Cardiac Journey: Part 2

We scheduled a consultation with a cardiac surgeon at that appointment. My parents also considered talking with other surgeons around the country that specialize in Marfan syndrome. I knew this surgery was a big deal in theory, but this made me realize it actually was. After talking to the local surgeon, we decided to stay in town for the procedure. I was very lucky to have the option to choose a surgeon who was familiar with my condition and made me feel comfortable.

I was going to have a valve-sparing aortic root replacement, also known as the David procedure. They would remove the enlarged section of my aorta and replace it with a graft. This procedure would be done on full-cardiac bypass, meaning they would essentially stop my heart and use a machine to circulate my blood. Fortunately, my aortic valve was still functioning well which meant that they didn’t have to replace this (hence valve-sparing). It was an “open heart surgery” meaning they would have to cut through my sternum (also known as the breastbone) and spread the two sides apart to get a clear view of the heart. This part of the procedure is known as a sternotomy.

My surgeon explained all these details very thoroughly. He drew pictures and answered all of my questions. Having an idea of the basic steps made the procedure seem much less daunting. My surgery was scheduled for July 12, 2017 which would give me just enough time to mostly recover before the start of my sophomore year of high school.

While I was definitely scared for the surgery, I spent the months prior worrying about “breaking the news” to people in my life. At this point, I still had a lot of trouble talking about my condition. I liked to be seen as overall healthy and normal even if the medicine said differently. Some of my best friends didn’t know the full extent of my condition, at least not based on what I had told them. In order to explain everything, I meticulously put together a powerpoint presentation with visuals and definitions to help them understand. As dramatic and extra as this might seem, I had conditioned myself my entire life to hide this part of me. So for me, having surgery was exposing this vulnerability of mine. I let my parents tell everyone else, including family members.

July came quickly, as does anything dreaded. While I was nervous about everyone knowing of my condition, the outpouring of love and support from so many people in my life was the first time I actually believed and internalized that having Marfan syndrome was something that made me strong, not weak.

The day before surgery, I went in for lots of pre-operative testing. My family and I went out for dinner that night, my choice - sushi and ice cream. Our front door was decorated by family friends wishing me luck. We dropped off my brothers at a friend’s house for an “extended sleepover.” I felt good going into the day of surgery.

My parents and I got to the hospital early the next morning. I remember waiting in an empty side room, being given medication to start calming me down, and then finally being rolled back to a bright room. They alerted me that they were about to put the anesthesia mask over my mouth and that I should start counting down from a hundred once they did. I remember being caught off guard and asking, “Really? Now?” A surgery I had anticipated my whole life was minutes away from starting.

I was told that I wouldn’t remember much from the days after surgery. I didn’t totally believe this ahead of time, but it definitely ended up being true. I vaguely recall waking up in the cardiac ICU with countless IVs/tubes attached to me and an oxygen mask over my face. I know that within that first day or two, I slept most of the time. I woke up mainly to adjust my oxygen output or to push the button that administered morphine through one of my IVs. The pain wasn’t like I expected. It was super deep pressure that was almost paralyzing. It was hard to breathe. I was awoken for chest x-rays in the super early hours of the morning. I was almost incapable of movement in those first couple days so someone had to lift me, slide a board under me, and take the chest x-ray from my hospital bed.

*Content warning for anyone that is squeamish* I was warned about the removal of my chest tube before surgery. A tube of at least a centimeter in diameter was inserted through a separate incision below the sternum meant to drain blood following surgery. I was told that I did have some unexpected bleeding during the procedure which they were able to control. This tube would help the doctors make sure I didn’t start bleeding again. Chest tube removal is known to be extremely painful. I was reassured that I would get a significant dose of pain meds and something to help calm me. The goal was that I wouldn’t remember the experience afterwards. I was worried this wouldn’t be enough, but luckily I don’t remember a thing from the removal. All I know is that they left a gaping hole in my abdomen covered by a large bandage. It would take months for this to heal as it closed up from the inside out. *End content warning*

After my chest tube was out, I was expected to start moving a bit. I was still hooked up to machines, but I was encouraged to attempt going to the bathroom on my own. The nurses brought a portable toilet into my room and placed it less than a step away from my bed. They helped me transfer so that I barely had to stand to get onto the toilet. I still remember just how difficult it was to sit up straight for only 30 seconds. I couldn’t even stay there long enough to actually go to the bathroom. I remember laying back down into my bed - winded and exhausted. That was when I fully realized how hard recovery would be and I was terrified.

With my unsuccessful attempt at going to the bathroom, the doctors inserted a catheter instead. I was so defeated. I needed a tube to relieve my bladder for me. The next day, I tried transferring to a chair, so I could practice sitting up again. It was a bit easier this time but still draining.

I was soon being encouraged to try and take a few steps. I am typically eager to take on this kind of challenge. I like proving that I am capable and strong. I push myself, sometimes too hard. So it was out of character for me to be so hesitant. The difficulty of the action terrified me. I had expected pain post-surgery, but I hadn’t expected needing to relearn many basic actions of my daily life. With a bit of coaxing, I soon tried walking a bit. It did get a lot easier even with a few days of short walks. But I will never forget how strange and unnerving it was to be so incapable of activities that used to be automatic.

Time passed slowly in the hospital, especially once I was out of the ICU and starting to feel a bit better. I was gifted countless coloring books and card games to help me pass the time, but these are only entertaining for so long. I watched reruns of the same Disney shows on the hospital TV when I got especially bored. Finally, I was discharged about a week after surgery.

I had thought recovery would be much easier once I was home. I remember how unexpectedly painful the car ride was. I tried to sit as still as possible because even slight movements were uncomfortable. I thought that I would at least be able to sleep through the night at home since I wouldn’t be awoken for tests and IV changes. But it was super hard to get comfortable in my bed, and pain often awoke me in the middle of the night. Turns out, I still had a long road ahead.

While I was recovering at home, I had a lot of visitors. Childhood friends made the road trip to come see me for the day, cousins stopped by, and more people dropped off care packages. If nothing else would come out of this experience, I was reminded day after day how lucky I was to have such an extensive support system.

After the first few weeks, I started to feel pretty normal in terms of daily activities. I was still unable to do any physical activity or lift much weight until my sternum healed. But I at least felt like myself a bit.

About a month after my surgery, the high school tennis season started. Being a part of the team was one of my favorite parts of freshman year. I still really wanted to be involved even though I couldn’t play. Reaching out to the head coach, he told me that I could assist the JV coach throughout the season. While I am so glad that I was still able to have some kind of role on the team, it was super hard to go to practice and be unable to participate. I was embarrassed above anything else. I was almost “outing” myself as having this condition and opening myself up to questions. Fortunately, most people were super understanding and supportive.

At the start of the season, however, I did have one encounter that really stuck with me. During tryouts, the JV coach asked me to take notes on the matches and players since she couldn’t watch everyone at once. On the second day of tryouts, one of the upperclassmen came up to me and said, in an obviously sarcastic tone, “Omg, is that your special note taking pen? That’s so cute.” She wasn’t outright making fun of me, but I started questioning why I was there if I couldn’t actually play. It made me feel like I shouldn’t be able to be a part of these things because of my medical condition. It helped that everyone else seemed to consider me a part of the team, but this comment definitely added to the shame I felt about my condition at the time.

On October 4th, exactly 12 weeks after my surgery, I was given the green light to play tennis again. While I loved the sport and missed playing it, for me, this was monumental because it felt like I was taking control of my life again. I could have this “normal” thing back.

My junior and senior years, I actually played on the tennis team again. This time, I had a new perspective and felt lucky to even be able to swing a racket. At the senior tennis banquet, my coach gave me props for sticking with the team when I couldn’t play and commented on how far I had come since then. This was another one of those moments when I realized that Marfan syndrome was something that has made me strong and resilient. Hearing this was one step of many to proudly spreading awareness for this condition. Looking back, I realize that this surgery was not only a turning point in my journey with Marfan syndrome but also in my life.

I was extremely lucky in the way my cardiac journey played out. As soon as my cardiologist raised the alarm bell, I was able to schedule a consultation with an experienced cardiac surgeon and soon after that, schedule the procedure itself. I had good health insurance, financial resources, doctors with thorough knowledge of Marfan syndrome, and a vast support system (including amazing parents that were willing and able to clear their work/general schedules to be with me through the whole process). This is all to say that recognizing my extreme privilege, I can’t imagine how much harder this whole process would be without some of these advantages. The already complex surgery becomes that much more risky when performed on an already dissecting aorta. Surgery isn’t even a viable option for these people unless performed immediately. A lot of the time undetected aortic enlargement will be fatal.

Since my surgery, I have been very stable when it comes to my heart. I still take beta-blockers everyday to prevent any future aortic growth. I also have annual cardiology appointments (and echocardiograms), but there has been no aneurysm for almost 6 years now and the graft remains intact. I hope that the very raw portrayal of my experience can help other people with Marfan syndrome who might face a similar procedure.