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Patient story

Donna Pedersen

My name is Donna Pedersen and I am currently a 22-year old nursing student at the University of Maryland Baltimore. I'm also a huge sports fan, a Christian, an activist, a lover of traveling, of music, of friends and family...oh yeah, and I'm also a PH patient ;-) Although pulmonary hypertension has profoundly impacted my life and who I am today, I believe I will have lost the fight if I allow it to be the only thing that defines me. I am here not to worry about dying, but to worry about living and loving and appreciating the world for the crazy, messed up, wonderful place that it is.

So that is just a caveat to say that this is my PH story...but by no means is it my entire life story. If you want to know the rest you will just have to get to know me and ask :-)

Ever since I was about one year old I have been monitored by cardiologists because of a murmur from a small hole in my heart (a VSD). I suspect that the results of an echocardiogram probably eventually led the doctors to believe that I also had pulmonary hypertension. When I was 7 I traveled with my parents to Massachusetts General to have a second heart catheterization done and they confirmed the diagnosis. At this point I really didn't have any symptoms and, being seven, I didn't know or understand what any of it meant for the long term! I was put on nitroglycerin patches, which I wore for years. We still aren't sure if they helped much or not, but back then (1992) no other treatment options were available.

Growing up I was just like a normal kid - I had water gun fights with the neighbors, went to school, even played soccer and swam on the swim team. I had to go see the doctor about twice a year, but as far as I knew it was just precautionary and I would get annoyed with my mom when she would make a big deal about explaining "my disease" on permission slips. No one knew what it was anyway and I certainly didn't feel sick! I couldn't wait till I grew up, moved out of the house and could be done with the silly doctors for good.

But the warnings that my disease would get worse became more real over time. My freshman year of high school I had to quit playing soccer. By my senior year I had trouble even walking up one flight of stairs, although I dismissed it as just being out of shape. The cardiologist said that my pressures were getting worse and I saw a new doctor who told me that I would never be able to have my own kids. That probably the first time I realized the disease was for real - not something that would just go away, but something I would have to fight and adapt to for the rest of my life.

Still, I didn't feel all that bad physically - and I certainly didn't feel ready for the IV medications or transplants that the doctors were thinking about for the future. I quickly got back to my normal routine and on with my plans to go to a good college and make a difference in the world (somehow!). In the Fall of 2003 I entered the University of Maryland on a full ride scholarship (I had wanted to go to Yale but didn't have the money or the acceptance letter :-P). Although I really enjoyed college life and meeting new friends, I often found myself getting short of breath on the hilly walks to class. Often I would stop and rummage around in my purse or backpack in order to catch my breath without looking too strange.

In January I was scheduled for another heart catheterization, but just before the procedure they discovered that my blood sugar was 600 and I was diagnosed with type I diabetes (apparently not at all related to the PH). So I had to be treated for that first. I learned how to give insulin shots and monitor my blood sugar. Although it was an adjustment, I felt empowered by the fact that it was something I could deal with and treat on my own. Life again returned to normal - albeit a slightly altered normal.

In early February I decided to take a ski trip with my new college friends. I was so tired that I didn't even make it to the ski rental place - I passed out walking up the mild incline to the lodge! I thought it was probably no big deal, but once the doctors at Children's in DC found out they wanted me rushed there immediately. So I got to take a 2 hour ambulance ride from the ski place in Pennsylvania to Washington, DC. I even got to watch “Shrek” on the way - although I wasn't feeling great enough to enjoy it much :-P

After staying in the hospital for a few days I started on bosentan [Tracleer], an oral medication that would hopefully make me feel better and improve the shortness of breath and passing out. I returned to school but I was extremely tired. I remember thinking it a huge effort just to shuffle down the hall to the bathroom. On Valentine’s Day I was returning to my dorm after a visit with my parents and I called up to my friends to help me carry some things up (the doctors had advised me not to carry anything heavy). I ended up carrying only one very light bag of groceries, but I still passed out in the elevator on the way up.

I remember feeling the room spinning and then just everything going black. I heard people shouting around me and I wondered if I would ever wake up. Luckily my wonderful friends were very conscientious and insisted to the paramedics that, despite being on a college campus, I had NOT been drinking, and that I had a serious illness and needed to be taken straight to Children’s :-)

When I woke up I felt sicker than ever. Even after a few days of recovering in the hospital I still felt very weak; I needed help just to get up and to go to the bathroom. At 18, my mom had to help bathe me (a moment of ultimate humiliation). I knew something had to change – that I couldn’t go home like this, and I certainly couldn’t live long term like this. Although I had previously resisted doctors’ suggestions of continuous IV therapy, at this point I was eager to try anything that would make me feel better.

The doctors also confirmed my need for help when I finally got that heart catheterization I had not had in January and my pulmonary arterial pressure was 116 (normal is less than 20!). After some waiting around for insurance approval (many PH medications are extremely expensive and the IV therapies are definitely among them), which included some hospital Maryland-basketball-watching parties and lots of wonderful visitors, I started on IV eproprostenol [Flolan].

The night I started Flolan was very difficult; I felt flushed and terribly sick. I was vomiting and to monitor my blood pressure I had an arterial line in my arm, which was quite painful. The worst part was not knowing – not knowing if the medicine would work and, even if it did, how I would make the necessary changes to go on with my life. I knew that just living would be an everyday challenge and I wondered, why? If I was going to make such an effort just to stay alive, I needed a reason. I remember crying as I poured out my heart to the nurse in the ICU that night – someone I had never met, but somehow I knew that she heard me and that she cared. The answer to the “why” question would come with time, but for the moment I knew I just had to take each day as it came.

I thank God that each day that came after starting on Flolan was better than the one before. Just one day after I walked to the bathroom (with some help, but I did it!). We take these things for granted, but for me that was a great accomplishment! The doctors, nurses, and staff at Children’s were and still are absolutely phenomenal – they literally saved my life and their life-giving care for me as a whole person is what eventually inspired me to enter the health care field. Every day I felt a little stronger and the side effects (feeling flushed, vomiting) got a little better. I learned how to mix the Flolan myself… and finally I was ready to go home!

Unfortunately I was still too weak to be completely independent, so I moved back in with my parents for a few weeks (which was extremely difficult after being grown up and moved out of the house for awhile!). Even during that time I still drove to class (about an hour away). I had tried to keep up with my studies as much as possible while I was in the hospital, but I did end up dropping 7 of the 17 credits I had been taking for the spring semester. It was a struggle to keep up with the 10 credits I had left, but I looked at it as something I needed to do to for myself.

Once I moved back to the dorm I generally went to class in the morning and came back, exhausted, to sleep for the afternoon. Unfortunately I was usually too tired to participate in the other activities (Lutheran Student Association, tutoring, choir) with which I had previously been involved. However, I had already discovered my passion for English literature and I found that poetry in particular would become my life raft in a difficult time. Two of the classes I was still taking were literature classes. Even in doing the reading assignments for class, I felt the words come alive. I understood what Tennyson meant when he said,

Tho' much is taken, much abides; and tho'
We are not now that strength which in old days
Moved earth and heaven, that which we are, we are;
One equal temper of heroic hearts,
Made weak by time and fate, but strong in will
To strive, to seek, to find, and not to yield.

Much abides. Not to yield. That was it. “How dull it is to pause, to make an end, To rust unburnish'd, not to shine in use!” A friend at the time (now my boyfriend) reminded me that, indeed, I was a person who could and did shine in use. I could make an impact on the people and the world around me. Maybe that was my “why” for life and for continuing the struggle.

This point was brought painfully and powerfully home for me in a sad way. Just weeks after I got out of the hospital, I learned that a friend of mine from high school who had been in choir with me had PH too and was at Children’s, not doing well. This was a shock, seeing as the disease is very rare and Taryn and I had been two members of a select twenty person choir (what are the odds?!?). I was sort of excited, at first, that I would have someone to share and help in the struggle. I went to visit Taryn in the hospital… I showed her my pump and told her how much better I felt after being on it.

However, a week later I got a call saying that Taryn was not expected to make it through the night. I went to the hospital and waited with her various family members and friends as they took turns going into the ICU to see her and to say goodbye. It was hard to watch, even to just glimpse, what they were going through. I wondered why Taryn had to die and not me. I wished I had taken the time to get to know her better. I couldn’t help but wonder if at some point in the near future this same scene would be replayed with my own family members. In some ways I felt like I was standing against a brick wall and someone was pelting stones at me – one after another. “What next?”, I thought. “What next, God?” But I also knew that somehow He wanted to use me and to teach me, even in this terrible situation.

I spent a lot of time with Taryn’s family, just talking and sharing stories. We all agree that she is still here with us through her legacy of caring for others, of always being a smiling face and facing adversity with courage. Taryn’s funeral was a turning point in my life. Although it was extremely sad, it was amazing to see the people stand up one by one to describe how she touched their lives in ways she probably never realized. She made a difference…which leads me to believe that maybe I can too. Maybe there is more to the “why” than what is immediately apparent. I know that living without Taryn is still extremely difficult for her family and I thank them deeply for allowing me to enter into their lives during a time of great pain and for allowing Taryn’s life to inspire my own.

Slowly but surely, healing came for me – both physically and spiritually. After just a few months of being on Flolan I decided that it’s pretty much a miracle drug. I went from not being able to get out of bed to walking around comfortably, going to basketball games with my friends and planning to take summer classes to make up for lost time! Every day I felt better, and the hassle of dealing with the catheter and the mixing was nothing in exchange for having my life back!

I began to figure out creative ways to get around problems that presented themselves. When I wanted to go camping with some friends at a Christian rock festival, I got a cooler of dry ice to freeze my ice packs for the Flolan and with my mom’s help we arranged for a spot in the handicapped section (which my friends liked, since it just meant we were closer to the action :-P) and made sure medical personnel knew about my issues in case of emergency.

My refrain for dealing with the disease and the challenges it presents has been and remains, “How can we make it work??” I’ve had the amazing opportunity to go to Europe twice since being on IV medication… once on Flolan and once after switching to IV Remodulin, which is even easier to handle since it doesn’t require ice packs and the supplies are a lot less bulky to carry when traveling. Planning for the trips was a lot of work (a lot of which was done by my mom – thank you and I love you, mom!), but the effort was well worth it! During both of my trips to Europe I visited Spain, lived with Spanish families and had a great opportunity to practice my Spanish, as well as visiting many amazing historic and cultural sites. I give huge kudos to Dr. John Berger and Nurse Practitioner Michelle Oulette at Children’s in DC for believing that we could make it work. Their philosophy is that, despite the challenges PH presents, the ultimate goal should be to facilitate the greatest quality of life for patients that can be safely attained.

During the second trip to Europe I also visited England, where my boyfriend was attending graduate school for the year. Andrew and I started dating in the Spring of 2004, only a few months after my long hospital stay and starting on the Flolan. It was strange because for the first time in awhile, I wasn’t really looking for a relationship. I was really just trying to get my own life back together. In some ways I wondered if I was even worthy of love, or if it was fair to involve someone else in the struggle that living with PH can be. Especially of concern is the fact that I can’t have children… what would that mean for a future life together? What if I die? Would I be robbing someone I care about of what could otherwise be a “normal life”?

I still don’t have the answers to all of these questions, but I tried to be as intentional as possible about making sure Andrew knew the facts in the beginning. I didn’t want to hide anything and I wanted to make sure he understood what the decision to be with me might mean. After three and a half years of dating and having what I would consider to be a pretty normal relationship (we fight sometimes, we joke around, we love each other), I still worry sometimes about the effect my disease could have on him (and on all those I love). The most painful and fearful thing for me is to imagine them suffering. Yet, I know this is not in my hands. I know that God is a God of love and He would want me to give love rather than withholding it. I don’t know what the future holds, but I know He has a plan.

I do know that God’s gifts often come in mysterious ways, and that my disease has actually been a blessing in the sense that it has allowed me to connect with other people in really deep and meaningful ways. I have had the opportunity to share my story with others on a personal basis, and also by speaking to groups at Christian youth events and PH events. I often tell people how my struggle with PH has taught me to appreciate life and the many joys it brings, especially the joy of living in community with others. The ability to share my story has often made people feel comfortable sharing their own stories with me. I have come to realize that everyone has a story, often one that includes both sorrow and joy, and that it is by listening to each other that we can learn and grow.

It is this desire to listen and care that makes me so excited about becoming a nurse. I’m fascinated by the way the human body works because I want to know what that means for people and for their lives. Many nurses (and others!) made a huge impact on my life because they worked with me to make things better both physically and mentally. They enabled me to really live, despite the disease. I hope I can do this for others… and can lend some extra understanding, knowing what it’s like to be hospitalized and poked and prodded and scared.

So today it has been about 3 years and 9 months since I started Flolan. I switched to Remodulin in March of 2006. I have had a few bumps in the road since then – one major catheter infection last year and a leaky catheter this year which had to be replaced. I also just recently was hospitalized for an episode of “atrial flutter” – a fast and irregular heart beat caused by too many electrical signals in the atria of my heart. The doctors aren’t sure why it happened, but we're hoping it was just a fluke and not an indication that my condition is worsening.

I just try to take one day at a time, and for the moment I feel great (as I have for the majority of the past three and a half years)! I can comfortably walk four blocks to class and maintain a schedule of school, tutoring and church activities. Earlier in the fall and summer I even enjoyed riding my bicycle at the beach and around the city. And plans are (hopefully) in the works to visit Ecuador in the spring!

I feel very blessed to be in such great health that I can lead the kind of active life I enjoy. It is always a great compliment when I tell my friends about my disease and their immediate comment is, “Wow – I never would have guessed you were sick at all!”

Of course, when the bumps in the road occur, I often worry about when the disease will get worse and what it will be like. But I also think about the fact that I could have died back in 2004… I think about the many amazing things I have had the chance to do and the wonderful people I have come to know since then…and I realize how blessed I am. When it is my time to die, I don’t think I will have any regrets. I will know that I have loved life and loved people and that it has all be an amazing ride. And for that I am truly grateful.

 

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