Reunited with my best friend and worst enemy
Fate is a strange thing and so unpredictable it astounds me. If you have read my previous posts you know, I had a heart transplant February 2, 2017 following over 36 years of living with hypertrophic cardiomyopathy, a genetic heart muscle disease. I did an unusual thing and asked for my heart to be preserved for research and education. The program at University of Toledo that is preserve my heart with plastination was unsure how to “open” it, as they had never worked on an HCM heart. I contacted a friend, Dr. William Roberts at Baylor in Texas to ask this world-renowned pathologist to dissect the heart for me. The heart was sent to him several months ago and I thought it had been done. I received a note from Dr. Batista in Toledo, the team that will be doing the plastination, that they had not gotten the heart back yet. As fate had it, I was planning to go to Texas to visit a new HCM Program at University of Texas Southwest, located a few miles from Baylor. I called Dr. Roberts office and asked if my heart was ready yet the answer was no they had not yet processed it. I said, “What are you doing Friday morning?” They were available to fit me in and I arranged to change my return flight home and attend my own cardiac pathology.
I admit this is a bit unusual, wanting to be present to see my heart opened and see what was really going on all these years, but I needed to be there although I did not understand why until it was over.
I arrived at Baylor 30 minutes early in part to calm my nerves. It was like being reunited with a long lost family member, I was excited and emotional at the same time. Becky Banks, Dr. Roberts’s assistant a long time phone friend whom I have never actually met but we had worked together for many years, greeted me. She took me back to his office, which was exactly as I expected filled with files and images of hearts of every shape and size imaginable. I have always been fascinated with cardiac pathology and the way the heart can compensate to keep us alive. Dr. Roberts is a rock-star to me and I have followed his work with great interest for many years. Over a decade ago a young man who was originally thought to have HCM, but instead turned out to have Danon’s Disease, passed away at 15 years old, his family donated his heart for research. I helped arrange it getting to Dr. Roberts and as it turned out it was the largest heart ever recorded weighing 1640 grams. We learn so much from pathology that helps the living and broadens understanding of disease, which was way I was so excited to see my own.
Dr. Roberts and his lab assistant and nurse, Saba, took me to the lab where Saba had already prepared my heart on a tray, it was covered with a cloth and I knew it was only a few moments until I would see her again. Because my HCM Community had joined me on this journey, I turned on Facebook Live and let them join in on the moment (see video below. There are some grainy patches but stick with it, it will clear up) . The cloth was pulled back and there was my heart, much smaller than I imagined and covered with more fat then I wished. I held it in my hands and noted it was not as heavy as I thought it would be but it was dense and very hard. I have held hearts before, this one was different it was really hard. Dr. Roberts placed his finger in the aortic valve and asked me to do the same. He said feel the walls on both sides? I did they were hard and completely blocked there was not a cavity as there should be. He then did the same down the mitral valve and I followed, again it was tight with NO space and I could feel the tiny pieces of the mitral valve pressed against the septal wall. I late joked with my husband it felt like those “Chinese hand cuffs we played with as children”. Dr. Roberts had a large long razor sharp knife as he lifted it to cut my heart to reveal its secrets; I got very tense, sad and a little afraid, it was the only moment I looked away. I thought long about why I felt this way and in truth; it was fear of the unknown. What IF my heart didn’t look that bad and I would be seen as not tough enough to have lived with something compatible with life or on the flip side was it as bad or worse than anyone knew or thought?
The moment the knife was all the way through and the heart fell into two pieces I was completely shocked at what was in front of me. My heart was thicker and more scared then I had thought it ever was. I had never been able to have a cardiac MRI due to my implantable defibrillator and abandon lead so I had been relying on echocardiography for 25 years to monitor my heart. My last echo report said my septum (the muscle that separates the left and right sides of the heart) was 1.9cm, which is about twice the normal thickness. However as my heart sat in front of my eyes and it was measured the septum was between 2.5cm and 2.8cm. The scar was throughout the septum in a line and patchy throughout the entire heart. There are parts of your heart called papillary muscles and mine were completely scar, I am honestly not sure how they were even functioning. A thrombus was visible in my aortic valve; I have absolutely no idea where that came from. My mitral valve was thickened and the scar from my endocarditis (1990) was clearly present. What was must astounding to me was that I had no cavity left. My ejection fraction on echo appeared preserved at 50% but how much blood was really processing if the cavity was so tiny? My fear that my heart was too good turned out to be unfounded, the reality in front of me left me un utter amazement that, “she” my heart, let me live as actively as I did for as long as I did.
I took my heart to the CT scanner and had images taken so we can have copies made in a 3-D printer and then I said goodbye for now to her. She is tough and strong and I thank her for holding out as long as she did. Then I began to process what I had just witnessed and oddly I went back in my mind to my freshman year of high school when I was treated so poorly by the gym teacher at my high school. He once kicked me in the ribs in an attempt to make me do a push up while I was experiencing chest pain. I will be sending a copy of my heart images to Coach Bill Regan at Morris Knolls High School to show him what I was living with and what he lacked the compassion to make accommodations for. Even with a letter from my then cardiologist, my HCM was completely dismissed by this man causing pain and scars that will always be with me.
Life with HCM is a complex maze of trying to be “normal” and not let your symptoms impact your life too much while trying to be honest with yourself and manage your condition as best you can.
If I had not had the benefit of fantastic healthcare access to top level HCM experts like Dr. Barry Maron, Dr. Martin Maron and transplant teams like the one at Newark Beth Israel and Dr. Mark Zucker and Dr. Margarita Camacho my story may have had a much different ending.
Today my images collected in the CT scan are being turned into 3D images of my heart, stay tuned for their arrival!
For more information on HCM please visit the Hypertrophic Cardiomyopathy Association at 4hcm.org