Frequent Flyer

There is a truth in life that we all try to avoid.  Some of us go much longer than others without having to face the reality but one day, everyone, everywhere, in every-time, will come to the reality that we are not in charge.  That our life is not our own, we can not do whatever we want or follow our heart, or become anything and no amount of meticulous planning, saving, working toward a goal can stop the inevitable from happening.  At some point all of us are going to face a moment where we realize just how out of control we are....well ladies and gentlemen, I have reached that point.  Do not get me wrong I have had moments like this before.  Its kind of impossible to grow up with Cystic Fibrosis, get a lung transplant and have cancer while still living in the blissful world of having control.  Having lived life both ways, I am human after all and we are all prone to believing we have control at one point or another no matter how much life forces us to see we do not, I would argue that life is actually simpler and more peaceful when we learn to release control.  Especially when we release control to God, who if I'm completely honest with myself is and was in control all along.   Luckily for us God has a way of forcing us to see this and that is the exact spot I find myself in. (or was in last week when I started to type this) .

I am finishing this almost exactly a week later and my life has settled a bit.  It is still wildly unpredictable but I have had a week to ponder the unpredictable and have had some nights of good sleep in my own bed to help me cope and my peace has returned.

So heres the deal:

Last time I wrote I was starting out on this path to figure out why my lung function had dropped.  One short nap for me, and an exploration of my lungs for my docs and it was discovered that my silly old immune system is acting up again and attacking my lungs.  My doc decided to give me rATG again (the same treatment I did last December).  This med is given inpatient so I was checked into Duke Friday 2 weeks ago.  I was in the hospital for almost a week and got out last Wednesday. The treatment went fine again like it did last year.  I spent the days sleeping off the Benadryl high and the nights walking the halls, painting, watching movies and confusing my nurses with my night time awakeness and strange use of a bed.  We are hoping this treatment will help bring my lung function up at least a bit and stop the rejection as the bronch showed acute rejection which is a reversible kind.  As if this was not enough we also discovered that I might have specific antibodies that are targeting my lungs.  This is another part of the immune system and what a flu shot works on, you give the shot, your body basically makes a key that identifies the flu virus so that the next time your body sees it it can identify it quickly and wipe it out.  Great if it is the flu not so good if it is your lungs.  As a result of this we decided to attack my B cells with another treatment, these are the cells that make the antibodies (keys).  This treatment is one of the medications I received back when I did chemo so it is not a drug I have never had before.  The treatment will be a weekly outpatient infusion for 4 weeks.  It is scheduled to be given at Duke but we are in the process of getting it moved to Colorado.  I got my first infusion of it yesterday at Duke that went alright.   I had an allergic reaction to the medication, which happened last time as well.  My throat got very itchy and stingy and my lungs felt tight.  At the same time my blood sugar was dropping, unrelated to the med, which lead me to feel AWEFUL.  Some juice, a shot of epinephrine, some IV Benadryl and more steroids later I felt back to normal and was able to start the infusion again, this time with no drama, as usual I was super high from the Benadryl and forced myself to sleep it off, so you know there would be no prophesying.  

Hopefully the combination of the two treatments does the trick.  It is kind of silly because unlike a normal medication that helps your body to work better these meds are meant to wipe out my immune system as it is working too well, great for not getting sick much this past year, terrible when you are trying to keep foreign lungs happy in your body.  If these do not work we have a few more tricks in the bag and another transplant is also a possibility.

Although I have some answers and a mild idea of the next few weeks, most things are still quite up in the air.  I have to take a month or two off of work as my doctor feels I need to rest between treatments and should not be retuning to work yet.  This has been hard for me as I have really enjoyed my work and miss getting to interact with my clients.  I also feel bad as my clients have no idea what happened to me all they knew is I would be out for a few days and then back the next week.  Well that has now turned into over two weeks with no return in the near future.  Basically I just suddenly disappeared.  Which has been kind of strange for me to think about.  The other big thing is that I am looking into the possibility of moving back home.  I have been in Phoenix off and on the past 4 years and have been living here long term for the past year.  Although I constantly think about moving back home and miss my family I also really like the warm weather and am not looking forward to going to CO in the winter.  I still am not sure what I will end up doing but I have decided to at least go home while we figure things out so that I am not alone out in Phoenix as these treatments and my decreased lung function have really been affecting my daily functioning and leaving me fairly tired.  So that is the plan for now.  Is it set in stone, definitely not.  This could all change tomorrow.  My lung function is pretty low and we do not have much wiggle room, if I wake up tomorrow to a lower function this whole plan could be dropped and I could be rushed off to Duke to be hospitalized until lungs become available for me.  At this point anything can happen.  So we wait, trust God and go about daily life as best we can.  

TTYL

Anna  

beep, beep, beep

With nothing new to post in the world of transplant and the fact that I am still in the hospital and a bit board I figured it was time to back this thing up and give anyone out there wondering a quick, History of Anna (be sure to read that with the voice of one of those commercial voice over dudes! Trust me it adds to it!!)

So Grab on, here we go!
I was born, obviously, I'm pretty sure everyones story starts out the same.  I was born in Colorado, one of the few states at the time that checked for Cystic Fibrosis in the newborn screening.  My test was positive so further tests were done and it was confirmed a few weeks after I was born that I was the lovely "winner" of cystic fibrosis from the genetic lottery!

A quick note for those readers out there that are lucky enough to have no idea what Cystic Fibrosis, or CF as it is shortened to, is.  CF is a genetic disease that affects the mucous membranes of the body.  If you want to get all technical I could tell you that it specifically effects the protein that makes up the chlorine channels, causing them to either not work correctly or in some cases be nonexistent and is a recessive disease (meaning you need two mutated genes to get it).  This lovely mutation creates not so lovely thick sticky mucous that gets stuck in the various organs and organ systems it effects, mainly the lungs but also the digestive system.  The teeny tiny error in a CFers genetic code leads to some huge consequences, frequent and sever lung infections, digestive problems, malnutrition and insulin deficiencies being the main ones.  There is currently no cure for CF, at least not for most of the different mutations, but there are many medications that can help to slow the process.  In the end though, CF leads to death (though as I like to say, life leads to death....well that and cancer! )

Sounds fun, right?  

Okay children gather round as we dive back into this fabulous tale!

Due to my inability to develop a fantastically advanced memory at a young age I do not in fact remember most of my childhood so bare with me here as this information is slightly ...fuzzy.  I do not believe I had many problems with my CF as a young kid.  I did take enzymes when I ate and did nubulizer treatments I think daily.  I also did what is called CPT, which is where someone, my parents, would pound on my chest to help loosen things up. (I have no idea what it stands for I would guess chest percussion therapy...?)  When I was I think 4ish I got the wonderfully exciting vest machine (it is actually a bit exciting) which replaced CPT.  As far as hospital stays I did not have many, and none for "normal" CF reasons.  I was in the hospital I believe for testing when I was really little and then again at maybe 3 or 4 because I got pneumonia and was not getting better.  I also think I was in the hospital after having a bronchoscopy, where they put a camera down your throat and look at your lungs.  During the bronch they tried to open up my lungs more and instead ended up releasing a bunch of bacteria which made me pretty sick.  Again I have no actual memories of any of this so it is purely what I remember being told but overall I think I had a pretty healthy and normal early childhood.

And then I turned 9...
at 9 years old my friend CF decided to awaken and get busy.  It launched its first attack on me in October of 2000.  After seeing the doctor for one of my regular check ups he decided it was time for me to join the world of other CFers and experience the wonderful invention of a "tune-up".  A tune-up is where you take a person with CF from their normal life, put them in a hospital for at least 2 weeks and pump them full of drugs, while doing nublizer and vest treatments 4 times a day and shoveling in as much food as possible in a full fledged counter attack on CF.  Now I know how great this sounds to the normal person, but imagine for a second that you are a nine year old kid who frankly doesn't feel sick at all.  (Oh yeah and its also almost Halloween)  In addition to the horribleness they call the hospital, which I like to refer to as jail, it was also discovered that I was the happy home to the superbug MRSA.  This got me, at the time, a one way ticket to the "isolation zone" a crazy world confined to four small walls, where visitors wear yellow "banana" suits and the only access to the outside world is a window with built in curtains that faces a parking garage.  Again did I mention it was almost Halloween and I was 9.  Then because this wasn't exciting enough I was put on steroids, which don't mix so well with me.  These drugs give me a ton of energy, make me CRAZY and send my blood sugar through the roof, we're talking high 400s to 500s here (normal is 70s-low 100s).  This made for a very unpleasant experience for both my mother (who stayed with me) and myself.  Luckily I have mostly blocked out this and subsequent hospital stays, though I do remember some details, trick-or-treating in my room by knocking on the bathroom door and having my mom pretend to be different people and giving me candy being one of them.  Luckily as most policies in the hospital like to do, the MRSA policy changed, allowing a person to be taken off isolation if they had a negative MRSA test enough times.  I thankfully only tested positive for MRSA that one fateful October so when the policy changed I was taken off of isolation and let me tell you what a world of difference that did for me psychologically.  There is just something to seeing people in normal clothes and knowing I can leave my room if the desire were to strike.  

I was in and out of the hospital a few times a year from then on.  It became a routine almost, go in for a check-up be told I needed a tune up, be pumped up with drugs, go crazy, get out, be happy and then do it all again.  

By 7th grade the decision was made that I needed more support nutritionally and surgery was scheduled to have a g-tube put in.  I have had it for 8 and a half years now and have finally made peace with it.  I also, thankfully, don't really remember not having it or the entire process of getting it which helps.  I do know two things; it hurt and it took a while before it stopped hurting. I have just recently started using my g-tube every night while I sleep, which is the typical CFer way to rock the tube!  The night feeds are helping me though, so although my lungs are going downhill my weight is the best it has ever been! (well almost)

As far as my lungs go they are doing pretty bad, obviously.  In February or 2011 I got pretty sick and was in the hospital for a few weeks.  During that time it was discovered that my right lung had pretty much collapsed, we tried to get it to reopen but with no luck.  Since that time I have done fairly well and felt decent for me but have definitely been very limited in what I can do.  This past winter I made the decision to move to Phoenix for school.  I loved it in Phoenix but unfortunately due to the lower elevation had a lot of trouble when I would return home.  I ended up getting pretty sick over spring break and never really recovered from that.  That brings us to now.  I am currently on my second hospital stay since I have been home for the summer.  I was only home a total of 13 days before I felt the need to return here. I am also on oxygen 24/7.  I had known oxygen was needed when I returned to the hospital the last time but hoped I could be weened off of it completely.  This has not been the case and instead it is seeming that I will be entirely oxygen dependent until I get my new lungs.  Hopefully that will be sooner rather than later but until then I am learning how to deal with yet another aspect of my CF. 

What do all those crazy words mean????
Here is a list, with explanation of the various things I do on a daily basis for anyone thinking a nebulizer is some futuristic zombie tranquilizing gun and the vest, is the latest fashion trend that only CFers seem to be in on.


     Enzymes: Yes these are exactly what they sound like.  They
              are digestive enzymes to help replace the ones     
              that my body can't produce.  I take them any time I         
              eat.
    The Vest: It is indeed a vest though I would not say it is    
              very fashionable.  The vest part hooks up to a 
              machine and fills with air that is vibrated.  It 
              helps to loosen the stuff in my lungs.
   Nebulizer: Blows air through a special mouth piece cup thing 
              that holds a liquid, usually medication but 
              sometimes just salt water.  This liquid is then
              turned into a vapor and breathed in.  This helps my 
              lungs either by loosening or thinning mucous or 
              delivering antibiotics, it just depends on what
              liquid I put in the cup.
      G-Tube: A small tube that is inserted through the skin into 
              the stomach.  It allows me to be feed formula by a 
              pump while I am sleeping.
     Insulin: Used to regulate blood sugar.  Most people make it
              naturally but if not it can be taken as a shot.

This is by no means a complete list but it gives you an idea of what I do.  If you have any questions please feel free to ask, I like answering them!