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| His first ever Marathon yet he crushed it! |
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| EDS Canada shirt reads "Endless determination ands strength" |
This post isn’t a continuation of the
Cannabis Hyperemesis Syndrome disaster story from last August (we’ll return to
that another time, I think it will just take one more blog post to wrap that
story up). Instead, it will be a lot shorter in length given that it talks
about events occurring right now that have yet to be resolved. This whole new
series of unfortunate events began around November of 2021 when I began
developing what we assumed to be a series of stubborn ear infections presenting
as fluctuating levels of ear inflammation. We were very wrong, and gradually
began to realize I was being misdiagnosed given the antibiotics didn’t seem to
have any impact other than upsetting my stomach. The nail in the coffin for the
ear infection theory was the inflammation beginning to appear in both ears
simultaneously! My ENT then switched his suspected diagnosis to something far
more spooky: Relapsing Polychondritis (RP).
https://en.wikipedia.org/wiki/Relapsing_polychondritis
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| I wish I could say it was just a sunburn... |
This is a degenerative autoimmune condition that causes inflammation throughout the body. In 90% of cases the first place one gets inflammation is an ear, then the other ear, and potentially all kinds of places after that. This is very tricky to figure out because I do have plenty of inflammation in other places, such as my fingers, and costochondritis which is inflammation of the ribs. The complication is that costochondritis is also a common feature of having Ehlers-Danlos Syndrome (EDS). I also have tinnitus (ear ringing) so severe I can barely hear myself think, but that has other possible explanations too like EDS induced Temporomandibular Joint disorders. Further adding to our difficulty is the fact that the nature of this condition means that your immune system attacks healthy cartilage throughout your body.
There is no cartilage in the earlobes,
and so they are typically spared from inflammation. Mine were spared for the
first several months this was going on, then that changed. It was only by
joining an RP support group that we were informed on how newer research has
shown that severe unabating inflammation can eventually spread to the earlobes
too. Apparently tons of research such as new diagnostic criteria was just beginning
but then, like with so many other important projects, the pandemic put all that
on hold. This means much of the information out there on this disease is
severely outdated. Plus, the condition is quite rare to begin with, affecting
about 1 in 33 thousand. I was only the second case my ENT had ever seen in his
long career where the earlobes were affected.
When I switched off the useless antibiotics, I was prescribed corticosteroid cream, though I didn’t realize how weak it was compared to other such creams. Thus its positive impact was quite limited. The rheumatologist wanted to do an ear biopsy to find out more. Unfortunately, the newest research that has also not yet been formally published indicates that such biopsies have little diagnostic value (it will show inflammation, but not necessarily what’s causing it) and can make the current flare worse to boot. Given this, the ENT prescribed 50 mg oral prednisone immunosuppressive steroids but we agreed not to actually retrieve the pills until my appointment a few days later where I would consult my dermatologist and THREE of his colleagues.
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| Just a teeny bit of swelling... |
That was quite the surreal experience, I was examined by four doctors and a fellow simultaneously! I’m just sitting in the room with them, looking up and back and forth between them as they argue with each other about what’s causing me all these problems. Three of them thought I had a nasty skin condition called… wait for it… Chondrodermatitis Nodularis Chronica Helicis. Both a mouthful AND a literal and figurative earful, heh. The fourth dermatologist disagreed with everyone, saying it’s not the skin condition OR RP. The problem is, he had no suggestion on what else it might be. Soooo…
Regardless of the diagnosis I clearly had
rapidly worsening inflammation issues and the mild corticosteroid cream wasn’t
cutting it. And so the dermatologists gave me a new prescription for the
strongest corticosteroid cream known to man. When I looked it up I found out
there are actually seven classes of corticosteroid strength. The first cream
was the weakest tier, class 7. The new cream, being among the strongest tier,
was one THOUSAND times more powerful than what I had been using. So, I must ask
why it took so long for us to give up on such weak treatments? Because as it
turned out, the stronger cream still hasn’t been strong enough!
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| Not even a large slathering of white corticosteroid cream can hide the ear redness! |
The disease continued to develop in a manner more reminiscent of RP than CNH, even though I’m incredibly young to have either condition. RP is known for occurring to people in their 40s-60s, whereas the skin thing is usually developed by those in their 40s. My doctors theorize that my collagen being inherently weak from my EDS connective tissue disorder is why I am getting this in my 20s. And even worse, they theorize the potential for permanent damage to my body is also higher and we’d already wasted so much time with the misdiagnoses and the incredibly weak cream!
Therefore we made the decision to take
the very big step of filling the oral immunosuppressant prescription. This was
a very big relief, because I’m the type of person who would more likely internalize
frustration from hesitating at the right moment to act, as opposed to
regretting an action taken decisively. I get stressed holding the gambling dice
in my hand waiting for the last possible moment to throw them, but once I start
the oral corticosteroids the die is cast and you just have to wait to watch how
things play out. Because if we hesitate just long enough to allow this constant
inflammation to cause further permanent damage to myself, I know I’d never be
able to forgive myself…again. Even typing “again” just now got me emotional!
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| This was posted almost exactly one month before I caught swine flu, beginning a life of chronic pain. This shows my natural positive attitude state, I had no idea what was coming. |
The typical maximum prednisone dose possible is 60 mg, and I used 50mg with gradual tapering over a 3 week period. The potential for complications with oral steroids is so much higher given the risk for it entering your bloodstream, as opposed to topical creams. The side effects are also infamous for being far nastier. You think you’re hot shit, eh prednisone? Well my ears are shit and literally too hot to touch, so FIX EM! “These are my pills. There are many like them but these ones are mine.”
Oral prednisone has such a wide impact
that it can interact with basically all of the other copious prescriptions I’m
on, but we didn’t have much of a choice. I had only just felt like I had a new
lease on life thanks to a breakthrough with sleep where I’m now awake 28-30 hours
and asleep 18-20. This puts me on a 48 hour sleep cycle, and anything that
divides into 24 is stable, even if it’s a weird equilibrium where I still sleep
through the entire day, every other day. Until last month my schedule was
basically completely random, making planning anything with doctors, friends,
etc impossible further than 2 days in advance. With this finally changing and
me now being awake 38 hours each day, plus me getting the all clear from my
immunologist that I can end my years of very strict covid isolation, I was
honestly crying tears of joy. Then literally one week later I get an autoimmune
condition diagnosis that requires me to reenter isolation indefinitely. I only
had the chance to see one friend and my cousin indoors during that glorious
week, for now I’ll have to stick to outdoor gatherings only.
Imagine our current alarm given that the
inflammation is still not under control! If that much immunosuppression can’t
get this handled when it’s mostly limited to my ears, what the hell are we
going to do if it spreads somewhere far more dangerous? Given this, my doctors
said to cease the tapering plan and go back to 50 mg indefinitely. This was
devastating news to me, because I was supposed to fly to British Columbia for
my brother’s wedding in a few weeks. I can’t fly and risk getting Covid while
on such powerful immunosuppressants. And I’ve heard from other people with RP
that due to the extent the disease spread they have to commit to being in
isolation for the rest of their lives. It can spread to the eyes, impacting
vision. To the inner ear, deafening you. To your throat, robbing you of much of
your speech capabilities. Even the heart, where it can be life threatening. The
fear of the unknown is just so intense.
The prednisone isn’t working when it
really should, which was less surprising than you might think given pills never
seem to properly work as soon as they come into contact with my silly body. I
take two dozen sleeping pills before bed and I’m still awake 30 hours each day,
which is actually an improvement! Therefore I am going to start getting
subcutaneous injections of an even more powerful/dangerous immunosuppressant
called Methotrexate. They use this stuff for chemotherapy! It has a better
chance of helping, but even if it does it will take 6-12 weeks to kick
in! I'll stay on 30-50 mg of prednisone in the meantime. The side
effects are even more infamously bad than high doses of prednisone. I have a
plan, though! It causes extreme exhaustion, so others I’ve talked with who have
taken it have to designate one day each week to get nothing done and feel
miserable. However, since I sleep up to 20 hours each night, I plan to take my
injection right before I sleep so that the exhaustion can actually become a
good thing and be worn off by the time I wake up! We’ll just have to see if
this pans out. The prednisone and corticosteroid cream treatments are becoming
less effective over time, which horrifyingly suggests the degenerative disease
is worsening. The inflammation used to calm down for 11 hours after each dose,
now it's 6.
It’s just so hard for my family and
doctors to believe I have so many rare problems largely independent of each
other! I myself am plenty used to the incredulous feeling surrounding this.
Life threatening Serotonin Syndrome is super rare, it happened to me twice
anyway. Cannabis Hyperemesis syndrome is even rarer, it still happened. EDS is
a relatively well-known rare disease I have of course. And now this RP
autoimmune nonsense. If only they could dissect me and then put me back
together. We had our suspicions that RP is connected to EDS confirmed! EDS
weakens connective tissues and RP attacks the weakened throughout the
body.
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Around the same time I got this new diagnosis I watched the
new Batman movie with Robert Pattinson (I won’t spoil any plot, he’s an adult
Batman so his parents were murdered way before the movie story begins), and
given everything going on it really touched me emotionally. I really related to
this portrayal of Batman, it seemed the most realistic we’ve had in film. If
both your parents were killed in a robbery while you were a child you probably
won’t become a party-loving playboy like in other depictions. More than likely
you’ll be pretty messed up mentally and emotionally. A murdering criminal stole
his childhood from him, of course he’s going to be filled with rage at this
injustice he had to deal with from a young age. My Satan-spawned genetics stole
much of my childhood from me given how my chronic pain started at the age of
14. I keep trying to scavenge what remains. So I told myself: “Well I lost my
teens and my 20s due to barely scraping by in survival mode, but we’ll catch up
in my 30s. Even if it’s mostly in spirit.” But nope, instead destiny decided I
would develop this degenerative condition at half the onset age it’s known for.
Thanks, Mitch’s genetics. I can always count on you to be the gift that keeps
on giving.
The theme song they chose for this Batman
movie is a song called Something In The Way by Nirvana. Kurt Cobain wrote it
about how he kept trying so hard to get his life back on track with his
addiction issues because every time he made progress toward that goal there
seemed to be Something [else new] In The Way. I’m not failing. If someone born
paralyzed was entered in a race and came last did they fail the race? I had one
week. One fucking week to enjoy ending my isolation before I was diagnosed with
an autoimmune disease requiring indefinite immunosuppressants. I was JUST told
I’m not immunocompromised based on my blood tests. There’s always ‘something in
the way’, indeed.
I got worried this week, when my tears
and anger turned to sardonic laughter at my track record of if something
extremely rare can go wrong, it will. Especially this past year Murphy's
Law has been taken to an insane degree. Though I do like the quote by Victor
Frankl: “An abnormal reaction to an abnormal situation is normal behavior.”
That night I went to bed with my ears a lot better and dared to hope I was
getting a handle on this new crisis, but I woke up in the middle of the night
to find the ears horribly inflamed again. They’re far too painful and tender to
get a decent quality of sleep, which has already been put out of reach due to
prednisone likely adding to my insomnia. It’s not easy to think about all the
time we wasted on antibiotics and incredibly weak creams allowing this disease
to progress. About how it feels like I’m being targeted by something malicious
that caused me to get a brief taste of freedom from covid isolation only to
have to reenter it one week later. How my problem with metabolizing/absorbing
pills now has the potential to get me killed or more disabled rather than just
making it impossible to fully treat my sleep disorder.
The conventional treatments are failing, and even if they do succeed a new flare can always come back derailing everything and requiring yet more isolation. I was forced to cancel plans to work at a beloved summer camp for the third year in a row. I want to be able to return to dating while I've still got some youth left but I'm as far away from that goal as ever with the constant need for isolation. I finally got somewhat of a breakthrough with sleep that made life at least bearable again. Yet somehow in no time at all I’m diagnosed with a disease that can take the choice of living away from me if left unchecked. With all these thoughts swirling around, in bed staring at the ceiling, I started crying which worsened my costochondritis rib inflammation pain which caused me to switch to disturbing humourless laughter (crying about this constantly worsening disease ruining everything and the act of crying makes the disease symptoms even worse? what a lovely hilarious feedback loop to have, on my June 3 birthday no less!) which made the pain even worse again! It’s hard to tell if my sanity is finally cracking or if the prednisone is altering my mood, which is a common side effect with it. Regardless, I plan on surviving this and then will have an even more positive story than I originally promised within the documentary! But for now it’s the hard times again, so thank you for your patience.
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