“The prognosis is poor. After diagnosis, the mean survival time of untreated patients is 2 to 3 years.”
I was 34, sitting in a bed in the Lenox Hill Hospital emergency room reading this as I was doing what they tell you never to do, google your own illness. Earlier in the day, my cardiologist, with the most concerned look I’ve ever seen from a doctor in my life, told me I needed to get myself to the ER now—and I’d probably need to be in the hospital for a few days.
True to form, I asked him if I could just spend an hour to record my podcast episode first before I went. It was like three weeks before Nationals after all. So I talked to Michael Weiss about 2017 Nationals, went home and packed for a weekend in the hospital.
““The prognosis is poor. After diagnosis, the mean survival time of untreated patients is 2 to 3 years.””
Four days, CT scans, MRIs, echocardiograms, and something called a right heart cath where they ran a catheter through a vein and into my heart and lungs to measure stuff, they confirmed that I have pulmonary arterial hypertension (PAH), the thing I was googling while I was in the ER. PAH makes the pulmonary artery, which delivers blood from your heart to your lungs, constrict. And as it gets worse and the artery has to work harder and harder to pump blood, the walls of the artery harden, and things start failing. Mine is the idiopathic kind, which means they have no idea where it came from.
It’s a progressive disease. And it’s incurable.
Figuring it out
Over the next few months, as my doctors and I learned more about this ticking time bomb in hiding, a lot more of my own life made so much more sense. At the rink, I was always the one who didn’t have the stamina to even get through a short program, the one who stood by the boards drinking water and catching my breath between jumps in practice. I thought I was just not in shape like the other kids. At work, I would have bouts of heart palpitations during stressful late nights, but they would go away after the stress was gone. I thought everyone got the same thing when they were stressed. At home, I had periods of time when I was just fatigued, taking naps every weekend. I thought it was just because I worked 80-hour weeks and I was tired.
But it was different this time. I was on a particularly intense project that was giving me constant heart palpitations. One of my colleagues suggested that I tried wearing her FitBit for a day and see what happened—and I would just be sitting at a desk working and my heart rate would shoot up to 140 for no reason. It actually got to be concerning enough that my team suggested I go get it checked out. And truth be told, if there hadn’t been a hospital within a 5-minute walk of my hotel, I may not have gone.
And even then, it wasn’t that easy. The ER doc there told me it was probably stress and I should just rest. Then I went home, and my regular doctor told me it was probably stress, but gave me a referral to a cardiologist just in case. I kept having heart palpitations spending two weeks doing nothing, so I finally got myself to the cardiologist. If it hadn’t been for Dr. Roth, who truly could not have looked more concerned while he was reading the results of my echocardiogram, I don’t know if they would have discovered it as early as they did.
Work stress saved my life?
The doctors didn’t know what to do with me. I was not their typical PAH patient—I was seemingly healthy, they had caught it early enough that the disease had not made anything else worse, and I was certainly more active than most PAH patients they saw. They told me my pulmonary artery was so constricted that weekend that I was in the ER that there was a possibility it could’ve ruptured. They put me on a lot of medication to figure out what might work. They told me to keep being active but to not push myself too hard. They told me to keep a pulse oximeter on me to keep tabs on my blood oxygen level.
But what they couldn’t tell me was how my life was going to unfold. They couldn’t tell me if this was the beginning of a rapid decline in health. They couldn’t tell me what other patients like me went through—because they didn’t know any. They couldn’t tell me if I should continue working or if I should exercise more or if I was going to live past 40.
I guess we were going to learn about this together.
Meeting Martine Rothblatt at a work conference
After going on a few different medications, the doctors told me that I should be healthy enough to tolerate high doses of a relatively new form of a drug that was often delivered continuously through an IV. Martine Rothblatt, a lawyer turned inventor turned co-founder of Sirius Satellite Radio turned pharmaceutical entrepreneur, wondered why that drug couldn’t be taken orally. As it turned out, her daughter had been diagnosed with pulmonary hypertension, and she was on a mission to save her life. So she developed her (backwards) namesake drug, Orenitram, which was exactly what she was hoping to create—an oral version of trepostinil that could make PH and PAH more manageable.
I say now that a lot of things saved my life—that ridiculously stressful project at McKinsey, my love for figure skating that helped me stay active through my 20s, being in a hospital bed contemplating mortality in my mid-30s. But Martine Rothblatt and Orenitram gave me a whole new trajectory.
What really is possible?
It’s been over eight years since that weekend in the hospital. I am on a cocktail of drugs three times daily, with constant and unpredictable sets of side effects that are just my new normal. But my heart has never been in better shape; my pulmonary artery is over 50% less constricted than it was when Dr. Roth sent me to the ER. And outside of that, my outlook on life changed from that weekend in the hospital onward. Work is still important but the bad stress isn’t there anymore. I poured more fuel onto my love for figure skating—and I would’ve never applied to cover the 2018 Olympics; I thought it was impossible.
But as it turned out, it wasn’t impossible after all. And as it turned out, I am now in my 40s.
All of which, if you’ve read this long, takes me to why there’s a picture of me after a race here. Since I was a kid, I found ways to cover up my lack of cardio fitness. I would run programs but never even went close to full out so I could at least slog through them. I would tell people I had asthma (I kind of believed it). I would take just a little longer getting from one side to the other when I played tennis. I would tell my friends my skating coaches told me to not run because it was bad for my knees.
But one of the things that I hated most as a kid was those yearly fitness tests where they made you run a mile. I never ran one the whole way through, and it always made me feel lesser of an athlete. Over the past eight years, as my system has gotten more tolerating of cardio activity, I’ve leaned into getting better, because what is more satisfying in midlife than feeling like you could beat your teenage self in anything?
Trying not to embarrass myself
I ran my first real mile in 2018, and I thought that was as far as I could go. But I could improve my cardio now, unlike before. So I started experimenting with more jogging and running, though I still couldn’t convince myself that running more than 15 minutes at a time was possible. My doctors kept having me watch my blood oxygen levels because that’s where the PAH kicks in.
A couple of years ago, my lung function tests showed significant improvement after just being steady for a while (no one really knows why it happened). And all of a sudden, my blood oxygen levels no longer dropped to alarming levels during cardio. It felt like permission for me to push further. And over the past year, I’ve amped up my fitness routine to a different level (thanks, Barry’s!), and my new challenge was to actually run a race—a 5k even.
I wanted to show that kid who was embarrassed about not being able to run even half a mile that it wasn’t his fault. It wasn’t because he was lazy. It wasn’t because he didn’t try hard. He just didn’t know there was an invisible obstacle in front of him.
And I didn’t even want to write this until I finished the race. I run enough to know that I could get through it. But I also didn’t know how I would do in a competition setting (just ask the guy who didn’t win his episode of Jeopardy!). I had a goal of running it under a 10-minute mile pace, with a push goal of running it at a 9:30 pace. I got the first but didn’t get the second—30 minutes 28 seconds for my first 5k. Not awful but I have more in me than that.
But you know what? I actually did it. This was truly impossible in my teens, my 20s, and most of my 30s. And this 42-going-on-43-year old with a progressive disease and a midlife crisis actually did it.
So next up, making it through a full program?
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I am convinced that there are many more people out there with pulmonary hypertension than there are diagnosed. It took me multiple doctors and non-routine testing for it to get discovered. And if more people like me know what they have earlier in life instead of when it’s too late to manage it, it could save their lives. www.phassociation.org