Live Your Life With CHD

When I initially signed up to write an essay about my mother’s and my experiences with congenital heart defects, my mom was getting ready to move in with my family and me. A big step for anyone that knew her. Fiercely independent to a fault is a good way to describe both of us. She absolutely loathed feeling as though she was a burden to anyone. She never learned to drive, even though she tried many times. She could just never get past the anxiety. Even so, she took public transport independently until her mid 60’s—and then she had a cardiac event that ended up making walking difficult. Since she was unsteady, I started taking her to her appointments, which she hated, not wanting to be a burden. So, our cardiologist solved the problem by simply coordinating our appointments and echoes. They were the same day, one right after the other.

Left-Right: Gwenyth’s grandmother Mary Ellen,  her mom, Sharon, and Gwenyth

When my Mom moved in, in August of 2018, neither of us expected she would be gone by the beginning of January. To get to that January, we need to first start when she was born. 

My mom was the oldest of 8 children, born in a small town in Wisconsin in 1949. When she was born, the technology simply wasn’t there to identify her heart defect. I remember Grandma telling me once that the doctor who delivered my mother never said anything about her heart. It wasn’t until a few months later that anyone even mentioned there was something wrong. They didn’t know what, and so they did what was the norm for decades to come — they took a wait-and-see approach. One of the theories Mother remembered the cardiologists proposing was that maybe she had been a twin, and there was a mirror effect causing the weird heart sounds. Maybe she had Tetralogy of Fallot. In any case, she spent summers going on the train to Milwaukee to see the cardiologist there and undergoing catheterizations, which were considered major procedures back then, not relatively standard as they are now. Finally, around 1975 technology had advanced just enough that doctors could finally diagnose what was wrong with her heart. Congenitally Corrected Transposition of the Great Arteries with associated ASD, VSD, Pulmonary Stenosis, and Aortic Stenosis. “CC-TGA with all the bells and whistles” was how she described it.

In June of 1976 my mom had her one and only open-heart surgery (OHS). I don’t know if they just did things to last back then, or if she was just that stubborn to not need another repair. In 1976 heart-lung bypass machines were still relatively new, especially in small town hospitals, so it was a big deal to have this surgery. Now, for her defect, people can opt for the “double switch” operation. That simply didn’t exist back then. In the end they ended up patching a dime-sized ASD. They left everything else alone, deciding that they worked for her heart. As was the norm back then, she was declared fixed and sent out to live her life. Seeing the cardiologist only once a year or so. My mom lived her life, which in turn, showed me how to live mine.

Gwenyth as a baby with her mom, Sharon 

I was born 15 months after my mom’s OHS. I was born at 31 weeks gestation, and back then ultrasounds during pregnancy did not happen too often, and fetal echoes were unheard of. Her doctors had planned on a scheduled Cesarean section, but I had other plans. The doctor had to use forceps because I was in distress and I was small. I was born just 3 pounds and 3 ounces, with apparent heart issues. Two things happened when I was born; things that I heard repeated over and over enough as a kid that they are ingrained. One, the doctors informed my grandparents that while my mom would be ok, to not hold out much hope for me. They really did not expect me to make it through the night. Second, the team had my mom’s cardiologist paged out of a keynote address that he was giving at a cardiology conference to decide if they needed to helicopter me down to Children’s Hospital in Milwaukee. Apparently, once he heard whose baby it was, he said, “Oh, she’ll be fine like her mom.” That independent, stubborn streak is strong. They ended up not sending me to Milwaukee. I spent two months in the NICU before coming home at just over 5 pounds.

As with my mom, they opted to take a wait-and-see approach for me. While my mom’s defect was more structural in nature, mine is primarily electrical in nature. I got lucky. My primary heart defect is Congenital Complete Heart Block, or 3rd degree AV Block. In my 30’s, my cardiologist determined my aortic valve to be a bicuspid valve. Mine has 3 leaflets, just one is undersized and fused to another. Now is not uncommon for babies with my defect to have a pacemaker implanted. It’s the standard of care. However, in 1977, it just was not possible to implant a pacemaker into a three-pound baby. After all, the first pacemaker with a lithium battery had only been developed 3 years earlier in 1974. It was the size of a pack of cigarettes. So, we waited.

When I say I got lucky, I mean very lucky. I was lucky that my heart had an escape rhythm. The way complete heart block works is this: the upper and lower chambers of the heart can’t talk to each other. There is no communication between the two. Your body normally requires some sort of communication between the two, or else cardiac arrest occurs.  The body has a “back-up pathway” in the ventricles, but mine meanders on its own way. I heard it described at a conference once as the atria beating along as it should, and the ventricle follows along like a cymbal player, “oh, yeah, I should do something.” My back-up ventricle pacemaker chugged along while we waited for devices to get smaller, and more stable, and me to get bigger. I stayed very petite for a long time, probably due to having a heart rate in the low 40’s and 30’s. I was excited in 8th grade when I broke 80 pounds, and in high school when I finally broke 100.

While we waited, my mom and I just lived. I learned early to listen to my body as far as my limits, just as my mom did. I learned that having a heart condition was secondary to me as a person. When I was eight years old we moved to Milwaukee, which until that point had always just been a place my mom visited as a kid for heart check-ups. 

In the summer we would walk to the lakefront for a variety of ethnic festivals. Irish Fest, German Fest, African World Festival, all of them. Irish Fest being the one we visited every day it was open. The walk to the festival grounds was easily 2 miles, and we probably walked 5 to 10 miles back and forth on the festival grounds every day. We walked everywhere. We walked across a long bridge to the library. We walked downtown to the museums. We took the bus to the baseball stadium, but even then it was a long walk from the bus into the stadium. We walked, a lot. 

From age 12 on, my cardiologist started seriously checking each year to see if this was “the year.” I remained stable until my senior year of high school. Although, in hindsight, I do not know how I managed. I definitely did not have a normal high school experience. I was in bed by eight PM every night; and could easily sleep 16 hours. But that was my normal. As long as I was ok with it, and stable, we put off the inevitable. 

It was probably all the walking that kept us both in good cardiac shape for a long time. Through some luck, and lots of walking, I managed to wait until pacemakers had come down a lot in size; and had better lithium batteries. I was 18, and just about to head off to college when I got my first device. College would have been impossible without getting my device, and the longer we waited at this stage, the more likely I was to have a Stokes-Adam attack or other complications. While my stubbornness had gotten me that far, my pacemaker would get me even farther. The biggest change for me initially was that a fog had been lifted. I was awake. I could stay awake past eight PM. That was an adjustment. I had no problem walking for miles, but I never could run. I mean, I couldn’t run far. But I could at least attempt it. 

Sharon and Gwenyth at a birthday celebration 

At some point, a cardiologist told my mom that she no longer needed to see him. She was not even supposed to be alive, and so whatever she was doing, she should just keep doing it because it was working. So she kept visiting the festivals, hosting huge feasts for friends, and feeding roommates and friends I brought home for Thanksgiving. I don’t think she saw a cardiologist again until I found out about a new specialty for adults with congenital heart defects in my mid-twenties. I had to drag her to that first appointment. I had found the sub-specialty by a combination of pure chance and frustration. I needed a cardiologist as an adult as my pediatric cardiologist had passed away. After being told similar things my mom had been told by regular cardiologists I had seen to try and establish care, I got frustrated and called the Children’s Hospital. I was told that they have a brand- new program just for adults with congenital issues. So I made us both appointments.  

My mom was pretty well sold on the idea of never seeing another cardiologist by that point, but she went. When he wanted to do all sorts of things, and finally go in and fix the valves and VSD that were never fixed during her original repair, she told him “Absolutely not”. My mom got her way. She would tolerate putting in a pacemaker/ ICD combo device but that was it. Somewhere in all those years of not seeing a cardiologist she had developed complete heart block (common for CC-TGA patients), and A-Fib. She grudgingly started medications, and would tell me that they did help, but she didn’t want to admit it to our new cardiologist. She didn’t want him to be right.

I have no doubt that finding a doctor who understood us and our issues extended my moms’ life. With medications, and a pacemaker and follow- up, she kept on doing the things she loved. Like the festivals, reading and re-reading Tolkien, and making fancy menus for feasts with friends. My mom and I were always close, and I often helped with the food preparation for the fancy menus, and we always went to the festivals together. My mom taught me through showing me that my heart condition was secondary, it is just a part of you but does not define you.  Sort of like Finding Dory, you just keep swimming. That’s what she and I did. It is what I still try to do.

Just before Thanksgiving 2018, my mom suddenly got really puffy and full of fluid. Even though according to her medical records it was surprising that she was doing as well as she was, she had never gotten puffy and swollen. While she was in hospital for this, she made a rounding resident chuckle because she told her she had to get home. Why? She had to make the Thanksgiving pies, of course. From scratch. I don’t think I ever ate boxed food until I was on my own at college. The resident thought she was joking, but she was completely serious. With that stubborn streak, she managed to get enough fluid off to get discharged. She came home and she showed me how to make the pie crust and the fillings for the pies again.

She ended up going back to hospital for another short stay a few days after the holiday, but came home and we filled our days making cookies and bread for my husband and son. Christmas candy for the Holiday was our routine. We would get up, get my son off to school and then plan what we would make that day for ‘our boys.’ On the day that she passed away in January, that is exactly how our day started. I was going to try and get some things done around the house, but I was tired. That exhaustion that only CHDers understand. She planned to make some chocolate chip cookies for ‘the boys,’ at around two in the afternoon. She chose that time so that they would still be warm when they came home from work and school. That’s what she did, and all seemed to be the status quo that we had become used to.

I was starting dinner, and she was on her third batch of baking cookies. We sat down for our break. It was sort of joke among us- “We have done some activity, now let’s rest a bit”. It was something we had done thousands of times before throughout the years. Part of the “knowing your limits” that my mom taught me - take frequent breaks. And so, we sat down for our break. My mom mentioned that she might want to go to the hospital once everyone got home because something was off. I went to get my phone from the kitchen to check something, by the time I came back she had collapsed on the sofa. I called the paramedics, but I knew. They worked on her for a long time and then took they her to the nearest hospital, not our normal one. I knew before they left that she was gone, but of course I didn’t want to believe it. Up until the very last moments my mom was living her life.

Sharon with her grandson

Sharon with her brothers

I was not expecting this to happen. I knew at age 69.5 my mom was not old, but…decently aged for someone with her defect, especially with all the ‘bells and whistles’ as she called them. She had really been looking forward to turning 70. I think that was her goal, just to make it 70 and then she would be ready to go. Even though it was sudden, it was best for her. Our cardiologist made a comment when I saw him shortly after she passed, my first appointment without her in a long time, that this way was best for her. A long illness in the hospital would not have been what she wanted. He was right, of course. In hindsight, I think it was that same stubbornness that got her home from the hospital and kept her from going back until it was time for her to shuffle off this mortal coil, to use one of her phrases. 

My mom lived her life doing what she wanted until the very end. Backwards and weird heart be damned. I will continue to try and live my life as I was taught: to just keep trying. I think that is really what all patients with congenital heart defects should do. Live their best life, even if it sometimes a pain and hard to push through each day. Value the small things like baking a loaf of bread from scratch, and cookies for grandchildren, children, nieces or nephews, or even just friends. Sitting around feeling sorry for yourself does not do anyone any good. I mean, sometimes it’s okay, but it is not a lifestyle. Take my mother’s advice. Live your life.