Payton's Anomaly
Hi, my name is Payton. I was born on March 28, 2000 at Miami Valley Hospital in Dayton, Ohio. When I was delivered at 3:06 AM, I appeared so blue that the NICU (Neonatal Intensive Care Unit) was called in, and I was whisked away. My mother never got to hold me or even see me. All she knew was that a nurse was saying, “You have a red head!” when I was crowning. My mom got a strange glance from my dad, because no one else in the family has red hair! Little did my dad know that my Great Grandma MacDonald had fiery red hair and a ton of freckles!
My mom had to wait until about 5:30 AM to find out what was going on with me. Dr. Terrence Dillon took my parents down to the NICU to see me for the first time. I was lying in an incubator with several tubes going in and out of me. They kept me asleep with medication in the hours after my delivery. Dr. Dillon drew a picture showing my parents how I had a rare congenital heart condition called Ebstein’s Anomaly. The disease is very rare and it occurs less than 1% of all heart disease. In brief, my heart was deformed. My tricuspid valve was long and protruding, which caused regurgitation or back flow of blood into my heart. I was taken by ambulance to Cincinnati Children’s Hospital Medical Center (CCHMC).
I stayed at CCHMC for about 3 ½ weeks. The doctors thought that I would probably not leave the hospital without some kind of surgery. I was on special medicine to prevent my ductus from closing. Back then, it was really hard to operate on such a tiny heart and my chances were not very good. They kept me asleep hoping that the newborn pressures in the lungs and heart would calm down. My parents brought my big sister to meet me for the very first time in case something happened to me.
One day, my parents went to the hospital to see me and I was gone! You could imagine what was running through their heads! They had removed me from the NICU and moved to me to a regular hospital room. As they were walking to my room, they could hear a baby crying. It was I, fully awake and very cranky. As soon as my mom took me into her arms for the first time, I immediately stopped crying. It was as if I knew right away that she was my mom. It was the first time that they had seen me awake and moving. To them, I seemed like a normal, healthy baby. The doctors told my parents that they didn’t know what happened, but now I did not need surgery. This was a miracle in their eyes and the eyes of my parents. They put me on Amrinone, a heart rhythm medicine, and Lasix, which prevents fluid around the heart and the lungs. I also had to wear a harness that would record any irregular heart rhythms. I was on these medicines and had to wear the harness for about 6 months. Other than being colicky, I grew and thrived.
My health remained relatively stable for many years. I was diagnosed with allergy asthma at age 5. I had an emergency inhaler that I rarely used. I was given weekly, and later biweekly, and then finally monthly maintenance allergy shots for trees, weeds, cockroaches, and cats.
I did not have my first rhythm disturbance, called SVT (Supraventricular Tachycardia), until around age 6. I was diagnosed with WPW (Wolff-Parkinson-White) Syndrome, which stems from my Ebstein’s Anomaly. My SVT seemed to happen when I had a fever. My parents would have me try blowing through a straw, bear down, or dip my face in ice water to get my heart back to normal rhythm. Sometimes these tricks would work, but sometimes after 20 minutes of trying, my parents would take me to the emergency room. The nurses and doctors would hook me up to an IV and would give me Adenosine, a drug which is used to treat arrhythmias. Sometimes it would take more than one dose and each dose got more potent.
With these worrisome symptoms continuing, we consulted Dr. Dillon, who suggested that we see Dr. Knilans at CCHMC, who was the director of Clinical Cardiac Electrophysiology and a Pacing of the Heart specialist. I had my surgery at age 7. The doctors would burn nodes of extra electrical pathways hoping that one normal pathway would exist. They were not able to correct the problem.
As years went by, I would begin to have SVT episodes more frequently and that meant more emergency room visits. We kept a journal to figure out what triggered the SVT, but there was no discernible pattern. We decided to try the surgery again with Dr. Knilans at CCHMC when I was 10. He was not able to reach part of the bad pathway because it was on the outside of the heart. Dr. Knilans wanted to wait until I was older to try again. Instead, he suggested a new wonder drug that had a great promise for rhythm disturbances called Flecainide. This medicine did work for me and seemed to keep my heart rhythm in check. No more awful emergency room visits with dangerous drugs being administered.
Life was going great and we thought that my heart would be fixed when I was a little older. Until December 2011. I had a terrible dry cough. I began coughing about every 30 seconds at its worst. It was horrible. It was hard for me to get any sleep. My parents took me to the doctor’s office at Allergy Asthma of Dayton in Centerville. I took breathing tests and they thought I was having either an asthma episode or I had a bacterial infection in my lungs that I was struggling to get over. I tried antibiotics, steroids, and inhaler treatments. The cough still persisted. I tried a different kind of inhaler and other allergy medicines like Benadryl. Nothing worked to cure the cough.
Kids at school were starting to get annoyed with me coughing all the time. The kids began to notice my jugular neck beating. I was also starting to complain about how I felt in gym class. I would see black spots and feel like passing out even taking our dog, Lucy, for a walk in the neighborhood.
It was mid February of 2012, and I played my first volleyball game with uniform shorts on. After the game, my legs were purple and mottling. My parents noticed how bad it was. We decided to make an appointment with Dr. Dillon.
Dr. Dillon listened to the whole story about the cough and about how I felt when exercising. During his evaluation, Dr. Dillon had trouble getting a pulse on my foot. He did an echocardiogram and a Doppler echo. Dr. Dillon looked very serious, really upset and concerned. He suggested that I needed to have surgery on my tricuspid valve. There was too much regurgitation (back flow) of the blood. It looked like fireworks going off on the echo. I was not circulating the blood around my body very well. Dr. Dillon thought we should go to the best Ebstein’s Anomaly surgeon named Dr. Bove in Ann Arbor, Michigan, at the C.S. Mott Children’s Hospital.
Dr. Bove was on vacation, so Dr. Dillon monitored me every other day and took me off all of physical activity. Exactly a week after from the first echocardiogram, something told Dr. Dillon to do another echocardiogram and Doppler echo. He did and a blood clot was found in the atrium of my heart. He made it my parent’s decision about where to take me. He thought if we went to Michigan, I would be there when Dr. Bove returned from vacation. And he believed that Dr. Bove was my best shot at survival. We drove immediately to Michigan in pouring down rain with a large storm known to develop tornadoes following us. In fact, a tornado from that storm touched down in Cincinnati. This was March 2, my parent’s wedding anniversary and one that they will never forget.
The doctors at Mott Children’s Hospital were able to dissolve the blood clot with Heparin. Then they took my parents to a room and told them that my tricuspid valve was bad and so was my left ventricle. The left ventricle was not squeezing properly. Motts said that the only option left was a heart transplant. They left it up to my parents to either stay in Michigan or go to CCHMC, which would be closer to our family and us. The Michigan hospital arranged to transport me by a Mobile Intensive Care Unit (MICU) to CCHMC.
All the proper transplant tests were accomplished and I was officially put on the transplant list as status 1A. Status 1A is the highest you can be on the transplant list. We were told I might be on the waiting list for a heart for up to 3 months. I would have to remain in the hospital because of the medicine I was on to help the left ventricle squeeze which required an IV.
When word of my condition got out, we started getting Facebook prayers from all over the world and I received lots of packages and cards at the hospital. It was like Christmas for me! I got a lot of balloons, cards from different schools, snacks, games, puzzles, lotion, socks, and sketch pads that I could use to draw my favorite Sonic characters.
I was in 5th grade at the time and my school was so supportive and really helped us during this challenging time. My teacher, Mrs. Jecker, made me feel like I was right there in class, sending my homework down on Fridays and making a special visit on a Sunday to bring more! Mrs. Jecker allowed the class to Skype with me too. I was able to have a CCHMC teacher, so I could keep up in school. That was the best part. An hour a day with a teacher counts as a whole day of school!
We only had to wait 9 days for a heart. My surgery was on a Sunday, March 18, a second birthday that we now get to celebrate. After I woke up from my heart transplant, my first words were, “Thank you, God!” as I sat up for the first time after the successful surgery. The nurses printed out a poster with Sonic and my new birthday, March 18, 2012, that hung on my hospital door. The nurses also showed us chest X-rays of my old heart and my new heart. The old heart was so enlarged that it was pushing my trachea back and causing me to cough. They believe that I never did have asthma. I will get tested for asthma when I get off of steroids. Currently, the cough is gone.
We were told that while I was in surgery, it was announced to the runners at the Cincinnati Heart Mini Marathon that a child at CCHMC was in surgery having a heart transplant. Many of the runners that were interviewed said that they ran for me and that I gave them a boost and a purpose. A week later, a local TV news station interviewed me, because so many people were asking how the child was recovering. Not only did I have a new heart making me feel great, I was a celebrity!
I stayed in the hospital until my real birthday on March 28. We had to stay close to the hospital for a few months. We celebrated my birthday in a hotel where we lived until a room was open at the Ronald McDonald House. We stayed at the Ronald McDonald House for about 2 months.
I had an awesome care at CCHMC. I have had numerous clinic visits, so many biopsies that I have lost count, and almost weekly blood draws to regulate my anti-rejection drugs. A biopsy is done with a catheter through a vein in my groin or neck. The doctors take tiny pieces of the heart and a lab examines the pieces to see if there is any damage due to rejection. In the beginning, I had a weekly biopsy, and then it went to every two weeks, and then monthly. Eventually, I will have two biopsies a year.
I can do so much more now with my brand new heart. There is no more worrying about SVT and emergency room visits. No more annoying cough. No more beating jugular in the neck. No more seeing black spots and feeling like passing out during exercising. No more purple legs. Everyday is a blessing!
Dr. Dillon coined the term, “Payton’s Anomaly”, because he and the other doctors do not understand how I was able to perfuse myself with such a bad heart. This was another miracle! We are so blessed and thankful for all of the prayers and support from friends and family, for Dr. Dillon, for the doctors, nurses, and support staff at both C.S. Mott’s Children’s Hospital and CCHMC, for the transplant team, for my school and my teachers, and especially for the donor and his family.