OUR JOURNEYS

Patient story

Carson SmithCarson Smith

From an article written by Carson and published in the Paducah Life magazine (February / March 04) -- reprinted by permission.

There are so many butterflies in my stomach' I'm sure they'll escape at any moment. Then a voice reaches into my world of nervousness and pulls me back to the present, "Ladies, on your mark, get set, and bang, the starter pistol cracks!"

The race has begun, I'm on the heels of the girl in first, perfect positioning. The wind in my face on the back stretch, around the curve, my heart beating wildly, the last 100 yards, my feet pounding the beautiful blue rubberized track at Paducah Tilghman High School. I've passed the girl ahead of me, 50, 40, 25, more yards. I cross the finish line to the sounds of crowd yelling encouragement and congratulations. I did it! I won!

Suddenly the scene jumps and I'm in a hospital, being wheeled into surgery. I look into the eyes of my hopeful, fearful, family. Suddenly I wake up, and release a sigh that has a double meaning as I realize I was having a mixed up dream. It is a sigh of sorrow and a sigh of relief....

For complete story, please see Carson Smith's Journey: A Different Kind of Race.

Carson SmithUPDATE, JULY 19, 2007

Taking Deep Breaths – Waiting (and Hoping) For My New Lungs:
The Ups and Downs of My Transplant Journey
by Carson Smith, Washington University Sophomore
St. Louis, Missouri

College is about independence - being away from home, trying new things, tackling challenging academics, and meeting interesting people who are different than you. And in my case, most people are very different. I haven’t yet met a lot of new college acquaintances who are on the organ transplant list waiting for a new set of lungs … and for a second chance at life.

Sometimes it’s awkward to talk about. There are a few questions that are typical conversation starters. Things like, "Where are you from?” … "Are you living on campus?" … "Do you know your major?” … "What were you involved in during high school?” … "Did/do you play sports?" This last question is where I was always tripped up last year as a freshman. Because I still to this day so desperately want to play sports, I could not simply answer, "No."

Instead, I would say, "Well, I used to," or "Not anymore," which would inevitably lead to the question of why I didn't still participate, at least in intramurals. My answer depended on how well I knew the person and how interested I thought he/she was in really getting to know me. My standard answer became, "Well, I stopped playing in high school because I started having problems breathing when I was playing." I left it at that giving my fellow student a way out if he/she didn't want to continue the conversation, letting the assumption be it was asthma or something of that nature. If there were follow-up questions, I was willing to offer more information, but I didn't want to force it on anyone. In some instances, I confided that I have a lung disease and need a double lung transplant. Then the inevitable, uncomfortable silence and a head-down, mumbled apology because I had been pushed for an explanation.

The truth is I have Pulmonary Veno Occlusive Disease (PVOD). Most people haven't heard of it because there are currently less than 200 people worldwide diagnosed with it. In essence, the veins in my lungs have fibrous material growing in them. Consequently, blood pools in my lungs and my body cannot get the oxygenated blood it needs. My heart has to work overtime to make up for my slacker lungs. Eventually my heart will stop working if I don't get another pair of lungs. Even though the words flow readily now, it hasn’t always been easy for me to grasp this reality. I have traveled through lots of dark, unknowing days while doctors tried to figure out what was wrong with me.

To start with, I was born an athlete. It truly is that simple. My mom tells me that I was an incredible runner from a very young age. I ran track for six years, I played fast pitch softball for 10 years, I played soccer for 12 years, and I danced for 15 years. All of this while trying to figure out why it was getting harder and harder to do what I loved. When I was 12 years old, I ran a 5K race. During the race I started experiencing extreme shortness of breath. I slowed to a walk. As soon as I was able, I started running again. I blamed it on the cold, December air and my lack of conditioning for the distance. I forgot about it, until the next August at soccer practice, I experienced the same difficulties again. The temperature was blazing, so again my breathing struggles were blamed on weather.

I was concerned. It didn't feel like it was the weather. My mom, dad and I talked to my pediatrician and she gave me a mild inhaler and I continued my activities. I was excited about the upcoming track season because I thought my breathing problems were solved. But the reality was that the symptoms intensified. I increasingly used the inhaler to keep pushing myself. I completed the track and soccer seasons, resolved to push myself harder the next season to get in shape. I now know I was ignorant and foolish.

The fall of my freshman year at Tilghman High School in Paducah, Kentucky, I played soccer on the varsity team and loved it. But before the season was over, going a single day without using my inhaler was unusual. Spring sports were no better. My breathing problems were ruining track and club soccer. By this time, my parents and I were growing wiser and more informed. We went to a pulmonologist. After several visits and treatments, I didn't show improvement. We all became frustrated. The pulmonologist insisted I had to be hyperventilating. I remember being so angry. I knew what hyperventilation was and I knew I wasn't hyperventilating. At that point, he chose to stop being my doctor.

Meanwhile, I continued to play soccer, spending most of my time on the bench, unable to breathe. I hoped my new doctor would have a solution. Instead, he said I was overly stressed and he prescribed cutting some of my activities. My frustration mounted.

Up to this point, the doctors had not confronted my problem, only my symptoms. I was becoming increasingly irritated and discouraged. Not only was my body failing me by not letting me enjoy what I loved to do, but my doctors denied me the chance to try to push myself. I was told to quit all athletics. I was in shock. I had always identified myself as an athlete. I didn't know what to do with myself.

After several agonizing months of no physical activity, I was able to convince my doctors that I could dance without having trouble breathing. This saved my sanity. I depended so much on my family to help me through what I thought would be a short "break" from exercise. Instead, what I learned is that they are still helping me through this entire process.

While dancing, working hard to excel in my high school studies and dealing with the depression of not being able to do what I loved most, my life literally turned upside down. I started being seen by numerous doctors who all had different diagnoses. I spent hours sitting through medical tests and waiting for results. I felt like a lab rat always being poked and prodded. My family and I would go to St. Louis for a week and most of the days would be spent in the hospital. My family was wonderful about always making sure we did fun things when I was not undergoing tests at the St. Louis Children’s Hospital. We would go to a Cardinals baseball game, or to see a show at the St. Louis Muny, or out to enjoy a nice dinner in the city.

Looking back, it was during these times when I felt closest to my family because I could see on their faces how much they were trying to help, how frustrated they were, but mostly, how much they loved me.

These hospital visits were typically tense because we just didn’t know what would or would not be found during the next battery of tests. I vividly remember a medical fact-finding trip to St. Louis when I was to have a heart catheterization with a twist – while the catheter was in my neck I would be put on an exercise bicycle so data could be collected on my pulmonary arterial pressure while exercising. After the grueling test, I was in a pulmonary waiting room. I remember the doctor walking in with my mom, dad and sister following. I instantly became aware that my mom had tears in her eyes. I quickly look at dad’s face and he looked like he was having a harder time not bursting into tears than my mom. My stomach lurched, but I still had faith that everything was okay. I turned to my little sister and tried to make a joke, "Chandler, what are they crying about anyway? I feel great." Then my sister burst into tears.
The words I would hear next will stick with me for the rest of my life – the doctor explained I had a rare form of Pulmonary Hypertension called Primary Pulmonary Hypertension and it appeared to be exercise-induced.

I was immediately wheeled to the Intensive Care Unit. Everything turned upside down. I felt trapped in this glass box watching everything go on around me and I just wanted to scream that I felt fine.

The next week was rough. It was hard going home to Paducah and telling everyone I had a life-threatening disease that no one really knew how to treat. My doctor had recommended getting a second opinion, just in case. So my mom started searching. Columbia Presbyterian in New York City was her conclusion. "It's the best," she told me and with the help of the medical staff at St. Louis Children's Hospital we scheduled an appointment.

The doctors at Columbia Presbyterian were wonderful. They performed the same cardio-cath with exercise as I had undergone in St. Louis, but this time when they set me up I completely passed out. They gave me a minute, we tried again, and we were able to complete the test. The conclusion was that I needed a newly approved medication and I was sent home.

Just my dad and I went to my follow-up appointment at Columbia Presbyterian. We had such a good time on our daddy/daughter excursion in New York City, and I tried hard to forget why we were in the Big Apple. During our week-long stay, Dad and I took a self-guided walking tour through the city, went to Soho, visited a bicycle shop where my dad knew the owner, visited New York University’s grad alley and finished our time together by attending a Yankee’s game at Yankee Stadium. Wow!

Unfortunately, the end of the week didn't go quite as well as the first half. The radiologist saw some strange white markings (interstitial) on my chest x-ray. No one had said anything about them before, but the doctors didn't like the looks of them. We went home and received a telephone call from the medical team. The news? It could possibly be Pulmonary Veno Occlusive Disease, but the only way to know for sure was an open lung biopsy. We decided to have the operation done at St. Louis Children’s Hospital.

The first day of my junior year at Tilghman High didn’t find me in Paducah behind a desk; I was lying in a hospital bed being prepped for surgery. Mom was with me for the pre-surgery tests but she went home for my sister’s first day of third grade and then drove back to St. Louis for my surgery. My dad stayed with me and the youth minister from my church had driven three hours to be at my bedside as well. I was so glad they were there when the doctors explained the procedure. They were going to cut into my skin right below my breast and then use what I had described to me as, "a tool kinda like a miniature hydraulic jack you would use on your car" to spread my ribs open. Then they would cut a one-inch section from my lung to examine in the lab. My only real memory of that day is the searing pain that I felt when I woke up. I couldn’t sit up, roll over, move my arms or breathe without pain.

The biopsy results were the ones I feared. It was confirmed – I had PVOD … the rarer condition … the one without a cure … the one that required a double lung transplant. It couldn't be happening. Not to me. I was so active. I took excellent care of my body. I was a runner. Why me? My goal in life was to excel at sports. When I was able to run, I felt more alive than at any other time in my life. Now I needed new lungs.

I cried. My family cried. We were a mess of tears for about two weeks. Then we decided it was time to pick ourselves up and keep moving forward. We silently decided to stop grieving, almost in unison, and decided to tackle getting me “fixed”. There were, and still are, days when I do think to myself, why did this have to happen to me?

My hometown of Paducah, Kentucky, rallied upon hearing my news. It was amazing. The community wove themselves together to become my safety net, my support network. They are there still today to catch me if I fall. As a part of this community effort to do whatever needed to be done, two gentlemen researched the Children’s Organ Transplant Association (COTA) and brought this amazing group of people into our lives. At first it was hard for my family to air our personal and financial affairs in front of everyone, but it gave people a way to show they cared and a way to help. COTA stepped in and taught the community how to raise funds for transplant-related expenses and how to raise awareness for the need for organ donors. I was so deeply touched by the number of people who reached out to help my family and me during some of my darkest days. I decided I needed to spend my future years working in the medical field and to spend my volunteer time helping to get the word out about COTA and about the need for organ donors.

The Children’s Organ Transplant Association (COTA) is an organization that works with families of children and young adults who need a life-saving organ, bone marrow, cord blood or stem cell transplant. COTA guides the family and their volunteers through the fundraising process and serves as the trustee of the funds that are raised.

My COTA campaign began a few months after I was diagnosed with PVOD. With the help of COTA, volunteers in my hometown organized a fundraising campaign that raised just over $200,000. This money is set aside to help cover costs related to my future double lung transplant, as well as care before and after the transplant. It can be used to help with prescriptions, hospital bills, living expenses for me and my family while I’m at the transplant center, the insurance deductible and more.

I have always enjoyed speaking in front of people. Now, I had something important to say. I wanted to educate as many people as possible about organ donation and transplantation. In the two years before I left for college I spoke to every group who would listen.

Having this disease is not easy. I take more medications than anyone my age should have to swallow. The side effects of the meds I ingest give me constant headaches and make me more susceptible to getting sick. I noticed, especially as a freshman in college, I don't have any way of releasing my stress. I had always used physical activity and since I was able to continue dancing through high school, I had always had an outlet. Not now – not at college. So much of the time I feel isolated from everyone else around me. Even my friends and my family who love me and know so much about what I am dealing with are outside the bubble of this disease.

Looking on the bright side, I have learned so much (medical and otherwise) from living with this diagnosis and waiting for my double lung transplant. Eleanor Roosevelt was correct when she said, "People are like tea bags, you don't know how strong they are until you put them in hot water."

Through the years that I’ve struggled with this disease (and even before I knew I had it), my circle of family and friends and community have been put in so much hot water. They are truly some of the strongest, kindest, most loving people I know. Everyone who has helped me during my hard times, and celebrated with me during the good times, has taught me that people are inherently good. I have learned that no matter how much I want to push my emotions from PVOD into the shadow, it's okay to show weakness every now and then.

Having this disease has shown me what it means to be loved and what it means to let people help because they do truly care. It may sound strange, but if someone gave me the chance to go back in time and magically have a normal life I would respectfully decline the offer. The value of knowing how to play with the cards life deals you is way too important.

This is one race that I am confident I am going to win.

Carson Smith of Paducah, Kentucky, is currently a Sophomore at Washington University in St. Louis, Missouri. She is at Washington University as an Ervin Scholar and a Toyota Scholar. During her senior year of high school, Toyota selected her as one of the 100 Young People Most Likely to Change the World. Carson is currently on the organ waiting list for a double lung transplant.

 

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