Long day doesn't even begin to describe today!

Things I did/had done today:
took out the  blown iv in my right fore arm, replaced it with one in my left arm
adjusted my epidural so that my pain was under better control
got my Foley catheter out
pooped (they wouldn't let me eat until I did that, so I really didn't have a choice) in front of a male nurse on a portable commode no less... overcame a lot of fears right there with one act!
went for a walk
ate my first two post transplant meals
got a fruit bouquet, chocolates, balloons and a teddy bear to cuddle
incentive spirometer
acapella
peed a lot (lasiks helped out there b/c i was holding onto so much fluid after being on the bypass machine and prednisone)


goals for tomorrow:
get out of icu
one whole lap around the floor
one more chest tube out
break 1000mL on IS

Flash Me Friday!

Ok, I thought I'd get in on the fun since I enjoy everyone elses' flashes so much!

The view where I live (today):

To the southwest

To the southeast

Down (obviously)

This is MY History

What you see below is a plot of my actual pulmonary function results over a span of 11 years.  This is a graphic representation of the decline pattern of my unique case of Cystic Fibrosis.  On the graph there are two lines.  The blue line shows the Forced Vital Capacity (FVC) and is measured in liters.  It shows how much air I can exhale out of my lungs to indicate how much capacity I have.  The red line shows the Forced Expiratory Volume in the first second (FEV1) and is also measured in liters.  People with healthy lungs should be able to exhale about 85% of their entire capacity in the first second of forced exhaling.  Simply put, the FEV1 is the most widely accepted indicator of the lung function in a patient with Cystic Fibrosis, where as, the ratio between the two numbers tells how elastic (or scarred/inelastic) the lungs are, which is also important.  

Some Things to Consider When Reading the Above Graph:

The pink line indicates the period of time when I was still riding horses and playing tennis regularly.  As time moves forward, I played tennis less but increased my riding.  In the end of my riding and showing career, I was on at least one horse 6 days a week.

Between the orange hash marks, I was attending college.

The yellow dots represent the day I quit drinking alcohol.

The green circles represent the day I had my gall bladder removed.

FVC All-Time
High  3.44L 3/02/02
Low  1.22L  3/16/09

FEV1 All-Time
High 2.49L 12/23/03
Low .62L    7/11/06

I was evaluated for transplant in May 2008 and was listed June 11, 2008.

One interesting fact is that I had completely quit doing airway clearance therapy or chest physical therapy just before this graph begins.  I didn't even own a vest until September 2005.


I know that the combination of the end of my riding career (the end of exercising) and being away at college (slacking on treatments, drinking, staying up late etc.) had a huge effect on the ability of my lungs to maintain function.  Had I known that any of these things would have such a consequence on my health, I would have done it all differently - but what's done is done.  The only thing I can do now is share my story so that others don't make the same mistakes!


Lessons I Learned (and I hope you will, too!)

1. Do your CPT, airway clearance, acapella, flutter, vest, whatever you do - just DO it! I spent more time coughing during the day when I wasn't doing these things than I would have if I'd just taken a half hour a couple times a day to sit down and make an effort with whatever method I chose.

2. Get out of breath.  Whatever activity that gets you up and moving, something you like, keep doing it. It's much harder to get back strength and function after a period of absence than it is to continue maintaining.  If you have to wear oxygen, make it happen.

3. Alcohol in excess is an enemy.  It's a Central Nervous System Depressant, meaning it affects your breathing (decreasing the depth and frequency).  It may feel nice not to have the urge to cough while you're drinking - I felt normal, like I didn't have CF when I was drinking - but just as with other muscles we don't exercise, our diaphragms lose strength, strength that we need to cough and breathe deeply.

4. Acid reflux in Cystic Fibrosis is common.  I never knew my gall bladder could be to blame for some of the symptoms I was having, excessive bloating and shortness of breath (especially after eating).  After I had my gall bladder removed, I felt instant relief (once the pain subsided!).  I wasn't constantly popping Tums despite taking 20mg of omeprazole twice a day.  And I even gained weight, because I wasn't getting sick after eating or avoiding eating a lot because of the bloating.  If you're having issues with GERD, ask your doctor about getting some liver blood tests done.  If they show abnormal numbers, it might be wise to get your gall bladder an ultrasound.

5. When the doctor says it's time for IV antibiotics and a couple weeks in the hospital - DO it!  You don't get 4 airway clearance, all your IVs AND have time to live/work/cook/eat/clean/be a person at home.  Don't bother, it's more work than can possibly be done and you're supposed to be getting well!  So many times I opted to do home IVs and ended up more run down and tired than I would have been if I'd just gotten admitted.  I know it may be difficult to schedule someone to take care of the kids and the house or to miss work/school, but really, everyone wants you around for a long, long time, and if you're not doing all you can to make sure that happens, then you're not only doing yourself a disservice, but you're also hurting the ones you care about most.  It sucks giving up the control of your meds and time, to be woken up at all hours, to have a constant stream of people asking you the same questions over and over, to have really really bad food - but trust me when I say that I truly believe it is for the better.  The hospital doesn't JUST want your money this time.

6. If the doctor says you need to wear oxygen, DO it! (I think that's my new motto - Nike's not going to sue me are they?) So many times I've been too embarrassed to wear my oxygen in public.  I still struggle with it at times or certain places.  But, ultimately, it's just going to wear me down and do damage to my heart - the last thing I need is a lung AND heart transplant.



And here, despite everything, I feel absolutely wonderful.  I've made peace with the way things are, I don't regret the past, nor do I wish to shut the door on it.  I love my family, I have amazing friends and am connecting with so many awesome people in the CF community.  If you have a blog - I want to read it!  I want to know your stories, share in your problems and work together to find solutions.

I'll leave you with two pictures of me in my prime, doing what I love most in life.  One day, after transplant, I hope to be able to return to the show ring.

Left: Lexington, Kentucky 2002
Right: Columbia, Missouri 2003

Miss Know-It-All

Wednesday morning when I woke up 10 minutes before my alarm, restless, and got out of bed.  I couldn't figure out why my body didn't want to sleep anymore.  Aha, Dad discovered it, the oxygen dewar had run out in the night - whoops.  My sats weren't too bad, about 88%, so he switched it over to the other one.  One doesn't last me very long anymore.  I remember when I would go 2 weeks on one fill with only one dewar.  Now it is 2 dewars filled weekly.  My handy little Helios (which I fill from the dewar) keeps me supplied with oxygen while I'm out and about - though it doesn't last nearly as long as it needs to, and is quite heavy and cumbersome.

I decided I'd at least get up and do a vest and my nebs before I went back to sleep.  For some strange reason I never did go back and lay down, instead, leisurely getting myself ready to leave the house to run some errands and go to the doctor later.  It was a very productive appointment, as we were able to discuss my hospital stay, the things which were good and also the areas that needed improvement.  The good thing about being the squeaky (or maybe bossy) wheel when you're in the hospital is that things get done, and if you're lucky, they change.  I was SO lucky to have such willing staff, that they listened to my concerns, met my needs (with very little attitude, for as much as I was dishing out) and were eager to make the changes necessary to better understand and meet the needs of patients in the future.  It may be only a job to some, but to me, it's my life - I can't always wait for the next shift to say something!

At the appointment, I mentioned that since I had been diagnosed with diabetes 12 years ago, I could probably do with a refresher diabetes education course.  They agreed and had the diabetes specialist come talk to me before I left.  She brought with her a handy little booklet that I had never seen before called "Managing Cystic Fibrosis-Related Diabetes"... Apparently they know much more about it now! Great! She held it up and asked if I still had one - I've never even seen it! I all but snatched it away from her.  I was so excited to get my hands on something that would help me better control my diabetes.  For those of you with CFRD, I recommend asking one of your docs about this booklet.  It's put out by the CFF.

I was explaining to a friend of mine yesterday afternoon that I love learning, but then I corrected myself, instead stating that I love knowing things.  As far as I know, there is no way to download information instantly to my brain, so I either have to hear it or read it before I can pass it on.  I recently went on a book binge.  One of the 9 books I purchased is called Sick Girl Speaks!  I have only made it about 35 pages so far, but I wish I were finished with it so I could know everything inside.  It's not that reading isn't a pleasurable way to spend my idle time (of which I have much), it's just that there's simply not enough time to read everything I want to know.  (I'm not blessed with the speed-reading gene) And the more I read, the more I realize I don't know.  And what you don't know can definitely hurt you.

Almost forgot the PFT update: FEV1 31%, FVC 50%... I'll take it! :)

Home Sweet Home

Yesterday I broke out of jail.  No, not really, I would never call the hospital jail... let me start again.  Yesterday I came home from my amazing and health-restoring vacation.  It was a wonderful two weeks, full of educating the uneducated and, well, coughing!  I coughed so much more than any other patient anyone had seen.  That's how I get better! At least it made them feel like they were making a difference.  Some of the respiratory therapists who had me were shocked that I wanted CPT (chest physical therapy) longer than 10 minutes (or 1 minute per lobe/position)!  I was shocked that no one had ever requested it or taught them differently.  So what did I do?  Educated everybody who handed me a nebulizer (except for the two wonderful ladies I met who could have written the book on being a good RT: Mary Catherine and Diana R. - you ladies rock!!)  I was even asked one of the most ridiculous questions I've ever heard out of an RT's mouth: "Have you had CF since childhood, or did you acquire it later?"  I know he didn't understand that CF is a genetic disease, because he didn't ask when I was diagnosed or even when I got sick.  He asked if I "acquired" it.  Now, I posed this issue to a lot of other CFers and many say it's common to get that question - and this alarmed me.

From a Respiratory Therapist, that question should be automatic grounds for re-enrollment in school.  How can you take care of a CF patient properly if you don't know why they are there?  The patient has lots of STICKY (more so than healthy people) mucus in the lungs.  "But Justine" you may ask, "why is it stickier than healthy peoples' mucus?"  Well I'm glad you asked, it's because of a defective mutation (actually two, one would still produce the phenotype of a healthy non-CFer) in the CFTR (Cystic fibrosis transmembrane conductance regulator) gene which causes chloride channels to trap chloride, and thus attract sodium to neutralize them both into - SALT!

Now I digress to ask you a question: What happens when you pour salt on a slug?.... Yes, it dries up.

Now back to my lungs:  If chloride is trapped inside the excretory cells (lungs, sinuses etc), the salt forms and the water, instead of being freely excreted into the airways to form mucus, is not as free to flow because it is drawn to the salt in an attempt to create a homogenous salt water concentration on both sides of the cell wall.  Result: stickier (drier) mucus.

End of science lesson.

If an RT doesn't know CF is genetic, they wouldn't know that we have sticky mucus, nor why we have persistent lung infections (bacteria thrive in moist, dark, undisturbed places).  For all they know, we just have a bad cough, and they wouldn't be able to give us the kind of treatment we need, because they weren't aware of the root problem.

Please, the next time someone in the medical field doesn't know about CF, and should, educate them! Even if it's a simplified, two sentence explanation.  It's really sad to think about how many people aren't getting the care they need!  I know I am an overly involved patient, but you can't expect anyone else to be your (or your child's) advocate.



All in all, it was a great vacation.  My PFTs yesterday morning were an FEV1(function) of 32% and an FVC(capacity) of 51%!  Great, especially considering how sick I was when I went into the hospital.  My port is healed and I was given thorough lessons on accessing and deaccessing, even doing it once myself.  After two weeks it's healed very nicely, although it's still tender.  I'm hoping that as it heals, the scar tissue will solidify it's placement.  I took the advice of many other CFers with ports and cut a hole in a sponge to put over my port when using the vest.  I tried it today, and it's still too tender for that, so I have been unhooking the shoulder velcro.

Port

This morning I was awoken out of a dead sleep at 6 to my nurse flipping on the lights, "They're ready for you in 30 minutes."  It's port time!  I had them page respiratory so I could get an albuterol neb before they took me... gotta breathe, right?  The cute resident who came and talked to me yesterday was down in pre-op. :D He answered the rest of my million questions to the best of his ability - I'm a pretty involved patient (translation: control freak), so he wasn't really prepared to deal with me.  They wheeled me into the OR, I climbed onto the table and laid down.  They gave me some versed and started to wrap me up, "like a burrito".  The last thing I remember was muttering something about having a fallback career lined up at Chipotle.  When I woke up, I had some pain and a kickass incision near my shoulder:

 

A few hours later someone came to access it so I could start receiving my IV antibiotics through my brand new port instead of the peripheral IV they had in my arm (which are very painful for me!)

So there you have it! It was fairly simple and I can't believe I hadn't done it sooner.

I've fallen in love... with a new nebulizer!

A Breath Actuated Nebulizer... how cool is that?  There is no waste, whatsoever, and the treatment time is fast - faster than any neb I've ever had!  When you breathe in, the neb cup with the medicine pushes down on the air supply, nebulizing the medication, and when you breathe out, it lets up.  SO cool!  It's called the AeroEclipse and I'm going to get a script for them when I leave.  I have been doing research on nebs tonight and was wondering if anyone had any experience with using this neb with Tobi?

 

Also, I'm set for getting my port a cath in the morning, so I'll make sure to update, hopefully with a picture of it!

Tune up time

Went to the doctor yesterday, the PFTs were WAY down.  I think they are at my lowest, FEV1(function) at 23% and FVC(capacity) 32%.  I'm getting admitted to the hospital today.  While I'm here I'll be getting a port a cath so I won't have to get any more PICC lines (yay!).  I'll update more once I get checked in and settled. xoxo