a sufficient combination of additional electrolytes. Yet, and this was interesting to discover, even potassium won’t stick without sufficient magnesium! So guess what? I have one heck of a flock of guardian angels, since they first directed me to take Vitamin C and magnesium early on in 2012 post diagnosis, both of which served to strengthen my cardiovascular system. Then I intuited the potassium and fluids myself, and voila: my own remedy.

I know, not all are this lucky or mild, or will be. But you can get some definite help and hope for the condition with your doctor’s help beyond the above.

The last major area I see dysautonomia strike most is the digestive tract, in the form of dysmotility and gastroparesis (literally, stomach paralysis) in some. I have only the mildest case of same – I get full really quickly now and have for years. But my food is still moving through me well, thank God. I did have a little trouble swallowing at the height of my storm, and my elderly aunty is permanently on thickened liquids in her care home now too. Thankfully my swallowig improved again on its own just like my CCI above.

But I have literally seen some fellow patients die from gastroparesis, as their food is literally stuck in their systems, or nothing moves through, and they are unable to receive proper nutrition. Yes, this is thankfully rare, but very real. I think those of you with impinged vagus nerves suffer the most from this. (The vagus nerve enervates your GI tract among other things.)

Why I have such good vagal tone I’ll never know, but I don’t doubt my daily Reiki practice plays a role and helps some as did acupuncture previously. Along with just plain more good luck knock wood again. (Yes, I knock on a lot of wood all the time, trust me.) And again, an re-strengthened neck from the C and magnesium helping keep my brainstem and the rest of my neurology un-impinged (free, unsquished) and happy.

Again, this is why I haven’t said much about the condition, because I’ve been blessedly spared from much of the worst of it all this time. Oh, sure, I freeze all the time, and swing wildly hot and cold on a dime, but that’s not generally show-stopping. Just highly annoying. (I wear three sweaters daily in winter.)

And this is just the tip of the iceberg of ways in which dysautonomia can affect people. I think it lends to more trouble with sensitivity to lights and sounds (I am very sensitive to both now), as well as how your pupils dilate. (Not always evenly, nor properly, even without taking any drugs.) Among many other things.

Again, there is a small but stubborn subset who insist they truly “just have dysautonomia” (usually POTS) and nothing else. But I’ve been hard pressed to find any in any of my support groups that isn’t secondary to some form of severe chronic illness like Lyme or EDS, and who truly don’t show signs of hypermobility at least if not also MCAD.

I believe there is allegedly an autoimmune form of POTS or dysautonomia, and I won’t argue with this: heaven knows autoimmune disease can attack any part of the body so nothing surprises me there. But I believe you folks are the exception. I still mildly suspect you of likely having part or all of what I call the Chronic Constellation and would love to know if you’re hypermobile or not. (Willing to bet some dark chocolate on it even.)

I often say “dogs don’t bark for no reason”, and neither do our bodies if you’ll allow me to mangle another phrase. (Om nom nom, I eat phrases for lunch, or my brain does at least, can you tell?) I really do think dysautonomia is more of a symptom cluster, or result of other things wrong in the body. I.e, it would be odd to experience in the absence of a clear biomechanical or pathological driver of some kind. But heaven knows some of you very black and white thinking types will insist on this just the same. (I’ve stopped arguing with you: you have literally steamrolled me to less than a crepe at times.)

But this is my blog, and that’s my theory and I’m stickin’ to it. It’s also late and I need to get to bed. It’s a very large topic, and very disabling alas. I hope I’ve at least shone a little light on why it can be so confusing to define: like much of what ails us, it’s a “syndrome” in its own right. Find my Dysautonomia Resources page here.

Best of luck to you as you manage this.

Jandroid

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What is Normal

Image of the head of a zebroid horse zebra cross animal with white body and black and brown striped head

Are we semi-common “zebroids” after all?

I know, “what is normal” is a really loaded question or statement, but I’m not intending it to be. My normal is not yours, nor theirs, nor anyone else’s. Well truly have our own “normal”.

I’m just trying to help distinguish between the majority who are not hypermobile, and those of us who are, but may not realize we are, nor what all that entails.

Since we tend to be attracted to each others both platonically and romantically, those of us who are a bit ore more “bendy” have “normalized” the milder manifestations of at least the most common and currently so-called Hypermobile form of Ehlers-Danlos Syndrome (hEDS) all these years as I write this in December 2016.

Update March 2017: I obviously now include all the newly recognized and described forms of the new diagnosis of Hypermobility Spectrum Disorders that were just “invented” by the International Working Group on Hypermobile Ehlers-Danlos Syndrome as announced by the Ehlers-Danlos Society on March 15, 2017 along with the new EDS nosology (first in 20 years.) This category is meant to catch all those not quite bendy/symptomatic enough to fit the newly tightened hEDS (hypermobile EDS) criteria now. <end update>

Other types may experience these things too, but are usually much more easily diagnosed due to grosser issues earlier on in life. I.e, what are things you experience with milder often undiagnosed hEDS or the new (as of March 2017) Hypermobility Spectrum Disorders besides the increasingly common fibromyalgia that the majority with “normal” or better aligned collagen do not?

The most obvious all these years is our gross flexibility, usually, lending to extreme bendiness and some contortionist talent. Yes, I highly suspect the lovely Sophie Dossi from the 2016 season of America’s Got Talent, among others. She is very hypermobile at a minimum, mistakenly taken by most doctors to mean just flexible, hopefully nothing more. (EDS = hypermobility plus medical issues. Some people are still believed to just be hypermobile by experts still.) Along with anyone else who is “double-jointed”, meaning you can sublux your joints at will, not that you have two joints.

NB Others can still have joint instability and other EDS signs despite not being flexible. That is, despite the majority with hEDS exhibiting great flexibility, not all will, but can still injure very easily and have soft inner tissues that rupture or tear still. Learn more here.

Popping your thumb or a hip out and back in easily is an example of a joint subluxation. It’s called a full dislocation when you can’t get it back in easily. Don’t play with your joints! One day it may not go back in, seriously! (Speaking from experience, ahem.)

Although this post will smack of my When Else to Suspect EDS list, it’s slightly altered with a mildly different focus on things we may not realize aren’t common to the majority of the world around us. This is likely because we are surrounded by like minded and bodied people in both our families, and among our friends – many of us find we are highly attracted to each other platonically and romantically. Whence our kids often get diagnosed first, they pull the “short straw” and get it from both sides, right?

But don’t stop mating, just be mindful as you do – we’re increasingly finding the gifts that come with it, and I believe with improved nutrition and medical support, we truly can have improved quality of life so that it’s not a burden for anyone. Just lower your inflammation, boost your nutrition and detox your lifestyle as much as possible pre-conception for the best outcome for all. We are an incredibly gifted bunch, some call “twice exceptional”.

But I digress…

Some other common “party tricks” we “zebroids” often enjoy:

  • reverse Namaskar sign ability (can put arms in “prayer post” behind your back)
  • Gorlin sign – can touch your tongue to tip of your nose
  • ability to flip your eyelids inside out easily
  • very flexible ears
  • great gymnastic or contortionist ability (can do the splits easily)
  • stand on the sides of your feet
  • W-sitting” (don’t do it!)

Other things that are common for us, but not “normal” for the majority of the human population include:

  • Weakness or being prone to more injury on one side of the body especially, not necessarily your dominant side either
  • Piezogenic papules (isn’t that a great word?) or “fat papules” on your heels
  • Highly visible flaccid bulging veins in hands and feet that “balloon” out (fill) with gravity or high BP / temp but conversely will recede/disappear (empty) quickly upon raising limb over head
  • Running much hotter or colder than others on average (usually colder)
  • Trouble falling and staying asleep
  • No filter – trouble with interrupting others a lot (look into ADD/ADHD)
  • Sensitivity to vibration, sunlight, temperatures (hot or cold), stress (see MCAD)
  • Some can injure easily without significant pain to warn them it’s happening. (Conversely, others of us gasp like a fish in pain at the slightest touch alas. Neither extreme is good.)
  • Blue tinged sclerae (a bluish tint or appearance to the “whites” of your eyes)
  • Highly sensitive INFJ personality and senses, score high on HSP scale
  • Lots of esoteric, “out of the ordinary” experiences, ESP, telepathy, psychic ability, or may identify as Indigo Children
  • Tendency to carry an electric charge and “blow” surrounding electronics and light bulbs. Also experience static shocks easily in winter, or almost any time. (Use vacuums carefully, they tend to build up static in us. Try to grab a desk, chair or other piece of metal before grabbing your phone or laptop to “discharge” this first and save your gadgets. Also try to eat lots of veggies or drink alkaline water to balance your acidity which lends to this.)

There are more, but I’m just throwing these out off of the top of my head this afternoon. I’ll try to add to and flesh out this post a bit later in time as things bubble up. I was just inspired to share this after a great conversation about one side of our bodies often being weaker for some of my Twitter followers today.  (This was a revelation for some.) It prompted me to think “what else don’t people realize is attributable to hEDS?” And voila, this post. (Welcome new followers here and there!)

No, not everyone will experience all of the above. That is never the case. (Boy are we a highly black and white thinking bunch!) Just as no solution will work for everyone either, it’s as individual as fingerprints. These are just some common themes I see crop up in the very large online support groups (over 20,000 on Inspire now) periodically.

Finally, I complete a post before midnight! Mark it on the calendar.

TTFN,

Jandroid

 

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Finding Your MCAD Triggers

illustration of a hand-held magnifying glass

Life-long MCAD detective

As many of you know, we’re unfortunately finding Mast Cell Activation Diseases aka “MCAD” of all kinds (forms of masto or the newly recognized MCAS) to be relatively common in Ehlers-Danlos patients, which is why I’ve written about them at length.

I’m so grateful to Patricia Murray-Wood for following me as @Mastocytosis on Twitter back in 2012 helping me to fall down this additional rabbit-hole and explain a whole other chunk of my symptoms and life experience that didn’t make sense from a purely physical standpoint of a “genetic collagen defect” (aka EDS).

Like, how could defective collagen cause me to have anaphylaxis to iodine contrast dye and various anesthetics and bees? Or cause my blood pressure to suddenly drop, have hives or itch madly in response to various foods or chemicals? Among many others symptoms and issues. Here’s a partial list here:

  • Hives (including from stress) and/or urticaria pigmentosa (a form of cutaneous masto)
  • Easy flushing red of any part of the “mantle” (upper chest, neck, face), sometimes just in blotches
  • Angioedema  experienced as swelling sometimes with a bluish tint almost anywhere, but around lips, eyes, and belly are most common. Unlike gravity driven lymphedema it comes and goes, and isn’t usually bilateral. (I.e, the swelling around your ankles is likely lymphedema, secondary to weak tissues.)
  • Esophageal spasms
  • Sudden onset nausea, diarrhea or constipation (and quick flips between these) aka “IBS”
  • Traditional allergy symptoms (itchy, runny nose, watery eyes, asthma, etc.)
  • Sudden spikes and/or drops in BP
  • Sudden shortness of breath, bronchospasm or unresponsive asthma (it doesn’t respond to inhaler, or your throat starts to swell shut and close off your airway)
  • Sensitivity to alcohol, mold, heat, cold, stress, vibration, sounds and EMF
  • Increased cerebral-spinal fluid pressure (“brain juice” headaches)
  • Any and all signs and forms of low or high level anaphylaxis including sudden onset anxiety or irritability and other mood swings or sudden mutism especially in children

Again this is a very small, partial list of all the ways mast cell activation can afflict us. Dr. Lawrence Afrin’s new (2016) book Never Bet Against Occam goes into detail about the myriad potential ways it may be manifesting and getting missed by driving other better known diseases currently. (Highly recommended reading.) I ultimately found it to be driving my high cholesterol and raised BP for a few years. Both have dropped since removing various triggers. I think the spotted chameleon should be our mascot accordingly, it masquerades as and drives so many other diseases and issues including psychiatric.

Bottom line, while “true allergies” require an IgE-mediated response to a protein such as a food or pollen of some kind (I grew up with horrific IgE-mediated grass-pollen and cat allergy myself), we can react to so much more that can’t be easily measured by the allergist, including chemicals like fluoride and chlorine, scents, smoke, dyes, foods (in absence of IgE reaction), sunlight, vibration, temperature, pain and stress. 

People with female-predominant hormone panels have the added double whammy that not just one but both female sex hormones estrogen and progesterone are mast cell triggers for our lose. So all those migraines you get around “that time of the month” if you cycle? Likely mast cell induced. Though men and boys are definitely not exempt from the “joys” of MCAD, not at all, and can be just as sick too, from other triggers. (And some have more estrogen and progesterone than they may realize also.) So there’s plenty of misery to go around alas, sorry. I highly recommend joining The Mastocytosis Society to find local support for same if you haven’t already.

We also tend to fall into two general “camps”: leakers or shockers. (One or the other.) Though even leakers can and will “tip over” and shock once their histamine and/or stress levels are high enough, we tend to avoid needing to use our epi-pens much if at all knock wood. You shockers tend to know who you are: you’ve probably been to the ER several times, and either are a master at epi use, or are about to be. Epinephrine via these auto-injectors called “epi-pens” is what is prescribed for administration to break you out of anaphylactic shock. This is what school nurses are constantly on the lookout for in our kids. Talk to your doctor about your proper use, it varies for everyone.

Leakers may still experience any and all of the signs and symptoms of anaphylactic shock, but at a much lower and slower level or rate (over hours to days) such that it will rarely hit the medical radar as “shock”, but more likely be seen as “anxiety”. Shockers will tend to lose their airway due to constriction from a swollen tongue or airway and or have such radical and rapid BP drops leading to unconsciousness that fewer medical personnel question them. I.e, they experience all the symptoms just much harder and faster (in minutes to an hour or so at most). So they tend to need to epi more to avoid dying.

Reactions in everyone can be “bi-phasic”, meaning, after you’ve quelled one, another may arise right after, which is why epi-pens now come in 2-packs by default. Make a mental note to keep as calm and trigger-free as possible if you sense a reaction starting, or have just finished one – you are likely to be easily triggered for a day or two after. In fact, some will have “rebound” reactions for a week or two after for this reason. Again, talk to your doctors about when it’s right to medicate and how for you.

In all cases, keeping your overall histamine levels down in your body tends to help the most: it reduces the rate at which we react, both in quantity and severity. But some poor folks will just shock more easily than others for as yet unknown reasons. There’s no lack of research to be done in this field. (Could be more Kit mutations? They’ve only been able to finger one for anything consistently so far.)

That said, once I realized what was driving some of my symptoms in addition to my weak connective tissues making my joints come apart, I set out to identify my triggers, as I realized that that was going to be the best way to reduce my reactivity in the absence of sufficient medical care. My HMO does not carry two of the better known mast cell stabilizers: Gastrocrom or Ketotifen, so I’m on my own with OTC remedies for now, whee.

I got lucky, and got the Basic e95 IgG/IgE panel done by a naturopath I saw in late 2012 to address my increasing “IBS”. Thanks to likely leaky gut syndrome (I’m positive most of us have), I was reacting to all the foods I ate the most, sigh. So I was facing a radical “elimination diet” beginning in 2013. But first my aunty took sick, so I went to visit her. While I did, I learned about the GAPS diet for helping to heal leaky gut online, which would accomplish the same thing: eliminating all the foods I was reacting to, and in a bonus, maybe start helping to heal my gut. So I opted to try that instead, in a slightly more radical move than my ND was asking.

Well guess what? Within a week of starting it, my brain fog vanished, pain reduced 3-4 points overall, sleep improved, flushing reduced, IBS reduced (though has never fully cleared up), hives reduced, blood sugar evened out, and I shed 20 extra pounds in 2 months without going hungry or even trying! (Note: I had them to spare. I also then maintained an optimal weight for my size afterwards, i.e, I didn’t keep losing until later, when I started reacting to chlorine.) Oh, and my cholesterol dropped. I can’t think of a single med that could do all of that, can you?

That being said, it is a very labor intensive full reverse elimination diet to try, and requires loads of discipline, both your own and your family’s if you have one. I’m lucky to live alone, so only had myself to worry about and cook for (thank heavens). But man was that experience worth it: you don’t know how inflamed you are until you actually eliminate it!

And since it took me down to just one safe food: a bone broth of choice (chicken for me, I react to beef), it was easy to see by very slowly adding foods back in just one at a time, only once every three days at most, just what I was reacting to. If you add foods in more quickly, you can’t tell if you’re having a primary reaction to the second food, or a secondary (bi-phasic) reaction to the first. And you want to keep your diet as broad as you can to maintain nutrition. So you have to be very disciplined as you conduct this: not easy with kids! But it’s one of the surest and fastest ways to figure out what foods you are reacting to if you can.

Also, be sure to eat only organic whole unprocessed foods that you prepare yourself without any boxed or bottled ingredients if at all possible. I.e, scratch cooking all the way. If you cook a chicken, but slather it in some kind of zesty sauce with 10 or more ingredients in it, you’ll have no idea what you’re reacting to. That same chicken with olive oil and salt is a lot easier to distinguish as a trigger. And yes, organic vs not makes a difference: I just reacted to some cheap inorganic chicken I bought the other day in desperation for the first time in four years. So yes, the extra money is worth it if you can squeeze it out. And trust me, I’m on SSD and food stamps, so it wasn’t easy, but it’s been worth it in lowered reactions. Pay the farmer now, or the doctor later right? (And also, what did the chicken eat??)

The alternative to this is doing a food journal and or trying to eliminate one food at a time. The problem with this is, if you are reacting to many things or eating complex meals with lots of added or processed ingredients (boxed or canned or bottled foods), you may never figure out the real triggers. Or it may take a lot longer to do so. I.e, you can eat the simply cooked rosemary chicken you make, but when you eat your friend’s zesty seasoned chicken or with some bottled barbecue sauce, you react. Was it the chicken? The seasoning packet or the bbq sauce? Which ingredient?

If you just eliminate gluten for instance, but are eating freely otherwise, including processed foods, who knows which of the hundreds or more other ingredients may be triggering you? Sure, you may feel a little better off the gluten, but this improvement may be masked by reactions to the tomato sauce you still use (with preservatives) or who knows what else.

And, you could be reacting to the fillers in your medications (keep a journal of those too), or dyes in the foods or meds, or scents, or hormones or… you get my drift? The closet detectives among you won’t ever be bored, smile.

That said, here are my best tips and tricks and clues to watch for:

a) Start any new medications (and foods) one at a time if at all possible with your doctor’s permission, so you can differentiate what you are reacting to. This includes vitamins and any other “natural” over-the-counter (OTC) supplements, no matter how seemingly benign or beneficial. We can truly react to any thing, and do. Avoid multi-vitamins and “combo” supplements as a general rule for the same reason: figuring out which component you might be reacting to if you do. (Alternatively, figuring out what’s working!)

b) Try to keep a food & med journal, even if you do a full reverse elimination diet like the GAPS diet I described above. Even if it’s just for a month and just says “Wed Dinner: lasagna”. You’ll learn more in that one month that you might in a year otherwise. What’s 30 days compared to 30 doctor visits? I’ve saved thousands by this one move alone.

c) Notice your moods and stress levels in general at all times: are you fighting with your partner/kids/employer/teachers over anything? Did you lose a job? Struggling not to? Getting divorced? Pregnant? Sick? Anything that alters our stress levels can make us more reactive in general and on average than at other times. I will get esophageal spasms if you stress me while eating almost anything, including “safe” foods. But I haven’t gotten any in two years now since figuring this out and lowering my stress and histamine levels. (Be sure to rule out eosinophilic esophagitis as well if you also get spasms like that – I’m negative.)

d) Track your monthly cycles if you have them – we are often much more reactive around our periods, especially right before they start, when progesterone rises. It’s the drop in progesterone that triggers your cycle in fact. And both estrogen and progesterone are mast cell triggers. And progesterone makes us more lax – whence all the clicking hips and knees and extra headaches and pain we experience then. Combine getting emotional from the high histamine from likely histadelia and we’re sometimes sunk, sigh. Many female hormone predominant persons finally get diagnosed with either hEDS and/or MCAD of some kind at peri-menopause too if they weren’t picked up at puberty. And peri-menopause or radical hormone changes can happen almost any time after age 30 I’m finding.

e) Avoid high histamine foods. There is ongoing controversy over what constitutes “high histamine foods”, and I refuse to provide any lists accordingly. I will only say in general: avoid ground meats (try steaks instead of hamburger), avoid fish more than 6 hours old that isn’t flash frozen (even if cleaned, but especially if it’s not), avoid leftovers or freeze them until needed again, avoid aged cheeses and fermented foods and drinks, all of which are naturally high histamine. Yes I struggled to take the sauerkraut juice in the GAPS diet, so resorted to an oral probiotic my ND recommended. I like to joke that my last meal will be beef lasagna and red wine… 😉

f) Watch for constant inflammation: if you find you are starting to “react to everything you eat” and have trouble eating almost anything at all, this is a sign it’s something you are ingesting almost all the time, likely daily. In my case, I finally slowly figured out I was reacting to the chlorine in my tap water, and the fluoride in my toothpaste. (This happened after swimming 2x/week for 3 years in a row post “storm”.) I ended up dropping down to 106 lbs without trying and my doctor begged me to “please eat”. Uhm, dude, I’m a former foodie who ate her way through France back in 2001: why would I suddenly stop liking food? It stopped liking me! (I’m better again now.)

Another friend just started reacting in a similar way (unable to eat almost anything) to potatoes she had been eating daily recently from stress, and a food journal helped her determine this. This is when you have to play detective and suspect… every and anything.

g) Notice your environment: are you surrounded by the smell of cooking grease from nearby restaurants who may be preparing your triggers for their patrons and wafting them into the air? Do you use scented laundry products? (Do your neighbors?) Is there mold in your walls or ceiling or carpet? Are you really sure? It’s often hiding in air ducts, which is sadly hard to find. Even if you can’t do anything about it (e.g. clean the ducts or move easily), just knowing this will help you to understand why you may have a higher baseline level of reactivity than someone who does not have these constant environmental triggers around.

h) Avoid sugar. Seriously. I know, our modern western diets are infused with it, and cupcakes are the latest craze. But it is truly our enemy on so many levels. It increases candida in our guts which we are prone to, and feeds the wrong gut bugs, lending to increased leaky gut from increased zonulin. (So does gluten.) It also increases pain levels and potential tissue damage, which is the last thing anyone with a connective tissue disorder of any kind needs right? It is highly inflammatory and they’re starting to recognize that sugar and carbs may be driving high cholesterol now, not healthy fats.

So, if the above list was too long for you, I’ll shorten it to this:

  1. Eat a whole foods diet, as much as possible, preferably organic. No processed, boxed, bottled or canned foods with more than 2 ingredients. Ideally organic and fresh. This way you know what all you ate, vs guessing which ingredient of the 20+ per sauce or packet you just added. Avoid sugar and leftovers. Yes this is labor intensive, but you’re worth it.
  2. Keep a food & med journal, and try to eliminate known triggers either wholesale (e.g. GAPS diet), or one at a time, so you can track and notice any patterns in your reactions and your meals. E.g., your lungs fill every time you eat tomatoes, or you get diarrhea every time you drink coconut water, or you choke every time you walk by the dryer or into the bathroom. Try to rotate foods so you can avoid being constantly inflamed by anything and also get a varied, nutritious diet. Watch out for accumulating effects. Include medications if can – folks with corn sensitivity or allergy may react to the corn starch fillers in many capsules e.g. Note whenever you start a medication or supplement, try to only start one at a time and wait at least 3 days before adding or starting another, at the lowest dose possible to watch for reactions and tell your doctor about any and all supplements you’re adding. (They can interact with medications.)
  3. Be mindful: observe your self and your environment. This can be harder for those of us with commonly comorbid ADD and OCD – our brains often get either distracted too easily and/or “stuck in a rut” (hyper-focused) and we fail to notice that we’re getting really hungry, or PMSing, or cold or ______(fill in the blank). (I speak from experience.) Yet we take amazing care of our children and other people. Just add yourself to that list, and in first place. Do this to avoid stress, or to notice if you’re too hot or cold etc. which might be contributing to your reactions. (E.g. I can eat chicken when calm, but get esophageal spasms if stressed or rushed.) Try to eat slowly and mindfully, not in a rush or while watching TV etc. Obviously we won’t succeed all the time, and many with children just plain can’t much, if at all I know.

I had my first sudden airway spasm experience a year ago in the summer after eating out at a friend’s house for a high histamine stir-fry dinner. We then topped it off with ice cream with a touch of alcohol on the side for dessert – a no no for me. Then we got in an argument and I realized too late I was over-reacting from the dinner triggers – sudden mood swing, hello! (Some brilliant person in my support groups calls this the “histameanies” – genius!)

So left in a blur of apology and cried really hard all the way home on my bike, harder than I’d cried in years, such that when I walked into my unexpectedly coincidentally smoky apartment hallway that evening, I instantly reacted to the smoke in the air. Someone had burnt their sugary dessert and the hallway was all filled with the smoke from that. My lungs instantly went into a spasm leaving me unable to breathe properly (I could only emit a short barky “cough”), and I realized I had to get out, and at a minimum take Benadryl and get my epi handy. Thankfully as soon as I got some clean air and swigged some Benadryl it eased right away, but it made me realize two things:

  1. Why I try to stay calm as much as possible and notice notice notice how I’m doing (and why). My friend had distracted me so much with conversation during dinner that I failed to notice. No wonder I reacted so badly upon encountering the smoke in my hallway. I might not have reacted as badly/quickly if I’d been calmer just prior. No, we can’t always help this, but it was very helpful for me to know what was going on – and why. I was able to avoid a trip to urgent care or the ER with this insight and self-management. (Again, if you are going into full shock, or have full signs of anaphylaxis for YOUR BODY – please call 911 / 999 or a friend and use your epi as directed! I was lucky to stay just below my “bar” for using epi that time.)
  2. Why I carry my epi-pens and liquid dye-free Benadryl everywhere, even though I’m not a “shocker” and rarely need them. You just never know when events will conspire to “tip you over”. (What if you’re in an auto-accident? Or trip and fall? etc.)

All’s well that ended well obviously, and I haven’t had any “full” anaphylaxis since some surgery years ago thankfully. My first was to iodine contrast dye in an IVP administered at 14 when they forgot to ask if I was allergic to bees first, oops. But it was a sobering experience, and made me really glad I spend so much time in the kitchen now, cooking nutrient dense low-histamine foods as much as possible. And no, I don’t always succeed. But the effort has been worth it, as much as my very limited fixed budget allows.

I hope the above is helpful as you thread the fine needle of life with any form of MCAD.

Jan

 

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Think marathon… not sprint, when trying to diagnose and treat a connective tissue disorder. (Large doses of patience and persistence are in order at all times.) – Jandroid

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Reframing autism and more

Stylized graphic of a flower made of question marks with a red center on top of a growing black plant vine

Who knows?

I know, you came here originally to learn about a connective tissue disorder called Ehlers-Danlos Syndrome. I originally came here to write about a connective tissue disorder called Ehlers-Danlos Syndrome after experiencing a massive onset “cascade” or storm of symptoms that got me diagnosed finally at 45 in 2012.

But like Alice down the rabbit hole encountering the fickle Cheshire cat and Queen of Hearts and even the Jabberwock, I quickly encountered several other major comorbidities, including the nebulous, hard to define and even tougher to qualify condition we currently call autism.

Not autism spectrum disorder, or disorders anymore. But just… autism.

Because thanks to the recent Awaken to Autism Summit (as I’m writing this originally November 15th, 2016, or “A to A” for short) and some other input from “out there” and daily Reiki, I am realizing we need to stop thinking of it as a disorder or disease, and start recognizing it for the package of gifts it embodies. Just very well disguised to our western allopathic pathologizing eyes as a “disorder”.  My own eyes included, formerly. (I’ve pulled my original post on autism and EDS now for this reason, so don’t go looking for it.)

Along with all of the so-called “mental illness” we are striving so hard to cure with modern pharmaceuticals. How about we start calling these conditions “mental differences” and leave it at that, without all the judgment and stigma inherent in the terms “illness” or “disorder”? I’m increasingly convinced that all who experience them are on a healing path, possibly even a shamanic journey and have a lot to teach and offer us, if we would just listen. I think this is why we see so many troubled artists and musicians – that is what begets the art, which is oft times the only way they might express themselves.

No matter how hard we try -and I’m seeing some noble efforts on social media including Twitter (The Buddy Project, more), anything including the term “illness” or “disorder” in it is by definition going to trigger a negative connotation to it, no matter how loving the intended audience, or speaker. (Doctors, parents.)

The A to A Summit opened my eyes to the incredible gifts and spirits of the adults and children with autism arriving daily. And not just some of them – all of them. Including or even especially non-verbal ones like The Horse Boy and Joao Carlos Carlin. Or medical mediums like Anthony William.

Sure, not all of you may have found your child’s gifts yet (or even your own), or balanced their environments and spirits enough to stop head-banging enough to even enjoy a calm after noon much less feel “gifted”. But I’m willing to bet we’ll find something there if we look. Even in the most “difficult” individuals. And I don’t just mean developmentally so. I mean, emotionally so.

I.e, our borderline patients, who often play victim to gain attention more than necessary, or bipolar patients who struggle to self-regulate their moods, up or down. They are not broken. Just imbalanced, in multiple ways that can be re-balanced with proper attention to diet and lifestyle and their true selves. I.e, finding the diet that supports your body best, not just the most convenient or desired. (Yes, this is hard if your child only wants white foods, e.g. or you only have access to processed foods due to budget or other constraints – my heart goes out to you. Try any way you can to improve your nutrition or your child’s.)

And being in touch with your inner self, finding what you really want, not just codependently giving yourself away to any and everyone who asks. (Been there, done that.)

And there is increasing evidence that most of what ails us called “depression” can be eliminated through detoxing our homes and bodies and improving our nutrition in a bioindividual way as described by Trudy Scott and Kelly Brogan MD.

And I am willing to bet every one of them (us) has some sort of artistic or creative or other gift to share with the world if we would just let them. I have yet to meet one single EDS or other patient with either an outright diagnosis or signs of various so-called mental illnesses or disorders (as of 2016) who doesn’t have some kind of amazing creative gift or message to share if they can get it out. Truly. You all are the single most creative and talented bunch I know. Just not always well focused alas.

Don’t misunderstand, we still want to alleviate suffering. Working to heal and treat the body is still key to our well-being and will go far to help do so. But may not be the whole picture. At the risk of alienating a few of my audience I now think we’re just missing part of the driver for it, something we still rarely speak of: our spirits.

I say this as a Reiki Seichim Level III practitioner of 3 years now who has had her own mild psychic experiences, one hell of an intuition all these years (it’s always been right, always!) and a deep sense that I’m here for a reason besides clocking in 9 to 5 or beating computers into submission on a schedule. (My former living.) Along with my own artistic vision and intellectual talents. (A semi-photographic memory and highly pattern-seeking and seeing brain, though I know I don’t hold a candle to many of you even still.)

The more Reiki I’ve done (I treat myself daily in the morning for 20-60 minutes), the more amazing insights I’ve received, including the above. The more Reiki I do, the more answers I get and resources I line up – almost without trying. It’s like someone’s helping me with all of my challenges and chores every day, and I’m finding solutions to all of my “problems” one by one. I’m also about to achieve my 50th birthday wish: making my last Lofstrand crutch disappear. Almost. I’m so close I can taste it.

I can’t quite walk free hand fully yet. (I fail on hills and stairs yet.) But I’m this > < close! (So exciting! I feel like a giant overgrown toddler, lol!) But, even more importantly, I’m also totally at peace with my new life post disabling onset “storm” of 2012.

And I’ve seen numerous posts in all of the groups through the years about people having semi or fully psychic experiences, or “sensing” things, and everyone complains of being “too sensitive” or empathic. (And how.) Many are self-described “Indigo Children” and upon looking into it, I had to admit I feel like one too – just older than most. (I thought I was too old until one of my sources nixed that, saying we started “coming in” in the last century, so it’s apparently still possible.)

I’m decidedly not “New Age” except for practicing Reiki, but I feel myself ever so slowly turning toward that world, and am increasingly convinced we all have a really rich inner world that drives a lot of what we enjoy or not. Why this goes with our broken bodies, I’ll never know. Are we all “old souls” who can “take it” perhaps? All shamans? I have no idea – yet. But I’m starting to form one, the more I observe.

I know, the more pedantic and pedagogic among you will be hopping up and down since not only do I not have scientific studies to back this purely anecdotal observation, but… it’s about something bordering on the esoteric. And we left-brainers tend to eschew the esoteric – until it bites us in the bum! Which I think is starting to happen to me. My Reiki is not only slowly healing my body, but helping me to “see” so much more and connect so many dots.

And here’s the thing – while some of you will outright fully reject what I’m saying, I do believe a majority of you may quietly nod secretly to yourselves about your own “esoteric” experiences with psychic phenomena and life as an empath. With SPD. And OCD. And ADD. Yes, one heck of a package! 🙂

Bottom line: autism etc is perhaps not to be “cured” so much as supported. We are not broken, but “twice exceptional” as someone smarter than me wisely described us. And if the experiences of the A to A presenters are to be believed (and I do believe them, I got chills watching), once we start to listen and follow each other, and stop trying to force everyone into the same learning (and medical billing) boxes, we’ll all do better.

That’s not to say that the most afflicted among us don’t need plenty of medical help and support. But it’s not the same as saying “cured” outright. As in, not being you. (And yes, I’m alluding to the totally non-verbal, sometimes violent children some get to support.) My heart does go out to the parents having to deal with this.

But I’d like to start promoting autism acceptance as this site does so elegantly and eloquently, not just awareness, which implies a problem and pathology. And stop trying to “prevent” it entirely, as I think that may be a futile task as I think it may be our evolutionary response to our current environment which we cannot quickly change. Perhaps we have just too many years of accumulated stress being passed down in our epi-genetics?

And needless to say, with all the nutritional imbalances and deficiencies we find from underlying connective tissue trouble, we’re likely in for a lot more such varied neurology and biology. Yes, especially in the so-called “First world”. It is precisely our disconnection from nature and original foods that I think is helping to drive this. (I may be wildly wrong, who knows, your guess is as good as mine, since that’s all we can still do: guess.)

Just eat real food, folks! According to your sensitivities of course. No more boxed and instant microwave meals, however convenient. Trust me, I spend a bulk of my time in the kitchen now, which is why I’m not writing more, I’m too busy cooking and cleaning for myself, but it’s working.

My hair and nails have never grown better or longer (heck, they’re actually growing for once!) And my tendons and ligaments are every so slowly holding me together better and better (whence my walking dream coming true).  And my pain is half of my 2012 levels, which is to say – way more bearable most of the time.

Does everyone with EDS or Hypermobility Spectrum Disorder have some form of autism? Hard to say, but I’ve observed one heck of a high comorbidity rate unscientifically in all my large support groups on and off line. (Are you a Hidden Aspie? I feel like one.) And vice versa, except that the autism world is still so highly focused on behavior, they’re not connecting the physical ailment dots and seeing the hypermobility yet. (This site just caught on recently thankfully.) All I can say is, the Autism Intensive Summit last year sounded a lot like they were describing EDS and MCAD patients to me. And everything that worked for them worked for me in my recovery. But that could just be one happy coincidence.

But so did this latest Awaken to Autism Summit. (Sound like EDS and MCAD patients to me.) Consider me awake. I think I get it finally. I think this is our wake up call to clean up our lives and our environments in all ways – physically, and emotionally. (Notice higher levels of illness and suffering of all kinds in adults with higher ACE scores.) Toss in the RCCX Theory (okay, it’s still technically a hypothesis, we get it, Oh hair-splitter!) and epi-genetics, and this “package” just doesn’t surprise me any more.

Behold, the new normal. Zebras appear to be inheriting the earth. Better start calling us horses. 😉

G’nite,

Jandroid v. 3.14159265

 

 

Posted in About EDS, Autism | Tagged , , , , , , , , , , , , , | 9 Comments

50,000 Shades of Grey

ShadesOfGrayFanSorry to disappoint you erotica fans, but this post is not going to be nearly as sexy as the recently viral romance novel whose title it is reminiscent of. But I hope having lured you in, it will keep your attention just the same, as this is a topic I’m feeling very strongly about.

I’m expecting to catch heat from multiple sides for this post, as I’ll likely rattle a few cages in the process, but c’est la vie. I can’t sit on this view any longer.

That is, the difficulty I find in both doctors and fellow (suspect and diagnosed) EDS patients alike to prematurely leap to conclusions, stick to them once drawn, and strive to classify the snot out of any and everything into tiny little black and white boxes that preclude having multiple conditions, factors or issues. (I call this sticky thinking, or “velcro brain” now. And yes, I’ve been guilty of same in the past too. I strongly suspect mild ADD and OCD to lend to this proclivity in us but that’s just my personal hunch yet obviously.)

E.g., upon learning you have Rheumatoid Arthritis or Lupus for instance, promptly ascribing any and all tissue pain and issues to that disease, and failing to see the comorbid hypermobile forest for the arthritic trees. (We find autoimmune diseases highly comorbid in the Ehlers-Danlos community.)

Or getting diagnosed with Fibromyalgia and refusing to see how every single symptom and manifestation of it falls within the EDS circle of experienceevery one. Including the familial inheritance pattern and your flying bird hands you wave at me in firm denial while your veins visibly pop and you squint your eyes at me through your myopic lenses while insisting you just have fibro! 

Yet you will refuse to consider the possibility you might have a so-called “rare” (!) genetic connective tissue disease even after I run a Brighton Critiera. No, you are special, you have the hard-won (being clinical, and thus purely subjective) diagnosis of the still poorly defined (and ever -expanding diagnosis of) fibromyalgia after way too many years of suffering and pain so you will not let go of it for love nor money! (Yes, I’ll still allow for a small handful to have “just fibro” but I would dearly love to know the cause that isn’t included in the Chronic Constellation when you find it.)

I can’t say that I blame you, any of you. After all, you’ve fought hard for the diagnosis you do have (or give, if you’re a doctor). And you’ve only been told about the grossest signs (extreme flexibility aka hypermobility, frank dislocations, and extremely stretchy skin or vessel ruptures) of the rarest types of Ehlers-Danlos. And been told that it’s really rare. (The Hypermobile form is very likely not. Leading experts now cite as high as 2% of the general population may have it, since 2012.) And that you’ll be in a wheelchair if you do have it, like I was in 2012. (Hey, I’m back out for the record! Sure, I still limp, but I’m on foot again, watch out!)

But when I point out the less famous signs and run the Brighton Diagnostic Criteria backwards you still resist saying “everyone does that!” Of course you do: you’re surrounded by people just like yourself both in your biological family, and your circle of like-minded friends (birds of a feather). (We’re finding we’re highly attracted to each other both platonically and romantically, and most who are clinical enough to get diagnosed end up seeing it in both sides of their family.) And yes, this includes many doctors and nurses who I increasingly suspect also.

I feel strongly these myriad extra-articular (meaning non-joint-related) health factors and issues may well underlie why Hypermobile type Ehlers-Danlos Syndrome is so rarely diagnosed IMHO. I feel it expresses and falls much more on a continuum or “spectrum” if you will, much like the extremely commonly comorbid high functioning autism spectrum I increasingly find it also comes with.

In fact, I’m semi-convinced now that “high functioning” or mild autism (and the related sub-clinical alphabet soup (as I call it) which many also exhibit of variations of ADD/ADHD, OCD, SPD and more) is merely a symptom of the underlying altered neurology I think we’ll find common in HEDS patients one day. (They need to autopsy or image a flock of us to prove this still, but Temple Grandin‘s brain scans already hint at our saggy hind brains. Squished occipital lobes aka Chiari anyone?) I think whatever drives hypermobility also drives or lends to neuroplasticity and thus highly variable neurodiversity. (And don’t get me started on the DSM~!) But I digress.

The problem is our very binary, aka black and white, either-or, all or nothing mindsets: either you have a genetic SNP (defect) or you don’t. No other possibility. No partially having it, or having it mildly. Or having it plus anything else a la Hickam’s Dictum.

Just what would you say I had for the first 44 years of my life prior to my long-awaited diagnosis of Hypermobile EDS pray tell?? I most certainly did not “catch” HEDS at 45! It’s not contagious, like the Zika virus (thank God). I’ve had it since I was a zygote. But no one noticed the milder telltale signs as I was growing up:

  • odd growing pains we couldn’t explain
  • large head on a wobbly neck as a colicky baby
  • IBS
  • extreme flexibility as a child (I could have been in this number with the Ross Sisters)
  • nearsighted by age 10
  • crowded teeth prone to cavities despite good dental care
  • easy bleeding and bruising
  • chronic fatigue, easily tired, “weaker” than most, clumsy as a child
  • “stretch marks” on my skinny thighs at age 12
  • early onset varicose and spider veins at 14
  • trouble sleeping
  • signs of comorbid MCAS

I could go on. My point being, my condition worsened as I aged, until it finally suddenly became “clinical” enough to hit a doctor’s radar. My late father’s and paternal aunt’s health all snapped into focus suddenly once I finally suspected the condition. And then later my late mother’s health also snapped into focus (full dentures, varicose veins, phlebitis, thin skin that tore at a glance, easy bruising and bleeding, headaches, bad back, depression and anxiety, miscarriages and much more).

Apparently I pulled the short straw, and got a double dose from both sides of my family (mom AND dad). Yes, my older sister (only sibling) does also show plenty of signs (even passes the Brighton Diagnostic Criteria IMHO though she won’t let me run it on her yet).  But I guarantee no un-savvy doctor would ever suspect my sister. Not based on the current literature and information!

And I wouldn’t blame them. She’s a highly functional full-time engineer after all who rarely to never misses a day of work, just like my late dad was. (And equally stubborn, TYVM.)

So not only can you have variability in the number of alleles (copies of your genes affected, e.g. single vs double), but you can have plenty of other environmental factors that may contribute to your level of severity. Especially if the RCCX Theory proves to lie behind the most common Hypermobile form, the level of stress in the home and your life will cause great variability in severity and age of onset. (And very nicely explains my level of severity along with a progesterone storm on top, thank you very much.) Oh yeah, progesterone makes you more lax by the way. Notice any trouble during your cycles ladies? Peri-menopause? Oh yeah. We get all the fun.

I was also the child of older parents (they were 40 when I hatched), who smoked and drank while conceiving and pregnant with me, and were starting to fight (live dysfunctional alcoholic lives) when I was growing up. (I’ve already mentioned my likely CPTSD in this post about Anxiety and EDS.) So I already had a couple strikes against my health when I was conceived as well as developing. (I suffered some benign neglect along the way my sister can vouch for.)

I also feel strongly a la Hickam’s Dictum that it’s not impossible to have multiple SNPs (genetic defects) or even a mashup of types or SNPs on multiple genes in the same patient that still lend to our issues, even if not all are directly disease causing. I.e, this is some (very B&W thinking) geneticists think you can only have one type that runs “true” in the family. Based on 4 plus years of observation of several thousands of patients and their families in my online support groups, I humbly beg to differ.

The level of variability and crossover of symptoms and types is almost unlimited. (And almost everyone has mild or more signs of Vascular type EDS and thus wonders if they have it. By all means rule it out by testing if you suspect it! We ruled out IV and VI in me based on family and personal medical history and presentation.)

Sure, a handful will appear to “run true” and be easily identified with a familial Mendelian inheritance pattern (usually autosomal dominant), but not all. I’m also pretty sure these are the exceptions (truly rare patients), not the rule. And perhaps you may just be a “carrier” for a rare type, even if it’s not fully expressed in you. (Much like how Dr. Meglathery suspects many of us may be carriers of CAH, even if we don’t have CAH directly in her RCCX Theory. She calls this “CAH1”.)

And never mind the effect of epigenetics in all of this! (Not all genes are expressing all the time, or not fully, etc.) It’s really much more of a spectrum to me than a clear black and white, all or nothing disease any more. (So many variables, so little time.)

Toss in myriad genetic variations in your methylation cycles all of which I’m sure can contribute to our overall well-being and variable ability to detox among other things, as well as variable diets and environments, and it’s a wonder they even thought it was genetic to begin with! (It’s looking less so the deeper I dig, or at least, less mono-genic anyway.)

[Aside: no, I do not fully agree with Debby McQueen that MTHFR “causes” Hypermobile type EDS, but… I DO agree it may well amplify or lend to it for the reasons she delineates and that treating any MTHFR defects may well help as she found with her family. I say this because MTHFR is known to run in at least 40% or more of the general population. And so technically if it did “cause” HEDS, then 40% of the population would have signs of hypermobility – and even I don’t think it runs quite that high! (I’m willing to bet dark chocolate it’s at least 10% and maybe as high as 20% currently, but not much more – yet.)

Also, the collection of Mast Cell Activation Diseases is collectively known as MCAD, not Mastocytosis aka “masto” as she states, and which is just one of many forms of MCAD. Not trying to pick on her, just trying to clarify correct information and keep us all discerning. I do like her thinking, and am impressed with her findings and results. /End aside.]

Once again it’s late, and I’m tired, and so I’m going to leave this here for now. I may well modify this post later as I think of more juicy reasons I find EDS so highly variable in both presentation and onset, leaving it so poorly recognized still due to being mis-characterized as “a genetic collagen defect” (or even “collection of genetic collagen defects”) lending to the black and white all or nothing thinking in the medical world about it. (Just because your doctor leaps off an information cliff like a lemming, does that mean you should too?)

I’m increasingly convinced it’s likely a huge collection of collagen, Tenascin-X and other connective tissue defects with several potential amplifiers (epigenetics and methylation being the two biggies), not all of which are equally expressing. We’ve all just been seduced by the early findings from the low-hanging diagnostic fruit found to date.

Again, I eagerly await what the working groups come up with since the International Symposium met last May 2016. But I’m willing to sit on my hands and cut them some slack in light of what I just opined above – this is an extremely large “animal” they’re trying to get their arms around, and I’m pretty sure it’s a steadily moving target as science continues to catch up and uncover more types and variable features.

I understand the hesitation to include the seemingly benign albeit grossly bendy patient (like I was as a child) who is able to run around and dance and perform like some on America’s Got Talent etc. But I’m willing to bet they either have or are related to someone who has some form of EDS at the very least! And you shouldn’t have to wait 25 years from first major complaint and become wheelchair bound to suspect it. (And no, blessedly many will never have any major problems – but gee, wouldn’t it be nice to know so you can prevent any major injuries by avoiding things like roller coasters and high velocity neck thrusts at the chiropractor’s?)

All I ask is for everyone to be more dialectic in their thinking – that is, feel free to hold an opinion, but allow for other possibilities as you proceed. That is, don’t stick like glue to the first or only thing you’ve seen about the disease to date, where ever that may have been (medical school, the internet, or an outdated text book). Take it as a starting point or guideline, but listen to the patients who are living with the disease!

I dearly wish I could download all I’ve got stored in my brain after reading thousands of posts from thousands of patients for the last five years as I fell down this rabbit-hole to everyone reading. (One of my groups alone has over 12,000 patients in it now, and I’m in about 20 groups. Subtracting for overlap, I’d conservatively guess I’m seeing at least 15,000 distinct patients total across the collection all complaining of their issues – sometimes more than they do to their own doctors! And I have a semi-photographic memory, whee.)

Yes, I’m working on my book to that end, just struggling to describe this very large animal I’ve alluded to so far while lacking scientific backup for much of it. But I’m getting there. (Nearing the half-way mark as we type, finally, thankfully, ahhh… splat.)

But hopefully, at the very least, it’ll change a few mindsets to be a bit more nuanced and allow more ‘Shades of Grey’ than we’ve seen to date. I hope you’ll join me in thinking outside of the black and white boxes we’ve been pigeon-holing everyone in to date. Feel free to return to those exciting romance novels, but I defy you not to think about hypermobility now as you read them, smile.

To your health,

Jandroid

Posted in About EDS | Tagged , , , , , , , , , , , , , | 16 Comments

Patience Grasshopper

Image of a grasshopper

Patience, grasshopper

Update February 7, 2017: Via The Ehlers-Danlos Society (aka “The EDS”) on Facebook the full new EDS nosology will be published on March 15, 2017. Meanwhile, some preliminary documents have been shared ahead of time, including one that talks about the new “framework” for recognizing and diagnosing the most common, hypermobile form of EDS.

So, lots will be changing, and thanks for your patience as I slowly get around to updating everything based on the information as it comes out.

February 18, 2017: The EDS just provided this FAQ about the pending new EDS nosology to be published March 15th, 2017. Again, patience, they want to get it out as badly and quickly as we wish to see it, I promise! (They also share our pain, I assure you.)

And follow me on Twitter and Facebook for the latest always. (See links to the right.) Thanks!

Original post from 2016:

So you all probably know the latest Annual Ehlers-Danlos Global Learning Conference under the auspices of the newly formed International Ehlers-Danlos Society (aka “The EDS” who are succeeding the EDNF of yesteryear after merging with some colleagues from the UK in May 2016) is just freshly ended (July 14-16, 2016).

And those of us who couldn’t attend are all on pins and needles awaiting the outcome from it. Especially regarding how to recognize, diagnose and classify the most common form of EDS, Hypermobile Type EDS (aka HEDS, formerly EDS III) in the absence of any clear genetic markers as I’ve mentioned numerous times.

Well, we all need to be a little more patient, as the Hypermobile committee has not yet reached a consensus on the matter, and that is actually apparently all that was shared at the conference about it. (That it’s still a work in progress, being cussed/discussed, various ideas floated around but not agreed to.) So Lara Bloom, the new British co-Executive Director of The EDS (with Shane Robinson of the former EDNF in the US) just shared this on her Facebook wall to stem the tide of inquiry they’ve gotten ever since:

“Many of you have asked when you will be able to see the work that has come out of the new International working group and symposium. The article will be published in the American Journal of Medical Genetics with the new nosology and management and care guidelines in March 2017 (date may be subject to change).
The article will be available on our website for free as soon as it is published, and a lay version will also be available.
We will release the slides and audio from the conference which is the work presented at the symposium and shows the working process we are doing to get to the article.
Be patient people – we have waited nearly twenty years for this, a few more months wont hurt… I can assure you we have over 90 people dedicated to giving us the very best chance we have so that the medical professionals actually take on these guidelines.”

In their defense, it’s a really large amorphous and heterogeneous (widely varying) animal they are trying to get their arms around, all while continuing to keep the criteria stringent enough to warrant medical attention, yet broad enough to include a majority of those truly suffering with it. (Lots, as you know I believe of course.) Never mind that we can go from seemingly benign to suddenly quite afflicted as I did so dramatically in 2012 (hey, the world didn’t come to an end – just mine as I knew it!) so it may appear you can “catch” HEDS at some point in your life if we’re not careful, despite having it since you were a zygote.

The bad news, we have to wait a bit longer. The good news, as I see it, hopefully this extra time will help them to hash out a more palatable and easily understood set of diagnostic criteria than the current Brighton/Beighton soup of confusion still leaving a majority un-diagnosed in my opinion. (Or misdiagnosed with Fibromyalgia still instead, sigh.)

Meanwhile, for those who just can’t stand to sit on their hands and let the committee finish working, I have some good news. Oregon EDS group President Heather Purdin just shared a compilation of the slides shared at the Learning Conference back in May at the 2nd and latest OR EDS Conference held Saturday October 22, 2016 at OHSU in Portland, OR which I attended. These are now available here on the OR EDS group website.

She made it clear in her talk that essentially what I said above is still true: they’ve found some more one-off mutations and types, but… the majority Hypermobile type is still being cussed and discussed by the hard working Hypermobile Working Committee as Lara Bloom stated above. I know, as I just had to gently inform my friend Dr. Bravo that no, I am not invited to attend the upcoming dinner in November 2016 as I’m not a doctor, nor invited, smile. (If only!) Stay tuned everyone, all in good time.

Not enough to tide you over? Then go back to sleep or sit on your hands or go play some Pokemon Go – carefully. (Watch where you’re going!)

Nothing worth doing is ever easy, and all good things come in time. We’ll get there. Hopefully it’ll be worth the wait.

Patience, grasshopper.

Jandroid 3.14159265

Posted in Uncategorized | Leave a comment

EDS and IBS

abstract image of twisted red lines

Feeling a bit twisted up

One of the most common comorbidities of Ehlers-Danlos Syndrome (and, no surprise) Mast Cell Activation Diseases is IBS, or Irritable Bowel Syndrome.

Yes, we’re going to talk about our innards and toilet habits folks! This post is not for the squeamish. But then, life with EDS is not for the squeamish, and… this is a major part of our reality for a vast majority of us, if not all. And yes, you’ll wanna settle in again, it’s another longish read.

So what is IBS? For those unfamiliar, much like the term “Fibromyalgia”, “IBS” is another “catch-all” bin condition that is what you’re left with when you’ve eliminated all other possible causes of your GI distress and pain such as the flu, Crohn’s or colitis, or a parasite, or… etc.

In layman’s terms, it is a condition where you experience any and all forms of gut pain and discomfort and can experience wide variances in  your bowel habits, from lots of constipation to sudden diarrhea – and sometimes both even in the same day. (And in one strange experience of my own recently: even at the same time – yes, truly – hold that thought.)

Yes, you might want to put down any food you’re eating for the duration of this post! We’re gonna get real. Guess what? While unpleasant to talk about, everybody does poop – or surely should, and if they don’t, they’re in for some serious trouble if they’re not experiencing it already.

What goes in must (mostly) eventually come out, and in most cases (you folks with stomas excepted), it comes out the “other end”, or your bum, as the English say. (Those with ileostomies & colostomies please be patient with the rest of us.)

So pooping is a natural bodily function, and ideally should happen once a day, if not a couple times even. (Many folks find themselves ready and able to go in the morning after their first coffee or other stimulant of choice.) Yes, some people regularly poo two-three times a day depending on their diet and gut health (biome).

But some poo only 2-3 times per week. According to this source, anywhere from 2-3 times a day to 3 times per week is an acceptable range. (Beware of annoying pop up ads on this site, sorry, turn your speakers down.)

And if you’re healthy, you will form a semi-solid “log” daily (the proper term is “stool”) that lands right around a “4” on the Bristol Stool Chart. (Ideally, they say you should poop a “5” a couple times a day. I’ve only done this a couple of times in my life, but then the deck is stacked against me as you’ll see.)

Yes, there’s a poop chart, who knew! Now you can amuse your friends and torment your doctors (or is that torment your friends?) with your new-found insight into your poo. (I have bragged about my recently regular “4” to my doctor – he should be proud – it wasn’t easy to achieve with this lousy condition!)

My naturopath told me our poo is made up of 50% bacteria in fact, so the healthier your gut biome (collection of intestinal bacteria we live with that helps us to digest food), the healthier (and better formed) your poo. (And usually easier to eliminate or emit.) And generally, a high natural (vegetable) fiber diet with plenty of water (not soda or dehydrating coffee) will help with this too.

But hold that thought. No surprise – where there is Ehlers-Danlos, there are always exceptions! (If you’ve been reading my blog for any length of time you’ll be familiar with this drill by now. We put the “syn” in syndrome, and blow just about every curve you can find.)

Unsurprisingly, I was diagnosed with IBS early on in my diagnostic journey, not long after my appendectomy at age 20 in college. (I’ll never forget that, I tried to blame the dorm food – no takers.) The IBS pain I first experienced around age 23 started low and in the right quadrant, right around my appy scar. So we naturally looked for adhesions with ultrasound, but found none. This was in the early 1990’s too, mind you. (We knew a lot less than we do now back then.)

As I was a mildly stressed college-age female from a rough childhood in an alcoholic home, I was quickly written off as just “depressed with IBS”. Which wasn’t entirely inaccurate, but I now know many more of the drivers of both conditions, which I’ll now gladly share.

The medication given didn’t do much at all for me, so I ended up giving it up (probably wise in 20/20 hindsight). This was also true for the SSRI’s I tried through the years – in fact, one (Wellbutrin) made me mildly psychotic once. Yet, because doctors have SSRI hammers, everything is an SSRI nail.

Yet the pain never subsided, and in fact, slowly grew to encompass my entire lower innards, from the duodenum on down. (I now hurt from my stomach on down, constantly.) I suffered wildly varying bowel habits, with regular bouts of constipation punctuated by sudden bouts of diarrhea with lots of bloating and gas on top, and rarely the occasional “balanced” day or two in a row.

I ate a very “Standard American Diet” at the time, including lots of bread and other carbs (pastas, cakes, cookies), even though I probably ate more fresh and cooked veggies and less candy than many. (I was brought up on a very mid-western style/German home-cooked meat and potatoes diet with some veggies on the side and not much junk thankfully. Eating out was a treat.) I did also drink a little alcohol – not a lot, thanks to my childhood experience. (I no longer drink at all – it’s a trigger now.)

Needless to say I learned early on about adding fiber to battle the constipation but ironically found doing so swung my system wildly to the other direction – and too much so. I loved gardening and included plenty of my own fresh veggies and tossed in a decent amount of fruit though I found it hard to digest. But nothing seemed to break this mad GI cycle, and I suffered mostly silently through my young adulthood with no true relief.

At one point I had both upper and lower GI studies done, only finding I had a mildly slow transit time, but nothing further. (No ulcers, diverticuli, etc. knock wood.)

I slowly learned to navigate my diet and found a combo of carbs, meat and veggies that helped me achieve a slightly more “normal” elimination schedule and experience. But I never got any good answers to this problem and stopped consulting doctors who unfailingly prescribed…. yup, SSRI’s. (If only I could have seen a naturopath 20 years ago!)

Fast forward to early 2012, and my sudden EDS onset “storm” or “cascade” that took me from walking to wheelchair in just 3 weeks at almost 45 years old. And suddenly I began to find some answers to all my issues – including, no surprise, my IBS.

Not only did I find it to be extremely commonly comorbid in the EDS support groups I was in (on and offline), but also in the comorbid MCAD support groups I ended up in also. I also got to consult a naturopath (finally) in latter 2012 who recommended both finding all my food sensitivities via the Basic e95 food panel (blood test), and taking a very expensive but comprehensive therapeutic level probiotic she highly recommended as the only one that gave consistently good results in her experience.

Needless to say my blood panel came back showing I was reactive to all the foods I had been eating the most – especially eggs and gluten and beef. But also tomatoes, dairy, yeast, and more. So much for my fried eggy sandwiches! (I’ve since done an IgE test and it was negative, so all of these are IgG driven obviously, knock wood. I’d have been much more anaphylactoid otherwise I’m sure.)

My ND admitted it might be hard, but urged me to try my best to go on a full elimination diet after the holidays in January 2013. (That is, eliminating all the foods we found me moderately or more sensitive to.) I was interrupted in this plan by the need to visit my poorly aunty (a Catholic nun) back east when she suddenly entered hospice in a coma in February 2013 and I flew back to be with her for two weeks. She rallied and I just saw her again for her 70th Jubilee (at almost 92) but needless to say, I had to eat whatever I was offered as the guest of the convent, so all dietary bets were off.

However, I’d landed in a great holistic EDS support group online in the meantime, where I was exposed to all kinds of great functional and holistic medical info, including an introduction to leaky gut and the much touted GAPS (Gut and Psychology Syndrome) Diet for fixing it there. (There are other diets, and you should work with your chosen practitioner – whether ND or MD to find the right one for you.)

Upon looking up leaky gut, I realized this made a lot of sense in light of not only our weak epithelial layers, but all of our likely comorbid gut dysbiosis and proclivity to gluten sensitivity. Which some say gives everyone leaky gut whether they know it or not.

I quickly realized the GAPS Diet would kill two birds with one stone: start to help heal my gut, and definitely eliminate all my food sensitivities! So I started it in earnest upon returning.

The GAPS diet takes you down to just a bone broth of choice to start, then you slowly add only whole foods back in just one at a time, only one every 3 days so you can truly detect any reactions and sensitivities. The collagen filled bone broth helps soothe your poorly gut and help it start to heal. Absolutely no hard fibers or roughage allowed for several weeks so the poor thing can start to truly heal.

Lo and behold – I found my pain reduced, gut calmer, sleep improved, brain fog improved, memory improved, and so much more. Not least of which was elimination improved also. Who knew what all inflammation could do until you eliminated it. (Well okay, all you smart functional medicine types out there, I know I know. Bear with us while the rest of us catch up!)

I then took a webinar on leaky gut and Small Intestinal Bacterial Overgrowth (aka SIBO, pronounced “see-boe”) and learned that you can have a stuck ileo-cecal valve between your large and small intestines that can allow gut bugs to back up into your small intestine lending to this overgrowth.

They proceeded to describe how you can actually manually massage your ileo-cecal valve closed if it’s open, but I haven’t had the nerve (er, guts? sorry) to try this myself. But it struck me that this is likely true in folks with EDS – who have weak valves and connective tissues all over. I’m betting we are the so called “tough cases” these guys talk about so much.

This excess of bugs there then also lends to leaky gut as the little buggers produce something called (I’m not making this up!) “zonulin” (so Trekkie sounding, lol!) which helps loosen your tight-gap junctions in your gut, causing “leaky” gut allowing over-sized food molecules to pass through.

So instead of perceiving it as a friendly food protein, it’s seen as an invading substance and your immune system is triggered to activate and “attack” it. Whence all the inflammation, which can help drive autoimmune diseases too. Poor innocent lentil!

Did I mention that sugar encourages all the wrong/bad bugs to thrive, who produce the most zonulin? Of course they do. Cuz, EDS.

Toss in low stomach acid on the top end failing to kill enough bugs on the way down, and voila: SIBO which causes loads of excess gas, bloating and discomfort.  And lots of belching, farting, etc. Ding! I had all the signs of this also, no surprise.

Good news, you can get better with either natural herbal remedies, or in worst cases a course or two of anti-biotics to clear up the excess. Which you then want to quickly repopulate via health pro-biotics before any “bad guys” can get a foothold and produce more zonulin. Going low/no-sugar including natural sugar can really help too.

That said, I’ve not been surprised to find this a recurring problem (nay, even intractable) among EDS patients, as I’m betting our weak connective tissue lends to a chronically leaky  ileo-cecal valve constantly allowing for this back up.

And I’m trying to remember what Dr. Meglathery told me may be causing the chronically low stomach acid (possibly a thyroid imbalance if my presently leaky memory serves? I need to look this up again – hypothyroidism is rampant among us too, no surprise), but it came up while discussing her RCCX and Illness Theory this past spring (2016). I think I caught a comment recently that CRH or cortisol may lower stomach acid. Still looking for backup on this.

And don’t let experiencing GERD fool you either – it doesn’t mean you have sufficient stomach acid either. I’ve also learned that your esophageal sphincter won’t fully close until it senses enough acid in your stomach – and the stomach produces acid in response to histamine (whence H2 blockers being prescribed for lots of MCAD patients, aka Zantac, Pepcid).

So no wonder so many experience GERD with EDS and MCAD of any kind, despite actually having naturally low stomach acid at baseline. Thankfully mine cleared up as soon as I stopped drinking coffee years ago – apparently a trigger for me. I don’t miss it, frankly.

Toss in common hiatal hernias and weak sphincters all over and no wonder we’re sort of doomed digestively speaking. But wait, there’s more! (Of course there is cuz… EDS!)

Then, I learned that diarrhea and GI upset (nausea, etc.) are all common signs of MCAD reactions and low level anaphylaxis, and I began noticing and tracking many more reactions than before. Bye bye coconut, sigh.

But, between eliminating most of my triggers, upping my anti-histamines and adding quercetin (a natural mast cell stabilizer), lowering my stress, eating more organic whole foods (almost exclusively now), adding a therapeutic probiotic (more on that in a minute) and avoiding sugar, and voila: my healthy “4” on the Bristol Stool chart. Usually. 😉

Now, having said that, I got a wild hair one day this last year to eat a bunch of steamed fresh broccoli after I’d had a rare bout of constipation from a medication I took. No, not an opioid either, though those are a common problem for us. Gee, what could possible go wrong?

Uhm, well, the broccoli could turn out to be a trigger (I hadn’t had any in a long time, and not in that quantity), and I ended up experiencing something I later termed “diapation” (alternatively: constarrhea?). This was where I had the strongest urge to eliminate ever, just like I was about to have explosive diarrhea, and yet, not a blessed thing came out!! Nothing! Not even a tiny little rabbit poop. Nada. Sigh.

Oh my word, talk about anguish. The old bathroom stall rhyme came to mind:

“Here I sit, broken-hearted, tried to sh*t but only farted” (author unkown, see bathroom stall walls worldwide). Whoever crafted that is genius. And probably had IBS. And MCAD.

Normally when I have constipation, I don’t also have such a strong elimination urge. I just feel… full. Yes, I want to poop, but I can manage to function albeit unpleasantly at a semi-normal level for a day or two. What surprised me about the broccoli was the absolute certainty I had that I was about to explode with terrible diarrhea, and yet… nada. Truly like a combo of both diarrhea (the urge) and constipation. (Inability to move.) Weirdest experience ever!

Someone in one of my MCAD support groups suggested this was my large bowel dry spasming from my reaction to the broccoli. I believe it, as I felt the broccoli go all the way through my system in a complete spasm, like a rock. All. The. Way. Through. Yes, painful. Take an esophageal spasm, one of my other deep joys through the years, and extend it through the entire digestive tract, following the food bolus as it goes down.

Thankfully, I clearly at least have decent motility – that is, ability to push food through my system and out the other end eventually. Some poor folks with EDS are plagued with another complication called Gastroparesis: literally stomach paralysis. This causes your food to simply sit and rot in your stomach or small bowel, making you quite sick. Basically it’s like constipation but much higher up the system. (Aka dysmotility.)

They suffer terribly from malnutrition and malabsorption as the food never gets properly moved through the system in a timely fashion. Some poor folks end up on feeding tubes and formula just to stay alive. I’m so very grateful that’s not me! I only experience some mild early satiety and anorexia (lack of appetite, not nervosa) in general. Both worsened a lot when I started to react to chlorine in water from swimming and tap water in late 2015. I did lose ten pounds then and grew quite weak until I could eat again.

Needless to say, food sitting and fermenting in your upper GI tract will also lend to increased gas and bloating (and inflammation). So those poor folks have yet another driver to that fun. Some of this is driven by MCAD, but even more by neuropathic trouble likely from an impinged vagus nerve.

I’ve come to recognize sudden my bouts of gastric pain (gastritis) and bloating followed by either constipation or diarrhea to be typical with my MCAS reactions now. (MCAD is the umbrella term for all forms, including MCAS and mastocytosis, NB.)

And of course, as too many of you know too well, opioids induce constipation big time. Thankfully I only suffered a couple months at my worst, as I quickly found mild strengthening as soon as I started Vitamin C, so I could wean off my pain killers. I now only need one on occasion, at most 3x/week generally only if I bust a move. but no more constipation from that thankfully.

I did not take any regular pain meds besides OTC Tylenol and Ibuprofen before 2012. I now live at an average of 5 overall on the pain scale and am fine as long as I can stay there. I have some hot spots that like to spike higher pretty quickly, but I can handle it most of the time, whence only the occasional use now vs daily at my worst in 2012.

No, no fun. Yes, it’s been quite a journey. I’m so grateful I ate my way through France back in 2001 when I was relatively mildly afflicted. Not unafflicted, just less so. So when my doctor ordered me to “please eat!” earlier this year during my chlorine reaction (until I figured that out), I nearly smacked him – he should know I’m a foodie! (I love food, it just doesn’t all love me.)

Happily, I’ve returned to my new peri-menopausal “normal” since eliminating chlorine and fluoride from my life this year. (I’m also back up to a healthy 115 lbs at 5’4″.) And oh yeah, did I mention that hormone changes can make all of this worse for women? Yeah. Toss that on the steaming heap of drivers of our gastric pain and discomfort. Estrogen is a mast cell trigger, progesterone makes you more lax, whee. Girls don’t wanna have that much fun, but alas we get it! And they call us wimps…

Protip: if you gradually lose your appetite altogether -aka anorexia (not nervosa) – and struggle to get almost anything down for a period of time, it’s probably something you’re eating/drinking/are in touch with daily like chlorine in your water, or fluoride in your toothpaste and/or water. (You can get a cheap 5 stage filter from ZeroWater from many larger department stores to help eliminate both if you live in an apartment on SSD like me.)

And oh yeah, MCAD can cause sudden onset of nausea and vomiting at any time. I knew I forgot something! I’ve just had mine so well controlled for so long I’d forgotten – lucky me. But so can twisting wrong (often to the left) and suddenly impinging your vagus nerve. So many causes… so little time! Remember to solve for both x and y with this lousy condition – always!

I warned you it would be quite a journey didn’t I? Hopefully not too much for you! To those who love us and are trying to support us, thank you or being there with us through thick and thin – literally. To those of you suffering with all of this along side me, and who take every meal you keep down as a success, high five and hang in there, and keep those probiotics coming.

It’s not an easy fix, but like all of our issues, I find a multi-faceted approach to work best. Mine consists of a combo of detoxing, stress, sugar and trigger elimination (both food and chemical), an organic whole food diet that works for me (mostly GF DF and grain-free high nat sat fat paleo diet except for oatmeal now), a really good probiotic (I now take just L. Rhamnosus as Culturelle on the Cusack Protocol), lots of steamed veggies that I tolerate, some kale juice on occasion, and my daily Reiki.

Oh, and lots of water, salt and potassium along with all my supps (C, D3, calc, mag, zinc and more) so it all “sticks”. You need magnesium and potassium and salt to actually re-hydrate fully. Plain water tends to go right through us – see the RCCX Theory for likely reasons why pertaining to the CYP21A2 gene.

I still have chronic constant gut pain, but again, my bowel habits are much more regular and ‘smooth’ compared to before. I can live with this much better than the alternative and will, TYVM. All drug-free. (That is, no drugs taken specifically for “IBS”.)

I hope this helps some of you sort out some of your issues too, EDS or no. (And remember, those of you with “Fibromyalgia” are probably hypermobile too IMHO.)

Wow, I’ve actually worked up an appetite writing this. I’m gonna go eat my rations for dinner and call it a night. At least I got something done while all the fireworks were going on – outside this time. 😉

Happy US Fourth of July,

Jandroid 3.141592658

 

 

 

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