What do RSD/CRPS, Elhers Danlos and Systemic Mast Cell Disease have in common …. besides me?

Well, it’s a new year and then some. I’m sorry I’ve been away from the blog so much, but it’s been a doozy already. Since Christmas, I’ve been overwhelmed; neck deep in health challenges (new and exciting along with old and obtrusive), life stresses (major and minor and some particularly painful), research, research, and more and more and more of each. In truth I’ve been way too stressed, too scared, too frantic, too busy just trying to stay afloat…

     (though I'm not sure I looked this cute while making the attempt)

However, the research part of this equation has born fruit. Although I am still full of questions, I have some answers, and have come to some rather life altering realizations. Some of these realizations are about things I’ve lived with all my life, and how they connect with what’s become of my life. Through the course of the last few months, I’ve not only felt the deep resounding bell of connection in the many ways that these things are relevant to me, I’ve realized that some of these connections might be relevant to some of my readers. I’ve also realized that although in some, (well, let’s have some humility here; in ALL) of these areas, I have much to learn… but none the less, by sheer virtue of living with these conditions for so long, I do have much to share… and with a larger audience than I have been addressing.

 So. First of all, what do RSD/CRPS, Elhers Danlos, and Mast Cell Disease have to do with me? Well, as some of my readers already know, I have suffered from RSD/CRPS ever since being rear ended in a car accident in 1999 (and had a previous bout of it in my teens after knee surgery, which thankfully went into remission). My readers also know that treatment of this particular Beast has been complicated significantly by the fact that I have a rather severely dysfunctional immune system involving widespread, often life threatening anaphylactic and “anaphylactoid” reactions to a vast array of pharmaceuticals, among a panoply of other things. Furthermore, I have, since childhood, suffered from “some form of Marfan-like collagen disorder” (as my parents and I were told at the time of the many aforementioned knee surgeries that resulted from a childhood filled with injuries and dislocations related to a rather excessively “bendy” body).

        (Guess I missed the career opportunities of the situation...)

The docs since my childhood either didn’t see this history as relevant or basically said; “Oh yes, probably Elhers Danlos Hyperflexibility, probably nothing you can do about it, so don’t worry about it”. I more or less followed that advice. In the course of my networking with other RSD/CRPS patients, and through the research I’ve done for this book and in my own efforts to master this Beast, I had come across the information that Elhers Danlos was sometimes a risk factor for RSD/CRPS. With a moment’s thought, this makes sense… all those injuries… and things just generally not healing right. But it made me wonder about what having the disorder really meant for me, and now, with this wonderful thing called the internet, I had the opportunity, finally (after such a long life with a bendy body, completely unsupported by knowledge or kindred) to learn more.

What I learned astounded me. So much of my life and the struggles I’d been through seemed linked to this condition… all except for the immune dysfunction and anaphylaxis that has so dominated my existance for the last 30 years. And the more I read the more unsure I was that EDS Hyperflexibility, or EDS III as it is now called, was in fact the type, or the only type of Elhers Danlos I had. I am now waiting for the results of testing by a wonderful specialist and very kind practitioner and scientist with the Provincial Medical Genetics Department to find out exactly what the diagnosis is… it makes a difference, especially because, if, as my doctors and myself have begun to suspect, I may have the vascular form, it may rather largely impact the surgeries that are looming rather pressingly on my horizon…. not to mention such little details as life expectancy…

                            Meanwhile, I delved deeper…

...and then came the kicker. I kept seeing references to Mastocytosis, or Mast Cell Disease in the co-morbidities and common conditions that went along with EDS. (In the same list with RSD/CRPS). I had a vague idea that mast cells were something to do with blood, or maybe bone marrow. I figured I’d look into it sooner or later, but doubted it had much relevance for me. Eventually, however, I stumbled onto a page that mentioned, casually, that mast cell disease had anaphylaxis as a prominent feature. Really?? Now I had to investigate further. And when I did, I was beyond astounded; I was stunned. Stunned and speechless, as I read the lists of symptoms, which read like a litany of much of my life’s physical sufferings. Of the 58 symptoms listed on the Canadian Mastocytosis Society's site, I had all but 2. The only things not included were encompassed by Ehlers Danlos and RSD/CRPS. Here, in all their “glory” (or was that “gory”…) were the many splendoured symptoms that I long since given up finding “the answer” for.

I looked through page after page of information. Everything I had suffered for so long, the drug allergies, the chemical sensitivities, the food intolerance, the multi-system, endless list of seemingly unrelated diverse systemic dysfunctions (which, yes, I had reason to suspect as immune mediated) were there. There, in a diagnosable condition along with its mechanisms, testing and treatment protocols, predicted lists of drug sensitivities and emergency care procedures… all discovered, apparently, during the last 30 years… during the time that I had stayed as far away from “modern/Western medicine” as possible, learning the non-pharmaceutical ways to survive with the constant threat of anaphylaxis and poly-system morbidity after suffering a pointless, terrifying stream of medical assaults during my months within the hospital system when it all began and pretty much any time I’d had to deal with Western medicine since.

      Back then, they’d left me with more questions than answers.

More to the point, they'd left me barely able to survive. And left me more than a little “shy”, after more than a few vicious “bites” from modern medicine. In those intervening years, I had avoided doctors and hospitals with all my might… and rightfully so. In many of my unavoidable medical experiences, I’d been treated as if I were mad, in environments, and with treatments that inevitably led to further terrifying and life threatening experiences with anaphylaxis. The combination of ignorance, arrogance, and toxicity were deadly to me. They had actually killed and resuscitated me more than once with their “care”. And so, I had spent 30 years dealing with literally multiple hundreds of life threatening anaphylactic reactions without medical aid, along with multiple thousands of lesser reactions. I had raised two children who had obviously inherited this horrific immune condition, keeping them alive and thriving primarily by keeping them away from doctors and their useless, frequently harmful “bags of tricks”. And yet, now, here before me were clear, scientific explanations for the mechanisms behind all we had experienced…. here were diagnostic criteria, actual “treatment protocols”... everything.

   … I was truly in shock (not the anaphylactic kind, for a change!)

Since then, I’ve taken this information to my doctors, all of whom blinked, and said, “By George, I think you’ve got it!” or something to that effect. We’ve hit roadblocks; testing that is unavailable in Canada, or available but not covered by our provincial health insurance in particular. One of the only available tests, serum tryptase, which ideally should be repeated a number of times, costs close to $200 a pop; not a manageable expense for us, after the last 15 years of RSD/CRPS with no pension of any kind. So, thanks to a kind, caring, and smart GI doc, who’d been struggling to make sense of my mysterious profuse intestinal bleeding, six year bout of chronic diarrhea and other hijinks for some time, who, when faced with my information hot on the heals of attending a conference where GI manifestations of mast cell disease were discussed, was willing to give me basically a clinical diagnosis and proceed with treatment in the absence of confirmatory testing.

When it’s come to treatment, we hit more roadblocks… many of the better medications aren’t available in Canada at all, are still on patent and not available anywhere as pure powders (the only hope I have of tolerating meds at all is in the pure drug form, to be compounded without the colouring, preservatives, binders, coatings and other chemicals that make the likelihood of my body accepting them slimmer than a camel who’s dedicated its life to fitting through the eye of a needle…) or are available in pure drug powder form elsewhere (usually the USA) but not Canada. Even with those pure pharmaceuticals we’ve been able to access, the drug testing has been a nightmare; no darned fun at all… it seems especially offensive to be having anaphylactic reactions to the medications designed to prevent anaphylactic reactions! However, one by one, I am finding the particular mix that is making some significant difference to my quality of life. And after all my frustration with the limitations of being a Canadian patient, I have to say I am filled with gratitude for being Canadian. Currently, the crucial inhaled and oral mast cell mediating Sodium Cromoglycate runs my government a cost of over $1500 a month, and is changing my life; I am more grateful than words will ever convey to live in a country with universal health care. “Oh Canada!”

      (Besides, what other country has cops as cute as these two?)

So here I am, now the proud “owner” of 3, count ‘em 3 (not 1, not 2, but 3; and wait there’s more! You also get the free jujitsu knife!! Okay, I digress… I seem to be prone to that too…) rare, incurable diseases. Phoooey! Just what kind of contest am I trying to win?? I guess, to use my own parlance, I’ve got a small pack of beasts to tame! The fact is, that though most folks who have the misfortune of winning the Crappy Prize Lottery of rare incurable diseases will only, thankfully, experience one of these Crappy Prizes, there are indisputable links. And I felt that if those links were relevant to only a small percentage of my readers, I still absolutely had to share. So here we are.

First of all, let’s recap. Exactly what are these diseases? First, here’s links to my 1 page specials. Please feel free to use the printable versions to educate friends, family, and/or medical professionals, but please don’t reprint without crediting yours truly. Also, I apologize that these are not cited. I created these as 1 page grab &goquickie educational sheets, and meanwhile, I’m working on cited versions which I hope to be sharing soon.

• What is RSD/CRPS?
• What is Ehlers Danlos?
• What is Systemic Mast Cell Disease?
  

Later on, I hope to be posting links to some of the bigger and/or better organizations, support groups, blogs,youtubes, and informational sites for each condition, but remember what I said about struggling to stay afloat? (I'll elucidate at the end of this post). Meanwhile, use google!

Okay. Now that we’ve got a handle on the 3 conditions, how about the links between them? It seems that having Ehlers Danlos creates an increased risk of having/getting either RSD/CRPS or any form of systemic mast cell disease. As Ehlers Danlos is a genetic disease, one we are born with, I don’t think we can cite either of the other conditions as increasing its risk, but many who are diagnosed with either RSD/CRPS or systemic mast cell disease are later found to have had Ehlers Danlos all along, without being aware of it. Apparently, having any form of allergic immune dysfunction, including systemic mast cell disease may, by virtue of the inflammation and central nervous system stimulation involved in the condition may make RSD/CRPS a higher risk. The links are there, in the research and in the population. Although I may be a rare bird, I am not alone. I have met and spoken with many fellow sufferers of at least 2 of the disorders I suffer from; Ehlers Danlos seems a frequent companion to either Mast Cell Disease or RSD/CRPS, and a 2 second internet search finds others with all three.

RSD/CRPS is now understood to be a disease of nuero-inflammation. As research into the causative factors for RSD/CRPS progresses, more immune players are identified; glia, microglia, cycotines; and Here’s a study positing mast cell activation as causative for neuropathic pain conditions including RSD/CRPS! And discussion of potential anti-inflammatory treatments, stating that scientific evidence supports elevated activity of mast cells as one of the key processes involved. Over and over, we see discoveries of the importance of mast cells in neuropathic pain and the implication that RSD/CRPS may in fact be an immune disorder first and foremost; here's CRPS UK's 2013 listing of studies related to this concept. And the inflammatory processes involved in RSD/CRPS may possibly even put one at greater risk of mast cell mediated disease.

So here we go loopy loo, here we go loopy lye… Dr. Robert Schwartzman's "Systemic Complications of RSD/CRPS"       for a wonderful article, which seems to have a great deal of overlap with the symptoms list from the Canadian Mastocytosis society.(remember the link above). Dysautonomias and Postural Orthostatic Tachycardia (Here's a great blog explaining this in more detail!) are common to all three of these disorders, as are migraines and some degree of chronic pain. Anyhow, you get my drift. There’s a whole lot of potential connections.

(And those connections can make for one heavy chain, bayyybeee...)    

Now. Just because you’ve got any one of these does NOT mean you have any of the others. Don’t go borrowing trouble, and don’t go self diagnosing in too big a hurry. In fact, because of the high level of symptom overlap, the odds are that you DO have some of the symptoms of the others, even if you DON’T have them. Here’s a couple of the distinctions. Many people with Mastocytosis and other systemic mast cell diseases have some degree of chronic pain, sometimes even neuropathic pain. And comparisons are odious, and the pain you’re in is by nature, the worst pain ever, but RSD/CRPS is in fact considered THE MOST PAINFUL CONDITION KNOWN TO HUMAN KIND.  Check out one of my besties, Elle's blog at elleandtheautognome.wordpress.com/ for a modern version of The McGill Pain Scale (wherein scientists gauged the pain levels of various disorders). The pain of RSD/CRPS is not bad sometimes, it doesn’t come and go… yes, there are more tolerable moments and there are acute flares, but this neuropathic pain condition is 24/7. It doesn’t stop. And though it involves many other kinds of pain (including bone and muscle pain and migraines) there is always burning, electrical nerve pain that is felt to be UNBEARABLE. RSD/CRPS has very high suicide rates for a reason. If you think you may have it, make sure to get a diagnosis as quickly as possible, and find a practitioner with actual knowledge of the disease to help you do so; those who are not knowledgeable can do you serious harm one way or another.  (And if you don’t have this particular beast, you really don’t want it. Trust me on this.)

On the flip side, many people with long term RSD/CRPS will have immune system dysfunction to a greater or lesser degree. Remember Dr. Schwartzman’s paper on the systemic effects? Another pioneer in RSD/CRPS research, Dr. Hooshmand, talks about changes in immune functioning and RSD/CRPS. And, in this world of massive and continuous chemical exposures, many, many people are developing chemical sensitivities, allergic reactions, and/or food intolerances. This does not mean that you have mast cell disease. There are specific hallmarks. Anaphylaxis is one of them. Not every case, but probably the vast majority of systemic mast cell disease patients are very familiar with that term. Anaphylaxis is a terrifying, full body, multiple organ, systemic and life threatening allergic reaction. Without prompt treatment, usually with epinephrine/adrenaline, and sometimes even with it, the patient is at serious risk of death every time it happens. It’s a nightmare. Some patients with systemic mast cell disease have other forms of systemic reaction including neurological reactions; seizures and migraines, but these are in response to the same sorts of triggers as other mast cell patients, and they too may be life threatening. The only way to avoid these systemic reactions is to severely limit exposure to half the trappings of modern life; scented products, cleaning and laundry products, personal care products, processed foods, construction materials, non-organic everything. With or without medications, most people with severe systemic mast cell disease lead lives that are very isolated and isolating. Their world becomes very narrow and there is no choice about it. If you think you may have it, do find knowledgeable and/or open minded docs to help you get a diagnosis, because with this one, at least, there are some actual treatment options. Again however, this is a terrible, challenging disease, and if you don’t have it, you don’t want it.

Ehlers Danlos? Well, if you have it, you are probably very, very bendy (not always, and not every form of it, but almost always and almost all forms). Hyperflexibility is one of the hallmarks. That and extreme bruising to the point that parents of EDS children may be accused of child abuse, and challenges with veins such as terrible trouble getting blood drawn or having IVs placed. Also lots of typical skin and GI symptoms. Do your own research and if you really, truly think you may have it, see a geneticist; many of these can be ruled in or out through genetic testing, and those that can’t can be diagnosed by a knowledgeable geneticist. And, again… you don’t want this at all; and you don’t want to give it to your children. So if you don’t have it, heave a deep sigh, and give thanks.

                Meoooowleluiah! Meowleluiah, meowleluiah...

For those who may have any one or, heaven forbid, more than one of these beasts; I am sorry, so, sorry! However, despite all the dire things I’ve said above, don’t despair. You absolutely can tame these beasts, and not only through the miracles of modern science; there are lots of effective, gentle, natural alternative! I know it. I’ve done it, and had fantastic quality of life for decades at a time, though the challenges move on… and if I have half a chance, I will do everything in my power to share the things I’ve learned both through a longish lifetime of living with these beasts with limited treatment options, and those I’ve learned through all of the wonderful fellow beast tamers I’ve had the privilege to have met and are meeting each blessed day.

I apologize for the many times I've suggested googling or doing your own research in this article, instead of providing links as usual, but things are pretty challenging. I started this article in February. It’s April the 11^th now, after some truly terrible travel ordeals I am posting this with free wifi outside a closed tire store and the help of my technology brownies (my son Nick and daughter Naomi) while waiting for the winds to clear to let me past the next known anaphylaxis trigger, the pulp mill at Tacoma. I am partway through my journey to the Portland Providence Center for Cancer and Less Invasive Surgeries on High Risk Patients. I knew I’d be coming down at some point… I’ve had some surgical incision sites herniate in the last year or so (thanks, Ehlers Danlos!), some perfectly good molars that need to be removed because of bone loss from osteopenia in my jaws (thanks, RSD/CRPS!) and can’t tolerate ANY local anesthetics or standard surgical techniques to take care of these little issues (thanks, systemic mast cell disease!). In the process of testing for my long standing GI issues in the last few months (thanks, EDS, RSD/CRPS and systemic mast cell disease!), I had a CT which showed an abnormally thickened endometrium (lining of the uterus). That, along with several years of acute menstrual bleeding led the local gynecologist to state that I must get down NOW, not later, to a center willing to deal with a patient with 3 pet beasts and resulting complexities, to ”urgently rule out endometrial cancer”. (…oh…)

Now that was a blow from absolute left field. I had no idea we’d be dealing with that particular beast. But here we are. We deal with the blows that fall, we ride the waves that come, and hopefully, we make it through. If I have my way, I’ll be back, to update this website, this book I’ve been working on, my journey taming this beast (RSD/CRPS) and sharing my knowledge with fellow sufferers to encompass the great wide range of people with RSD/CRPS, Systemic Mast Cell Disease and/or Ehlers Danlos. I hope that I can be of service, help you on your journeys, help you tame your Beasts. I’m scared to death to tell you the truth. Surgery, with all these complexities, and my terrible medical history, is my worse nightmare. But I have wonderful professionals preparing for me in Portland, dedicated to getting me through. And my incredible family, with me on this trip, and keeping the home-fires burning, are supporting me with love and devotion and hard work as always, and I would give anything for more time with them. I’ve just barely healed my arms enough to regain the ability to paint. I’ve got an awful lot more to do, an awful lot more to give. SO. Keep me in your thoughts and prayers, please, kind friends and fellow beast tamers. My surgery is scheduled for April 16^th around noon, pacific time. With a little bit of luck, and the help of loving friends and skillful pros, I’ll see you on the flip side.   

 

 

 

 

 

 

 

 

 

 

 

 Here's my wonderful home-fires team (my son Nick, his gal Suzi, and our sweet four-leggers, Sunshine and Solomon) making magick from back on the farm...

And here's me and my super dedicated mobile life-savers, my husband Terry, and my daughter Naomi, finding a moment of sunshine in a challenging journey. See... I've got an awful lot to live for.... so here's hoping and praying I'll be seeing you all soon...

 

Author: Lili Wilde
Date Posted: 2014-04-13   Date Last Edited: 2015-09-15 06:39:01