The Best Laid Plans of Mice and Me...

Well, I had, as they say, the best of intentions... When last I posted, I  thought I was "getting my sea legs again", and would be posting shorter, but much more frequent posts, with weekly rather than daily pup-o-grams, and I would be focusing on getting the book in some sort of manageable shape for others to finish if I don't get the chance. OH my.... Progress Report: "FAIL". Yup. I'm so sorry, friends, I truly thought I could get there, and I couldn't... at least, I didn't, and it sure feels like I couldn't. I guess I won't waste your time beating myself up more about it now (I'll save that for later, if I really feel I need to... :P )

So, what have I been doing these past 6 months? On the health front, I've actually made some headway... I've gone through the dangerous and scary desensitization procedures for Aspirin, and am now taking the prescribed daily dose for both heart disease and the potentially elevated prostaglandin levels of Mast Cell Disease. I was hoping it might have an impact on pain, thus facilitating a decrease in other pain medications, but that hasn't happened. It's mightily hard on the tummy, but I am tolerating it, and it was a previous anaphylaxis trigger, so that's pretty huge!

(Well, ok, it was big, if not quite as big as advertised here...)

I've also successfully gone through the testing protocols for Montelukast, *finally* off patent, and available as a preservative and additive free pure powder (though only in the USA). Montelukast is a leukotriene blocker, and an important mediator of a potent mast cell degranulation product for many patients. It has been on my list for a long time! However, it was again, a scary procedure as this medication can have nasty neurological side effects, up to and including hallucinations and suicidal ideation; both of which I could do without! Lucky for me, neither these, nor any other of the potential side effects has entered the picture thus far. Instead, I experiencing a drastic reduction in the degree and frequency of anaphylaxis and laryngeal edema! YAYYY!!!! So far, the impact has been entirely positive... but to fully explain just how positive (yup, it actually gets even better!) I need to get back to my heart.

My heart... tick tock tick tock. During the first part of this year, things continued in not such a nice way, with brutal chest pain and weird sensations of the arm or arms accompanied by an intense sense of impending doom had my doctor thinking "heart attack".... except that the test results were confusing. The ECG said "evidence of previous infarction" due to a bloggy blip down where it should have been a sharp poke up (gotta love the scientific way I present this info, eh?), yet the cardiac enzymes remained unchanged. These episode increased in frequency and intensity... and they were usually sudden, late at night, and bracketed about by nightmares... loads o fun... all we'd have needed was a clown or two to make it a regular Cardiac Circus...

    (Now that's just too scary... but then, so were these episodes!)

However, something happened... "What?", you may ask... well, Montelukast. Since getting established on a Mast Cell Disease appropriate dosage, the episodes have all but completely stopped! And it all started to come together... All through these months I'd continued to monitor the support groups, though I haven't been anywhere near as active on them (thank you soooo much for being there, all of you wonderful folks, even if I haven't been able to say much, I've been appreciating you from the wings!), and I also continued to research, research, research...  'cause... that's what I do when there's trouble. And between a mention of "Kounis Syndrome" on the wonderful  Integrative Masto site that's been such a boon to me, and some simultaneous search results leading me to the same, I found it. Mast Cell Mediated episodes of coronary artery spasm with acute cardiac pain and all the symptoms of a heart attack, but without changes in the cardiac marker enzymes!  On further reading, Kounis Syndrome with all of its complexities (yup, of course there are complexities!) seemed to me to almost certainly be part of what I am dealing with. The docs are now on board with that, and I have been formally diagnosed with yet another rare disease (though really, it's probably "just" another facet of the MCAD). Interestingly enough, leukotrienes have been established as one of the primary mediators responsible for Kounis Syndrome (thus, the effectiveness of the Montelukast has been considered evidence of Kounis Syndrome in my case).Here's a couple of links, just in case it's of help to any of my readers.

The cardiologist who made the diagnosis of the Kounis Syndrome has been a lovely surprise. He's kind, quite brilliant, and communicates so well. He works in the local hospital that all cardiac patients on the island are sent to... one that I can't even enter without anaphylaxis... but he is happy to be part of my team, helping with monitoring and planning and working with the wonderful pros in Portland who (we hope, oh how we hope!) will be helping us with assessment and coming up with a plan of action, and eventually, if and when the time is right, giving me a new chance at life by giving me a new aortic valve (of whatever kind proves the most likely to work well for me).  My situation is so complex, I know that even the good doctors at my wonderful Portland Providence and its sister hospital St. Emmanuel's (where the Valve Clinic is located) are somewhat daunted at the idea of attempting heart surgery on me, but I am hoping and praying that they will see their way to helping keep me here just a little longer... there is so much to do, so many paintings waiting to be done, so many ways I that believe I can be of service, especially to those who, like me, are suffering from this inter-related family of rare diseases. Not to mention this family of mine who so badly wants me to stick around as long as I can... 

However, there is some really wonderful news. This good local Cardiologist, and the dedicated head of the Valve Clinic in Portland, have concurred in the new idea that my Aortic Stenosis may be closer to moderate than the very severe rating it was being given a year ago. Despite the terrible deterioration of the valve from 2012 to 2014 (during which time I was living in a mouldy situation, and had to travel to Portland, go through surgery among other challenges, and experienced countless episodes of acute mast cell activation, from laryngeal edema and anaphylaxis to the more mundane but profoundly unpleasant "lesser" reactions); the echo from this last year (during which time the mould has been as ameliorated as humanly possible... aside from the fact that you can't de-mould the entire ecosystem in which you live... and the mast cell disease has been much more stable, especially with the blessings of the profoundly positive new medications, and I've been working hard at implementing more and more anti-inflammatory meds and alternatives including very regular and very skilled osteopathy care) showed NO new deterioration! So I think we're getting somewhere... In any case, rather than being told I have 6 months to live, I am now being told "it may be one year, it may be 5 or 6; it certainly won't be 10" (until I need to have heart surgery or plan to "meet my maker"). That still sounds short, but it's more than 6 months, and maybe we can make it more than they think at this point... prayers, good wishes and other assorted good mojo gratefully accepted!

So at this point, we've decided to delay the trip to Portland for assessment of the Aortic Stenosis for 1 year in any case... hoping that I can get the mast cell disease more stable, investigate options for medical imaging (despite acute reactivity to contrast materials), and perhaps get moved to a slightly less mouldy micro-eco-system. Later this month, I will be heading to Vancouver for the stress test (non invasive exercise test) that I was denied last time (by technicians who would have been perfectly happy to have me take multiple tests that would be undoubtedly acute anaphylaxis triggers, yet were afraid I would drop dead on the one test we knew that I could tolerate!), and since this trip will unfortunately likely encompass my birthday, perhaps my darling and I will get some more wheelchair camping experiences under our belts. It's  great to have something potentially really nice to look forward to besides all the medical stuff!

Meanwhile, I am also currently waiting for test results for two more rare diseases I am suspected of having... (seriously???)... Hereditary Angioedema (Yay! Finally got government funding for this important genetic testing due to the continuing behind the scenes work of the wonderful Provincial Geneticist on my behalf!)  and  Sjogren's Syndrome (because I am suffering from acutely and distressingly dry eyes and mouth all of the time). I don't know... Despite the headway I hope I am making on the heart disease, I'm sorry to report significant worsening of my whole body RSD/CRPS since undergoing the mainly very successful multi-pronged surgery last year. It's found a few new corners to inhabit, and has become more severely symptomatic in places that had previously stabilized... and the pain levels have not returned to their pre-surgery levels. And though I've made considerable headway on the mast cell disease, it is still a constant battle to stave off the symptoms from minute to minute and hour to hour... I just have more tools for the job, and as I age the Elhers Danlos has found new ways to rear its ugly, bendy head... All in all, I kind of think I've got *enough* rare diseases, don't you! I'd just as soon not be the Imelda Marcos of rare diseases... *Sigh!* I don't want any more diagnoses on top of those I already have, but, you can't work to fix it if you don't know what it is, and I am determined to get as well as I can, so really, I'm grateful.

Also meanwhile, I've continued to paint, working on my series of angels... that is, the angels of my life; some of the many people who have helped to keep me here on the planet, if not "safe" , then a whole heck of a lot safer than I would have been without their sometimes mysterious, sometimes mundane, often surprising and always appreciated intervention.
           ( "Late Night at the Train Station" copyright S.L. Wilde,                                   so please don't use without my express permission, thanks!)

Despite the fact that I now hope I have at least a little more time than we were first led to believe, I’ve also spent a lot of time thinking about death… my own, obviously, but in general too. Some of this process has been really challenging, really difficult, but some of it, and some of the places my research (Cuz… am I researching the topic? Yeah, you know it…) have led me have been inspiring, intriguing, and heartening.

Since some very harsh experiences at a very young age, I have been filled with horror and disgust by the modern funeral industry; the unnecessary, grotesque and invasive things they do to human bodies (which have become so standard, so unquestioned by so many), the way they pressure people who are at a weakened, vulnerable time in their lives, the joyless repulsiveness of modern practices… it has been an anathema to me all of my life.   Imagine my surprise to find that I am not alone, and that there are devoted people world wide who are working to create positive change in every aspect of how we deal with death. I'll likely be posting more on this topic in the coming months and years; please don't be concerned or put off by it... as in every other area of life, good comes from thought, and the thoughtful sharing of ideas, and we need good news when it comes to death.. it's something we will all have to face, sooner or later, and I truly feel that some conversations on the topic wouldn't be amiss even if it's rather off the intended original purpose of this blog.... some of it is even rather beautiful. Like this lovely, imaginative stop motion animation (be warned; though it's super sweet, there may be tears...) :

Now, this post, as always, is getting too long! One last word on the "intended original purpose of this blog", and I'll say "Until Tomorrow". My intentions are mainly the same... I do want to get back to the blog, and the book, in as full a way as I can, when balanced with the demands of survival (which seem to be more time consuming every time I turn around... just doing the basics plus one more small something is the max that I get on a good day, now) and being here for my family, and my ongoing work attempting to achieve mastery over the most unhelpful of mediums (acrylic paints; rather less toxic than many options, much much faster to work with than most, but cranky as a fish wearing work boots, at least with me)... well, let's just say I likely will be less able to fulfill my intentions than I'd like. However, I hope to post again within the next few days... a long delayed post for my darling friend Marie, and not only all of my mast cell compatriots, but my chronic pain pals too, because this info is relevant for all of us...

.... coming soon to a blog near you (that is to say, Taming the Beast)...

                                   " DE-NUKING, DE-MYSTIFIED "  
Until then, my friends, I wish you all health, happiness, learning and loads and loads of comfort and love.










                                                (Until Tomorrow...)


Author: Lili Wilde
Date Posted: 2015-07-04   Date Last Edited: 2015-07-04 21:33:09


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