ears to heart, heart back to ears. repeat.

One evening not so long ago I was listening to “This American Life” while jogging.  At the end of my hour-long run, the podcast was ending as I was super close to home, and the closing song came on…

“if the kids .. are united .. they will never .. be divided!”

The punk rock song (by Sham 69) went from the ear buds past my hearing faculties and just straight to my heart.  My legs started pumping, sprinting me up the hill, my brain took me through this super vivid flashback- thinking of being a young sweaty middle schooler, at a 7 Seconds show with some friends.  7 Seconds played their cover of If The Kids Are United, and I remember feeling so empowered.

I was a kid.  We were united.  I thought we could take on the universe.  My fists were pumping (and not in the Jersey Shore kinda way), and I just felt that the couple hundred of us huddled into some crappy Daytona dive venue, we could change the world.

random mosh pit pic i found on a google image search

Back in 2012 my jog wrapped up, the song was over and I got to thinking: my heart and hearing connection has always been a close one when it comes to music.

Some of my earliest memories are of my dad’s mix tapes.  My dad’s a total mix tape genius, oh man.  I grew up in this world of themed tapes for every mood.  Bruce Springstein by the pool, Wham! when we’re kicking off a road trip.

As I got older he introduced me to his most precious gems: The Beatles, Simon and Garfunkle. Concert soundtracks for Woodstock, No Nukes; all these amazing 70’s artists.  We’d drive around, because listening to music is best done in the car, and he’d explain the feelings each song brought to him.

me and my dad, a couple years ago

I best understand my father’s pain and sadness during my parents’ divorce via “Only Living Boy In New York.”

I have to compose myself here, literally, tearfully wincing as I’m writing.  It’s that emotional for me.

For every event there is a song.  A life-long soundtrack that encompasses all genres, envelopes all emotions.

From my ears, to my heart – music goes. 

When I started to find my own music: Sublime, Alanis Morissette, NOFX, Blink-182 – they spoke to me too.  I would sit in my room, “doing homework,” just listening to my CDs on repeat.  93KRO was the alt rock station that existed when I was in middle school in Daytona Beach – I’d listen nonstop, letting them teach me about the Cranberries, Poe, the Toadies, etc.  I remember getting ready for school every morning to “Dude Ranch,” putting my blue mascara on.  Yeah!

seventeen year old me

And high school? College?  Oh man.  As punk gave way to emo, I went in head over heels.  I was a chest-pounding misty-eyed emo dreamster, speaking to my closest friends strictly in song lyrics.  Seriously, we’d communicate in lyrics.  Some of us still can do it.  Crying at the Dashboard concert kinda stuff.  Sprinkled in with some pothead memories, a lot of Dispatch and Ben Harper, of course.

my friend in green and in white = me, gettin funky at a dmb concert years and years ago

So as 28-year-old me is uphill jogging and I’m reflecting on this ear-to-heart beeline, I ponder: does it go both ways? 

For any of y’all not in the loop: I currently suffer from a condition my doctors call “peripheral neuralgia,” or “atypical facial pain.”  For the past 12 months my right ear has been hurting so badly.  The pain radiates from my ear, to my cheekbone, forehead, jaw … and it’s so debilitating that I can’t even think.  I’ve been on a whole mess of meds, treatment attempt after treatment attempt has failed, and it sucks.  I just want my ear to stop hurting.  The meds I’m on hold me for the time being, with a limited amount of breakthrough pain, but they aren’t a cure.  I can’t conceive while on these meds, they have side effects that I don’t like.

total fail - didn't work.

icing my neck on a day my ear was hurting real bad!

I feel broken.  It’s infuriating, depressing, frustrating, and sad that we (my amazing team of doctors and I) can’t get to the bottom of this.  I’ve had better success with two kinds of cancer.

My ear hurts every day.   Just the right ear.

And well, the little things hurt.  When a baby cries, when a mic reverbs, a whistle, a horn, my neighbor’s super loud and obnoxious bass – all of these sounds and noises, they HURT me.  Physically.  I hate it.  I have to wear an ear plug in my right ear a lot of the time.  I always have to wear it at church, it’s way too loud in there.

The ear pain started last January (2011), in the aftermath of the miscarriage I had. The stress of the miscarriage did a lot of damage to my body in general (the grotesquely mismanaged miscarriage, during which I bled heavily for 15 days, for a total of 30 when all was said and done).  I have a genetic blood disease, porphyria, and stressors take a harder hit on me than they do your average young and healthy person.

But maybe there’s a more simple answer: that heart-to-ear expressway.

Losing that baby was so sad.

Sad is such a lacking word.

I wanted to be a mother.  I was so happy to be pregnant.  I was so excited to have a baby.  Overjoyed, blissful, grateful, peppy.  It was the best I’ve ever felt in my life.  And then… it was taken away from me, in a rather undignified way, a drawn-out, painful, way.

I’ve experienced sadness and loss in my life – of course, who hasn’t? – and I can honestly say the greatest heartache I’ve ever suffered was losing that baby.

And it surprised me with how terribly sad it was.  I had no idea that a miscarriage was that hard to go through.

From my heart, back to my ears, so the connection goes? 

The idea is intriguing.

I mean, there are other things that I know to be true – I have musculo/skeletal damage on my right side, in the nerve path of my ear/face.  I have nerve damage. This isn’t the first time I’ve had this one-sided ear ache, it’s just the only time it hasn’t gone away after a week or so.

As far as mending my heart, I think I’ve come a long way.  I was never angry about the miscarriage, no one is to blame.   I was always just sad.

If anything, the event was part of a path the reignited my faith and brought me even closer to God.  It brought Duggs and I closer together.  I’ve been to therapists of all kinds – regular, neuro-psych, pain psych, etc., and they all say I’m doing okay, that I’m happy and well adjusted, doing the best I could be doing, given the circumstance.   In the past year, I’ve emotionally come a long way.

A great deal of heart healing has gone on. 

So am I just waiting for the ear to catch up?

In an effort to tackle the problem from the other side, I’ve jumped back into music with a new-found passion.  I don’t spend that much time in the car these days, and I’ve gotten kinda addicted to TV, so re-immersing in my jams has felt good.  I totally have Spotify to thank, I’m so obsessed.  And running; running again gives me reason to rock out.

cake will be here in hawaii later this month - i think i should go on therapeutic grounds!

So this inner express lane from my ears to my heart, it feels roundtrip.  Maybe the way to cure my facial pain isn’t through Lyrica, but lyrics? Ha. Do you like what I did there? Lyrica is one of my meds.  That’s funny.

If anything, perhaps soaking myself in my most favorite of tunes will be a way to help me feel better in general and/or distract me from the pain?  Maybe my heart has more healing to do than I’ve realized?

The connection feels real to me though: ears to heart, and back again.

….

As always, thanks for reading!!  Love, happy hippie rose

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someone get this fat suit off of me

I debated on whether or not to post this… it’s PETTY and bratty.  But it’s also honest.  I used to be an athlete, a swimmer and a lifeguard who lived in a bathing suit, I even did a little modeling.  I also battled an eating disorder in high school and my early 20’s; so that said, just like many women, I have body image issues.  As my medical condition, my age, and my lifestyle take a toll on my waist size, I’m becoming increasingly frustrated and hard on myself.  I just don’t feel like me anymore. 

Someone Get This Fat Suit Off Of Me!!!!

I’m not even going to glorify this post with an accompanying photo… No way.

But, I’m not very pleased with my current weight status.  My face is getting all puffy again, like it was back in the dark and dreadful Prednisone days.  I feel like there’s a fat suit on me, seriously.  When I move around, run, stretch, etc., I feel these extra parts of me that never used to be there.  It’s weird, and surreal and frustrating.

Now.  Don’t get in a huff and puff at me.  I KNOW I’m not obese, or technically even overweight. My BMI is actually in a very healthy place, in the mid-range for my body.

But I’m not changing body shape in a way that I’m okay with, or enjoy.  It’s side effect of the meds I take, of the steroid shots I get.  A lot of it is water weight, and even though I take a diuretic daily, it’s hard to stay out ahead of.

I try to eat okay… but let’s not kid, I have the palate of a child and all I want to eat is ice cream, candy and cake.  I have the sweetest sweet tooth ever.

I work out a lot, when I have the energy to do so.  I seem to go in chunks of good weeks and bad weeks.  Some weeks I’m doing two-a-days, and some weeks I can’t get out of bed.

But.  I’m going to be patient.  And focus on eating balanced, proportionate meals, working out and just waiting out for something to work (medically, health speaking) so that I can taper off of my meds.  All of the ‘roids I get shot into me are the worst culprits, followed closely by Lyrica. Ugh.  I’m grateful for the pain relief, but I could do without the barrel of side effects.

Anyways.

That’s my gripe!  I know it’s not that big of a deal, but it’s just what’s on my mind today. Blah.

/rant.

I’m going to focus on the good things my body can do, and be grateful for the aspects of my health and fitness that are intact – of which I have so many to be thanking God for.  I just need to respect His blessings and my body by getting my toosh into shape and into gear!!

my docs are on my nerves!

Literally!  I got a nerve block on Tuesday.  Hehe, wasn’t that a funny title?  I’m so witty.

we pulled up to TAMC and saw a rainbow, i thought for sure that was a good sign

Anyways.  Yeah, I got a Stellate Ganglion Nerve Block placed on Tuesday morning.  And the results are in:  they missed, it didn’t work, mah face still hurts.  Total bummer, I know! But, at least nothing went wrong, and I haven’t suffered any dreadful side effects.  So, it could have gone better, and it could have gone worse.  It’s a draw, and I’ll take that over things getting worse, any day!

The plan was to go into my neck and inject some meds to help cool off the nerve area that we suspect of causing my chronic facial pain.

quick background info:  since january of 2011, i’ve been suffering what is being Dx’ed as “atypical facial pain casue by peripheral neuralgia.”  they at first thought that i had “trigeminal neuralgia,”  but that was ruled out.  my docs believe that an acute exacerbation of the blood disease i have (porphyria) caused this nerve issue to happen – the blood disease, when exacerbated, can cause all kinds of nerve pain and damage.  doctors have been trying to find the source and treat my nerve pain all year long, and so far we’ve only come up with band-aids.  the nerve block this week was my first attempt at using a physical intervention directly into the area like that.  i’ve also done rounds and rounds of trigger point injections to try and treat the area in a referred way, as well as taking oodles of meds.

 

The plan was really solid, and I had been so very hopeful.  I had so many people rooting for me and my doctor, praying for us and the procedure.

The procedure itself was rather unpleasant.  It hurt a little more than I had been expecting, and just because of old experiences back when I had cancer, I don’t appreciate people rooting around in my neck with long needles (when I had cancer, I had this core biopsy of a lymph node in my neck go terribly awry, ugh).   So yeah, not the most pleasant way to spend a Wednesday morning, but it was manageable.

I did get about ten minutes of sweet relief, during which I cried happy tears and referred to my face as “blissfully painless.”  And then… IT CAME BACK.  The pain started in my ear, again, and just spread out.

that's a little snap shot of my procedure the doc took for me!

right afterwards, yeah i don't look thrilled

Don’t worry though!  We have more plans.  There are different kind of nerve blocks to try, and we can repeat this very one too.  There’s a chance the doctor missed the location by just a smidge.  Or maybe, the inflammation/aggravation is so intense, it’s going to take a LOT of blocks to get in there and hose out this fire.  I’m not sure.

hey! that's not a friendship bracelet!

Bad news: My face still hurts!

Good news: there are more things to try, hopefully a solution will be found and I’m still optimistic!

here's a photo of duggs taking care me after the procedure... oh wait. he's snoozing and the pooch is taking care of me! what a nice pooch.

here i am, one day afterwards and i can't get that permanent marker off! doh!

For the past couple days my neck has been kinda stiff and tender, especially where they poked me.  I woke up today feeling pretty much back to usual though, which is nice.

Yes, it’s back to the drawing board.  But I’m confident we’ll find something that will work soon.  It’s all a process of trial and error, and I’m okay with that.  Thanks for all the well wishes and support, I’ll post as things play out and as I find out more info myself!

i’ve got an L422X on my PPOX, duh!

i’m officially, documentedly, DNA-testingly, ONE OF A KIND!

My Porphyria DNA panel finally came back, and with it came an official iron-clad diagnosis: I have Variegate Porphyria (VP).   And, the specific mutation sequence that I have been found to have in my genes, is the first of its kind ever discovered, ever tested in a lab!

the dna test report

there ya have it!

So what does all this mean? 

1.  It means that I actually know what’s going on with me.  Having a specific diagnosis is the best way to get better.  You can’t treat what you don’t know.  For years doctors have assumed, based on symptoms and simple biochemical testing, that I have AIP: Acute Intermittent Porphyria.  Knowing that I actually have a different type helps us (me, my hubby, my fam, my docs – everyone involved in my care) better assess, prepare, prevent and treat my exacerbations/attacks.

2.  This explains why my symptoms haven’t always meshed with the model of Porphyria that docs are looking for.   Because this version of porphyria is something never before discovered, who knows how different my actual case is than others within the family of the Porphyria diseases?!?

3.  This doesn’t just apply to me, this applies to all of my family members with the disease, as well as those suspected.  It explains why diagnosis has been so difficult (it’s very hard to diagnose porphyria anyways, let alone having a one-of-a-kind mutation of the disease). It’s most likely that the mutation I was discovered to posses, it the same genetic sequence/mutation that my family members have (so I’m not literally one of a kind, we probably all match, but still – we’re breaking the mold, as a family!  which knowing us… makes a LOT of sense).

So things are moving fast for me now:  My Nursing Case Manager and team of doctors and geneticists are working to get me to the mainland to see a specialist.    My emergency protocol has been updated, the to-do list of ways to prevent an exacerbation have been updated.

This is all great news.

This information will help me be able to get better, more specific care for myself, as well as for my family!  We’ll now be able to get into the elite clinics and be seen by the specialists (their criteria to see patients is pretty intense, since there are only half a dozen or so of them in the USA).

Having a finite diagnosis sets me free.  It gives me direction.  It helps remove the gray area.

It’s a little scary.  I kinda miss that ole “intermittent” in my diagnosis.  I liked the idea that it comes and goes and bit more than this Variegate seems to hang around.   But, it is what it is, and when it comes to quality of life, I have no room for complaint.  I’m more than able to enjoy my daily life.

From my ER Protocol:

Variegate porphyria is a form of hepatic porphyria most common in white South African population.  This autosomal dominant disorder may produce acute attacks (as in acute intermittent porphyria) as well as skin photosensitivity.  The condition is caused by mutations in the PPOX gene which lead to deficiency of the enzyme protoporphyrinogen oxidase.  Acute attacks are managed and may be prevented as in acute intermittent porphyria.

Acute porphyrias cause acute attacks of neurological symptoms that can be life-threatening.  ACute attacks are triggered by environmental stressors such as certain drugs, sex steroid hormones, reduced intake of calories and carbohydrate, alcohol, and unknown factors.  Many of these factors stimulate heme synthesis in the liver, which in the face of a metabolic enzyme defect, leads to increased production of heme precursors that may be neurotoxic.

So yeah, it’s pretty crazy!  While some of the verbiage sounds scary or intimidating, I assure y’all that this is good news and there’s nothing weird or scary that’s come to my attention that I didn’t already know.   Having this solid of a Dx is just going to help me move forward, and for that I’m beyond grateful.  I’m so appreciative and happy for that.

don't you fret, i haven't changed a bit - still me!

And as for being so special?  We’re all special, and we’re all literally one of a kind.  But having these test results come in was nice.  It was like God whispered in my ear that day, and reminded me that I am made differently and specifically, and that I am so special.

All of this has really helped re-shift my focus towards the future and I’m just in roll-up-the-sleeves-and-get-to-work mode.   I’ll update as I do!

And if you’re my family and you’re reading this, I promise to scan and email soon.  We’ve been so busy.  I know that’s no excuse, I promise I’ll get the info to y’all very soon.  I love you all and I’m so glad that we’re going to tackle this whole thing together, as one big awesome team.