Lone Cyprus

Lone Cyprus

Tuesday, September 29, 2009

I don't wanna shake your hand. Just wave.

Mom and I drove to Nashville today to see my doctor at Vanderbilt. I have an update and somewhat good news. The thing is, before I started going through all of this, I didn't get a lot of this lingo and there's no way I would have understood most of what I'm going to tell you. So, I'm gonna tell you what the deal is, and then make it as simple as possible for those of you who aren't medically minded and are more like I was pre-scleroderma.

So, here's the deal, pickle. My rhematologist, who is the doctor that will treat me for scleroderma, is referring me to a pulmonologist to address my pulmonary hypertension issues. She'd like me to see a pulmonologist at Vanderbilt that sees only patients with pulmonary hypertension. So, this guy knows most all there is to know about it and exactly what it is I'll need. Hopefully, I'll get in to see him in the next few weeks.

In addition, we discussed and agreed that I will begin taking a drug called CellCept. CellCept is an immunosuppressant and is used as an anti-rejection drug in organ transplant patients. I'm taking the drug in hopes that it will address my pulmonary fibrosis and inflammation. My doctor told me today that she has a few patients who are currently taking it and have stabilized. That means that they aren't necessarily better, but they haven't gotten worse either. As you can imagine, this is quite attractive to me given my options. I've been told that most people tolerate it pretty well with few side effects and IF it works, could definitely buy me some time.

The down side to this? Well, two things really. First, the cost. Recently they have come out with a generic for this drug, which is great! The unfortunate part is that even the generic is incredibly expensive. The plan is, let's try it and see if it works then we'll worry about how to pay for it long term. (Plus, I'll apply for assistance with the manufacturer.) Second, the immunosuppression aspect. I'm gonna have to, in part, go against my nature and chill with the physical affection. I have to begin to be more aware of putting myself in situations where I can easily get sick. Less crowds, less handshakes, less hugs...not just because it's flu season and having crappy lung function mixed with the flu is a terrible combo, but also because the drug will make me more susceptible to infection. Don't get me wrong, I'm still gonna do what I want and go where I want, I'm just gonna be more mindful of higher risk situations. I guess I'll have to go with more smiles, winks, thumbs up, pats on the back and blowing my kisses.

Overall, things feel a little brighter these days. I still have diffuse systemic sclerosis. It still sucks. I still have hope. Period.

Tuesday, September 22, 2009


For the past week, my heart has been especially heavy. There's no denying some sadness and fear exist but even more prevalent is the increasing awareness of the necessity of family. My feelings and beliefs about family, including their purpose and specific roles in my life have been pushed to the front of my heart and mind. I understand that we don't choose who our family members are. I didn't pick out my mom or my dad, brother or sisters. They were given to me. It is no accident that we're connected. I feel this includes all of my extended family as well. Look, I'm not crazy, I realize every member of your immediate and extended family won't be your best friend. You may not even like some of them. That's ok. But I will say this, now more than ever, I appreciate and understand the gift of family. I have taken advantage over the past several months, and even more so the last week, of connecting with and beginning friendships with many cousins, aunts and uncles that I have not been close to since childhood. The warmth, comfort, love and happiness this has added to my life is welcome and cherished.

I have been blessed with much. I have great step-parents, step-siblings, nieces and nephews, a hilarious and loving grandma and many long-time friends who are like family to me. And man, my immediate family? My mom, my dad, my brother and two sisters...they are my constant. They are always on my side, always loving me, defending me, encouraging me, looking out for me, and laughing with me. I cannot and do not want to imagine it any other way. My parents are amazing and my siblings truly are my best friends.

My older sister, Kristi, is the nurturer, the one I can count on to encourage me and check on me when I'm sick. She's who I call when I can't sleep and where I stop when I want easy company. She's always there. Although she's five years older, she always listens to me and respects my thoughts and opinions. She thinks they're important. Although I realize there's no such thing as perfection, I look up to her as a wife and a mother and in my eyes her flaws are few.

Jarrod is two years older than me and he's the big brother every girl should have. He is truly one of my favorite people on the planet to hang out with and I dare you to try to find someone cooler. You can't. I have always admired him and wanted to be like him. He joined the swim team in high school, so did I. He learned to play guitar, so did I. He went to ISU for college, so did I. He got involved in the Baptist campus ministry, so did I. He got his PhD and is a research scientist, so...I went another direction. :) He's my realist. He'll talk facts with me and let me feel what I feel. We can talk sports, family, religion, politics, current events or just make fun of any and everything. It's simple really. He likes me. And I feel lucky.

My little sister, Carin isn't actually little anymore. She's 2 years behind me and our relationship is the one that has evolved and changed the most over the past few years. I used to think we were very different, but now I see that's not true at all. We're a lot a like. We're both emotional and sensitive. We're both smart and a little sassy. (Her more than me, of course.) And I can always count on Carin to defend me and rise up to fight for me. The great thing is, I can also count on her to cry with me if I need and she never fails to remind me of my worth. She's the pesky little sister that's grown into a best friend.

Then there's my Dad. I confess my Dad and I have only grown close over the past few years. He's always been around and available but I've also always been away or running around. His love for me is great and I've never known that more than I do now. Since I've settled here, I've gotten to know my Dad as more than just my daddy but as a man too. Everyone loves my Dad and now I can see all the reasons why. He's funny and genuine. He's generous and full of life. He's the kind of guy who would own a successful restaurant and stand out back, cooking on a Friday night surrounded by all his friends who just want a fun, welcoming place to hang. Hmm...sounds familiar.

My mom may be mentioned last but she'll never be the least. My mom is supermom. She has loved me beyond measure. She has accepted me and supported me. Rescued me and let me go when needed. She has allowed me to be who I am and although it has likely been hard at times, she tries not to question too much. She taught me to love, respect and accept others and her example of a strong, smart woman is not lost on me. She has sacrificed much more than I deserve and there aren't words to describe the love this daughter has for her momma.

Not everyone has what I have. I know this. But there is absolutely no time like the present to think on and share your love for the people in your family. These gifts of mine are priceless.

Tuesday, September 15, 2009

Not your typical, everyday post.

Before I begin with all the information and explaining, I need to put a sort of disclaimer on this blog. My emotions are raw, my nerves are shot and my heart is sad. This is the hardest thing I've ever had to write, so I will try my best to make sense of it for you.

Yesterday was my 31st birthday. My mom and I were in Chicago for all the pretesting for the stem cell transplant and my friend Claire was visiting. We were all three hanging out in our hotel room and the phone rang about 10:30. It was my nurse and she told me that the doctor needed me to come to his office at 3:00. He had gotten some test results back and wanted to discuss them with me. My heart immediately dropped and I became instantly anxious, but she couldn't tell me anymore. The next several hours were unbearable as I waited. I feared he had found something wrong with my heart and wouldn't be able to do the transplant. The doctor had stressed over and over how important the health of my heart is for survival through the procedure.

The time came and my mom and I went to the office and waited. The doctor came in and as the words came out of his mouth, I heard what I had known for hours, months maybe, even years perhaps; I'm dying and there's nothing anyone can do to change that. As reality replaced my disbelief, panic and nausea followed, tears stung my eyes, I felt his hand on my shoulder and glanced at my mother's face. The pain and sorrow I saw was more than I could stand. There are no words for that kind of devastation.

You see, the doctor explained that my arterial pressure is too high in my heart. The blood doesn't flow easily through my arteries from my heart because the fibrosis in my lungs is so bad. My lungs sort of act like a brick and keep the blood from easily getting through the arteries which builds up pressure in them. In addition to this, my right ventricle wall is damaged beyond repair and to an extent that would, when coupled with the high arterial pressure, cause death during transplant. So, no transplant for me.

The next question is, what else can I do? The short answer is, not much. My plan at this point, is to return to my rheumatologist in Nashville for treatment. I will be able to get some meds and oxygen that will make my quality of life better. There is no cure for scleroderma and outside of the stem cell transplant, there is nothing that addresses scleroderma directly, only meds to control symptoms. There are a few experimental drugs being used for treatment of scleroderma but none of the results have been particularly consistent or promising. I will of course look into any options, including these drugs and follow the advice of my doctor.

Does this mean I have months or maybe years to live? I don't know. And I don't want to know. I plan to find some way to be at peace with this, do what I need to do to stay as healthy as I can for as long as I can and I plan to soak up every minute I have left with the people I love, doing the things I love. I have so many people who are always on my side. Many of you worked hard to support me and see me through the past several months and I am grateful. I wish I knew how to express the depth of my gratitude. "Thank you" is all I've got.

Friday, September 11, 2009

Project Stem Cell Transplant: Day Four

Short and sweet today. My right heart cath has brought much anxiety and fear over the last few weeks. This morning, I found, it really isn't so bad. I was lucky because they could access a vein in my neck so I had a short recovery time. I had to stay for about an hour and half after the procedure only because they had given me a small dose of a mild sedative...told ya I was nervous! Beside the fact that it's creepy to have something in your neck, it really was quick and relatively painless. My arms are now official pin cushions as I had 2 more sticks today. The good news is the pressures in my heart and lungs are high but not high enough to keep me from getting the transplant. And even better, week one is over! I made it!

On a more "fun" note, my mom bought me an early birthday present! A City Pass, which is a booklet of tickets to go to some of the coolest tourist places here in Chicago. I'll get to go to the Adler Planetarium, The Field Museum, The Museum of Science and Industry, The Shedd Aquarium and today after a long nap, we went to the Observatory atop the John Hancock Center (94 floors high!). I took some cool pictures and there are amazing views of Chicago and Lake Michigan. I have company coming to visit this weekend and no tests until Tuesday! And right now, the bathtub and bed are calling my name!

Thursday, September 10, 2009

Project Stem Cell Transplant: Day Three

There was going to be no blog today. I planned to go to bed early but have laid down twice to try to fall asleep and can't. I have to be at the hospital for a heart cath in 8 hours and I guess I'm feeling a little nervous. I figured writing about it might ease the anxiety a little. I keep thinking that after tomorrow morning, I'll be officially through my first week of tests. Feels like it's gone really fast.

This morning I had an MRI of my heart done. Lots of the tests I'm having done are to check and double check the condition of my heart. Dr. Burt explained the other day that the heart is uniquely important in transplants for Scleroderma patients. He says it's very important there's not damage or decreased function or we won't be able to go ahead with the transplant. So far, so good though. I've had a CT scan and stress echo and both seem fine. When they get the results from the MRI and the heart cath and then I meet with the cardiologist next week, we'll know for sure. P.S. The MRI was no fun. This was my first and I wouldn't be upset if it were my last!

The MRI took so long we had to hurry to my dentist appointment. I'm having my mouth and teeth checked because the mouth is one of the most vulnerable places for infection and they needed to make sure there were no causes for concern. I checked out OK, which better have been the case considering I feel like I spent the summer at the dentist getting work done! Something I got really excited about is the dentist I saw happens to be particularly interested in Scleroderma and has a very good understanding of what patients with this disease deal with. She was so kind and took the time to personally make me some custom toothbrushes that are easier to hold! Since my grip is not good and I can't close my hands all the way, she molded some acrylic to the handle of the toothbrushes to make them easier to use. They're so great and it helps a lot!

I had to hurry over to my appointment for my vein check after that. Mostly all that means is someone explained to me that during harvesting, I'd have to get a catheter put in my jugular vein to remove the stem cells. I already knew this, but she educated me and my mom on what to expect and how the day would go. The nurse was a funny lady and after weighing me told me that I should take this time in my life to be thankful that I'm not a size 5. Apparently, women with quote, "a little meat on their bones" have an easier time with harvest and usually only take about 4 hours opposed to 7. So, indeed I am thankful to not be a waif.

As I sit here and think back on this week, I definitely feel worn out. It's been rough at times. But I did it. Everytime I'd be in some uncomfortable position or forced to wait forever or someone has been rude and the thought to say "just forget it" has crossed my mind, I dismissed it and pressed on. (And believe me, those thoughts have come frequently.) Although I had no idea I'd be a pin cushion quite like this, with one stick from a blood draw and 3 IVs in 3 days, they've all been done with no problem. I guess the bottom line is, this isn't fun, as I knew it wouldn't be, but it's bearable. I can do this. At least I have my mom. And Dr. Pepper in the refrigerator. What else do I need?

Wednesday, September 9, 2009

Project Stem Cell Transplant: Day Two

Beginning these first two days blogging each day may be a bad idea. I'm not sure I'll be able to keep up everyday. You might actually be glad about that! Some days and things just aren't that interesting. In addition to how today went, I do have another reason for writing. Many people have asked for my mailing address while I'm here in Chicago and I finally remembered to check into that. So, if you're wanting to send something snail mail style, send it here:
The Seneca Hotel
Beth Fortwendel
200 E Chestnut St
Chicago, IL 60611
Keep in mind, this is only my address until the 2nd of October. It will change after that!

Today was a relatively good day. I had to be at the hospital a little earlier and started the day with a Pulmonary Function Test or PFT to those familiar. It's basically a breathing test and measures lung function and capacity. PFTs aren't my favorite tests but they are far from the worst! After the PFT I had a stress echo scheduled. This test, in my opinion, completely sucks. Really I'm not complaining about it because it just has to be done, but let me just give you the low down on what happens during this 90 minute test. In only a hospital gown, I laid on my side in a room cold enough to hang meat. I did have two blankets on but nothing on my hands and they get so crazy cold, it wasn't long before I was pretty miserable. But really this is the tolerable part. Besides being cold there's a blood pressure cuff on my left arm, which is the side I'm laying on, that goes off every 3 minutes. I have an IV in my right arm that is intermittently getting medication passed through. In addition the tech is jabbing an ultrasound wand thingy (I don't know what they're called) into my chest and ribs. And I mean jabbing hard enough it's painful. Also the tech is asking me to take breaths and hold it while he takes pictures and this is especially hard because I can't hold my breath as long as the normal person so we keep having to do the pictures over. And best of all, on top of all that, half way through the test they start giving me a medication that makes my heartrate go from around 80 up to 160. So my heart feels like its beating out of my chest, I'm shaking from being cold, I'm trying to remember to hold my breath long enough and hold my left arm out when the blood pressure cuff goes off. This test is NO fun and I'm always very glad when it's over.

So after all that, we had lunch in the cafeteria and got a chance to meet with the transplant doctor. Our visit was short and sweet. He reminded me of the risks of transplant and likely results. He told me that over a period of a few years I would see great improvement in my skin and potentially some in my lungs. He said there are some people who do not have much lung improvement and there are some that do. I've decided I'm ok with either, as long as they don't get worse. That's the biggest concern for me.

I was done by 2:30 so we came back to the room and had a short nap then we got a little dinner and walked through a few stores close to our hotel. I feel good about today and hopefully tomorrow is even better. One thing I have already found that troubles me about Chicago, I can't find Dr. Pepper in any restaurant I've been in. What's the deal? Didn't they know I was coming?

Tuesday, September 8, 2009

Project Stem Cell Transplant: Day One

Ok, so I'm tired already and we just started today! I spent about 8 hours at the hospital getting all kinds of fun things done. I got to meet with Kristin, the nurse liason for the stem cell program. She's great and she sat with us for about an hour and explained the whole process and what to expect during each phase. Some things seem way less scary now and some things are more concerning.

I had labs next. They needed 18 vials of blood for testing. EIGHTEEN. Ridiculous. It was smooth sailing with no problems at all.

After the labs I had to go to yet another floor and department to get an EKG. The test was quick and painless and not really noteworthy at all. What WAS noteworthy was the walk into the department. A lady about 3 times the size of me using a walker on wheels was called back just before me. Instead of waiting for a tech to come open the door for her, she attempts to pull the door open (which opens toward her) on her own. I'm just behind her and see that she's having a hard time so I step up right behind her and try to grab the door to help so she can pass through. Just as she tries to move backward out of the way, she loses her balance and falls into me, pinning me against the wall. I'm holding all of this lady's weight on me for a good 30 seconds before a tech gets there to help her gain her balance again. That was fun. Actually when I think back, it probably looked hilarious. Wish I would have seen it instead of being involved.

Then the real fun came! I waited for an hour and 30 minutes to get several xrays and scans done. I'm normally fine with the waiting. I understand things get backed up but I was in a hospital gown and it was freezing. I had a chest xray, a CT scan of my chest, sinuses, abdomen and pelvic area. I was told before that I wouldn't have to get an IV, but of course I get to the waiting room and they call me back to get one. I consider that minor compared to the nasty Barium drink I had to down. Three bottles of that stuff in an hour. Simply put, it was gross. So really, I can't say I love being in the radiology department.

It's been a long exhausting day. Running around the hospital to different appointments with different demands at each will wear you out! I feel better after getting to eat and take a bath (in the jetted tub!) and relax. I am very grateful for one lesson I've learned while here though. I'll save you the visual, but last night I learned that bathtub jets can be powerful enough to basically flood the bathroom. Good to know.