Hopkins Journey + ARVC

As you all know, yesterday’s appointment took up a lot of space in my head over the past several weeks. I had trouble sleeping the night before, because I knew that whatever I was going to hear was going to carry a lot of weight in terms of how I see my health, how to tailor my current lifestyle, and what to expect in the future.

I am so glad I went. I didn’t get miraculous “you’re actually totally fine!” news, but I already kind of was ready for that (I really do think it was good that I saw those MRI results).

In summary, here is what I learned:

1- Yes, I do have ARVC. There is no more mystery here, I meet multiple criteria for the diagnosis and in their words, “everything lines up.” (The VTach, the MRI results, some abnormalities on my EKG, and my Holter monitor results). They noted things that no one had pointed out to me previously on my Holter and EKG. It was honestly kind of comforting to be told “you fit neatly into this box” rather than “ehh, we don’t actually know what to do with you”.

2- I have the “best kind” of ARVC. I do not carry any of the known mutations for ARVC, which places me in the “gene elusive” category. This form is more common in athletes, but is thought to be likely also genetic, just polygenic instead of monogenic (otherwise . . . this would be way more common). The good news is that the form I have is less likely to progress if I remove the endurance exercise stimulus, and it is much less likely to impact the kids. (They recommended some screening every 3-5 years which I will definitely have them do, but unless anything is found, no exercise limitations for them and the risk is overall very low that they will have anything similar!).

3- Strength training is okay, as is walking. “Endurance exercise” especially at high intensity is the absolute worst for ARVC and they were thrilled I had stopped running. I talked about my current routine (strength ~4x/week, yoga) and they noted that while avoiding “burners” and HIIT-like training is important, I don’t have to stress too much about following any specific heart rate guidelines and generally strength training is thought to be much better (just does not stress the walls of the heart in the same way). I feel like this is a lifelong prescription I can live with, maintaining health/body com/reasonable level of fitness. They also said “one off” activities are fine – so an occasional hike on vacation, or skiing a week or two every year is still ok!

4- Speaking of heart rate . . . they want me on a beta blocker based on my monitor results. So, it’s likely that my HR won’t be getting that high on medication anyway. I am going to start with a tiny baby dose and see how I do. I’m a little leery of side effects (I do not really like to take medication) but I understand the rationale, and honestly I’d like to feel fewer palpitations as they do kind of stress me out. I’m also going to get a repeat monitor in ~3 months to see if things calm down.

5- I have the right defibrillator. They were thrilled I have the ICD that I have and recommend I have one for life unless there’s some major breakthrough where you can ‘cure’ this condition.

There was more (discussion of other potential future things I may have to deal with like ablation or other meds etc) but that was most of it and really, I left feeling . . . mostly a sense of peace. People have this, there are providers that know how to handle it, and there is a decent chance I could live with this in decent health for a long time. Obviously, there are probably variations in how people do and I can’t be cavalier in following the recommendations, but OVERALL it was positive. And the recommendations are livable. I’m mostly over my sadness about running, and generally am ready to focus on how incredibly lucky I am in all this (having my VT at the right place/right time, having no mutations to pass to the kids, being able to access world-class experts, still being able to strength train, having essentially normal heart function right now).

Oh, and I’m going to go back every ~2 years to see the team there, while doing other interim visits at home. This is a cadence that will allow them to oversee management over the long term while avoiding frequent travel to another state (and out of network fees – pretty sure that visit won’t be covered, but worth every self-pay penny).

Anyway! I wanted to provide this update in part because of how many of you offered advice and support these past couple of months — I truly appreciate you (including the 3 of you I got to meet yesterday– A, V, and K!).