Rage.

   Tonight, I'm ANGRY. I'm on a hormone surge that feels somewhat like what I imagine the Hulk feels like right before he starts morphing into the big green guy. I've been down all day, warding off a crying fit - which while you're surrounded by a bunch of females at work who are all mothers and insanely intuitive, is NOT an easy task. Mind you all, this crying fit, was prompted by my realization that I'm going to have essentially permanent bruises on my inner elbows, and junkie-esque track marks to boot. Now THAT'S super, mega hot.

   After I got home, my sadness turned into anger and rage. What caused the mood swing, you might ask? I was surfing around Facebook and noticed that Luke had "liked" a picture of an old girlfriend (we're talking 5 or so years ago). BIG FUCKING DEAL CHELSEA, YOU'VE DONE THE EXACT SAME THING BEFORE, IS THIS SERIOUSLY WHAT YOU'RE GOING TO LET CAUSE A PROBLEM TONIGHT?! is the current line going through my head. It's like Jekyll and Hyde up in my noggin, I tell ya - one part says it's okay for me to be insane about things that don't matter, such as my boyfriend "liking" a picture of someone whom he hasn't even spoken with in months and to go ahead and freak out about whatever I want because for some reason I'm entitled; while the other part tells me that in about 20 minutes this all will be over, I'll be back to a more plateaued state, and will look back and realize just how out of control my emotions and thoughts were minutes previously. I just need to ride out this roller coaster until then... 

   

   Welcome to my life.

   

   I proceeded to shut myself in my bedroom in an attempt to hide after realizing the picking-a-fight-over-nothing state that I was in - I am especially good at this. I was finally found, and started crying my eyes out because of all of the feelings overwhelming me. I'm in withdrawal. I have no control over what I'm feeling - can't turn anything off, or shut it out like I usually do. I'm exhausted, feel like I got run over by a bus despite the full nights' sleep I've been getting lately. I have no energy to do anything, but know that there are lots of things I need to do. I'm stressed about the holidays coming up, and just life in general because I'm good at worrying about things that are years down the line.

   I'm so angry that I have to deal with this. Going through withdrawal. Everything is heightened. Having bruises and marks on my arms that I can't hide, likely causing people to ask me "what's wrong?", which I don't mind answering, but I also don't want the one-uppers (yeah we all have them) doing their thing and making it seem like their ailment is so much worse - that's part of why I hid this for so long. Sure, it's nice to have support coming from places I never even dreamed of; but what REALLY pisses me off are the people who are listening to my story and either butt in, or listen to the whole thing and immediately reply back with how their lives are so much worse. I completely understand that most people have some shit to deal with, on an at least semi-regular basis, but you know what? I didn't ask what your issue was. You asked me. DON'T try to one-up me. Just don't. Because you piss me off and make me regret telling you in the first place because you make it seem like this doesn't control my life and isn't a big deal.

   Newsflash - this controls my ENTIRE life and is a HUGE deal. It's almost second nature to me now, but there was a point in time that I had to consciously think about every move I made and whether or not it was likely to result in a swelling episode. I'm usually a trooper about it, putting on a good face and just agreeing with people when they brush my issues aside. Today, I can't. Emotions, hormones and YEARS of harboring it in have let the flood gates crash wide open. I HATE that I have to deal with this every single day. I HATE that I have absolutely no control of what's going on with my body right now. I HATE that my options were to take steroids every single day and deal with the side effects, or need an infusion once every three days that will leave me bruised, scarred and dependent. Of course the latter option is better and I'm thankful that it is an option for me now, but in reality, it still SUCKS. And I'm LIVID that I don't even get to choose anything other than "deal with it."     

   I have a great support system; they will make me hold on even when I'm ready to let go and fall...which I've already been incredibly tempted to do. I know this will be better once the ride is over, but I'm ready to get off now. Hurry up, steroid free living, I can see you on the distant horizon...

   ~Somewhere between always and forever, Chelsea Leigh

Withdrawal

   Over the past few days, I've been asked by many people how I'm feeling - if I'm noticing a difference based on the new medication I'm on or not, usually followed by the question, HOW will I know it's working? What's interesting is the answer I have to give. I've been feeling fairly normal this past week...and that's pretty much the only way I'm going to know if the medication is working. I won't notice an actual change of symptoms per se, as one might expect; what I will "notice" however, is that I WON'T be having symptoms or full blown episodes. Yes, it does sound strange and kind of backwards from what you'd expect, but that's pretty par for the course in my life.
   Normal. To me, "normal" would be NOT constantly being afraid of minor activities throwing me out of commission for a few days due to immense swelling attacks. Normal would be NOT having bouts of sickness where my stomach does the weird, strange thing that it does - it's a sensation that I can't describe in words to anyone else, it's just a feeling that I have and I know what it is when it's happening. I used to get really frustrated with anyone who would complain about their lives being boring, or normal; it seems like a minuscule thing, but it's something that a lot of people take for granted, something that a lot of us aren't lucky enough to just have every day. Maybe it seems silly or ridiculous, but speaking as someone who would do unspeakable things to experience normal on a daily basis, if you're lucky enough to have normal - don't take it for granted, you don't know how precious it is.
   I'm not there yet, but I'm hoping (and the outlook finally is a really good one) that within a couple of months I will be one with the masses.

   The next piece of my journey is coping with steroid withdrawal and it's subsequent symptoms. Common withdrawal symptoms from steroids include weakness, fatigue and sleeplessness. Oh yes, count me in for the whole TRIFECTA. Add on top of those three, an emotional roller coaster and I feel like what I imagine a pregnant lady immediately going into menopause would be like hormonally, if such a thing were possible. I spent the majority of yesterday fighting off a teary break down, for no reason in particular - other than that I'm a week into knocking my steroid dosage down from 1.25 mg/daily, to 1.25 mg/every other day.
   Hormones are a finicky thing, and aren't anything to screw with. I'm psyched to be on this new journey and a lot of other things in my life seem to be falling in to place, so I'm on happy-high...but my medication is currently doing the emotional driving and dictating how I feel and what I'm able to express. I cannot WAIT until I reach the plateau I'm hoping is in my semi-near future; I'm sure Luke is right there along with me, bearing with crazy-girl syndrome in all it's glory, and being a real trooper.
   Hopefully I'll be able to curb the exhaustion, but I foresee a very restful next couple of months coming up. I'm not currently doing a great job of fighting off  the "hit by a bus" run-down feeling, as I write to you from the comfort of my bed, glass of OJ to my left and a sleepy golden retriever to my right.
   I have infusion number three tomorrow, flying completely solo and Ginny will be here later in the evening to discharge me from visiting nurse visits. I just have to keep on trucking through, beat the steroid withdrawal and then things should be smooth sailing from there on out.

   ~Somewhere between always and forever, Chelsea Leigh 

Day one - personal voodoo doll

   Today I had my first infusion of Cinryze! My home health nurse, Ginny, came around 10 am this morning and I felt like a little kid waiting for Santa Clause as I sat at our bar watching out the kitchen window for her to arrive. Ginny is a sweet old lady that has been a nurse likely longer than I've been alive and could probably start an IV on a goat if she had to; that's the kind of person that you want to have teaching you how to stab yourself with surgical steel on a regular basis.
 
   This is my two week supply that I mentioned yesterday. Yes, I get a box filled with all of these medical goodies once every TWO WEEKS...good thing we don't pay for trash removal!

  Out of that two week supply, this is what I will use (plus some random bar paraphernalia) for each infusion :

 
   I'm one of those weirdos that likes needle sticks, (tattoos, piercings, things of the sort), so I figured you know what? Day one, I might as well go balls to the wall and try to insert the needle into my vein all on my own - worst case scenario, I miss and either my nurse or my paramedic lovebug will take over and restick me correctly. However, being the perfectionist that I am, not accepting failure or defeat ever, you better believe I got that sucker on the FIRST STICK (!!!!!!!) with my non-dominant hand, mind you.

   My infusion went very well, without any adverse reactions so far, but I have my handy-dandy Epipen standing by just in case. This new regimen is going to be an added hassle, and is going to make things like traveling a little more difficult; but if I get the relief that I'm hoping for from it, it will be more than worth the sacrifice of time and effort.
   Ginny will be coming back on Sunday for round two, and hopefully Mom and Dad will be joining us - the more people that know how to do this for me, the better. I definitely am looking forward to the bonding experience that will be allowing my parents to stab me with needles...

   ~Somewhere between always and forever, Chelsea Leigh
 

Waited my whole life

  This post is going to be dedicated to explaining the new adventure I'm about to embark on in the form of at home, self administered IV infusions.
   Starting tomorrow, I will be giving myself IVs once every three days, in order to administer my Cinryze therapy. This is something I thought only existed in my dreams, and that I'd never have the luxury of experiencing - being able to give my body the exact pieces and parts that it is unable to make on its own. Lucky for me, Luke is a paramedic and has been giving people IVs for the past 10 or so years, and I have him at my disposal. I do have to learn how to do it myself and will probably do one every couple of weeks just so I don't get too rusty and to keep up to par for the days that our work schedules are completely opposite and I don't see him for 24 to 48 hours; but if I have a professional at my disposal, I might as well put him to work, right? :)
   You may be wondering to yourselves, "where does she get this medication, and all of the supplies to suddenly have the ability to get an IV in her living room while watching another episode of HIMYM?" Well my friends, let me tell you - health insurance and people with lots of money who DONATE to financial assistance programs are amazing things. Allow me to break it down for you - each dose of Cinryze costs approximately $7,000 and I need one of those bad boys every three days; that makes ten infusions per month, equaling an estimated $70,000 monthly. That's just the medication itself; that's not including the box of medical supplies I also receive biweekly containing butterfly needles, saline filled syringes, reconstitution water, empty syringes, saline locks, mixing needles, sterile drapes, individually wrapped sterile gloves and a sharps container.
   Now, I have a really good job that pays me fairly decently. But I'm not sure even a doctor's salary could actually afford the copay involved with those kinds of costs. Luckily, gratefully, amazingly there is a financial assistance program that I fully qualify for that will provide up to $100,000 YEARLY in copay assistance - and that's plenty to cover what the insurance won't. I can't even begin to think about trying to put into words the incredible feeling that surges through me when I think about how appreciative I am to that assistance program and all of the wonderful people who work behind the scenes to make things like this available to people like me.
   Speaking of behind the scenes, I need to take a moment to put my parents up on a pedestal. I never used to understand why my Mom would get so upset every time I got sick, and ended up with my face in the toilet puking my brains out for hours on end...until I got to a point in my life when I started thinking about how guilty and upset I'm going to feel if/when I pass this wonderful little defect along to my kiddos. I used to think that she was mad at me for BEING sick...and now I realize that she was pissed that this is a piece of her that she gave to me, and unfortunately can't ever take back. I never realized how awful it had to have been for my Mom and Dad to watch me be sick, and not be able to do a damned thing about it other than finally drag me to the ER when I said I was ready - there were sleepless nights for my whole family when I'd go to the ER at 2 am, and finally be brought back home with just enough time for my Dad to get ready for work and head back out the door. I know that as a parent, your role is to do anything for your kids, but some parents get dealt a heavier hand than others, and mine are no exception. They've been the base of my support system all along and I'm positive they're to thank for the strength that I have now - that's not even mentioning the financial obligation they had until I could afford my medications on my own, because let me tell you, steroids aren't cheap either. Living with this has made me stronger, and it's definitely made me appreciate the small things, not taking so much for granted, because I physically can't some of the time. Science and medicine are always making new breakthroughs and refining the knowledge that's already out there, so I'm confident that treatment and living with this genetic shitbag can only continue to get better and easier. So you know what, Mom? This is a bond that we share, that is indescribable to anyone else, and I'm ok with it; sure it's a little screwed up, but it's ours.   

   Stay tuned for an update tomorrow on how day one of being my very own voodoo doll goes.

   ~Somewhere between always and forever, Chelsea Leigh

Backstory

   Well, here I am, joining the blogging community, at a time that I've been waiting for, quite literally, my entire life. I realized earlier today, that I've kept what I've been going through to myself, my family, and a very few close friends. I'm not ashamed of my condition, nor did I purposely hide it from everyone; it's just hard to explain, especially when I'm not in a constant state of affect, and when I'm not in a full blown episode, I like to forget about it and pretend that I'm normal.
    Here's the deal, what's wrong with me - I have a genetic condition called Hereditary Angioedema. I've had it since the day I was born, thank you to an awesome roll of the genetic dice. Luckily for me, I was able to be diagnosed via a simple blood test, whereas my poor mother (who also has the condition, cue the hereditary part) went through years of absolute HELL to reach her diagnosis.
   Hereditary Angioedema (HAE for short) is super rare, occurring only once in every 10,000 to 50,000 people. For those of you science/biochemical freaks like me who want to know how it works on a molecular level, here goes (for those of you not in that category, stay with us, I'll give you the "not-so-science friendly" version in a few lines) - the class of HAE that I have, type 1, causes a defect in my genes that controls the production of the protein C1-Inhibitor (INH) in the liver. C1-INH's role in the body is to regulate the biochemical interactions in blood based systems, in the way of inflammatory response. Defective or inadequate levels of C1-INH, not performing its regulatory role, results in the production of unwanted peptides that induce the capillaries to release fluids into surrounding tissue, and subsequently causing all kinds of edema in any body locale including hands, feet, abdomen, lady parts, throat and face.
   *Not-so-science friendly version* - I don't produce the inhibitor that keeps the protein that controls the body's ability to swell in check; so, I'm prone to swelling - and by swelling I don't just mean the puffiness you get along with a black eye, I'm talking the kind of swelling that causes your skin to split open.
   So, I swell. Doesn't sound so terrible, right? I'm gonna go ahead and say no, wrong answer. Basically, there is no single, exclusive trigger, nor is there any specific body location that the swelling is "assigned" to. My personal triggers for an episode are stress, trauma, lack of sleep, emotional roller coasters, and the random "because my body just f****n felt like it"; the most common locations of my swelling are my hands, usually because they experience the most "trauma" - I use quotes because something as seemingly harmless as hitting a few golf balls at a driving range will cause my hands to do their thing and blow up on me.
   A few years ago, every couple of months I was making a trip to my favorite home away from home, Club Fletch's ER. The trigger there, (which I was able to see and accept years later after being removed from the situation) was my ever lovely and INSANELY stressful relationship with Scott. I will not use this space to bad-talk him or our relationship, other than to say, those were the most stressful years of my life and I have medical proof of it. Anywho, I'd go in to the ER after a few hours of vomiting thanks to intestinal swelling (my Mom has said that the intestinal pain associated with an abdominal episode is worse than labor pain - so kids will be a breeze, right?); subsequently, I was unable to keep anything down and was walking the fine line of consciousness versus blacking out due to dehydration. After seeing one to five medical students, a resident and an attending who all, I'm sure, were just basking in the medical rarity that is my life, I'd demand my two bags of saline, and a dose (sometimes two if it was a real doozey) of Zofran (IV anti nausea amazingness for any of you non-medical personnel). Then, I'd go home and sleep off my absolute exhaustion for two days and start all over. Rinse and repeat. 
 
   I'm sure that a lot of you are thinking something along the lines of, "well, isn't there anything that you can do, some medication you can take?" Until very recently, I had limited options available to me. At age 17, the summer before I went to college I started a daily regimen of anabolic steroids. These steroids are supposed to help, but I call bullshit. The specific 'roids I was put on, are often prescribed to patients who have undergone surgery and are subject to weight loss, and thus need to gain weight. I do NOT need help in that category, and having been on these steroids for the past 6 years straight. A few months ago, I tried to ween myself off of them, thinking I somehow miraculously would be able to just not need them anymore, and was horrifically unsuccessful.
   At this point, I should interject and give some serious credit and thanks to my boyfriend, Luke, for bearing with me thus far in the process. Let me tell you, trying to remove daily steroid use, will make a bitch crazy. PMS doesn't have anything on the mood swings and emotion rush involved with removing a daily dose of testosterone in a female body. Although I'm sure it would've been easier (and seemed the more reasonable and sane option at times) to run for the hills and away from me, Luke has stuck by me, been my rock through it all, and I love him even more for it. I assure you all, I was an absolute nightmare, ranging from crazy psycho, to a heaping ball of crying mess when I came to the harsh realization that if I can't remove the steroids from my daily life, then I'm not going to be able to have babies.
   Ah yes, babies. I'm 24 years old, so naturally my uterus is starting to give me the "I'm here for a specific reason" vibes, and although I'm in enough control to not just give it a fetus to house for 9 months, it is something that is on my radar. In my case, it's even more difficult and something I actually have to plan, because anabolic steroids + fetus = unfortunate outcomes; not to even mention the list of side effects that steroid use has a on a female body...which we need not get into.
   So, I set out on a mission to find a new treatment plan for myself. After some research, and reaching out to my patient advocate at the Hereditary Angioedema Association, I had a new plan to present to my physician - one that I wouldn't take no for an answer on.
   The new treatment I was after, is called Cinryze. Cinryze is purified human C1-INH esterase - EXACTLY WHAT I'M MISSING!! My doctor was/is completely on board with this plan of action, and immediately signed off on every piece of paperwork that was sent his way in the past month - and there have been MANY.
 
   There you have it - that's my story, one that I've kept to myself for a very long time and am finally ready to share. I appreciate all of the thoughts and prayers and kind words you all have already sent my way; you create an amazing support system that I have come to rely on more than I realized. I intend to use this blog to keep everyone updated on my progress with my new treatment, and hopefully new "outlook" on my future. In my next post, I'll give a deeper look into the upcoming Cinrzye treatment I'm about to embark upon.

   ~Somewhere between always and forever, Chelsea Leigh