An Interview with Katherine Standefer

Could you talk a little bit about the process of writing this book and how you decided you wanted to write about Long QT Syndrome and your ICD?

I have been a writer my whole life before I passed out in that parking lot at age 24. The part of me that had been born an artist understood immediately that I was living through something wild, to wake up in a parking lot, having mostly ignored my younger sister's condition other than initial concern for her life – once she had her defibrillator, and was medicated, it seemed like, okay, that's taken care of, and I had really avoided going in to get an EKG done.

And so when I woke up after my syncope that day, I woke up into this new understanding of the story I was living. I moved from Boulder where I had my first ICD implanted to Tucson in 2012, in order to do a Master of Fine Arts in creative nonfiction writing, and I knew that that's what I would be working on.

What I didn't know was that it would take on the added layer of researching where the materials inside my device came from. And I didn't know that my own personal story was going to require that I get so into the weeds – what we do know about Long QT Syndrome? And what we don't know about Long QT Syndrome? How does that change what are perceived to be the correct treatments? The longer I lived my own story with Long QT and an ICD, the more I understood just how much conversation and research there actually is unfolding.

Could you talk about your experience receiving treatment for LQTS and the challenges that you experienced along the way?

There's a lot of layers to the types of barriers I encountered. The most complicated part of my care in the beginning was that I passed out in a parking lot well uninsured in an era before the Affordable Care Act. To seek care at all was going to be expensive and out of pocket. And SADS conditions obviously require quite a workup in order to get any kind of answers. And to do that more than once, to get a second opinion, required a lot of financial resources that I was lucky to be able to access through my parents.

Then came the barrier of age, and no one in my life would have thought that at age 24, I should see a pediatric cardiologist, so I saw an adult cardiologist. The adult cardiologist I encountered was certainly much more used to offering care to those closer to the end of their life than the beginning, and did not seem to have a way of translating their language from the reassurances they might give someone at that stage of life to the reassurances or workarounds that someone on my stage of life might need.

My biggest problem, and part of what led to me getting an ICD in those early months, was that I had a bad response to beta blockers. Beta blockers were getting in the way of my living in a really intense way. I've heard Dr. Ackerman use the term beta blocker zombie now, and it was incredibly comforting the first time I heard him say that, because there just didn't seem to be an awareness of the level of distress that went along with just constantly spinning and being lightheaded and not being able to do basic things because of this drug.
 
I think it's impossible to conceive of what Long QT Syndrome is outside the systems in which people are seeking care. I think a good practitioner understands that they're stepping into the story of a life and that the way they advise you, see you or don't see you, changes how you experience yourself and changes the way you experience terror around your condition or the sense that things are moving in the right direction and you're protected.

How did your ICD impact you personally?

In the early parts of this book, and in the early parts of my story, I thought that by getting an ICD, I couldn't die anymore. And one of the things that happens in the course of my story is that I go septic, and I almost die. And then I take the shocks to the heart and I realized that ICD is can also harm the body, not just save a life. And then late in the book, my device vibrates to let me know that its battery is low. In the process of switching the device out, I end up learning that my wire is broken and we decide to remove the wire and in doing that, the wire snaps off. We learn the next year it actually also had been stripped of its insulation. So I ended up with a nest of stripped wire in my right ventricle. And to me, this really came to hammer home the point that we don't ever eradicate the possibility of death.

 All of us live with the threat of death every day. And that's sort of the unpalatable and ultimate human truth. And how we live with that consciously is such a big philosophical question. For me, the ICD became a way that human truth was implanted in my body and then revealed to me, and by being in my body. And it is also an invitation into a particular form of wisdom, maturity, vibrancy: how do we live well, despite the fact of death? A good life is not a flawless life. A good life is one in which we are alive while we're living and not running away from something. And that's what the ICD and then writing a book about the ICD did for me.

 This book also considers the global supply chain of the device. I went to mines in Madagascar, South Africa and Rwanda. Ultimately, the ICD also connected me with groups of people and ecosystems and other parts of the world and this fundamental question of, what does it mean to be in relationship to Earth? It's like my own years long meditation on the ICD and where it came from was ultimately a way of being more connected and rooted on this planet. To consider what the real cost of an ICD is, and to consider what it costs me to have one, and weighing that against its life saving potential, really just brought me into conversation with the whole fabric of things.

 What has been the response to sharing your journey?

It's been extraordinary. You know, I dedicated this book to myself, which felt like a weird thing to do at the time. The dedication reads: “to the girl in the parking lot: here is the book you needed.” And it's because when I was 24, and woke up with gravel in my forehead, assuming I must have the same heart condition as my sister and not knowing what would happen next, uninsured, I didn't have any orientation around what this would look like for my life. And so it has been incredibly gratifying to put the book into the world and hear back from the other people who are like me, who I had never really gotten to meet before. I have friends who have also lived with long, chronic illnesses, but not specifically people with these heart conditions, people with ICDs, people with snapped wires and technology stuck inside them. I have been overwhelmed by the open heartedness of the people who are reading the book, how seen they feel by it, how moved they feel, by the way, that it's traveling quickly from person to person.

Nobody likes to have the know-it-all patient who's writing a book. And I'm not a medical practitioner. I always want to respect that fact. And yet, I really did know what I was talking about, both in terms of some of the research and in terms of what my body had been through. But I've been hearing not just from patients, but from electrophysiologists, who say, no one should practice as a cardiologist without reading this book. And that just lights my heart up because I feel seen in the way that I didn't feel seen through so much of this book. And other people feel seen by it. Like that's the most beautiful ending.

What’s your relationship to the SADS Foundation, and how has it helped you?

I got referred to SADS right after I passed out, when we were trying to figure out what I should do, how to live on beta blockers as a person who was not having a good response to beta blockers, and who earned her living being physically active. I spent a ton of time on the phone with Alice in the summer of 2009. Just talking through what might be possible, what we knew, what we didn't know, trying to get connected with networks of people who might be able to help. I think a lot of my relationship with SADS over the long term is just as the collector of the latest and most relevant information. SADS spent a lot of time helping me. The SADS Foundation really holds room for living with the uncertainty of these conditions and connecting people with others who might make them feel less alone.