written by Landri
My day-to-day life generally consists of school, friends, playing video games, and participating in activities that interest me. Unlike the average teenager, one of my favorite things about school is learning new things and applying that knowledge in my future. My favorite subjects are probably Social Studies and language. I say language instead of English since taking the time to learn Spanish has resonated with me. I enjoy not being stuck behind a language barrier and having the chance to meet and learn about other people around the world. In June of 2026, I will be going on a school mission trip to Guatemala to help rebuild houses for families in need. Learning and connecting with other people is a big passion of mine.
One very important piece of me that many people don’t even pick up on is the everyday strain that comes with having a rare genetic cardiac condition. At age 1 I was diagnosed with Jervell Lange-Nielsen Syndrome; a rare form of Long QT that occurred from inheriting the KCNE-1 gene from my father and the KCNQ-1 gene from my mother. JLNS is a mutation in the potassium channel which also causes deafness. My parents were told I was deaf at birth and the Long QT diagnosis came a year later. My mom oftentimes explained to me the times before my diagnosis when she pointed out how low my heart rate was compared to other babies in the NICU; her concerns were dismissed and she was told it wasn’t causing any problems. A year later, while 1-year-old me was in recovery following my second Cochlear Implant surgery, a brilliant nurse pulled my parents aside showing concerns about my QT-interval on the heart monitor. The nurse had the anesthesiologist come over to talk to my mom about what she was seeing. The anesthesiologist said that he would feel bad if she lost one second of sleep over this, and that it was nothing serious and likely linked to medications I had during surgery. After the doctor left, the nurse pulled my mom aside and explained to her that even though the doctor didn’t recommend it, she had the right to request a cardiac evaluation on me before I was sent home. My mom said, “She [the nurse] said that if it was my child I would stand my ground and have this looked over.” This turned out to be the right advice when the cardiologist looked over my EKG results coupled with my deafness, they immediately were able to diagnose me with JLNS which was later confirmed through genetics. This was also a lot for my parents to absorb as they realized for the first time that they also had Long QT.
Many might assume that this creates barriers in the activities I participate in. This statement is not true at all, my parents have never held me back from doing what interests me due to my diagnosis. One thing
that most interests me is sports and that is one of the areas that generates the most anxiety for people in similar scenarios. I don’t let the anxieties hold me back and often, I don’t have any. When I do, I try and translate it into adrenaline similar to how you feel if you were to go skydiving. Being able to center your energy and use it to help fuel you is important. I have always lived under my mom’s philosophy which was always explained to me, “You go through so much with doctor appointments, procedures, testing, surgeries, and medications all so you can live your life to the fullest. You deserve to be able to trust your safety net and do everything you want to. That’s the reward. That’s why we do it. So you can live a mostly normal life. It would be a shame to go through all that and not trust it and hold yourself back.”
My safety net includes taking a beta blocker two to three times a day since diagnosis, having a left cardiac sympathetic denervation (LCSD) at age 1, having an implantable cardioverter defibrillator (ICD) at age 3, and then I also have cochlear implants. Since I was diagnosed at such a young age, my parents had to make a lot of these decisions for me. I have had three ICD surgeries one initial placement and two revisions; my first revision was at age 5 and my second was at 14. I have been taking a 60mg dosage of Propranolol twice a day since I was 9 when my dose stabilized. Because I had the denervation and have been taking beta blockers since I was 1, it is hard for me to explain what they’re like since I don’t know any better. I have never had an abnormal rhythm or needed pacing by my ICD. The average person has an ICD because they had an event that caused their medical team to react by implanting it so they can receive therapies when needed. This is why never having had an event and
still having an ICD makes me a little bit different. Mine was implanted due to my QT intervals being in the 600s.
I was inappropriately shocked at age 5 when one of my leads to my ICD broke. I remember being home with my mom and singing Let It Go from Frozen while standing in my living room. I felt a pain in my back and wasn’t sure what it was; my mom wasn’t certain either, but she suspected a shock. What followed was racing to my local cardiac team to have my device shut off. While we were walking through the hospital halls I was inappropriately shocked once more; I then got my first lead revision days later at Mayo. Initially, this caused me to lose some of the trust I once had in my device. I would have phantom shocks while in elementary school and still do from time to time. It would be hard for me to fall asleep at times since I was so anxiety-ridden about something occurring again. I am now taking anxiety medications at night which help combat those issues. I still have some struggles with night terrors, but I have learned ways to cope and come to terms with my past. Even just squeezing your fists when you’re anxious can go a long way or lightly pressing on your chest almost simulating a hug. When I was younger it was hard for me to grasp the fact that these thoughts and nervousness didn’t affect all of my friends or my teachers; I thought it was normal.
My regular appointments consist of yearly visits to Mayo Clinic in Minnesota where I get a chest x-ray, EKG, echocardiogram, blood work, device check, and an occasional stress test. In addition to the cardiac visits, I am seen annually to get my cochlear implant programmed. I’m grateful that Mayo is only a three-hour drive from where I live so it doesn’t interfere with my routine. We also have fun traditions that we have adopted with my appointments. For example, we go to Hollandberry which is a breakfast diner that serves authentic Pannekoekens. They are Dutch pancakes and are more thinner and eggier than a regular pancake. We also often take trips to their local mall and walk around there. Making the yearly trip a fun almost vacation is something I am glad we adopted.
I think that everything that I have gone through in the past has only made me more excited for what the future holds. I want to put my insider knowledge to good use once I am out of high school and help kids who are going through similar situations as I am. Being a device nurse is a career path I have been highly considering for the past four years now. I also hope that all of the active studies with ICD advancements, gene therapy, and trying to better understand everything that comes with Jervell and Lange-Nielsen Syndrome help supplement better treatment for everyone in the future. Until then, I hope that sharing my story can let people resonate and relate my life to theirs and help them create a more positive perspective on their situation.