Health Update Time: It’s All Good… Well, Mostly Good!

Howdy Readers, Friends, Loved Ones.

Much like the President and his State of The Union, I feel like coming here and posting a big ole update about my health and where it stands is something I like to do, and I do on a sorta regular basis.  I mean, it’s totally as legit at the State of the Union, right?

I mention my health in passing, and for the sake of advocacy, understanding, and information – I like to be an open book.  It’s been more than eight years since my battle against Stage III Lymphoma played out publicly in college – but my own health issues and ordeals are not over.  In going through all of that in front of so many others, I realized that by being open, I was helping others.  So many people who’ve dealt with cancer have reached out to me, and I’ve helped how and where I can.   Yes, it’s in small and tiny ways, but good ways nonetheless.  So, when I can… I’ve been able to offer compassion, support, empathy, and love.  And now with my sweet ole blood disease, I am just doing what I know how to do: be a blabbermouth, and if anyone needs help, I’ll do my best.

I have porphyria.

It’s a blood disease.  It’s genetic.  There’s no cure, and you have it forever.

It’s complicated, weird, hard to explain, hard to understand – yes, it’s all of these things… but, it’s manageable.

By far the biggest, best, and most amazing breakthrough in my life as a person with acute porphyria was the DNA test I had done in Hawaii.  I know the exact mutation in my genetic code that causes the blood disease I have.  And if you think you’re special – allow me to trump you.  I am, to date, THE ONLY person to have ever been tested with this exact mutation.  Talk about ONE OF A KIND!

Now.  I know I’m not really all that unique.  The likelihood that members of my family share this specific mutation is very high.  We are likely a one-of-a-kind porphyria family.  And given how genetic diseases and mutations work, well it’s not really that fancy.

So… onto the good stuff:

HOW AM I DOING?

I’m okay.  I’ve had some attacks lately.  Triggers-and-attacks is the cycle of my disease, with lovely periods of calm remission in between.  Triggers for me include stress, illness, medications of certain kinds, alcohol, preservatives, lack of sleep, and more. Around the holidays I was eating tons of processed meat and cheeses, which I love, but which are oh so bad for me.  Add to that the ole Santa Pub Crawl I couldn’t miss out on, and a lot of travel and staying up late, and well – I created my own little perfect storm, didn’t I?

at the santa pub crawl we held in fredonia, much like the real giant santa pub crawl that takes place in buffalo every year!

a totally weird and silly pic from that night

i did too much of this

and delicious as it is, i ate way too much of this kind of stuff during the holidays.

i stayed up all night while visiting my sister for christmas eve. d’oh! i need sleep!

 

I did.  I made some bad choices… and I payed up for it.  I felt pretty crummy for a good chunk of December.  I modified my behavior though, because I love my body and want to give myself the best shot possible.  Even though some choices seem tough in the moment, in the long run they’re important.

so i abstained from booze for the rest of the holidays, even christmas day and new year’s eve!

outfit repeat, but really – here i am as the d.d. on new year’s!

After a while of clean living and good choices, I seemed to have really pulled out of things.

at duggs’ work’s winter party – we had a blast, and i felt pretty good.

I am feeling better now.  At last month’s visit to my docs, we adjusted my meds.  I am now happily on a new form of medication that I get through a patch.  Isn’t technology rad?  Since porphyria affects my liver and its enzymes and red blood cell production, getting meds into my body without bothering my liver is excellent.  Yesterday I went back again, and we tweaked the doses a bit, so once those get filled and I regulate out… I’m hoping my day-to-day will be even better.  It’s really nice to be on meds that don’t have so many long-term effects on me and side effects, etc.

I’m seeing a couple new doctors, too – and they are really wonderful and very committed to helping me get the treatment I need, when I need it.  I’ve just recently opened up the lines of communication with my local in-town doctor, so the next time an attack happens – I can get into a hospital nearby and get the IV treatments that help me stop an early attack in its tracks.  And now the meds I need, should a serious attack occur, will be known and available (in Hawaii, this was pretty serious – they didn’t have ANY of these meds on the island before I got there… eeks!).

Coming from the military system to civilian world of medical care has been… well, halting.  The expense of my meds alone was enough to crank up my stress to attack-inducing levels.  Finding the right meds, that my insurance will cover, has been a task.  It was pretty crazy there for a little while, but things are looking up for me now.  It’s also been hard to piece together a communicative team of experts.  In the military system, all my docs could see each other’s notes, save for my primary care – they were all in the same building.  So the convenience factor has shifted a bit, it’s been a learning process, but it’s getting there.  I am happy with the team I have now, and I trust their expertise and compassion – very much so.

Otherwise, nothing really new or crazy or wild to report.

I’m still me.  I’m still human.  I try and live life just as me, weirdo wacky Rose… and not as a person afraid to experience life and deal with the affects on my body.  It’s a balance though.  And in every attack I experience, I wise up about my triggers and the lifestyle choices I need to, and should, make to avoid bringing issues upon myself.

So, there you go.

I still have Porphyria.  It’s not going anywhere!  But, everyday I think I get a little better at navigating what it means for me and coping with it all.  And umm, I’ll give a shout-out to my handsome life partner, Duggs (my husband) because he’s been an awesome man to have by my side through all of it.  My whole family is very supportive, and once again – I find myself feeling very blessed to have access to competent medical care.

so this is the most recent pic i could find… oh boy, not so flattering, haha. but as you can see, i look normal and i’m outside and being wacky, laughing, etc. can’t really ask for more than that… so, life is good!

 

Thanks for reading!  And thanks for following along the wacky and weird and up-and-down journey that is my health.

XOXO,

HHR – Happy Hippie Rose

PS: Just because I share what I go through, please don’t think that my porphyria is all I am or all that I focus on.  I’m a real, breathing, dynamic person.  And since my health is something people who know me worry about… and by being open and posting about it publicly I can help add to the available points of view out there on porphyria (since there aren’t many), reducing stigmas and encouraging others to talk about it, learn more, and so on… I do tend to mention it more often online than I probably do in real life.  But it’s just like anything that’s in a persons focus – real, but only one part of a whole, multifaceted life.  

Ready To Pull The Trigger

So. I know I’ve been silent for a while.  A long while.

I drafted a post months ago… in early December, actually.  It’s well-written, it contains a message, and it’s very very me.  But I have not pressed publish.  Why?  Well, lots of reasons.  I was sick for a while in December, porphyria attack.  And then again in January with the flu… the remnants of which seem to not want to truly leave me be.

Work.  The Holidays.  We’re buying a house.

Reasons… kinda, but really they’ve become my excuses.  These are excuses.

You see, this post I wanted to share was about hunting season.  It’s drafted title is, in fact, I killed a Deer and I liked It.  (get it?)

You’d have to be living under a rock to not be in the midst of the gun-control melodrama that’s taken over our collective conscience lately.  The issues, outcries, politicizing, rallying, fear-mongering, paranoia, anger, and all the rest of it have just been a lot to deal with.  As a responsible gun owner, a legally licensed hunter and skilled shooting enthusiast – am I ready for what could happen if I publicly declare that I am okay with guns?  I wasn’t sure.

I’m still not totally sure about how to deal with any potential fallout that could occur.  And I know that’s likely.  I have friends and loved ones deeply entrenched on both sides of every proverbial line in the sand.  And while I’m pretty durn open and honest about who I am, and what I believe. (I mean, my Fb has plenty of pics of me holding and shooting guns… and not just hunting guns, but handguns, and yes – even an AR-15).

In the online blogging silence that ensued, my own internal voice grew stronger.  I do know who I am, and I love who I am.

And I, take it or leave it, am a gal with a gun.  I killed a deer, and I liked it.  I am not irreverent, malicious, cruel or casual in my approach to hunting, killing an animal, taking the meat from his body, and preparing it to be my own food.  In fact, I’m in awe of such power; I’m full of respect and admiration for the processes of life and the insight unto them that I have gained when looking down the barrel of a gun and feeling my finger on the trigger.

This is NOT a battlecry for gun rights.  This is NOT about Newtown or the CIA or our gun laws.

This is just a blog about me.  And at times, I am a hunter.  A happy hippie hunter.  Don’t think it’s possible?  Give my post a read and hear me out.

I’m pushing publish on it now.  I’m ready to be okay being me.

Black Lung Out, Insomnia In: A Lyrical Look At My Health These Days.

Howdy readers, pals, random web browsers who have stumbled here and have no clue of the incoherent weirdness with which you’re now facing head on!

So, last we chatted about my health I was going on and on about the mold issue in our home in Hawaii.  I’m happy, hell – ECSTATIC, to report that since leaving the home of the mold, I’m feeling much better.  Markedly, gloriously BETTER.  It’s amazing the difference, actually.  I think at first I was nervous to come right out and say it, out of fear that it would be short lived or something else.  But alas, it’s been a few months now and I just feel better all the time.  So, for me, the debate on the mold issue is settled: there was mos def something in that house that was crippling me, killing me softly.

Overall I feel great day to day.

I’m down on meds, at a very low level and in the process of a final taper.  Which given the past 18 months I’ve faced is quite incredible, honestly.  It feels amazing, I’m more than blessed to be writing from this vantage point right now.

I mean, clearly there’s no cure for Porphyria.  I’ll deal with that my whole life.  I’ve actually had one small flare since moving here – last month was a (ready? TMI is a-coming now…) right ovary month, and the cysts must still be there.  After a few months reprieve, last month was AWFUL.  Some of my close gal pals can attest, I was tethered to a heating pad and hunched over for several days.  It was so severe, it caught me off guard.  And of course in my domino reaction of a body – one thing can and will trigger something else.  Thus, the stress of acute pain sends me into a Porphyria-based decompensation (fancy way of saying “an attack”).  I carb/glucose loaded though and headed it off at the pass.

I’d like to thank my darling husband for his extreme attentiveness and ability to identify the early warning symptoms better than I can (honestly).  It’s what really helped us to keep things down to a little blip on the radar and not a colossal melt down.

So, the ovarian cysts.  That sucks, yes.   Not tragic though, so don’t cry for me Argentina.  It’s fine!   This month is business as usual and it seems as though Lefty-the-Other-Ovary is fully functional and normal.  To inquiring minds: fear not, I’ll seek the proper medical attention about all of this.  No more winging it, I promise.  But really, it doesn’t feel too urgent to be seen these days as  I’m basically fine.   Well.  Insomnia – that’s the only other ailment I really could mention.

The funny thing about Insomnia though, I don’t really hate it.  I’m a legit insomniac because I’m not ever tired.  I don’t drag all day, and at this point I’m consuming little-to-no caffeine.  I just don’t sleep.  It’s weird.  I always feel in the early hours of evening that I’ll be nice and sleepy come night time… and then, a flip switches and I just get that second wind.

the sunsetting over lake erie last weekend – as night comes on, i wake up.

Night time is a magical, quiet, intoxicating time.  I adore the sun and daylight, believe me.  But night is oh so nice too.

Last night I stayed up until about 5:30am today – at which time I rested for a couple hours.

Don’t scold me!  I know with the upcoming marathon this won’t do at all.  I know how paramount sleep is to proper recovery and healing, as well as overall health and injury prevention.  It’s not an every night, up all the time thing.  It just sometimes strikes me and that’s it- no sleep (’till Brooklyn).  I think it’s genetic.  I know my mother is a crazy night owl and often prone to Insomnia as well.  And my Dad, i don’t think he’s averaged more than four hours a night my entire life.  He just doesn’t sleep like the average person does.

Last night was one of those great nights to be alive.  After these hot, cloudless days – the crisp night air is an incredibly welcome change.  I actually ended up going for a nice long run around 1:30am.  It was gorgeous.  I love night running… the low moon hung huge, in a pretty golden crescent and the cool air felt awesome on my skin as I ran miles all over this sleepy rural town.  Afterwards I came home to a quite house, and I stretched and showered.   Still wide awake, a devilish idea struck and I found myself at Tim Horton’s moments later.   After a surprisingly good bagel breakfast sandwich I was able to feel sleep lapping at my body and mind.

drive thru, with my twenty pack giving me the eye

a very dark and kinda creepy, mostly terrible pic of me in the drive thru

thumbs up for getting the egg white version, thumbs down for bacon. oops!

Overall, I have no complaints!

When I was so consumed with the mold and it was taking over my body, I was sleeping SO MUCH.  Like, 16 hours a day kind of much.  Lethargic is an understatement.  Having energy and stamina now is a welcome blessing!  After the year and a half I’ve had, I’m very ready and willing to sweep the inconveniences under the rug and focus on the monumental improvement of which I feel.   And better I do feel – it’s amazing actually, liberating and nice.  It feels good to be awake, energized, and alive.

It’s been a long time coming.

FIN.

As always, thanks for reading!  xoxo, hhr  

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

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.

Part Two on my Health: An Update on my Current Condition, Porphyria

Part Two on happy hippie rose’s health: Current Update

Did you read Part One?

It’s about my medical history and explains where I’ve been and how I’ve gotten to now.  It’s the lead up.  Go check it out if you’re interested!

People have been asking me what’s going on with my health, so I keep promising to write an update.  And then I keep finding reasons to put it off.   I have so much sunshine to spew out that I hate to post about the crappier side of my life.  The down side, my illness.  But life isn’t all rainbows and giggles, and I can appreciate being real and down to earth, even if that means being Debbie Downer for a hot minute.

My Health Update

What’s going on with me now? I have porphyria.

duggs and i at one of my oodles of appointments

Acute Intermittent Porphyria, to be more precise.  I’ve known (for sure) that I have Porphyria since 2007, but I’m just now really dealing with it.  I kinda dealt with it back in Leesburg in 2009 when I was in nursing school.  I had a really cool hematologist who educated me some.  But I’m just now REALLY dealing with it.  It’s def been the focus of my 2011 and if I’m painfully honest: I’ve been pretty ill this year.

I’ve been way more sick (or is it sicker? I’m an English major, hello, I should know this kinda junk… I’ll go with sicker) I’ve been much sicker this year than you probably know, more worse off than you can glean from my facebook profile, not doing nearly as well as I let on.  I promise I’m not lying to everyone I know, I just don’t focus on the bad stuff.

My illness is insidious.  It sneaks up on me.  I can hide it sometimes.  I don’t have an obvious tell:  no bald head like a cancer patient.  No cast like someone with a broken bone.  I look and seem normal.  Sometimes, some days I am normal.  But a lot of times I am sick.  I wanted to write a post titled “I wish I had cancer,” and vent about how it is to have this untellable disease.  Back in the cancer days everyone knew I was sick and I was given this sorta breadth of understanding. People just knew what was up, as soon as you see the bald chemo head, you just know.  And there’s this quiet respect that’s offered.

But when people see me at church or in the grocery store now, when I post pics of snorkeling and hiking, when I leave my health out of conversations, it seems impossible that I’m anything less than spot-on healthy.  So it’s hard to get the understanding that I used to get back when my illness was apparent.    It seems like I’m not even sick sometimes.   And strangers have NO idea, they can’t tell at all.

When you have cancer you still do tons of normal things.  In between chemo treatments you function and live your life.  So that’s how I am now.   In between I have a life.

Of course I don’t really wish I had cancer.  But I wish that people just knew that I don’t feel well, but I’m still alive, and kept that in perspective.  I don’t want or need special treatment from my loved ones, friends, and acquaintances, just understanding.   Not kid gloves, by compassion maybe?  Everyone knows what cancer is; explaining porphyria is tedious.  A lot of people readily realize that having cancer is a like a full-time job: so many appointments, doctor’s visits, diagnosis testing, treatments, taking medications around the clock, etc.  I wish people realized how time-consuming my porphryia is for me.  It is my full time job right now.

And then when I’m not at the doctor, on the days I do have energy: I can be normal-ish.  I can hike for a few hours.  I can go boating.  Some days I can run.  It feels good to be active, it’s good for me.  Anything that keeps me moving and reduces stress is excellent for me.  But just because I’m well enough to sit on a boat or walk to the dog park with my husband doesn’t mean I’m totally well.

me, at the dog park one afternoon!

That’s the hard part.  If and when I am able to do things, people who see me don’t believe or grasp that I’m ill.  At worst they think I’m faking, and at best they just don’t get it.   Or sometimes when I feel miserable, people have no idea.  So when I’m slow to get my wallet out of my purse at the store, people get annoyed.  There’s no tag on my shirt that explains my hands hurt from my medicine.   Every time I answer the phone people keep asking if they just woke me up, so I know something must be wrong with my voice.  I just don’t feel good, that’s all.

Porphyria ebbs and flows.  I have good days and bad days, good days with bad nights. That’s another thing: I sundown.  I sundown badly (I’m crying as I admit this part, it makes me feel elderly, or weak, or just something not myself).  I sundown; meaning I get worse at night, sometimes a lot worse.  My pain gets worse.  Sometimes I get confused.  Last night I curled up in a ball shivering so intensely.  Duggs put a blanket around me and I cried that I don’t think I’m ever going to be better.  But yet…. just hours before I went on a three mile run.  It’s hard for me to accept and understand, it’s hard for everyone to understand.

Once, back while Duggs was still deployed, I tried to rest.  People were insisting that my activity level was hurting me.  So I spent several days in bed.  Literally.  I was propped up like a princess.  Back when I had my laptop, I stayed in bed all day long chatting online and watching shows on Hulu and Netflix.  I ate my meals in bed.  I didn’t do a thing.  And it made me feel worse.

My doctors actually encourage me to be active.  Yoga, walking, running, getting sun.  Sun is good: the natural Vitamin D I produce in the sun is better absorbed than the supplements I take.  The activity reduces stress.  So I try to stay active, even if that’s confusing to people.   The daylight is good for me, I love daytime.

A few hours of action isn’t wellness though.  It’s a break.  It’s a gift, honestly.  I’m not working at all other than the tie dye business.  Thankfully people haven’t questioned that too much or given me a hard time about staying home everyday.  The sad truth is that I’m just not up to it.  I’m not well enough for full-time work, yet.

Let’s back up… This genetic blood disease runs rampant on my Mom’s side of the family, having been passed down from generation to generation just like our recipe for red sauce. The disease works in an ebb and flow kinda way – sometimes popping in to make you feel crummy and sometimes going away for weeks, months, even years.  During the ebb times the disease can be almost untraceable, not showing up in simple blood or urine tests.  But when it flows, or rather when the patient experiences “attacks” or “decompensations,”  the disease can announce itself in many colorful ways.

Literally colorful, one of the hallmarks is making your peepee dark.  Other symptoms are relentless tummy aches, headaches, neurological problems from tremors to seizures, and on and on.  GI problems, neurological problems, anxiety, insomnia, low blood pressure.

Like I said, it’s a colorful disease.  And the disease has different forms, and people with different kinds have different symptoms.  It’s complicated to say the least.  Most of my doctors hardly know more than a textbook paragraph or two about porphyria.

But what about ME?  What’s this all got to do with me now?

The miscarriage I had stressed my body out and caused me to “decompensate,”  or in other words: have a porphyria attack.  The bleeding, the physical stress on my body, the emotional stress, the crazy amounts of Motrin they treated me with: it all contributed to a porphyria attack.  As part of that attack I suffered some nerve damage/inflammation in my neck/face, on just my right side.   Porphyria can cause nerve cell death/destruction all over the body.    So I emerged from the miscarriage feelings worse and worse, and my face and ear started hurting.

(Side note: my docs actually think that i don’t have crohn’s disease, that its really been porphyria all along, causing nerve cell death in my GI tract. crazy, huh?)

Okay, anyways… this porphyria attack (that probably started) in January caused the nerves on the right side of my face to get wonky.   I have problems with cranial nerve five and six.   The location of my pain mimics “trigeminal neuralgia” and for a while they thought I had that.  I actually went into the ER thinking I had an ear infection, that’s how this whole thing started and how they got to the trigeminal neuralgia diagnosis.  I wrote a blog about it a while ago: Blog on Trigeminal Neuralgia.

I saw a neurosurgeon back in the spring, who treats trigeminal neuralgia all the time, and she definitively said that’s not what I have.  My situation is different.   So, now i’m diagnosed as “peripheral neuritis, caused by porphyria.”  They also call it “atypical facial pain.”  So laymen’s terms: the blood disease I have flared up and in doing so messed with the nerves that concern the right side of my noggin; I suffer loss of sensation, facial pain, twitching, increased sensitivity to sounds, touch.  It’s odd and hard to pin down.  It changes daily.   But basically, the blood disease I have creates neurological problems, and for me, during this attack, it seems to be making my face hurt.

I get terrible ear aches, but anatomically, my ear is perfect.   I’ve had ultra sounds, MRIs, CT scans, X-rays, cameras stuck up my nose, you name it – they’ve done it.  I’ve had lab work drawn, tests run, everything.   And nothing is “wrong” with my ear!  Yet it HURTS. The pain is crippling.   When it flares up real bad, i’m rendered nonfunctional.    The ear pain starts deep down inside, it feels like an inner ear infection, and it’s only ever on my right side.

taking more of my blood for more of their testing

The pain starts in my ear and radiates out.  So my teeth, my jaw, my cheekbone and my eye socket all hurt.   I get headaches behind my right eye.   When I open my mouth and air touches my teeth, sometimes it hurts.   And it’s only on the right side.  It’s so weird!

So anyways, they have me on all kinds of meds.

our med cabinet, well... they're all mine.

Nerve meds, like lyrica. (i was on gabapentin, but that didn’t work out so well).   I’m on pain meds and more pain meds.   The Lyrica has all kinds of side effects: I retain water terribly.  So I’m on diuretics (lasix) to help with that.  But when you take diuretics, you have to take potassium, so i’m on that too.  It’s a crazy cycle!

When I wake up in the morning, the water retention is the worst.  My hands and feet get so puffy and swollen, I can’t even make a fist and it hurts to walk.  The skin gets stretched so tight on my fingers that I get these little cracks in my skin.  It hurts to type when that happens.  So I take the lasix in the morning, and I pee all day long.  If I take the meds on an empty stomach, I get sick.  So I have to time my meals around my meds.

Since I had a small bowel resection in 2004 and I’m missing a couple feet of small intestine (I have no terminal ileum) I can’t take certain time release meds, or anything that absorbs enterically.  I have to take stuff that gets absorbed in your stomach.  Then, with porphyria itself, there’s all kinds of meds that are dangerous to take.   Meds, meds, meds.  It’s insane.

the topical patches i use for pain

i wear the patches 12 hours on, 12 off. duggs calls me "patches" now. it's cute.

In May, before Matthew’s homecoming, I went to my docs and said I needed to get off all these meds.  The pain was finally under control and I was doing better.  So they started me on a taper.  I was tapering off the meds, bit by bit every week.  I had some rough side effects, like dreadful night sweats for one.  By the end of June, I was pretty much done with most of the meds.  I had like a week of tapering left to go.

It was amazing!

I felt like things were under control. I felt like the end was in sight and that I’d be back to normal. The big deal for me is that while I’m on all these meds I can’t get pregnant. They’re all really bad for in vitro babies. Sadface.  So, tapering off felt great. I was proud of myself!

Then, I cracked that tooth.  And I was in severe pain.  Dental drama is a big deal for me. The meds they use at the dentist is bad for porphyria.  Having dental work done stresses my body out.  And since I’m terrified of the dentist, my anxiety and stress levels were going up and up.

me, right after i had my bad tooth yanked

hey bad tooth, see ya never

So the doctors decided I should go back up on the meds.  Stressful events kept piling on… and they decided to up and up my dosages to “hold me.”  I mean, as soon as I cracked that tooth and my mouth started hurting, my ear started throbbing again.  I know I needed the meds, but it was a big bummer to come SO CLOSE to being done, and then go back on again.

So, all of my docs: my neuro, the neuro surgeon, my geneticist, the metabolic team, my case manager RN, the pain clinic, and my hematologist all are in agreement that i need to see a porphyria specialist.  That’s a pretty intense team, huh?  I love my case manager. She calls me all the time and she sorta keeps everything organized for me. Cathy.  She’s great.  My neuro is the chief of his dept, and he’s the one who has really taken the reigns of this whole porphyria ship.  He manages my meds and treats me for the ear pain.  He’s amazing.

we got a glass at mcdonald's and we gifted it to my neuro, it was pretty funny. maybe you had to be there? (the glasses are free, lol)

My geneticist has been eductaing me about porphyria and she’s been building a family case for me.  You see, the disease is SUPER genetic and about half my family (on my mom’s side) seems to be affected.  I won’t call any of them out specifically… but yeah. Anyways. The geneticist is amazing.  She made me an emergency protocol.  If I were ever hurt or let’s say – in a car accident – and brought in to the ER, I’d need special treatment because of my porphyria. So I have these papers I keep with me, and Matthew keeps a copy with him, the glove compartment, etc. – explaining what to do.

I have to wear a medical ID now. They made me a red dog tag, but since that’s ugly I have a Road I.D. bracelet. It’s awesome.

my medical ID

There are only five porphyria specialists in the US, and they’re all on the mainland. So my case worker has been organizing my trip to see one of these doctors. I still have no idea where I’m going or when – but it’s in progress. I’m having some genetic testing done now for one of the specialists. I’m probably going to go to Mt. Sinai in NY.

So yeah. My face hurts all the time. I wear my medicine patches on it. Sometimes I ice it. I get nerve blocks and shots of steroids in my neck and shoulder to help. I have a HUGE muscular knot in my back on the right side which might be playing a role in this.  The steroid shots are “trigger point” injections.  It’s a nerve path thing.  They shoot you in one area to affect pain in another area.  A shot in my back can aid the pain in my face, because that’s how the damaged nerve path works.  In theory.   I’ll try anything, so I get the shots often: sometimes in my head (that didn’t work well), sometimes in my back, sometime sin my neck.  We’re trying to figure out where the damaged nerve path is.  The shots have some numbing medicine (one of the -caines, like lidocaine) in there and some steroids.

my dad, my neuro, and me, during a trigger point session. my dad is an expert in trigger points it so happens.

the trigger point poster

my face, when it went half-numb from some trigger points once. it wore off after a few hours, no worries

I’m learning more and more about porphyria as all of this goes on. It’s been crazy. 2011 has been a wacky, roller coaster of a year!

From my porphyria notebook the genetics dept prepared for me:

Rose has the clinical features and a family history for Acute Intermittent Porphyria (AIP). The human porphyrias are clinical disorders reflecting defects in heme biosynthesis.  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.

my porphyria notebook

my emergency protocol

Does this link work?  It’s for a video i posted on FB of what happened to my face after some trigger point shots my doc gave me.  Oy.  Half of my face went numb!  I tried to upload it to this post, but I can’t figure it out.  My apologies if the link doesn’t work!

So that’s what’s been going on.  This weird balance of okay and not-okay, of heathly times and illness.   I seem to be getting better, in many ways.  In some ways, I’m stalling out.  I need to get to a specialist, one of the five porphyria clinics on the mainland.  Hopefully, that’s something that’s able to happen and I’m able to get better so once Duggs is out of the USMC I can work.  I want and need to be able to work, so hopefully something will give soon.

Any prayers for healing, good vibes, good thoughts, anything happy is VERY appreciated.  

 

And, I will update.  I’ll update sooner next time, I promise.  As always, thanks for reading.  It means a lot.   Love ya, happie hippie rose

PS – and let me just add… and i’m such a heel for not mentioning this earlier:  my husband has been so amazing throughout all of this.  He comes to as many appointments as he can, he takes care of me dutifully and with love.  He knows my whole medicine routine and regimen better than I do!  He is the best.  He cares for me with so much love and tenderness, it’s beyond words how blessed I am to have my Duggs caring for me day in and day out.   Thank you my love, for taking such good care of me.  I’d be a goner without that guy for sure, he’s the BEST.