The Bad News Post


Though I originally meant this blog to be entirely a venue for sharing, pre-publishing, some of the information from my on-going project, “Taming the Beast” with those who need it most, it has become at times a somewhat more personal place of expression as well. This is one of those times. Those of my readers who also know me as friends may be aware that I’ve been out of touch for a while, struggling to absorb a recent piece of very bad medical news. I haven’t known what to say, since I hardly know what to think or feel. I’ve been very distressed; I’ve been all over the map…. But I’m going to try to share this news with you now, along with (as I’ve been asked so many times of late without answering) “how things have been going”… in my usual wordy way. (For those who prefer the Reader’s Digest condensed version of things… mea culpa, I guess.)

So, here it is, in a nutshell… I’ve basically been told that sometime in the next 1-2 years I must have the most major and brutal of surgeries (which I am unlikely to survive, by the most optimistic accounting), without which I will die, during that same 1-2 years. After a lifetime of Elhers Danlos, a lifetime with systemic mast cell disease (that has dominated my existence since the age of 20) with a side order of asthma and chronic lung disease, and 14 years with whole body RSD/CRPS, and the dysautonomias that go with that, it’s probably none of these, directly, anyways, that will kill me… it’s my heart that is finally (and oh so prematurely, apparently,) breaking.

I’ve been struggling for some time, (increasingly in the last 2 years or so), with a set of symptoms that I and my doctors have pretty determinedly ignored, partly because (on my side, at least) they were too frightening to look at head on, and partly because they could each be explained away, to one degree or another, by one or more often multiple of my pre-existing conditions. During the months since my surgery, this symptom constellation has gotten very much out of control.

Here’s the list, along with the easy pre-existing-condition-go-to for each. This part is for those who really want to know, so feel free to skip this part if you don’t want the grotty details; suffice it to say that most of this *stuff* that’s been happening to me may be the symptoms of severe aortic stenosis… or not.

  • Unexplained weight gain; I’m not a big eater at the best of times, and lately have had real struggles getting food in (see below), but there’s been a very decided and inexplicable lurch up. Clothes I’ve worn for a decade or more are suddenly not so comfy. Not a happy camper; not at all. Well… ok, I’m disabled, I have activity levels that are severely limited by RSD/CRPS, and I’m pushing “the pause”. Mast cell disease can also cause unexplained weight gain. The recent jump seemed likely to be at least partially if not largely water, and in either case a mast cell mediated (and hopefully temporary) systemic complaint about surgical trauma… and it still might be.
  • Swelling of the legs, feet, and abdomen; Swelling is a very common part of anaphylaxis, and of mast cell disease in general and again, I have whole body RSD/CRPS, that began, at age 17 (though it blessedly went into remission after just 2 years) in my legs. So, although I’ve had NO leg swelling in the dozen years of RSC/CRPS preceding it, when the part time leg and abdominal swelling I’ve been struggling with so much these last 2 years became acute in the first days post-surgery, I (and my caregivers and doctors) blamed a combination of mast cell mediated immune response, surgical trauma, and, as the days turned to months, and the pain in my legs and pelvis continued to grow exponentially with the swelling, I began to suspect that the surgery had triggered a flare of the RSD/CRPS. That would not be unexpected, but I would hope and believe it to be temporary (as some of you know, my watchword, when it comes to RSD/CRPS is plasticity… meaning change is the only constant and some of it will almost certainly be for the better!) So I’ve been doing all my alternatives and trying to wait it out. We did do an investigation that supposedly ruled out post-surgical thrombosis, and then left it at that, hoping that time, elevated legs, compression stockings and an ocean or two of dandelion infusion would heal all (swelling) ills…. but it hasn’t remotely happened so far…. which doesn’t necessarily rule out any of the previous non-cardiac explanations.
  • Dizziness, sometimes severe; again, “pre-syncope” has been a part of my medical picture for many years, and I’ve blamed the rather radical recent increase primarily on irritatingly prolonged post-surgical mast cell hijinks… and it still might be.
  • Nausea and acute lack of appetite; yup, all of the above… though with the ever increasing weight it’s been more and more a case for Scooby Doo and the gang… or something…
  • Shortness of breath, persistent cough with very thick, sometimes darkish, often blood tinged phlegm; well, I’ve had a lifetime of lung issues, and I’m now on several meds that thicken or dry normal secretions (as a side effect to much needed pain relief and/or histamine blocking and/or mast cell mediating)… not sure why that would make it bloody, but… I’ve mentioned it to docs, but they, like myself, were prone to shrugging it off and hoping it would either resolve in time or that I could just live with it… and maybe that’s been the right tack to take…
  • Episodes of left shoulder and arm pain; well, duhhh, I have whole body RSD/CRPS which began its rule over my adult life in the upper body; granted it was the upper right quadrant, but it quickly spread. The kind of pain I’m experiencing is not really dissimilar to acute RSD/CRPS pain (though not precisely the same, either)… it starts with a heavy, crushing feeling in the L chest or more often back of the shoulder blade, with shooting pain and prickling numbness down the outer two-three fingers of the arm…. Really not sooo different to what was common for many, many years. That crushing sensation is a little bit of a twist, but RSD/CRPS is a many headed beast and oh how it loves coming up with a new face on any given day… and just to add to the confusion, there’s been about 20% right sided pain out of the increased episodes as well. I’d been distressed by this, it’s been scary, made me worry about losing incredibly vital hard won gains (that make it possible for me to write and paint again, among other things), but I thought of it as a (hopefully) temporary increase in the RSD/CRPS in the wake of the surgery… and it still might be.
  • Difficulty sleeping, increasing and sometimes intense anxiety; ok… is there anybody living with constant pain, central nervous system hyper-stimulation (normal to both RSD/CRPS and systemic Mast Cell Disease), and/or the never-ending constant threat of life-ending allergic reaction who doesn’t sometimes have, shall we say, a hard time relaxing/drifting off? So why worry now? I’ve been living with life threatening and frequently traumatic illness all of my life; I’ve learned long since to navigate the overwhelming dread that is part of anaphylaxis and the “freight train, goin’ so fast” aftermath of the adrenaline needed to save life when anaphylaxis occurs, as well as the jangling CNS hyperdrive that goes with a life of constant neuropathic pain. I’m pretty darn good at cultivating calm, if I say so myself… so why do I find myself just now fighting overwhelming anxiety that wakes me after 15 minutes (or an hour, or 2) of sleep, (with or without concurrent chest/arm pain), to flood my brain and body with a stark and nameless fear ? “We have nothing to fear, but fear itself”… unless of course, it’s that special fear the body generates to share information with the mind to the tune of… “Wake up, wake up, you wouldn’t want to miss this Exciting Cardiac Event!”
  • Weird headache in the back right quadrant of the brain; don’t even know if I should include this one, since it’s the only one of the list that is not utterly screaming CARDIAC, but it is another change that I’ve been pretty much ignoring. It’s a headache like I’ve never experienced (and I have had loads of types including several flavours of migraine); it’s prickling and numb and neurological feeling, it lasts hours or weeks, nothing alters it for long. The only treatment to touch it comes from my magic Osteo practitioner and even he can only give me temporary (though desperately appreciated) relief. It smacks of blood clots in the brain, to the paranoid part of my 3am mind. It’s pretty irritating… and nasty. (and probably unrelated to cardiac disease.)
  • Constant, overwhelming fatigue; this has actually been the very most troubling. Yes, I live with some exhausting health conditions, yes I have had my sleep disrupted by one thing or another (nightmares and respiratory problems from childhood on up, pain from multiple dislocations etc, vigilance due to unsafe conditions, the discomforts of twin pregnancy, the feeding and care needs of premature twin babies, vigilance of parenting two children with serious chronic health issues, a lifetime of frequent anaphylaxis and the aftermath of epinephrine treatment and near-death trauma anxiety, then RSD/CRPS, high level neurological pain and CNS agitation, and round the clock meds timers for the last few years) pretty much all of my life, and sure, it’s probably been catching up on me despite my apparent ability to manage anyways… but it’s been really out of control for much of the last year, and increasingly so. If I am holding still… no matter what time of day or night… and I am not actively involved in something cognitively engaging (and sometimes when I am) I increasingly, continuously, just…. pass out… fall asleep, mid sentence… talk sleep talk to people when they think I’m awake etc. And yes, I’ve had times of that through the years, when the sleep deprivation got especially bad, but this has become the new “normal”… I am the Pass Out Queen. Every move of any body part is a massive effort (unless I’m in the midst of my most intense physio of the day, when that lovely “I am woooman, I am invincible” thing kicks in… (yes, even now)), and the most likely words out of my mouth at any given time are “I’m so tired...” This level of fatigue has also brought with it more cognitive mess up-edness and Sally space-case-ism than ever before. Which doesn’t mean that it’s anything other than just a whole lot more of the same old thing…Sometimes, the information we receive from our bodies can be just a little... confusing.

There can be so much confusion in trying to sort out new symptoms... Those of us with multi-system complex chronic health conditions, ( let alone multiple, semi-inter-related and often obtrusively interacting multi-system complex chronic health conditions )  get used to just *dealing with* an ever changing bouquet of many splendoured permutations. Yeah, on this day your voice sounds like Darth Vadar’s and on this day like Mini Mouse…. Yeah, your waist (and face) size changes so many times in a month/week/day that you have to juggle multiple clothing sizes or give up and just wear jumbo lawn &gardenbags(with fabulous accessorization!), yeah the only thing constant about the pain you live with is the… constancy…. you just deal with the flavour of the month/week/day/hour/moment and try to have a life despite it all.

So you can see how all of these things have been easy to just add to the pile.

There is a one size fits all explanation for all of these things and it’s backed up with tests results that say “at least moderate to severe aortic stenosis”. And it’s pertinent to know if these symptoms are or are not (being caused by my heart). That 1-2 year clock starts ticking at the point of severe aortic stenosis when you become symptomatic. Until that point you are “merely” at a high risk of sudden death… if the ticker is running, it’s still behind the scenes.

I can’t say I had no warning… two years ago, I landed in hospital with “intractable asthma” (what we now understand to be an acute mast cell attack after a mould bloom in our rainforest environment). I traveled to the mainland because the hospitals here in Victoria have proven to be so toxic for me. While there, they did an echocardiogram and mentioned that they’d found some evidence of “mild to moderate” aortic stenosis with some minor atherosclerosis also in the aortic valve. While it wasn’t happy news to find out that I had yet another disease that is not expected to rear its head until one hits the 80’s (I also have cataracts, thanks a lot), it didn’t seem much of a threat, not really very “real”. The docs didn’t seem horrifically concerned, either; a bit surprised, saying it was unusual in one so young as me, but just let’s monitor this on a yearly basis and don’t worry about it. So I didn’t… worry (much).

Oh yes, I had a moment or two of angst, but the good (my family, friends on and off-line, my work, baby animals, butterflies, flowers and all those sorts of things) the bad (multiple chronic rare and difficult diseases and all that goes along with them), and the ugly (having to deal with some degree of poverty, mould, a polluted world, and a seemingly never-ending series of additional health crises) of my life rather put my aorta in the growl puppybackseat while months became years.

I did realize that time was passing, and that my pain management doc, who’d agreed to be in charge of monitoring the cardiac stuff wasn’t really monitoring, so as we began to get close to the 2 year mark, I asked for a repeat of the echo. His response was pretty irritating; he didn’t remember this, couldn’t recall seeing it in my files and, since it seemed highly unlikely at my age, he “wondered how I’d gotten that idea into my head”…. (grrrrrrr!) So I persisted, diplomatically pestering via email, until he finally, begrudgingly, agreed to repeat the test. (After I’d sent him a copy of my discharge notes from the hospital which stated that it existed and that he’d agreed to monitor it. Remember, folks, always get copies of reports and keep your files in order; don't count on the docs to do it !)

I went and took the test. Though I was the one who’d pushed so hard for it, I wasn’t wildly worried. I told myself it would probably not have progressed, though admittedly, I’d had a lot of troubles that seemed like they could mayyybe be cardiac-ish (the swollen legs, fatigue and all)… I didn’t actually know the symptoms of this particular beast, been too busy with other things, and figured it would be just another background worry to be dealt with later.

The plan was, that when the test results came in, Terry and I would travel to Vancouver to get the results, then head off on our long planned “Great Wheelchair Camping Experiment”. I’d been really missing the travel part of our past (pre RSD/CRPS) life together terribly, and though we’d n20140410_134420ever taken a week alone together since our honeymoon (back when our kids were 9 year olds), we had recently realized that they actually would survive our abandoning them for a week or so and were dreaming of taking time alone together in an alternate environment… one that not only wasn’t mouldy, but also was not fraught with renovations to be done, food to be put up for winter, bills to be paid, debts to dig out from, all those garden/animal/human/structural/financial needs clamouring for attention alongside the massive boatload o’medical ….Just a little space to actually Be… Together. Hey, we’d made it to Portland, not once but twice…. granted we’d had to, granted we’d had the wonderful extra support of our daughter along with us, but hey, if it wasn’t emergency travel for a change, and I wasn’t in critical condition (maybe just somewhat miserable, what with the balloon legs and bloata-belly and the overweaning fatigue and endless pain and all… but still, not critical), hey, maybe we really could do it, and maybe it could be fun….

(Here's a moment from our 2nd Portland trip... just to prove it wasn't a totally mad idea...)

So that was the plan.     Turned out to be rather less fun than we’d hoped. Starting with… oh, dear, do I even get into it here? Yes, because Systemic Mast Cell Disease is part of my reality, and some of my dear readers share in living with that particular beast, so; yes. OK. I live up the coast and out in the toolies, on an island. My best hope for reasonable specialist medical care (in or out of my own country) is a ferry trip and many hours of car travel away from my home. The Straight that I have to pass to get there is guarded by a many headed dragon of Pulp Mills. Depending upon the wind direction, I can safely get to medical care or run into sulphurous pulp mill pollution that means instant, throat swelling, life threatening allergic reaction; anaphylaxis. So my family study the winds, and we live by them. If I need medical care on the mainland, we watch the marine buoy reports and forecasts, and jump when the window appears (because it’s deadly more often than not and can change quickly). So when the reports said, “the window is now and may not occur again for over a week”, we jumped, despite the fact that we really weren’t prepared to go and hadn’t wanted to go for two or three more days.

            ( Ohhh Canada... where the "Forestry Industry" rules.... my life, anyways...)

Terry called in to cancel work with multiple clients, and the entire family frantically started packing. And when I say packing I really mean it! My visiting mother once observed that watching us prepare for a day-trip to town was like watching preparations for a trip to the darkest and deepest of far away jungles. And she’s right. We have to have EVERYTHING with us. For me to enter any public place, eat any unknown food (certified organic or not), let alone sleep on bedding or wear clothing washed in any but a known and tolerated product is Russian Roulette in the extreme. All of my medications are compounded preservative free and many are very difficult to obtain. We MUST be self contained and carry EVERY POSSIBLE ITEM WE MIGHT NEED unless we want to spend all of our time assessing and dealing with reactions, many of which would be life threatening.

It sucks, but we’re used to it, and prior to RSD/CRPS, we never let it stop us from having a life…. THAT particular beast nearly stopped us in our tracks, but now, after 15 years of it, we were at long last trying to take back some small bit of our lives… thus, the Great Wheelchair Camping Experiment. Which we were embarking upon now, albeit prematurely. So, we all ran into high gear; we made it to the boat, got on the boat, and made it across. YAY! But unfortunately, one of the other beasts of our lives, the ongoing poverty inherent to a family with a quarter decade of living with multiple members’ serious chronic health challenges, had been thrashing its nasty head more than usual of late, and due to a bureaucratic error (and a mean spirited civil “servant” or two) the very slim funds earmarked for the trip were held up and not forthcoming. We’d hoped to get things sorted out before we went, but the wind waits for no man… and certainly not for this woman in a wheelchair.

So there we were in the big city with nary a dime to deal with it while we waited for the doctor’s appointment and the funds to come through. The first night, we crashed by the heron nesting grounds near the ferry terminal, thankful for the summer weather. But the next nights were colder and thus much harder; we tried a couple of parking lots (which were extremely unpleasant and stressful for varying city sorts of reasons), and finally found a reasonable rest parked outside the Richmond Bog Nature Preserve. Then came the rain… a cold and persistent, driving rain… days, and nights of it. We had to drive between sleeping spells to stay warm, and we don’t have a camper you can stand up in, just our regular sized travel van with a little mod-cons trailer Terry built with kitchen and bathroom facilities (as long as we had access to a camp-site with water and electrical hookup)…. So lots of immobility, the vibration and motion of driving, spells of being overly warm as we tried to blast the van with heat while we drove, interspersed with increasing cold as we tried to sleep through the sound of the rain hammering on the metal roof of the van for as long as we could manage between times, the stresses of lurking in not entirely legal parking places in the city night. Those of my readers with RSD/CRPS will have some notion of what this all translates to…. Yeah, you know it; PAIN FLARE. 20140902_124215.[1](Nope, not an esoteric shot to represent the "cage" of pain... just a cute baby squirrel robbing a bird feeder at the Richmond Bog Nature Preserve on one of our rainy city wait days. )

We managed to steal a few moments of fun between struggles; it’s what we do best… we went for physio walks at the bog, and in Stanley Park, and were thrilled to find out that the Vancouver Art Gallery had it’s “By Donation” evening on one of the days we were there. We knew I’d pay, but by George, we were going to squeeze some pleasure out of the wretchedness, so we went for it. There was a very cool exhibit on the history of art in Canada, and we really did enjoy it, but the museum is undergoing renovation, and had obviously had some sort of mould or moisture troubles, so there were dehumidifiers roaring in every room, and a fuggy stink sharply highlighted by the scents of construction…. the environment was not a friend to one with Mast Cell Disease, or a chronic pain disorder…So I wasn’t in grand shape by the time it was the day of the doc’s appointment.

lili and op art copy

Here I am, in all my exhausted glory, with first piece of Canadian "Op Art" I remember my much loved artist Grandfather showing me when I was a child... I asked Terry to sneak this (rather blurry) shot under the Gallery guard's stern eye, since photos are verbotten. Naughty, I know, but I was feeling rebellious...

We were surprised when we arrived to be told that “both the doctors are running a little late”, since I was only seeing one, as far as I knew. Turned out the mystery guest was a young cardiologist, who my PM doc had called in when he saw the test results. He poked and prodded just a little, frowned over my "grossly edematous legs and feet", and then he dropped the bomb. I was told that the echocardiogram showed that the aortic stenosis and associated atherosclerosis had progressed abnormally quickly; it was now “at least moderate to severe”. The cardiologist said that if I was “a normal, otherwise healthy octogenarian” (like my mother, who at 89 was unhappy to receive this diagnosis earlier this year), he might give me 2-5 years before I would have to have open heart surgery or die. As it was, despite my abnormally young age, he gave me 1-2. He said that I appeared to have a “particularly virulent variety” and suspected it to be related to my “obviously acute systemic inflammation”. None the less, he said, he didn’t want to act. He doubted I would survive open heart surgery, though there might be some options soon available (he said vaguely). I said that I was certain that the people in Portland would know what those options might be. He asked a few questions, then said he would support my being treated there “when the time came”, as certainly no one here would want to make the attempt. He agreed to follow my case, said he’d be booking another echo in 6 months and that he’d see me then. And off he went.

The rest of the appointment passed in a blur. My PM updated my meds, chatted away (a bit weirdly) about my great smile, about how I “didn’t have a mean bone in my body” and should be Santa Clause if I was a man but of course I was too young and not quite plump enough and how he wanted me to agree to find a fake beard and dress up as Santa Clause 30 years from now. I told him I’d decided to stay on higher doses of meds for the time being in an attempt to up my mobility with harder physio pushes. He made a wry comment about how he had never disagreed with a single medical decision I’d ever made including bugging him with emails when he didn’t do his bit…When I tried to talk about the symptoms of the heart disease he brushed it off… told me that my grossly and obviously edematous legs (which every medical professional who had seen them in the last 4 months had been shocked by) were probably “how can I say this… just fat” so not to worry… the whole thing was a bit bizarre… but we were in a haze of disbelief and shock, so everything seemed a little surreal.

( Okay, you said...Open heart surgery?? 1-2 years??? Santa Clause?? Legs??

Wait.... What was that again? )

When we gathered our things to go, he grasped both of my hands, looked deeply into my eyes and told me that I would survive, that he wasn’t remotely worried. Sure I had all these “extra health things” but there was not much else wrong with my heart and I was young and tough and had already survived lots of things that nobody thought I would. Besides, in his career, he said, he’d seen that cognitive ability often corresponded to surgical outcome and there was nothing wrong with my brain… I was touched… I guess… but also very, very confused.

Now we were “free” to head off on our trip, except for that little financial matter, which couldn’t be cleared up for a few more days... so despite my massive pain levels and an overweaning desire to see my (young adult) babies, to just go home… with our path home barred by toxic pulp mill pollution, we chose to forge on. So the Great Wheelchair Camping Experiment began.

Great Whlchr Camping Exp copy.[1]

The Great Wheelchair Camping Experiment

This post is getting painfully long… in more ways than one. (Apologies to my forgiving readers and unforgiving body.) Suffice it to say that the next couple of days were utter hell. I divebombed into a desperate, frantic depression, flailing at fate, biting the hands that fed me. I misbehaved rather badly, I’m ashamed to say (apologies to my unbelievably patient and loving family, especially my stubbornly steadfast husband)…. I was half mad from pain and the rest of the way there from stress, and then more than over the edge with the neurological impact of pesticide exposures from the smoke and mosquito spray saturated public campground we were huddled in. Thankfully, part way through the week, we escaped that hell-hole, and found ourselves half way up Mount Baker, at the most wonderful campground, Silver Lake Camp, we met an adorable squeaky clean ex-boyscout park ranger who took mercy on me and gave us a personal recovery zone; the group campsite (meant for 37 campers) for our sole use. I swear that man had wings and a halo…

Having a safe haven in which to start to detox, wash our nuked clothes and belongings and actually stay warm and dry in gave us a chance to step off the merry-go-round of misery, calm my CNS and actually, weirdly, *enjoy* ourselves. We explored wheelchair accessible mountain trails (some in the chair, some for physio time), we picked glorious, intensely flavourful wild blue huckleberries (anti-oxidant heaven!), we swooned over mountain vistas, exchanged “words” with wildlife, we laughed a little, cried some, talked, held onto each other. For a brief time, we had what we had so yearned for… simply, Time. Together.

L and T blurry but nice1 copy

Me and my honey; in shock but mainly managing to live in the moment...

Since coming home, I have been struggling to encompass this news, reach some kind of acceptance, come up with some kind of plan. I’ve avoided talking to anyone but a very few of my nearest and dearest about it; I don’t know why… it’s like talking about it might make it more real. And I so desperately wish that it wasn’t. Meanwhile, writing about it here has made it actually seem almost less real; like this is a story I’ve made up. But it isn’t.

I’ve done my usual; immersed myself in research, trying to increase my understanding of what is, and may happen to me. What to expect for myself and those I love. And what I’ve learned has been pretty distressing. After the point that Aortic Stenosis is diagnosed and symptoms have begun, 50% of patients will be dead (without surgery) at the 1 year mark; at the 2 year mark 100% will be dead. Surgical outcomes are better than that (for those who can withstand the surgery), sometimes buying up to 10 more years of life, but open heart surgery is incredibly brutal, and involves so many drugs in families that are either known to be deadly to me or untested but highly likely to be deadly. It seems very unlikely that I could survive it. Dr. Rea has suggested that given my previous history, it is extremely high risk that I would reject any heart valve, but that artificial, bovine or porcine would be almost guaranteed rejection, whereas a human valve might stand a chance. The most modern option, the Transcatheter Aortic Valve is a mix of porcine and artificial elements, and apparently the human heart valve is no longer an option. I’m still learning more about all the options; none of them look likely for my survival… but I’m not ready to go. I still have things to do.

I contacted the cardiologist, asked for further testing. Told him and Terry and I both believe that I am symptomatic (which means the clock is ticking) and that it has been masked by my pre-existing conditions. He called me back, a little hostile, stolidly refused to consider any treatment or testing until after the next echo, 6 months from now. (…and the clock is ticking…) He says he will support me going to Portland “when the time is right”, but that “it isn’t right now”      (tick tock, tick tock…) and I’m to “leave the worrying to him” (though it seems quite apparent that he is, in fact, not worrying…) Honestly, my impression was that he had decided I wasn’t worth the health care dollars. I hope that isn’t the case, but I was left feeling very, very concerned.

I contacted my wonderful Providence surgeon. He was not at all impressed with the attitude of the cardiologist here, and strongly suggested that I contact the folks at the Portland Valve Center at Providence St. Vincent’s (the sister hospital to Portland Providence, where I received such incredible, exceptional care); where assessing high risk patients with aortic stenosis is“what they do”. He believes that there is testing and possibly multiple kinds of treatment that could prolong my life before we even have to look at which surgical option, and that these are the people who will know what to do and when. He gave me contact names, told me he would talk with them about me (let them know that though I am a complex patient, I am a cooperative one, and not crrrrazy, despite my zebra status); he gave me hope and compassion. Unlike the cardiologist, he appears to care if I live or die.

To live or die… that is the question that all of us with chronic disease, especially with brutal, chronic pain must assess… over and over again throughout our lives. Just this last week, the RSD/CRPS community lost another beloved warrior/angel, and I am among those who have also lost a friend; the grief has sent many of us into (or deeper into) terrible pain flares. Suicide is an ever present option when our lives are so unrelentingly hard… and yet… I live with not only with two diseases called “the suicide disease” (RSD/CRPS and trigeminal neuralgia share that distinction) but with one that involves near continuous life threatening reactions (Systemic Mast Cell Disease). I have struggled greatly, as so many of us have, at different points in the disease process, with thoughts of finally ending the struggle… and yet, even on a day when such thoughts have seemed near to overwhelming, should an anaphylactic reaction rear its ugly head… I fight. I fight with every ounce of strength of body and mind that I possess. So I know that despite all the challenges, despite all the pain, I still want to be here. I AM ABSOLUTELY NOT DONE YET

my 4 babies

 There are people (2 &4legged), who love me, some of whom maybe still need me, including my 4 beautiful babies; nurse dog Sunshine, my shining son Nick, my darling daughter Naomi, and silky heavy headed Solomon... as long as they, and my truelove Terry still want me around (despite how difficult I am to keep here), I'm not going anywhere... willingly.

So, given this new, seemingly unbeatable challenge, what shall I do? Do I accept the “fact” that my life is winding down and there is probably little I can do about it? (Knowing that I am unlikely to survive even the “less invasive” options, and knowing that sudden death is common with this thing, once it’s been graded severe, even if asymptomatic.) How on earth, after a lifetime of fighting to survive can I ever reach that kind of acceptance? And if, by some miracle, I manage to do it, how do I prioritize the time that is left to me? Aside from spending every possible moment with the people I love best, what do I focus on?

Do I, for once in my life, as recommended my loving daughter who knows me so well, be purely “selfish”… do I spend my last months/year(s) focused on what would almost certainly give me the most pleasure… that is to say, painting, creating artwork? I had truly believed, from the time I was a little child up until the total loss of my arms from RSD/CRPS, that I was “meant” to be an artist, that my work had meaning and was in some way “important”… after over a decade of enforced abandonment of my career to the Beast, I had finally won back my hands and arms, and was just starting to make some headway in bringing that back into my life… do I stop with focusing on helping others and give myself what may be the last months of my life to devote to my “real” work? Do I allow myself that glorious pleasure, that challenge, that utter selfishness?

1. Selkie  Sedna  Surcease

"Selkie/Sedna/Surcease" (copyright S.L.Wilde, 1999, all rights reserved... please respect my copyright and do not copy or use in any way without permission) ~ the much beloved piece I finished the night before the car accident that sent RSD/CRPS screaming into my life...

Or do I dive into Taming the Beast, on the grounds that people need me to? I know the struggles my readers and all those with intractable pain are living through…. and sometimes dying because of. The costs of medical care are a brutal burden to the families of those with RSD/CRPS and other forms of chronic pain, including and especially alternatives (since few insurance plans, government or private, will cover them). Can I abandon my efforts to provide the desperately needed guide to accessible, economical, holistic, home based alternative therapies for intractable pain? (Not to mention all the dysautonomias, medication side effects, social/psychological/spiritual challenges that go along with it…) Should I not, even with my last years/months/days be trying to follow through on that commitment? The knowledge that I have to share was so brutally hard won… And yet, it seemed a vast and sometimes forbidding task before this wretched “egg timer o’ death” started losing its sand so fast before my eyes … now it seems surely all but impossible to complete…

(Have I ever mentioned that a sense of humour…even a rather daaark one, is a vital tool to surviving constant high level pain? Yeah....)

And if I cannot, (or feel that I should not try to) reach that place of acceptance, what the heck do I do instead? I have so much to figure out, and the clock is ticking . I am so angry with myself for all the time that’s been wasted in my life. If, for example, you took all the time I have spent trying to educate and protect myself from medical professionals and petty bureaucrats…the time devoured by the struggles of poverty, illness and disability at the price of time for useful, creative and meaningful work... the love and time I’ve wasted on people who just couldn’t love me back… not to mention the very angst and time it takes to think these kinds of thoughts…. How much of my life would I have back??  

2013-11-23-JohnBauer_thumb3Enough navel gazing !

At this point, the Plan of Action is:

  • Get more information. Learn more, research, get more tests done to try to clarify whether my aortic stenosis symptomatic or if these symptoms are in fact caused by my pre-existing conditions, whether there are actually any medical or alternative therapies that could buy me more time, whether there is some unknown chance that I would survive this kind of surgery…
  • Ramp up on every kind of alternative and holistic anti-inflammatory that I can tolerate. Given that it seems that the aortic stenosis is very likely related to my systemic inflammation, and that we know that to be immune (read: Mast Cell) mediated. And that mast cells have been implicated in heart disease, as have leukotrienes and other mast cell degradation products, focus new testing and treatment accordingly.
  • With the same rationale, do everything in my power to decrease my mast cell burden, including considering a move to a drier property.
  • Try to get this current, ever present anxiety and fear under control. Get meditating, do every CNS calming technique I’ve ever learned and learn more. Seek calmness and clarity. Pray. Try to find grace.
  • Continue to push the physio with a will, even ramp it up, but moderate intensity until a stress test can be done.
  • Figure out my priorities…. Maybe try to cut my mountainous Taming the Beast into a much smaller resource, but one that covers the most desperately needed information?
  • Get my house in order, as the saying goes… in every way that I can.
  • Hang onto every single moment of love and happiness, especially those that I can also give to the ones who have given so much to me. Generate love. Live in the moment (especially the good ones, and in the hard ones, hang on hard to those silver linings).

Granted, all these things wouLili in Plether crop copyld be easier if my level of sheer physical suffering would back off a bit, if the galloping range of scary symptoms would give me just a little break… still we can’t always get what we want. So, regardless, I hope to keep you all posted a little more regularly (more frequent posts, but shorter!), and I hope to follow through on responding to some of the specific requests I’ve received, like a blog post on “denuking procedures” for my Mast Cell compatriots…. and hope I still get enough time to be of use, a chance to help you all in taming your beasts…

Meanwhile, please keep me in your thoughts and prayers in the coming days. I could use all the help I can get… and I have never underestimated the power of love.

So on that note; love to you all. I wish you low pain, low reaction days and nights, and much love and happiness. Despite the many clouds in your life, seek avidly for silver. Savour every moment. Take no blessing for granted…

 Until we meet again, I wish you all Well.






Author: Lili Wilde
Date Posted: 2014-09-26   Date Last Edited: 2018-05-18 17:10:28


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