Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) & Me

Hello and welcome. My name is Rachel, and I’m delighted to welcome you to my new publication, ARVC & Me.

I’ve started this publication because I have been living with ARVC for some time, and my condition has progressed significantly. It has taken me years to truly understand what I am living with and where my life is heading. Often, this information has been withheld from me by healthcare practitioners, which has only made the experience more difficult and challenging.

A quick little bit about me, I am 40 year old queer woman. I work in the tech sector where I'm trying to build my own tech for good startup. I love cooking, walking, eating out, learning, playing boardgames and being a bit silly. I am diagnosed with ADHD and probably also have Autism.

I am not a doctor or healthcare professional. I am simply a patient. Nothing written here is medical advice. If you live with, or are concerned about, ARVC, you should speak to your doctor.

If you don’t know what ARVC is, it stands for Arrhythmogenic Right Ventricular Cardiomyopathy. It is a heart condition that primarily affects the right ventricle of the heart. There are many different causes, and therefore many different outcomes. People with ARVC will likely experience arrhythmias, some of which can be life-threatening. I’ll talk more about ARVC in a future post.

In my case, the cause is genetic. I have a mutated PKP2 gene, which, as I understand it, means my heart does not produce the proteins in its muscle tissue correctly. Instead, I get damaged cells that don’t function properly. These can lead to arrhythmias such as ventricular tachycardia or even ventricular fibrillation, which is life-threatening.

I learned about my condition too late. By the time I was diagnosed, it had progressed to the point that I experienced a life-threatening event. I was cycling to meet a date one Friday evening, and I woke up a few days later in an intensive care unit (ICU). I am extremely lucky to still be alive.

By the time I was diagnosed, it had progressed to the point that I experienced a life-threatening event.

Over the next couple of weeks, I was diagnosed with ARVC, though not that the cause was genetic (that would come much later), prescribed medication, fitted with a protective device called an ICD (Implantable Cardiac Defibrillator), and sent home.

It had all happened way too fast and I was given very little information at the time. Tt was only through my own research and years of asking one question at a time during hospital appointments that I eventually came to understand what was wrong with me. I went home not truly appreciating why I needed medication, and why I had to have a device implanted under my muscles. I am probably still traumatised by it all over a decade later, and unfortunately this kind of thing keeps happening.

Right now I'm now at an advanced stage with my condition, my arrhythmias keep becoming more difficult to control, requiring hospital stays and more specialised medications. I’ve had a surgery called an ablation which was to help reduce my arrhythmia. It was initially effective, but unfortunately not even for a year. When I agreed to do it we had hoped for anywhere up to five years of relief. Things are under control right now but I am in the unfortunate position of starting to explore a heart transplantation. If my condition gets so bad that we cannot manage it out of hospital then transplantation would be welcome and would improve my life, however, it is not a silver bullet and comes with great consequences. I’ll talk more about that in the future.

I don’t want to die young, though that’s looking ever more likely, so I do a lot of research into how to adapt my lifestyle to slow the progression of my condition. Doctors advise us not to do strenuous exercise because it is known to progress the condition in patients like me, and they're doing research into how diet affects things too. This is the type of thing I want to know about so I can talk to my cardiologist about applying it to my life. And I'll be sharing these experiences, as well as the research here.

Before you run away let me assure you that this isn’t going to be a Deliciously Ella “I manage my chronic health condition with lettuce” blog. There is no cure for ARVC, and no substitute for medical treatment. But how we live our lives can and will have an impact on our future.

I’ve started this publication to raise awareness, both for people with ARVC and those without. I hope you find it useful.

Rachel x