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.