The past two weeks

I've been working harder to control my blood sugars the past two weeks, and it seems to be making a difference.  My trend line has been lowered by about 50 mg/dl, which puts me in the 150-225 average range, instead of the 210-250 range.

Progress, not perfection!

A couple weeks ago I was beginning to feel pretty punk, and went to the doctor.  My PFTs were only down a little, which shocked me.  I was expecting to have to be admitted or do home IVs at the very least.  We opted for a couple oral antibiotics instead, and I finished them last week.  I have to admit, they really did help.  But now I'm not sure if I'm going downhill again.  My left lung (just to the left of my sternum) has been hurting pretty badly at times and has been getting worse, not better, as time passes.  It's not to the point where I need to see anyone about it.  Even in the past, when it's been nearly unbearable, they really can't do a whole lot for me.  Maybe I will just get some new lungs soon?

I exchanged emails with my transplant coordinator last week and what I was told was:

• they have done 16 transplants this year
• they have been quiet the past few weeks (no transplants)
• I am essentially at the top of my blood type list because the other people who are my same size, with higher lung allocation scores, have other factors which make them more difficult to match - therefore, they expect that I should be next

This news has stuck in the forefront of my mind.  I know that the holiday weekends are typically high organ offer times.  With every place I go, every thing I do, every thing I plan, I keep in mind that I need to be able to get my ass to the airport stat if I get THE CALL.  So no riding in other peoples' cars, the cell phone is always visible, and my bag is (still) packed in my car, ready to go.  I even have my digital camera with me (battery fully charged) so I can get lots of pictures to document my journey, because I may or may not be too excited to remember details.  

I continue to pray for my donor and my donor's family, because they will be experiencing a great loss just as I am getting the opportunity for renewed life. 

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! :)

Daily Reminders

Even on days when I'm feeling (relatively) well there are always little (and sometimes big) reminders of my illness.

For starters, I wake up and cough. And I don't mean clear my throat... I mean COUGH - braced and hunching over until I'm red in the face, gasping for breath cough.  This happens whenever I wake up, so if I stir in the night, it happens.  I'll be up for 5 or 10 minutes just coughing so I can be clear enough to lay back down.  Another 10 minutes to fall asleep, if I'm lucky.

When I get up in the morning, the first thing I do (after going to the bathroom and grabbing a soda) are my breathing treatments.  This consists of aerosolized medications and a vest treatment - the vest shakes my chest to help loosen the congestion so I can cough it up. This takes anywhere from an hour to two hours -  and can be exhausting if I'm really congested.

Then I can start my day.

Get food (Need those calories to maintain my weight - coughing burns a LOT of calories).  Did I mention I wear oxygen? I didn't.  Ok - I wear oxygen.  I don't notice it so much when I'm sitting in one place, but when I begin to move around the house, the tubing leash I have trailing me can get caught, stepped on or run out.  Making food in the kitchen is a hassle, because it reaches just to the refrigerator, but not the stove, cutting board, dishwasher or sink... convenient when I don't want to clear my dishes, but not so much when I'm alone.  Off comes the oxygen.

After eating, I pop pills and shoot up.  No, seriously.  I take about 15 different pills every day... many of them multiple times a day.  I won't list them all, so you'll have to just trust me on this.  I also have CF related Diabetes for which I take insulin shots whenever I eat.  These two things are probably the easiest part of my health maintenance, and I'm really looking forward to a day when that's ALL I'll have to do.

One difficult part about CF is that because I need so many calories, I'm often eating a large volume in one sitting, which makes it difficult to breathe.  With the stomach pressing against the diaphragm and lungs, I end up having to cough and clear after meals.  Which is okay if I plan to be near a trash can or toilet. Yeah, sometimes I cough until I barf.  Although that has happened a lot less since I had my gall bladder (which would swell after eating) removed.

Before I get myself ready to leave the house, I have to make sure I fill up my portable oxygen device an hour before I plan to need it.  It has to build up pressure to be able to function properly, and if I'm unable to use it, my blood oxygen saturation will drop and that causes the heart to work too hard (and the last thing I need is a heart transplant, too!).

When I leave the house, it's usually time to give another good cough.  I often purposely cough when I'm driving places so I won't have to when I get where I'm going.  One of the hardest things for me to deal with is coughing in public.  I hate to gross people out or worry them.  People think I'm choking and get really alarmed when I have a coughing fit.  I find myself giving the dorky thumbs-up just to get them to leave me alone until I catch my breath.  I hate having to justify it afterward, "Oh yeah, that's normal for me.  And it's not contagious."

When I'm home during the day, a lot of times I take a nap, which for me symbolizes starting my day over: I get up, cough, and do a breathing treatment.  The added exertion from coughing and struggling to oxygenate my blood often wears me out in just a few hours out of the house and I need naps!

Before bed, it's the same routine as the morning: breathing treatments, vest and bedtime snack (food).

I share this, hoping that more people will understand when someone says, "I have Cystic Fibrosis".  It's not just a label, it's a lifestyle.