Morbidly Pleasant

I'm sitting in my room, facing my computer.

To my right is a window.

I open it and let in all the southern light that I can.

Zoe seems to find joy in early morning sunlight, and I do, too.

     It's a recent discovery for me - before I wanted the shades drawn, isolating myself inside my man-made cocoon of artificial lighting, creating my own solstices, deciding when daylight ends or begins.

All the rest of my life is based on artificial supplements: my oxygen, my digestive enzymes, my bacteria fighting abilities, even the ability to effectively cough has been assigned to a machine.  Why not shut out the sun?  It will probably give me skin cancer one day anyway.  But why, then, does it make me so happy?  When the day is new and I'm resting in between the cool sheets, my body still hot from sleep, I feel energized, motivated; I feel "normal".  And then I roll over, that familiar urge to cough strikes without warning and I shoot up into "cougher stance".  I reach for the kleenex to spit and drop it into my bedside trash can.  I have everything I need within reach.  I hate that my life has come to this point, being so medically handicapped, that I have to alter my living environment to accommodate the necessities.  The nice thing is, once I'm thoroughly exhausted after doing my morning treatments, I can sometimes crawl back into bed and pick up where I left off sleeping or reading, with a napping buddy at my hip.

It's just morbidly pleasant.   

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

OMLG Twinkies!!!!

The last time I was in the joint (for those of you who don't follow, I mean the hospital) a friend and her daughter were coming up to visit me.  She called while she was at the convenience store to see if I wanted any junk food - um, YES!

Side note: When I have been in the hospital in Nebraska, they have a convenience store in the hospital, so it's an elevator ride away to my heart's content, provided I don't mind lugging an iv pole and oxygen tank with me.

She was naming off items in front of her and I stopped her, "Do they have Twinkies?  I haven't had a Twinkie in years!"  I was in luck, she brought me 2 2-Packs and right before bed I thought I'd have one... Just kidding, I ate all 4 of them.  A I was sitting among sticky plastic and cardboard wrappers I knew that I would have to get more Twinkies, STAT.  My Twinkie addiction was off and running!

Luckily I was in the hospital in my hometown and Daddy, my dealer/enabler, is just a phone call away.  The next day he brought me a box of 10 of them, but warned me they might not be very good because the filling was green.  I opened the bag and saw what he meant - Shrek Twinkies!  I think they were even more delicious than regular Twinkies.

Boxes and boxes of Twinkies later, and several pounds gained, I have decided to make a diary of my Twinkie consumption on Twitter.

Follow me HERE!

Let's just see how many Twinkies I eat.  I will not be altering my pattern of Twinkie-eating in any way, so what you're reading will be actual, real-time Twinkie ingestion.

Bon AppeTwinkie!

A Poem

It is life, and it is misery.

Oh, thieving and sustaining demon,

you will one day be dead to me,

but for now, "ON"... I'll soon be breathin',

free of your time and space consumption.

Can you guess to whom or what the speaker is writing?

Long Update!

I've been meaning to update this for a while now. So let me back track:


June 2nd I went back to the CF doctor here in Wichita for a 1 week post hospital stay visit. My lung function was holding steady (31%, 50%) I felt great.

The following Tuesday, the 8th my dad and I loaded up the car and made our way to Omaha. I watched the sun set over Lake Manawa, sitting at the end of the dock... a beautiful place to sit quietly and meditate, or just listen, not think. It was a very welcomed moment in what I knew to be a busy next few days.

Wednesday, the 9th I saw my ENT (ear, nose & throat) doctor, who did my last sinus procedure the summer of '09. My sinuses look about like you would expect a CF patient's sinuses to look - inflamed and butchered. I was glad to hear that they looked "normal" because my primary care doctor in Wichita was somewhat alarmed at my sinus CT scan - CFers know what I mean. After that appointment I saw the endocrine and CF teams at the hospital all the way across town. The visit was less of a check-up and more of an exit-appointment (although my PFTs were the higest they’ve been in over two years, FEV1 was 35% and FVC was 57%. I've decided that I'm going to transfer my CF and CFRD (CF related diabetes) care to Wichita, following solely with the center here, instead of both Wichita and Omaha. I'm torn about this decision for a couple reasons:

Firstly, it's no secret, but Omaha sees more individual patients than Wichita (larger population in the vicinity) - thus giving them an overall greater experience in dealing with a variety of cases and issues. It's always comforting to know that when you go to your doctor for a problem, he's seen it a million times and knows exactly what to do.

Secondly, I "grew up" under the care of the Omaha CF team. I began to get involved with my own care when I went away to college, I transitioned to the adult clinic about the same time, so I was really learning about CF in a new way, as an understanding and autonomous adult. When I go to the doctor alone, without a parent, they have to talk to me. I get all of their attention, focus, eye contact. I'm free to ask questions, joke, and bulid raport as the patient. When a parent enters the room, even if you can manage the dauntiung task of getting them to remain quiet, they automatically fall into the parent role, regardless of how old or competent the patient may be. They get half the eye contact, they get to ask questions, make comments, go off on tangents or tell semi-related anectdotal stories… ad nauseum. And the doctor humors them. What’s really happening is the parent is trying to gain back the control over their child’s disease and care. While I want to keep Mom and Dad in the loop, it’s important for me to maintain my independence in this area, even though I’m unable to be independent in other areas.

Those things aside, I know that splitting care between two centers is not as beneficial as it seems it should be. At first I thought that I might be getting the best of both worlds – but really, it was the opposite. The new center didn’t feel like they had the authority to act immediately, rather waiting for consult from the old center before prescribing anything, meanwhile I’m waiting to get IV antibiotics or a PICC line, getting frustrated myself, wondering what the hell is going on that all of this can’t be done sooner. Everyone ends up frustrated and touchy and the patient (ME!) suffers. I feel like now that this is settled, I really will be getting the best of both facilities, because Wichita is now the sole caregiver and we (as a team) are able to consult Omaha when there is a question regarding my history or anything else, without feeling like we’ve burned any bridges.

The 10th saw Dad and I on the road again, headed to Minneapolis. Early on the 11th I had appointments at the U of Minnesota. My PFTs were still the highest Minnesota has seen at 31% and 43% but down a little from two days before – goes to show how humidity, barometric pressure, air quality (car air) and allergies can affect pulmonary function from day to day, or even hour to hour. I wasn’t too disappointed in the dip.

On the 12th we headed back to Omaha and then Wichita on the 13th.
Later that week I got my Lung Allocation Score from my visit, and it was 34.93 - which I think is lower than it's been in the past. I didn't have a 6 Minute Walk test done while I was there, so I have to get it done here. I think that will help my score out, since I know I require more oxygen than I have previously. When I get that done and sent to Minnesota, they will update my LAS.

[Side Note: A 6 Minute Walk test is where you wear a pulse-oximeter which reads your pulse and the % oxygen saturation in your blood while you walk for six minutes. A respiratory therapist records your pulse, oxygen saturation, oxygen usage and distance every 30 seconds for the whole six minutes. This gives them an idea of how "disabled" you are. I think they should add a video element to the LAS. To see me cough gives a very clear picture of my dis-ease.]

Ideally, I would get called NOW and wouldn't have to do it! :) I was hoping that the holiday weekend might offer at least a call to me or my friend CysticGal - but no luck as of yet. She has had two dry runs so far. I feel like it's her turn. Or mine. I have been waiting for over two years. I told CysticGal that I feel like we are the last two to get picked for kickball. In all reality, that probably would be the case, since neither one of us would really have a lot to offer an athletic team. But that won't always be the case.

At the end of June, the CF community, nay, the world, lost a dear boy named Conner Reed Jones to CF. His mother shared via her blog, his passing, with amazing clarity and beauty. The world celebrated his life last Wednesday - all over people released red balloons for Conner. Red was his favorite color. His funeral service was even shared with the internet community. In it, his mother wrote the most beautiful and touching eulogy. Anyone who read or watched her family's journey would be touched. It's always sad to lose someone we love so much, especially a child, but her strength is a wonderful testament to her faith in God and in love - concepts which Conner knew well, despite his young age of seven. Which is why I released seven red balloons into the sky on Wednesday. Attached was a card, on which I wrote, so that whomever finds the balloons will know who Conner is and why he was so special. Watching the balloons float upward, I had to shed a tear or two - it was just that powerful.

That's about all for now! I need to do better at updating this - it worries people when CFers disappear for any significant length of time. I know I worry when I don't hear from my CF friends!

Help me pack for transplant!

My senses tell me that transplant is on the horizon.  I have a bag packed, but want to get some last minute input from those who have gone before me.

My plan:  I will fly 2 hours from my hometown to Minneapolis.  When I get there, if it's a go, what will I need with me immediately before and after surgery?  Comforts? Bare minimum?

What did you bring when you got "the call"?