EP Perspectives

My story begins on February 3, 2003. It was just another ordinary Monday. I finished with my classes for the day at Hoover High School in Birmingham, Alabama and headed to basketball practice. My basketball team was preparing to play for the area tournament in the state playoffs. I loved everything about sports — it had been a part of my life since I was five years old. I was now fifteen and playing on the varsity basketball team. I considered myself a good athlete and in very good shape. However, on this day, my life changed forever. Around four o’clock in the afternoon, after taking a routine water break, my teammates later told me it looked like I had bent down to tie my shoe, and then slumped back against the wall. After calling my name and getting no response, they knew something was terribly wrong. I was there on the court lifeless, limp, blue in the face, and unresponsive. I had gone into cardiac arrest. One of my teammates rushed upstairs to get our athletic trainer, Coach Sheppard, while my coach ran to get the external defibrillator, which was located only a few yards from where I had fallen. The trainer arrived within a few seconds after my collapse and began performing CPR, along with another coach. After three minutes of CPR and three shocks from the external defibrillator, my heart went back into a normal rhythm. I was taken to Children’s Hospital and remained in and out of consciousness for three days. 

I remember very few details about my stay at the hospital. I was in the ICU for five days, and during that time, I only remember two things. The first was waking up and seeing my dad sitting next to me. I still remember what he was wearing, and I remember asking him if I was going to die (I didn’t yet realize that I already had). The only other thing I remember was Kyle, the handsome nurse who took care of me. He had dark, wavy hair and glasses. It is funny how the brain works. Of all that went on, the visitors that came to see me and the hours my family spent with me, I remember those two things. After numerous tests, the doctors diagnosed me with a genetic heart disorder known as Long QT Syndrome (LQTS), and implanted an ICD. After days in the hospital, I just wanted to go home and get my life back to normal as best I could. Soon, I realized that my life would not be normal — no more basketball, softball, or competitive sports of any kind. However, this story is not about what I could no longer do, it is about how my story became my passion. 

In the toughest moments, I recite my favorite bible verse, Romans 8:28: “God works for the good of those who love Him, who have been called according to His purpose”. I have always been interested in the medical field. Growing up playing sports, I always thought I would become an athletic trainer, physical therapist, or even a physician assistant for an orthopedic doctor. Medicine, specifically sports medicine, fascinated me. During my last two years of high school, I was able to work with Coach Sheppard, who was now a trainer at Birmingham-Southern College in Birmingham, Alabama. This experience had convinced me that I was going to follow in his footsteps or go into physical therapy. After graduation from high school, I enrolled at the University of Alabama taking classes for a degree in Exercise Science. I hoped to apply to the Physical Therapy program at UAB. 

However, early on an August morning during my sophomore year in college, God had a different plan in mind. I was at my house off campus in Tuscaloosa and as soon as I got out of bed that morning, my ICD shocked me. This was the third time I had been shocked in the two years since my ICD was implanted. As I sat on the edge of the bed calling for my sister (who was sound asleep in the room next door), I was shocked again. I did not want to move at all. I felt scared and paralyzed. How could I keep this device from shocking me again? Since it was a Saturday, my pediatric cardiologist, Dr. Yung Lau, told us to meet him at his office. He would be waiting for us with a representative from St. Jude Medical who could interrogate my device. That is where I met Craig Bernard. He had never checked my device because Barbara, Dr. Lau’s nurse, routinely performed this every few months. However, since it was a weekend, he was on call. Dr. Lau and Craig determined that the lead in my heart had fractured and caused the device to deliver two unnecessary shocks. The only solution was to replace my defibrillator lead. Because of my age, they did not want to leave the lead in, as it could potentially cause further issues down the road. A few days later, Dr. Neal Kay extracted my fractured lead and a new lead was implanted. Thankfully, my surgery went as well as could be expected, and although I was sore for the next week or two, I had brand-new leads, and was happy that I could get back to my ‘normal’ life. 

During that semester of classes at the University of Alabama, I began to question my chosen career path — I felt I was being called to a different purpose in life. Not coincidentally, Craig showed back up in my life for the second time in a matter of weeks. He offered to allow me to “shadow” him, and I took him up on that offer not too long after. I was sold — no turning back — and fell in love with everything about his job as a sales representative for St. Jude Medical. I loved the patient interaction that came with the job. I felt that I had a story to share, and that I could help other device patients understand how to cope with living with a device as well as how to move forward with a positive outlook in life. It seemed challenging, but I was up for the challenge. I was also intrigued by the complicated workings of the heart and wanted to learn as much as possible. Over the next few years, as I was attending college, I continued to “shadow” Craig. I knew that it was no coincidence that this door was opening for me — this was my calling. Craig later put me in touch with Kristen Barnsby, a clinical specialist who worked for St. Jude Medical in Huntsville, Alabama. I remember calling her and asking for advice. What classes should I take? What avenues should I take to get into the business, etc.? We corresponded through email. Doors continued to open and four years later, St. Jude Medical offered me a job in Huntsville, and Kristen became my mentor! I knew so confidently that this was what I was meant to do. Everything that I had been through had only made me appreciate my job even more. Philippians 1:6 says that “ I am certain of this, that he who began a good work in you, will continue his work until it is finally finished on the day when Jesus Christ returns”. This verse was my foundation on how I wanted to approach each day of work. I knew I had something to offer to the patients that other people could not. My experiences help me relate to, teach, and offer guidance to device patients, and if I can be a positive, Godly example to them, then I am living out my purpose. 

Many years have now passed since I went into cardiac arrest that day in 2003. After doctors first diagnosed me with LQTS, my twin sister also later tested positive for LQTS. We were told early on in the days after my cardiac arrest that LQTS is what they believed may have caused the event. However, for some reason I was never at ease with this diagnosis — all of the puzzle pieces didn’t fit together for me. Post arrest, my QT interval was prolonged. Symptoms correlated, but other heart disorders connected to my symptoms as well. After being in the medical field for several years, I knew genetic testing had become much more advanced, so I took it upon myself to have another genetic test. They tested me for multiple cardiac panel anomalies, and it took about six months for the results to come back. Was I ready for the answer? The day before my 26th birthday, my EP in Huntsville, Dr. Paul Tabereaux, approached me with the test results: “Ashley, you have Arrhythmogenic Right Ventricular Cardiomyopathy, or ARVD/C for short.” Being in the medical field and treating some device patients with ARVD/C, I knew a few things about the disorder. However, when the words came out of my doctor’s mouth, I immediately broke down into tears. I didn’t really know why I was crying. Was it that I finally had an answer and it was a sense of relief, or was I scared of having to live with this disease and move forward? 

Like most patients who learn about a new diagnosis, my immediate response was to get on the internet and find out every little detail about this cardiovascular disease. I googled everything I could about ARVD/C, joining support groups and online chat rooms, searching patient stories, and reading the Johns Hopkins website on ARVC. While the internet has some great information, and much of it was beneficial research, it was difficult not to focus on all the negatives. The phrases “progressive”, “no cure”, “control” the arrhythmias, only “light exercise”, and “multiple ablations” all caught my attention and stuck out in my mind. The fear set in. This was a progressive disease that was completely out of my control. There was nothing I could do besides stop exercising to slow the progression. I had no clue how I was going to live with this disease for the rest of my life. It was almost like I was starting over again.

According to Johns Hopkins, ARVD/C is a leading cause of sudden death among young athletes; however, it can affect people of all ages and all activity levels.1 The right ventricle is the chamber of the heart that is affected, and dysplasia means there is an abnormality of the structure. ARVD/C is a specific type of cardiomyopathy, and as a result, the condition is also referred to as ARVC for short. Simply put, ARVD/C is a genetic, progressive heart condition in which the muscle of the right ventricle is replaced by fat and fibrosis, which causes abnormal heart rhythms. ARVD/C is estimated to affect one in 5,000 people. Further genetic testing revealed that my dad has the same condition. I still follow up with my EP in Huntsville (Dr. Tabereaux) as well as with Dr. Hugh Calkins at Johns Hopkins. They have great doctors with a wealth of knowledge and ongoing research for ARVC patients. I try to enroll in as many research studies as I can, in hopes that I can help improve the future outcomes of people with ARVC. 

Thinking ahead, I still worry about my device and about questions on living life with a device — will I get shocked, how much can I work out, will my lead fracture again? I feel like I have been through a lot in my short 27 years of life: cardiac arrest, multiple shocks (some appropriate, some inappropriate), lead fractures, lead extractions, generator changes, you name it. Each challenge presented a different obstacle to overcome. For me, it hasn’t been an easy road. 

I realize how blessed and fortunate I am, but sometimes it still seems surreal what happened to me on that day — like it is someone else’s story. Like most patients with ICDs, I sometimes struggle with anxiety, panic attacks, and bouts with depression. I have to remind myself that my device is there to potentially save my life, and if I can help other patients understand what living with a device is like, all that I have gone through is (well, maybe one day will be) worth it! I even created a local support group because I know the effect an implanted device can have on an individual’s life. My hope is that patients can understand that they are not alone in this struggle. Having faith, as well as getting support from family, friends, and the ICD patient community, can help us overcome whatever comes our way. 

Now that I work in the medical device industry, I see myself in the patients I treat and care for. The challenges they go through are the same that I continue to deal with. Although this disease and the treatment for the disease (the ICD) will never be over, I will not let it hinder my life. I believe my experience has made me better at my job. I am working at a job I love, with people that I adore. I want to be an example to others and show patients that the one thing that changed my world in 2003 is now the one thing I am most passionate about. My passion is to be a positive example that life with heart disease can exist. It is not the end of the road for us — it can be a new beginning. We write our own stories. 

Disclosure: The author discloses she is an employee of St. Jude Medical.  

Reference

1. Arrhythmogenic Right Ventricular Dysplasia (Arrhythmogenic Right Ventricular Cardiomyopathy). Johns Hopkins. Available online at https://www.hopkinsmedicine.org/heart_vascular_institute/clinical_services/centers_excellence/arvd/index.html. Accessed September 17, 2014.