The Heart of the Matter

So, things have gotten a bit more complicated health-wise. For more than a decade, maybe 15 years, I’ve occasionally had an espisode when I’ve pushed myself to exert physically—running through Penn Station in New York to catch a train, hustling through a huge international airport to make a flight connection—and have gotten weirdly out of breath. Not the normal huffing and puffing from running, but actually finding it hard to breathe. And for all these years, the working hypothesis has been that I have exercise/stress-induced pulmonary hypertension (PH).

PH is a known late stage complication of scleroderma, and my cardiologist has monitored me for a long time via regular echocardiograms, which have shown normal pulmonary pressures, for the most part. I’ve also had many, many pulmonary function tests over the years to check my lung capacity and diffusion rate. But over the past year, in particular, these episodes have happened more frequently, with the added bonus of feeling like I’m going to pass out. Fortunately, that has never happened. But it’s become quite worrisome. I’ve also had a heart arrhythmia for many years that has gotten more pronounced.

So, I’ve had a series of heart diagnostics, including a Holter monitor study, a heart MRI, an echocardiogram stress test, and a couple of weeks ago, the gold standard for PH, a right heart catheter stress test. This was not fun. It involves having a catheter inserted into the vein on the right side of your neck, which is then threaded into your heart and the pulmonary artery to the lungs. Your mouth and nose are covered with a tight mask and breathing tube, which collects data on carbon dioxide exhalation, and you have to pedal a recumbent bicycle until you can’t anymore. I lasted about four minutes before my breathing became difficult.

After three conversations with three of my specialists (PH pulmonologist, rheumatologist, cardiologist), the diagnosis is clear. I have Type 2 exercise/stress-induced pulmonary hypertension. Unlike Type 1, which involves changes to the pulmonary artery, Type 2 involves stiffening of muscle on the heart’s left side. Basically, as I understand it (and it’s taken some time to wrap my head around all this), when I exert too fast or get really stressed, my blood pressure spikes, my heart rate increases, and although my heart does a great job of contracting to pump blood to my lungs, it cannot relax readily—kind of like a clenched fist that can’t easily release—which then causes my pulmonary pressures to spike, some fluids to leak into my lungs, and the shortness of breath and near fainting.

It’s a lot. The standard treatment is to go on a diuretic, which is what my new pulmonologist prescribed, with follow-up blood work and appointments to monitor potassium levels and how I’m doing. However, and here is one of the big lessons of this experience: while very knowledgeable, my new pulmonologist doesn’t know me, yet, and we didn’t have enough time to talk, due to her schedule getting backed up, so I never got to ask a basic question. If I have Sjogren’s, which causes severe dry eyes and mouth, how would a diuretic affect me? Also, she only read the most recent stress test results, and not the rest of my heart work-up.

So when I saw my long-time cardiologist the next day, I asked him, and he nixed that plan and put me on a calcium channel blocker. He said my other heart diagnostics had shown I am not retaining fluids in my heart, and a diuretic like Lasix, which she had prescribed, would probably leave me feeling pretty crummy and dehydrated. The calcium channel blocker is supposed to relax my heart, lower my blood pressure (which has been all over the place) and slow my heart beat, also possibly ease some of the arrhythmia.

Even on the first day of taking my new medication, which is time-released over 24 hours, I began to feel better. Only had a slight headache, and by day two, that was not noticeable. By weekend’s end, I was marveling at the fact that my heartbeat seems to have faded into the background, for the most part, and is not demanding attention as it has been for months now. So, off to a promising start.

I’ve chosen not to write about this until I had a clear diagnosis and some answers. There is no way to know, without a heart biopsy, which I have no intention of doing since the information wouldn’t change my treatment plan, whether scleroderma is the cause of the stiffening of my heart. But it’s likely a culprit. No cure. My approach is to work with my team on the right balance of medication, increase my aerobic exercise as tolerable, and also try yoga to see if it helps me with meditative breathing, strength and flexibility.

I’d be lying if I didn’t admit it’s been scary. The diagnosis last week was quite sobering. I’m grateful for my medical team, and that I have a very experienced and knowledgeable cardiologist who really respects me and knows my history. He also has the calmest voice and manner, which was incredibly soothing when we met on Friday. Scleroderma is complicated. You have to advocate for yourself and build a trusting relationship with medical professionals. And while there is no cure, yet, there is medication that certainly helps.

There is also a lot I can do to give myself the best odds of managing this evolving disease process. That’s been my strategy for the past four decades—living with scleroderma, not letting it run my life. And that’s what I intend to continue doing.

Evelyn Herwitz blogs weekly about living fully with chronic disease, the inside of baseballs, turtles and frogs, J.S. Bach, the meaning of life and whatever else she happens to be thinking about at livingwithscleroderma.com. Please view Privacy Policy here.

Image: Jamie Street