Pre-Transplant Fun... Kinda

Before I started the transplant process, I basically had to get my whole body checked out. We did gastro, cardio, psych, etc. The gastro guy was like- "yeah- your gut sucks". Obviously we knew that. But then we found got unexpected news. The cardiologist looked at my ECG and noticed that my readings were a bit funky. They asked me if anyone ever told me I had Wolff-Parkinson-White Syndrome (WPW). NOPE. They told me that it appeared that I had an extra electrical pathway in my heart. Seriously? Oh great. Something more to deal with. They wanted me at my peak of health (or as close as I could get) before the transplant to give me the best chance of surviving. So I started undergoing extensive testing for this cardio issue.

First, they wanted to see if they could get me to have issues- yes they wanted me to have a heart issue- by making me exercise extensively. It was no fun and they couldn't get my heart to react. Finally, they told me I would have an exploratory surgery. They were going to stick I probe down my esophagus and place it next to my heart. Then the probe would try to get my heart to beat really fast which could put me into cardiac arrest if I had the disease. We all were like "you wanna do what now?!?". But they assured us that they would have a defibrillator on hand in case I did arrest and that it wasn't too dangerous. We had to do it if I was going to get the transplant so we agreed. I was put under and woke up a few hours later. They told me everything was good and that while I did have an extra electrical pathway in my heart, it did not lead to anywhere significant and I would not have any heart problems. So that was a relief.

However, I hear that after I woke up, I was quite the handful. The drug they gave me put me in more of a "twilight" (that's their word for it) instead of a full anesthetic sleep. The doctors said that usually for girls it makes them weepy and for boys it makes them aggressive. Well guess what I did? I got FIESTY. I apparently was very demanding and short with everyone. I even yelled at my dad at one point. For those of you who know my family- just imagine me yelling at my dad. And everyone just sat there and took my demands and abuse apparently. I don't remember any of this and still believe to this day that I am a perfect angel... Hahaha.

After that we met with the main team of doctors and head nurses that would be in charge of my care during the transplant. Unfortunately we had to meet during my high school graduation. So as of now- I haven't walked a stage. That was disappointing for me. The team of medical personnel included the transplant goddess- Dr. Filipovich. I know that without her sage recommendations I would not be alive today. They told me the bare minimum of what was going to happen to me because I think they didn't want to scare me. Here's some of what they said (but in my words):
I would get a shit ton of chemo for 10 days straight and then have to stay in isolation because I would literally have no immune system. On the 10th day I would receive my sister's (Mercedes') bone marrow. Then I would recover in isolation for as long as it took- usually about 30 days. I would have to be very careful not to come in contact with any public because if I contracted any illness I could possibly die. Etc. etc. etc. My first questions after their spiel: Will I lose my hair? YUP. Are there any other things I should expect? Most patients get horrible mouth sores. I have no idea why. They just do. Finally, the doctors told me that we would begin on August 12, eleven days after my 18th birthday. So that was that. And the real journey begins.

PS- I redid the email subscription. It is at the top of the page. Please re-subscribe if you have done it before. I hope that it works this time!

PPS- I love seeing your comments! Whether they're reminiscent, encouraging, or even sad- it is nice to know that I'm not alone in this. It always brings a smile to my face (and sometimes a tear to my eye) to know you all care! :)

The End Date and Fate

I think I was going to just a regular check up at NIH. I don't remember there be anything out of the ordinary. But during that visit everything changed. I was 17. It was the middle of my senior year in high school. My doctor, Dr. Holland, sat down with my parents and myself in a private room. I don't even remember how he said it. But what I do know is that he told me that I needed to consider a bone marrow transplant because if I didn't get one, I was at risk of getting an infection and dying- probably within the next two years. My life had an end date.

The usual response would be tears. And maybe I did cry- I don't remember. But I was also excited. If I got a BMT (this will be my abbreviation for bone marrow transplant from now on), that meant I would be cured. I wouldn't have to worry about infections all the time, about remembering to take my pills, about avoiding mold and fungus. We had known that a BMT was an option, but I was never sick enough to warrant one. But now I was.  So our next question was "where?"

We asked trustworthy Dr. Holland at NIH. He had been treating me since I was five and he was like family to us. He said that if it were his daughter that needed a transplant, he would take her to either Duke or Cincinnati Children's Hospital. So we thought about it. In the meantime, we also started getting my sisters tested to see who was the best match to be my donor. It was amazing that they were all so willing to do that for me. And it turned out Mercedes was my best match. At the time, she was finishing up med school and starting her medical internship search with her boyfriend (now husband) Dave.

Eventually my parents and I decided that Cincinnati was the best option. And within a week Mercedes and Dave were matched to a hospital for their internship... It was Cincinnati Children's Hospital. That's when we knew it was truly meant to be. It could not have been any other way. Or in my words- so crazy!! It's like it was a sign that this was going to happen and I was going to be okay.

That was in March of 2008. Between then and when I got my transplant that August, I was on an emotional roller coaster. I specifically remember just walking out of the choir room and sitting on the ground crying just because the thought was overwhelming. Also, I was getting sicker. I had to drop out of the high school musical. It was a minute thing, but it meant a lot to me. I got thinner and a bit weaker. But everyone was there with me- excited and terrified.

The First Hospital Stays

After I was diagnosed with IBS, it slowly got worse. It wasn't unmanageable, but it eventually was labelled as colitis- a worse form of IBS. Then, when I was 15, everything took a turn for the worse. I had begun to have incapacitating pain in my abdomen every once in a while- it felt like I was trying to digest a knife. When I would go to the bathroom, blood would always come out. Finally, I got an infection. It ranked in the top ten most painful things in my life. I could barely walk. I was hospitalized for at least two weeks. They had to do an exploratory surgery and put me on intense IV antibiotics. The surgeon said that on a scale of 1 to 10, I was an 11. He said he didn't know how I was even able to walk from my hospital bed to the bed to take me to surgery.
But some funny and happy stories came from it. When they went to place a PIC line (a super large IV that goes directly to the heart) in my arm, the doctor said, "I've done over 5,000 of these and never missed." Now you know he just set himself up for failure. He wanted to do my left arm so that I would still have my dominant arm completely free. I told him it was a bad idea! I told him that the veins in that arm were wimpy and small. But no. Don't listen to the patient. So he attempted to place the PIC and it didn't work. Surprise surprise! He became so flummoxed and frenzied that he had to walk out of the room to compose himself. I ruined a doctor's perfect record! Haha! I know it wasn't funny for him, but I found it (and his bit of arrogance) hilarious.
Now the happy anecdote was about how much everyone cared. My friends Loren, Cara, Amanda, and Sarah all came to visit me. They brought me balloons and all of the assignments I missed in school (okay- so maybe I didn't want those). But they cared enough to come and that meant a lot to me. Also, all of my sisters came to see me. At the time they were all in Austin. Carina had moved there with Jason. Mercedes and Brandan were in college. But they all came to see me even though their adult lives were so busy. I couldn't ask for a better family.

So that was the first infection and it finally bumped me up from having colitis to Crohn's disease- the worst of the intestinal diagnoses. To combat it they put me on a drug I had to have every four weeks through an IV. However, this drug worked by lowering your immune system response. This was not the best for me. I already had an autoimmune disease that caused my white blood cells not to work so I was already prone to infection. Consequently, infection after infection began occurring. The most memorable for me was the pneumonia I got about a year later when I was 16. My chest began to hurt more and more. Finally, while I was on a regular visit at the NIH, they took x-rays (because they always took x-rays) and found that a quarter of my left lung was covered in infection. Again, I had a hospital stay and a lot of antibiotics.
While those years were hard for me, I had support. I am always thankful for my friends in senior high. They understood that I had issues, but never asked me about them because I think they knew I would be embarrassed. I would go to lunch with Briana, Angela and Christy and often I would drive. But as we came back from lunch I would almost always get pain in my gut and then have to rush off to the bathroom. They would go so far as to drop me at the doors and then park my car for me. They just somehow understood. Once, I went to the bathroom while we were at Chipotle for lunch and they took my keys and pretended they had left me! To get back at them, some of my other friends were at also at Chipotle and hitched a ride with them. It scared them so bad and they called me wondering where I was and I told them I was already back at school. To get back at me they parked my car in the farthest lot! It was hilarious.

So this post was the beginning of the bad/sad entries. And it is not going to let up for a long time. There are always some bright spots though. But there are things you should know before reading the next post. I am one of the bravest- maybe not THE bravest- but one of the bravest people you know. I'm not saying that because I'm conceited or because I think very highly of myself. It's because to go through what I did, I had to be. Also, I think the most important part of my personality that helped me through everything was my stubbornness. When you refuse to give in, it makes it kind of hard to let yourself die. All of this may sound ridiculous to you now, but I hope later you will understand. I also hope that by writing this, maybe one day I will help someone else who has had a lot of health issues. Being this honest with the public is hard, but I think it will help me immensely to get over what has happened, it will help everyone who knows me to understand me better, and maybe it will help someone else one day.

Tummy Troubles and Weddings

I was going to start this by saying I'm sorry I hadn't posted in a while, but I have been busy. But I was on spring break and I had the time. I think that I'm just nervous to start the next section. It gets a lot more uncomfortable and personal. So if you choose to keep reading, then you're going to learn a lot that you may not want to know about me.

When I was 10, I started developing IBS. For those that don't know, IBS is where you have intestinal and colon issues and you have to use the bathroom a lot. Like A LOT. Your insides can get irritated easily and that irritation can cause pain and bleeding. I had my first flare up and my doctors started me on steroids. The steroids caused me to get round in the face and a bit chubby. However, that was better then the alternative. Before, I was ridiculously thin because my intestines wouldn't absorb any of the food I ate. I was a walking stick. Some of my friends would joke that if I turned to the side, I would disappear. But the steroids weren't good either. You eat a lot, you have horrible moods, you become hyper, you can't sleep. Luckily, I don't think it was too bad for me when I was that young, but it definitely had its effects later.

At the time, my oldest sister, Carina, was engaged to be married. She and her husband, Jason, got married when I was 11. It was a beautiful wedding even though I don't remember it too well since I was so young. Of course, during it I was in the middle of a high dose of steroids. And that was the first wedding that I had been a part of (as a flower girl) and it began the precedent of me never looking "normal" in any wedding I've been in. Of course, I know that the weddings are in no way about me, but I think I will always be a little sad that in all of my sister's and cousin's wedding pictures, I never look like what I want to. I feel embarrassed to look at them and even more embarrassed to be so selfish. Even as I'm writing this I feel myself wanting to cry. My family won't even let me look at the pictures of my cousin's wedding that happened when I was 19 because they know what it would do to me emotionally. While writing this, I hate that I'm complaining about how I looked at someone else's wedding. It's stupid and egotistical. But the brides were all magnificently beautiful and it was a wonderful day for them which is what really matters! The other weddings will surface later in the blog, but this was when I first started getting sick and what it coincided with.

Welcome to Texas!

At six, my family moved out to Texas for my dad's job. My dad told all of us that everyone rode horses to school and we would need to bring our own trees (for foliage beauty as well as for air)! To me, it wasn't that big of a deal because I was so young. Of course, it was a lot harder for my sisters, but they eventually came around. I made friends and had so much fun with them. But every time I got a tiny cut, I would have to go inside and wash the cut, put hydrogen peroxide on it, put neosporin on it, and cover it with a bandaid. And that was just how it was.

Now, when I was in elementary school, the playground was mulched so I was unable to go outside for recess with everyone. This could have been so sad, but my teachers made it great for me. I was able to invite a friend to stay inside with me and we could play on the computers. So I became one of the most popular girls because none of the girls wanted to go outside in the hot sun! And I had so much fun! That school was so great to me. The principle even changed one of the playgrounds (there were two) to a pebble base so I could go. Of course, we had no idea she was doing that, otherwise we would have told her that since the other playground was still mulched (and the bacteria could be picked up in the wind), I still wouldn't be able to go outside. But it was one of the kindest things anyone has ever done for me. It showed me that even though I had a disease, people would look past it and help me get around it. :)

Needles and The Butterfly

When I first started taking my medications, I only thought that I was taking pills. But it turns out that my parents had somehow been secretly giving me injections every other night while I slept. So one night I had passed out on my parents bed. All of a sudden I woke up to my parents holding me down with a needle in my leg! I freaked. Like seriously freaked. I was bawling and asking what was happening. So after that night, they told me that I had to get a shot every other day to help me with my disease. And I did. Sometimes it was a struggle- anyone would be reluctant to have a needle shove into them. So I have so much respect for diabetics. Shout out!

There was even an incident when I was probably seven when my parents were out for the night so my sister Mercedes had to give me my shot. And oh my, it was hard for both of us. First, I had become VERY particular about how the shot was given. It had to be in the exact spot I said. It had to be at this angle. Et cetera, et cetera. Second, Mercedes had never given me a shot before and she was probably 15. We sat in my grandmother's room for 15 minutes waiting for me to give the word to let her do it. Finally, when I gave the word, she began to give me the shot, I squealed a bit, and SHE TOOK THE SHOT OUT. OH MY GOD. I was already freaking out (as was she) and then I scared her so bad with my gasp that she took the shot out of my skin! I was sooooo mad. It is completely hilarious now, of course. I mean, it's a tiny shot. But then we had to start all over. These two nervous girls- 7 and 15. It was horrible, but I also trusted her from then on to give me my shots whenever my parents were gone.

I also had to start getting my blood drawn every three months when I was diagnosed. Now THAT was horrible. I would scream freaking bloody murder every time they tried to take my blood. My parents joke that they made me a room with a closed door because I screamed so loudly (there really was a room made in the blood draw area). So my parents would hold me down and I would become a banshee. But that was the problem. THEY HELD ME DOWN. Finally, when I was five (about a year later), my grandmother gave me her butterfly pin to wear during my butterfly blood draw (the blood draw needles look like butterflies because they have wings). Also, since I was five, I was able to more easily articulate what I wanted. The butterfly pin gave me the strength I needed to speak up. I told them that I just didn't want to be held down! Seriously. It was that simple. I just hated being held down. So the next time I just walked up and sat in the chair and held out my arm. They put the tourniquet on and drew my blood. No fuss. And that was when butterflies became my thing.

This is a very similar pin to the one I have in Florida. 

I may upload a picture of the real one later.

Diagnosed

I grew up in an amazing family. I am the youngest of four girls- Carina, Mercedes, Brandan, and then myself- and we have the most loving parents. And I am so grateful that I have this wonderful family because without them, I would never have been able to go through all that I did.

Now for the story- which is all based on family members' recollections (I was 4 at the time and cannot remember). My sisters and I were performing in what we called the "Basement Productions"- our fake theater in our grandparent's basement where we put on little shows- when my parents seemed to notice something on the back of my legs. They called me over and I sat on their laps as they examined me. Apparently, I had these large red bumps on the back of my legs. We had no idea what they were or why they were there.

So our search began. We went to many doctors. They would biopsy the bumps and become puzzled. It was a reaction to a normal bacteria that most people's immune systems should be able to fight off. But why wasn't my immune system kicking in? Why was it not fighting?

Finally, one doctor performed a simple blood test on me to see if I had a rare disease called Chronic Granulomatous Disease, or CGD. Now the odds of having CGD is 1 in 250,000...
And I had it. Once again, I must remind you that I remember none of this. At least, I didn't until I was checked into the hospital to treat the infection with IV antibiotics given through a large tube in my chest. My first memory is of waking up at night to see my parents sleeping in chairs in my room. That's it. Just a snapshot of my parents snoozing at the foot of my bed etched into my mind.

I can't even fathom how my parents felt. I imagine it was something like this: They had three healthy children. Why did this happen to their fourth? How much do we know about the disease? Is this fatal? How will this effect her life? How will this effect our life? But mainly, how do we make it better?

So that is the beginning of my story. My first memory is of a hospital and my parents at my side taking care of me. And I guess this is why my version of normal has always included illness.