Loving, Living Life

It has been FAR too long since my last post!  I had actually deactivated my blog temporarily in the fall and am just now in a place where I can start it back up.  This past year has been CRAZY but in so many good ways.  I am in my last year of grad school, so I spent the last 6 months or more applying for actual "big girl" jobs!  It's insanely stressful- as I'm sure it is for anyone on the job market, but after spending essentially the past 10 years of education working to get to this place, and to have everything come down to a few pages of self inflating mumbo jumbo that I modify (just enough for it to take forever with each new app) for the specific position (of which there are MANY) is just....exhausting.  I also tend to not be very comfortable or good at talking myself up, so that doesn't help.  But, I was somehow able to convince a few schools that I was what they were looking for and actually managed to get some campus interviews!  One of the schools was an unbelievably perfect fit in every possible way- location (back HOME), co-workers, student body- everything!  And they must have felt the same, because I got the job!  Yay!! So in the fall, I will officially be a professor!!!

It's still not completely sunk in that, 9 years after finding my dream job, I'm actually there.  Sure, there's this pesky thing that I need to get out of the way in the meantime (finishing my PhD), but still.  Much of the work is now behind me.  This past year has been RE-diculous (spelling for emphasis).  I have had weeks of constant work all day, to come home to work all night, and still feeling like I am way behind.  I still have those weeks, even now that applications are done.  Evenings and weekends are like shining beams of light- not because they are work free, but because I get to do it from home, on my time, in sweatpants, with the kitty babies snuggling, and David with me.  BUT, I really love my job.  The only times I feel drained are when the work is labor intensive.  I wouldn't do it if I didn't love it.  Just Friday night, and Saturday morning, I was uber-geeking out because the tissue I had stained looked AWESOME and I'm still super excited about it!  And it totally blows my mind that not only do I get to be paid to do this stuff for the rest of my career, but I get to spend my days showing how awesome it is to future "lifers."  It's seriously not even fair how totally awesome it is.

And what could make all this even better?  I have been hospital and IV med free for 15 WHOLE MONTHS.  That means I made it through a winter without a stay in the hospital.  Not one.  Not even close.  Even considering the stress of job apps and short 1.5 day interviews passing through multiple airports full of sick people.  And no major PFT drops to boot.  My last appointment had me about where I was last summer, meaning I'm not once again spending the summer recovering from winter lows, but continuing the steady rise!  So we'll see how things go from here...  WOOT!!!

PAs and PFTs

There are some days where my health causes both really high highs and really low lows- sometimes within a very short period.  Well, today was one of those days.  Since returning to my Maine home after my 2 week visit to my New York home, I’ve felt like I’ve been fighting a cold or an infection.  I’ve done well fighting back, what with my crazy awesome compliance streak as of late, but every few days I felt like it was kicking my butt a little bit more than I was kicking its.  I decided I might as well nip it in the bud, so I called in for a script for some antibiotics on Thursday and scheduled a visit with the doc.  I have also been taking Zen Pep for my pancreatic enzymes over the last few days, which doesn’t work as well for me as Creon (my script had run out and I decided to wait until my appointment today to get a new script for it).  I’ve lost a little bit of weight and have been having some pretty bad nausea recently, so I was hoping that getting my Creon would help curb that trend.

Now, it’s important for me to note here that I make a point to go to this one pharmacy, even though it is out of my way.  As someone who fills a lot of prescriptions, I may look for different things in an ideal pharmacy.  I rarely if ever expect to be able to run in and out with no wait.  I don’t usually expect the process to be free of complications.  What I do expect is to be treated with respect.  Specifically, I expect that they listen to my questions and concerns and answer them without being rude or acting like I’m wasting their time.  I do expect to be able to walk to the desk when it’s my turn and be waited on, especially when there are 5- 10 people (that’s just counting those that I can see) working.  

There is a pharmacy 2 minutes from my home that is RIGHT on my way to and from work.  I do fill some standard scripts there- normal antibiotics and such, but no more than 25%.  I actually drive to a pharmacy all the way into Bangor (about 20 minutes away) for the majority of my medicine.  It’s definitely out of my way.  I definitely have to wait MOST of the time.  And they are ALWAYS super busy.  But they are super polite, they make a point of explaining any issues, and most of all, they are awesomely understanding about insurance issues- sometimes I’ve gotten my scripts before getting billed, or given a discount, because they recognize that it’s important for me to get the meds I need, even if there’s a temporary problem with insurance. HOWEVER, they obviously can’t change insurance requirements.  My insurance covers a lot of things I need, which is awesome, BUT it is a pain in the butt when it comes to prescriptions.  Many of them need “Prior Authorization” from my doctor before they’ll pay, which takes a couple days to go through and get approved- and they need to be renewed every year.  And in fact, right now is that fantastic window of time in which all of my PAs are expiring and need to be renewed!  Awesome, right?

So, now that I’ve rambled on with the back story, I’ll explain my day.  Following my doctor’s appointment, I went to my usual pharmacy to fill my enzymes and vitamins as well as to pick up my hypertonic saline that they had been out of over the weekend.  I waited about 20 minutes for them to fill the saline, but they hadn’t gotten the other scripts that my doc’s office was supposed to call in.  So I called the office to remind them and after about 5 minutes the people at the pharmacy let me know that they had received it.  Score.  So I’ve waited another 15 minutes or so when a woman came out from the back and called my name.  That’s NEVER a good sign!  Turns out BOTH my enzymes AND my vitamins need a PA.  AHHHHHHHHHHH!!!!! I was so frustrated -this is the 4^th time in about 2 weeks that I’ve had to wait due to a PA- but I tried to be polite.  As frustrating as it is, it’s not their fault and at least I got my saline.  Ultimately, that was a bummer and I get pretty caught up in being angry when trip after trip has the same result.  It’s bad enough that PAs extend the wait time, but it’s obnoxious when you have 10-15 prescriptions to fill every month!  Oh well, I get over it.

Ok, so now for the good part of the day.  After struggling with a potential infection for a week or two, with less than a week on antibiotics, as well as being without my saline for a day and a half, I was expecting my pfts to have dropped since the end of June.  It’s been about 2 months since I started being really great about doing my treatments including my vest and making sure to get the most out of it.  I also just recently tried starting to get some cardio on the elliptical.  I haven’t done a lot of it, but I do have more stamina than I expected to.  So how did my pfts look?  Better!!  My FVC was down, but last month was abnormally high and my doctor tends to be less concerned about that measurement.  My FEV1 went from 51% to 53%!  Considering I’ve felt sick, that’s pretty awesome.  I came home to add today’s data to my graph and it looks like I’ve been slowly but steadily increasing since December when I was at 47%!  I’d say that’s something to celebrate and be proud of!  So overall, it was a pretty good day.  :)

She was a Swan

My sincere apologies for going so long without blogging.  These past few weeks have been a little rough to say the least.  Very shortly after my last post, my maternal Grandmother went into the hospital and, within the day, into hospice care.  About 6 months ago, doctors found a few large tumors in her brain, later diagnosed as adenocarcinoma, and though they tried a few things, she just decided that she was tired and wanted to spend the rest of her time being comfortable.  We knew from the beginning that there wasn’t much to be done, so as heartbreaking as it was, it was calming to know that she’d made that decision. 

I was able to get home a week after she went in, and on the same day that she was moved to the Aurora House, a wonderful 2 bed hospice home in Spencerport, NY.  I spent a week and a day visiting with her, talking to her, and doing my best to be an extra support for my Mom.  She was kept unbelievably comfortable, receiving constant care by each pair of hospice volunteers, who rotated every 4 hours.  They were incredibly supportive for my family- keeping track of who we were, and most importantly, keeping informed about the day to day details about my Grandma and family- a surprising task considering we rarely saw the same individuals more than once or twice.  I can’t express how comforting, peaceful, warm, and touching this hospice organization is.  I have lost a handful of people in my life, but have never been present for the process and these wonderful people who volunteer their time made it more peaceful than I could hope.  She passed comfortably late one night, with my Mom by her side.

It’s hard to really believe that she’s gone now.  She was such a lively person- even more so than I ever really took the time to think about before now.  She was a strong woman.  She was graceful and had so much poise.  But she was a goofball too.  She would do things and make jokes that seemed out of place coming from an older woman (and CALLING her an older woman is not something she would appreciate!).  Years ago, she went to my cousin’s game and wanted to surprise everyone by dressing in a mascot costume.  After failing to acquire a mascot costume, she decided to wear a random costume (I think it was a penguin) and wandered around the game, incognito! Why? Just for fun, of course.   She often made light of things that most people would be broadsided by- whether she didn’t get bothered, or wouldn’t let anyone see her bothered, I’m not entirely sure.  But she was definitely stubborn!  We often had a lot of “Ohhh, Grandma” moments.

As a child, teenager, and young woman, I sometimes struggled with my relationship with her.  I never disliked her by any means, but we were very different.  I was a huge tomboy- rarely ever wearing make-up, dressy clothes, couldn’t do anything fancy with my hair (still can’t really), and I hated the idea of carrying a purse.  I wanted to be a farm girl and play in the dirt and grass with horses and dogs.  And, if you haven’t figured it out by now, I am a MAJOR nerd.  Though I honestly did try (a little), there wasn’t much about me growing up that was remotely “girly.”  And to sum it up, my Grandma just didn’t get it.  She was a girly girl through and through.  Always dressed nice, hair done, nails done; shoes and purses galore.  She even raised two girly girl daughters.  And I was her ONLY grand-daughter to shower with girly girl dressings!  She was understandably a bit thrown.

I don’t quite remember at what point that changed.  It may have been gradual.  I started to find my inner girl- or at least my version of it.  She was thrilled the first time she saw me carry a purse.  But that wasn’t all of it.  Sometime during finishing college with honors with some research under my belt; sometime around starting graduate school in a PhD program; sometime around me really finding myself and figuring out who I was, she really did too.  She started to “get” me.  It’s hard to explain even still, but over the past few years, I really felt that she saw my life- who and where I was in my life, and that it was who I really wanted to be.  And she was proud of me.  Not because I did what SHE thought I should do.  Not because I became who SHE thought I should be.  But because as an early 20 something, I had decided what I loved, I reached for it, and I was doing it.  It may not seem that big of a deal.  People are proud of their loved ones every day.  But the ability to step back from your own eyes, from how you see the world and how it should be, to even take the time to try and see another person’s perspective; that’s not something that a lot of people are capable of, or willing to do.  I will always have that with my Grandma, and thankfully, I was able to tell her on her last day with us how much that meant to me. 

 I hope that I can learn to be more like her in some ways.  Take things a little less seriously sometimes.  Have a little more poise.  And, of course, let my inner girly girl shine every now and then.  Most of all, I hope that I can always do my best to understand other people- the way their brains work and their perspective on the world- such an easy thing to forget, but what rewarding results we get when we just take the time.

Urine for a CF Anti-Treat…

So I have had a busy few weeks lately!  The Friday before my last blog post I went to walk in care for what was diagnosed as a basic bladder infection.  What was strange (apologies for the upcoming TMI) was that I wasn’t experiencing any of the typical symptoms I tend to get.  I wasn’t having any pain or anything of that kind, but I was more than a little concerned by the atypical color of my urine (think iced tea- YIKES!), and occasional kidney aches.  After a swift kick in the butt by my mother, I realized waiting until Monday to call the doc was probably not the best decision I could make.  Turned out that the local walk in center was 30 minutes from closing so I jumped into the car and I’m sure made the day for the nurses and desk clerk who, due to the previously empty waiting room, were likely expecting to close early.  After making them all the more excited about my presence when I struggled to pee on command, and having the capped cup accidentally thrown at me (thankfully it didn’t spill), I was prescribed a standard 7 day antibiotic for a bladder infection.  

Fast forward to two weeks and one more antibiotic later, peeing was painful and the kidney aches had gotten worse.  After looking through the information from my samples at all 3 visits, the doctor suggested that I might actually have a kidney stone- AWESOME.  Between traces of blood, lack of bacteria, and kidney pain, it made sense.   I’m fairly certain that I have had very small stones in the past, but I never knew for sure.  I was glad to have a possible explanation for the ineffectiveness of the antibiotics, but I was terrified of having kidney stones.  I’ve seen people go through that and I knew it wouldn’t be a walk in the park- even if during said walk you kept falling and smashing your face on the pavement.  She sent me home with a pee sieve and told me to wait for a call about scheduling a CT scan of my kidneys and bladder.  

The next morning, while I was doing my airway clearance, I started experiencing that same peeing pain, even though I was just sitting on the couch (and not peeing on it).  Luckily, I finished my treatment shortly thereafter and headed to the bathroom.  Now, I have experienced some serious physical pain in my life.  I’ve broken a leg, collarbone, and some toes, and have been hospitalized for a bowel obstruction.  My left butt cheek has felt the full clamping power of an angry German Sheppard’s jaw, I’ve fallen out of trees, up and down stairs, and even been hit by a car.  What can I say?  I was a curious and not very cautious kid.  But STILL, the pain that I felt when I tried passing that stone was ranked right up there at the top of the list.  And when I say I “tried,” I mean I TRIED. UNSUCCESSFULLY.  After panicking and crying, I called the doctor again to find out what to do- IF there was anything I could do.  She brought me back into the office, gave me a script for bladder pain, and sent me out for the CT scan.  I stopped back at my house to give it another shot and passed it immediately.  After everything, my doc suspected that the stone was actually lodged and chilling out in my urethra between my two “sittings.”  The scan showed that I didn’t have any more stones, so for the time being, it’s over.  It turns out that CF puts us at a greater risk for kidney stones, potentially due to malnutrition, so there’s another joy to add to the CF-Fun-Time list.  But hey, it gives me a funny story to share!

In other news, I am pleased to say that I have been blowing my treatments out of the water lately!  I just bought a baby bottle sterilizer for my neb cups and have been using it for a few days now.  I also told myself I was going to stop making excuses for avoiding airway clearance, buck up, and do my job.  We want to have kids sometime in the near-ish future, and I want to be able to spend as many years with my family as possible.  The best thing I can do to make that happen is to take responsibility for my health.  For about 3 weeks now, I have not skipped using my vest once!  I don’t think I’ve ever been able to say that before.  I also took some time to figure out how other CFers do their treatments and tried a few of them myself, to see what will maximize my treatment time. 

I’ve settled on a way that I think works great for me:  I do 2 puffs of my Xopenex inhaler (this is a broncho-dilator and it helps open up my airways so I can get as much in and out as possible), wait a few minutes for it to really open me up, and then I do my saline neb.  I just started holding it in for one or two seconds before breathing it out (something I read on CysticLife and what I feel has made the biggest difference for me), to really let it do its job.  I follow that up with Pulmozyme and then use my vest after I’m done with those.  After all that, I do my Cayston/TOBI, and my lungs feel so much cleaner.  I can’t TELL you how much more effective it is- I almost feel silly for not doing it this way before!  It takes so little effort to get junk out, it’s almost like it’s jumping to get out of my lungs, which for me, is HUGE.  I’ve never found it easy to cough up mucus- a big reason I wasn’t a fan of airway clearance.  If you don’t have CF, I don’t even know how to describe the difference, but it’s amazing.  

Tomorrow, I have my first clinic appointment since I’ve started this new plan.  I’m SOO excited to see if it’s made a difference in my PFTs.  However, I have prepared myself for the possibility that it hasn’t.  I think I’ve been fighting a bug recently and that can certainly make a difference.  But either way, I FEEL so much better, whether my PFTs reflect that or not.  I won’t let a lack of an increase reverse all these great changes I’ve made.  Wish me luck!

Me = CF^2

Obligatory super geek title!  Though being Christy Fessler with Cystic Fibrosis (ps- EVERYONE who makes the double CF connection thinks they are the first), there should probably be a parenthesize, but it wouldn’t look as good…

So after last week’s review of CF in general, I thought it would be a good idea to talk this week about my own specific CF history and symptoms and such. So here goes!

 It all started one day, about 26 years ago, when a beautiful baby girl was born.  And by beautiful, I of course mean what has been described as something along the lines of slightly creepy with dark eyes and a full head of crazy thick, black hair.  At 6 months, I was failing to thrive- I wasn’t gaining weight, had no appetite, lethargic, and behind developmentally.  I didn’t have that cute chubbiness that babies often do.  My parents took me to doctors, had many tests done, with possible diagnoses being thrown every which way.  The CF test at the time was a sweat test, where the amount of chloride in your sweat is measured using an electrode and a solution on the skin (remember that bugger of a protein that I mentioned last week that doesn’t work for us CFers?  Well, it’s called CFTR and its job is to allow chloride to pass through the cell walls.  If that doesn’t work, then chloride doesn’t go where it should, which, among other things, is going to change the levels in our sweat, and VOILA!  There you have your super salty sweat!)   Well, for some reason, there were problems getting a consistent result from me with this test, so the diagnosis was slow going.  In the meantime, the doctors started me on some medicine which ballooned my cheeks out like CRAZY- seriously, I looked like a hamster with my cheeks full of seeds, ready to pop.  Eventually, my older brother was actually diagnosed, followed be me shortly after.  For two people who were only carriers (whose odds of having one child with CF are 25%), my parents had 2 out of 2 kids with CF before knowing anything at all about the disease, let alone knowing that they were carriers.  

Despite my health problems early on, I lived a very normal childhood.  Before graduating high school, I wasn’t hospitalized for respiratory infections or “tune ups,” I didn’t take nebulizer treatments, and I could probably count on one hand the number of times I did airway clearance, if ever.  The only impact that CF had on my life, aside from the occasional playground question about cooties or what would happen if other kids took my pills, were the daily visits to the nurse for enzymes at lunch time or puffs of an inhaler before PE class, and annual or biannual trips to the specialist. 

 I did go through a period of bizarre and inexplicable arthritis flare ups when I was in junior high that crippled my whole body for about a week at a time.  My parents would have to lift me out of bed in the mornings because so many of my joints were stiff, sore, and swollen that I couldn’t even roll myself out.  I went to every relevant specialist, but no one could seem to fit my symptoms under any one umbrella of a diagnosis, and thankfully over time, the flare ups just stopped occurring.  

It wasn’t really until college that my lung functions started dropping out of the healthy or normal range.  I had a couple episodes of hemoptosis, which were brief but scary.  I did my first round of nebulized antibiotics (albuteral, pulmozyme, and TOBI) during my junior year, in order to avoid having to withdraw from my classes and go into the hospital 3 weeks before the semesters end; I had been hit with a few bugs and didn’t want to bother myself with a visit to the doctor, resulting in my lung functions dropping to 40%. 40%!!! For someone who was used to over 90%, that was a cold hard reality slap right to the face.  Now, I had probably been below 90% for a while, but I was so sure that I wasn’t a “sick person” that I never really bothered to pay much attention before that moment.  The semester ended, I got my first PICC line ever, and my functions were back to 60%.  It was pretty hard to accept that it wasn’t getting much higher than that, but it was an improvement at the very least.

Fast forward to my first semester of grad school.  I had been relatively healthy, but once again, I got sick a couple times and not having learned the first time, I didn’t do anything about it.  My chest hurt, I couldn’t sleep from coughing, and I was so run down that even sitting down at my computer and writing, I couldn’t manage to finish one coherent paragraph for a final paper.  I went home for winter break, hoping to recoup some energy, but it just got worse with every day.  My heart was racing constantly and my air was getting cut off.  My Mom would sit on the edge of my bed, rubbing my back just so that I could calm down enough to fall asleep.  Christmas night, after I woke up once again with my heart racing, she took me to the emergency room.  They wheeled me in, rushed me to a room, and started hooking me up to tons of machines.  My blood sugar was over 600 (normal is about 90- 120) and my oxygen saturation was at 40%. I couldn’t even manage a lung function test if I’d tried.  To be honest, I really don’t remember a whole lot of anything for the next couple days.  I was in the ICU, being pumped with antibiotics and steroids and insulin and sleeping all day long.  I was hooked up to a BPAP, or BiPAP, to help me breathe.  Finally, I woke up with a voracious appetite (thanks prednisone and food network!) after a couple days and started getting better.  I’m not sure how many weeks I stayed on IV meds, but I flew back to school, about a week later than intended, with a PICC in my arm.

I made a promise to myself, sometime shortly after, that I would never let it get that bad again.  I went through a lot of pain, wrecked my body (not to mention my grade on that paper, I’m sure), and worst of all, I put my parents and family through hell; and for what?  To avoid a short trip to the doctor and having to maybe just once consider that I may just BE a “sick person.”  I’ve learned that I was looking at it all wrong- it’s not black and white, much like, well, freaking everything else in life.  If I sit idly by and let my body do whatever it wants, well then duh, it will.  Since when does taking care of yourself mean you are admitting to being sick? Come on Christy, that’s just plain stupid.  It still crushes me with guilt to think back to the sight of my Mom sitting helpless at the end of my hospital bed, and know that, no, circumstance did not do that to her.  I did.  I can’t change that.  BUT, I can make sure that I continue to do what I can to stay as healthy as possible.  

So, what are those things that I do to stay healthy, you ask?  Get to the point already, you say?  Sorry, I’ve never been accused of being too reticent.  As you may have gathered so far, my main issues that I deal with are respiratory, digestive (though only in the past couple years have I had any trouble keeping weight on), CF associated arthritis (symptom free after junior high, but diagnosed when it came back in grad school), CF related Diabetes (since that 600+ moment), and I also have scarring in my liver that seems to have been there since I was a baby.   I treat each of these every day with various medications.  The scarring has caused my spleen to be enlarged as well, and while it is monitored, it isn’t a risk as of right now.  I have very low vitamin D levels, so I take supplements in addition to taking a more general vitamin daily.  I typically have low platelets, which is suspected to be a result of the liver and spleen issues.  This doesn’t seem to affect me a whole lot, except for the fact that it prevents me from being able to donate blood (though it hasn’t stopped them from drawing vial after vial for tests!).   

Every morning, I “neb” with saline and TOBI or Cayston and every night I do it all over again with Pulmozyme stuck in the middle.  I’m working on getting better about airway clearance because it’s definitely not my favorite thing.  But I ask myself- honestly, how difficult is it to sit with a vest on when I’m already sitting and puffing on salt water?  Not very.  And hey, as someone who likes to break out into song at inappropriate times, the added effortless vibrato is a nice plus (Though, better for comic relief than being pleasing to the ears).  I’d like to get back into running this summer also.  I’ve gotten a million times better about taking care of myself to avoid being that sick person that I’ve always been afraid of being, but the reality is, I’m not perfect.  I can always do better.  I can always work harder.  Good news?  I’ve got the rest of my life to keep working on it, and the rest of my life starts now.

Cystic Fibrosis 101

So today's post is a little bit of a cheat, but I think it's important.  I wrote this as a guest post for a good friend's blog and realized I don't even have a good review on my OWN blog!  As a last little salute to the end of May (which also happens to be Cystic Fibrosis Awareness Month), I wanted to take some time to really explain what CF is, some common, though very reasonable, misunderstandings about it, and what it means to those of us who "suffer" (though I hate using that word) from it.  This isn't intended to be a means to start feeling sorry for CFers- most of us are not fans of pity, but rather just a way to understand the details of what makes our lives different.  If you know someone with CF but don't really "get" it, this will attempt give you a better idea- hopefully in a straight forward, no biology degree required, way.  For my next post, I'll go over my own personal experiences with CF- that is, the specific manifestations,or symptoms that I have, how my health has changed throughout my life, and what my specific treatment regimen is like, day to day.  I hope you all enjoy!

Cystic Fibrosis (or CF for short) is a recessive inherited disease, meaning you are born with it and have to get it from both parents.  Now, to avoid getting WAY too scientific about it, CF is essentially caused by faulty genes that then cause a certain protein throughout the body to not work correctly and leads to thick mucus, and really salty sweat.  It is described as a respiratory disease that affects the digestive system as well.  But just like SO many illnesses, there is so much more to it than that.  I hope to give you a short glimpse of what CF means to a “cyster” or “fibro” (what we call girls and guys with CF).

One common misconception is that CF is a “childhood” disease.  That, sadly, was absolutely true years ago- when I was a child, CFers were hopeful to live to 18 years.  However, thanks to amazing researchers, the average life expectancy of a CF patient in the US is currently around 37 years.  For us, that’s a whole LIFE longer than it was 20 years ago and there are now many adults with CF.

Another common misconception about CF is that it is a JUST respiratory disease that also has a digestive component.  While these are absolutely the primary life threatening issues, CF is a disease that affects our cells and as a result, our whole body.  In addition to frequent lung infections, CF causes pancreatic insufficiency which leads to malnutrition and difficulty maintaining a healthy weight.  I know some girls out there may think, “Must be nice!” and I can’t TELL you how many times I’ve heard that.  Sure, I can eat a lot more than the average person- but it doesn’t come free.  EVERY time I eat I have to take a handful of pills that do the job that my pancreas can’t.  What if I don’t?  Bloating, pain, and constant trips to the bathroom- not fun.  In addition, frequently getting sick requires energy.  Having no extra fat on your body means that you get really sick really fast. While those are the obvious symptoms of CF, there are many additional problems that patients deal with.  We often suffer from  CF related Diabetes, CF associated arthritis, liver scarring, enlarged spleen, serious sinus issues, possible heart complications, infertility, osteoporosis, dangerously low vitamin levels, and I’m sure many more that I don’t even know about.  One problem is that we are constantly dosing our body with antibiotics to extend our lives and stay healthy, but those could ultimately be causing other problems that can worsen over time.  

We do inhaled nebulizer treatments and “airway clearance” (parents or significant others will pound on our chests or we can use other instruments that vibrate the lungs to loosen the mucus so we can cough it out) 2- 3 times a day which can take several hours, we give ourselves shots and IV treatments, and we take multiple handfuls of pills multiple times a day, every day.  We often get over tired, and frequent respiratory infections and coughing often causes a lot more pain than you can imagine.  Hospitalizations are commonplace and familiar (how many 20-somethings have favorite nurses in various hospital wings?? I do!).  We get weird looks and are asked “do you need some water?” when we have coughing fits.  Don’t worry, we aren’t contagious and thanks, but water won’t help.  We worry about things most people don’t have to, like where the closest bathroom is at all times, whether a public place has a lot of sick people present, getting home from social engagements with enough time to do our hour plus of treatments and still get enough sleep, figuring out how to spend weeks in the hospital with as little impact on work or school as possible, or waiting for someone else to lose their life in order to get a second chance at ours with a new pair of lungs.  We HAVE to work hard, not to necessarily get healthier, but to stay as healthy as possible and prevent getting worse.

But as awful as this disease is, it gives me and others a different perspective on life that I wouldn’t trade for the world.  I don’t WORRY about getting old; I see every year as another milestone.  I enjoy every breath that I take and any day that I can breathe, can move around pain free, and have normal blood sugars is a great one.  I celebrate a 5% increase in lung functions (I generally function at 50% lung capacity).  I am lucky enough to have found my passion and career early on and get to come home to a wonderful man who adores me, CF and all, and enjoy our amazing fur babies (two 5 month old kittens) together, every day.  Sure, I get down in the dumps sometimes, but what good is it to spend my days feeling sorry for myself- especially if those days are limited?

In the words of the inspirational Eva Markvoort, a cyster who devoted her life to spreading the word about CF, and tragically lost her battle at the young age of 25, “Cystic Fibrosis means drowning on the inside, it means learning how to access my own med port at age 13, it means closer family bonds, as everyone pulls together to get through the tough times.  It means making jokes about beating your children, and salty girls tasting better.  It means living my life not for the number of breaths I take, but for the number of moments that take my breath away.”  Right now, there are amazing drugs in development and getting FDA approval that bring us a thousand times closer to that big, amazingly scary exciting C word that has always seemed so impossibly far off in the distance for us.  Every day, we fight our battle, individually and as a community, so that we can be one day closer to the famous day that CF stands for Cure Found.