Spring 2013. I was in my final semester of graduate school at the time. After reaching a 100-pound weight loss milestone just a couple years prior, I had adopted a fit lifestyle that included running 5 miles daily. One night in March, I noticed I was fairly winded very early in my run so I lowered the treadmill speed to a walking pace to try to catch my breath. Only I wasn’t catching my breath as I had expected and I suddenly felt myself getting lightheaded and a feeling that I was going to “shut off”. And then I did shut off.
When I came to, I was lying at the foot of the treadmill and the belt still running. I got a friction burn on my chest due to the treadmill belt pulling my shirt. A woman who was using a nearby elliptical machine rushed over to turn off the treadmill and asked if I was okay. I was in shock that I had apparently passed out and couldn’t answer her right away. I felt okay enough to get up so I walked to the entrance of the gym and sat in a char. As I was sitting, I began to feel waves of light-headedness and shortness of breath and palpitations in my chest. And then I got in my car and drove home. A few months later and after several more episodes of fainting and nearly fainting, I would be diagnosed with CPVT.
Catecholaminergic Polymorphic Ventricular Tachycardia (CPVT) is a rare heart condition that affects 1 in 10,000 people. CPVT causes irregular heart rhythms (arrhythmias) during physical activity or emotional stress. In simpler terms, imagine your heart as a drummer in a band that usually keeps a steady beat. But sometimes, when you're exercising or feeling very stressed or excited, this drummer starts to play an erratic rhythm that doesn't match the song. This is what happens in CPVT: the heart beats in an unusual and rapid rhythm that can lead to dizziness, fainting (syncope), and sudden death.
In essence, CPVT is a condition where the heart's electrical system, which usually controls your heart rate, doesn't respond well to the natural stress signals, causing the heart to beat very fast and in an uncoordinated way, especially during times when your body is under stress or during physical activity.
CPVT is caused by various genetic mutations. Mutations of the RYR2 and CASQ2 genes account for 1/3 of CPVT cases. Remaining cases are believed to be due to mutations that have not yet been identified.
There are good days and bad days when living with CPVT. For me, a good day is one where I do not experience any symptoms and bad days are where I experience symptoms and/or a shock. Sometimes bad days occur because I may have missed multiple doses of my heart medications. And sometimes cardiac events break through despite sticking to the medication. There are days where an arrhythmia may start out of the blue with no apparent stress trigger. Read the ICD shocks and Electrical Storms sections of this post for more examples of bad days.
In the first few years of my diagnosis, I would have a lots of short cardiac events, non-sustained ventricular tachycardia or NSVT, at all times of the day (before/during/after work). While my device didn’t need to shock me for many of these, I would still feel the symptoms that come with the arrhythmia - palpitations, shortness of breath and lightheadedness. For me experiencing the symptoms is worse than the shock because I have to wonder about whether I’m okay and safe in those moments.