Living with Scleroderma

Reflections on the Messy Complexity of Chronicity

pulmonary hypertension

The Heart of the Matter

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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

Just Breathe

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This past Friday morning, I had double-header appointments at Boston Medical, an echocardiogram followed by a routine appointment with my wonderful rheumatologist, who has been my specialist for at least two decades, now.

My last echocardiogram was done a couple of years ago, one of those tests I have to repeat occasionally to monitor signs of pulmonary arterial hypertension (PAH), a late-stage complication of scleroderma. The only symptom, so far, is extreme shortness of breath if I commence intense aerobic exercising without a serious warm-up. We’ve been watching this for years, now, and I’m on prophylactic medication that seems to be protecting me from worse complications.

Most of the time, the test doesn’t bother me. It’s non-invasive, and, depending on the tech, just mildly uncomfortable. Like I said, depending on the tech. This time, let us say, it was more challenging.

First, the easy part. You lie down on your left side, with your head on a pillow. The lights are dimmed so the tech can see the computer screen more clearly. A transducer, which looks like a short, hand-held rod with a gel-covered rolling ball on top, is pressed against your ribcage, neck and diaphragm, to send high frequency sound waves through your chest wall. It’s like an ultrasound for your heart. The sound waves bounce back to the computer, which translates them into moving pictures of your heart muscle. Occasionally, the tech will turn on the audio, and you can hear your heart beating away, kind of a squishy, pumping sound that seems to reverberate from a deep well.

Now for the hard part. You have to hold your breath during certain parts of the test, so that your diaphragm doesn’t cause your heart to move around and your lungs aren’t so full that they interfere with the heart imagery. I’ve never had an issue with this in the past, but my tech on Friday had a very specific way that he wanted me to empty my lungs, first, and then take in only a small sip of air. Then hold. And hold. And hold. While he pressed really hard with the transducer on my ribcage. I have no padding there. It hurt. And I couldn’t wave my hand or ask, “Can I breathe now?”

I really started to wonder, at a few points, if I would actually be able to hold my breath long enough. Fortunately, each time, just as I thought I wouldn’t make it, he said I could breathe again. It was also reassuring to hear my heart beating when I felt like my lungs would burst. “You’re doing great,” he said. I guess so. Test results will be available this week.

By the end of the half-hour, I was very glad to get dressed and head over to the Rheumatology Department. The sun was bright, the air crisp. As I caught up with my rheumatologist, who, like me, is in his sixties, we chatted briefly about retirement. To my relief, he has no plans of retiring anytime soon. This time, I needed no permission to breathe.

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 Credit: Eric Witsoe

Tending Barre

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Around New Year’s, I decided to shake up my exercise routine and join a community fitness center—to access a greater variety of classes, to use the fitness equipment, to break up my work day with a workout.

Good intentions. But, to be honest, I’ve been less than diligent about going. I’ve had plenty of excuses. It’s been way too cold out. I don’t like changing in and out of exercise clothes in the middle of the day. I’m too busy.

Then there have been a few mishaps as I’ve tried to find my place—like killing my knees in a Zumba class and getting short of breath in a “Senior” exercise class. The latter experience left me mortified (can’t they call it something else?), but it was a serious workout and I arrived late, didn’t warm up enough and started feeling faint during the aerobics portion of the class. I recovered, but not without scaring my instructor. Later in the day, I received a thoughtful follow-up email from the fitness center director to be sure I was okay and to suggest a few more options.

Really, the big issue is being careful that I don’t accelerate into strenuous aerobics too quickly, which seems to trigger what my physicians suspect is stress-induced pulmonary hypertension. But it spooked me, and I wasn’t sure what to do.

Then I discovered Barre Exercise. I’ve always loved dance, and over the past ten years, I’ve taken jazz, modern and Middle Eastern belly dancing. As my feet have become more sensitive, I’ve had to cut back. It’s very hard to keep my balance on the balls of my feet as the fat pads have significantly thinned out due to scleroderma.

But this class uses a ballet barre. So I have something to grab onto.

It’s been many years since I took a basic ballet class, and I am no Pavlova. But I had forgotten how much I enjoy the form and grace of ballet movements. All the Pilates classes have paid off. I know how to align myself and engage my core. And I still remember the fundamentals—foot placement, arms, the essentials of a plié, tendu, dégagé, coupé, attitude, battement. I can’t quite hold my balance in an arabesque, but I can approximate the position.

The workout is quite intense—deceptively so, because each movement is limited and controlled. But I work up a sweat, and the cold room no longer feels cold after about 15 minutes. The pacing works, so I can keep up with the aerobics without getting short of breath. And there is plenty of stretching at the end.

Most of all, I actually feel graceful. This is the best part. My range of motion has been so constricted over the years by this disease that the fact that I can actually make a beautiful shape with my body is astonishing and wonderful. I leave the class feeling refreshed and a little more confident each week.

I still need to figure out a way to get myself to the center more often. I know I should probably do the treadmill or stationary bike to build up my aerobic endurance, even though the prospect is boring as all get-out. I’d like to find another class that I enjoy. But at least I’ve been able to tap my inner dancer, once again. Whatever my physical limitations, this is what I always return to. 

Photo Credit: quinn.anya via Compfight cc

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.

Multitasking

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Friday morning. While brushing my teeth, thinking through the day ahead (must leave the house by 10:00 to get to my 11:40 annual cardiology check-up in Boston, must take my laptop with access to work files for the inevitable waiting-room doldrums), I suddenly wonder: I see my rheumatologist in two weeks, but I know he ordered a pulmonary function test to be done prior to the visit. Is it today?

I check the calendar on my cellphone. Sure enough—PFT at 2:30. I never transferred it to my desk planner (yes, I prefer a paper calendar for a weekly overview, easier to get the gestalt).

My entire afternoon is now in flux. I had a lot of work planned for when I got home. Now I really need to be in full portable office mode. I check emails before I leave. One of my clients needs to discuss a consultant’s proposal. I suggest a 1:30 call. I should be out of my first appointment and waiting for the second by then, and I can park myself in the lobby outside the diagnostic lab for the conference call. Laptop, cellphone and charger stowed in my purse, I head out the door.

Fortunately, traffic is moving well, and I arrive for my first appointment ahead of time. My doc is running a bit behind. There’s an electrical outlet near one of the chairs in the waiting room. Perfect. I set up my laptop with the charger, so I won’t drain the battery later, and begin to work through emails. Of course, this magically conjures the cardiology tech, who calls me in for my appointment.

Juggling purse, coat, computer and cord, I make it through the preliminaries of weight check-in. As she records my blood pressure and oxygenation level, my mind is on my work. I sit on the edge of the exam chair, waiting for her to calibrate the EKG machine, and watch the black second-hand of the wall clock. Click-click-click-click-click.

EKG recorded, I set up my laptop and log into the WIFI. I’m about to start up with the emails, but stop myself. Oh, right. The reason I’m here is to see my cardiologist. Better make some notes about issues to discuss. I jot these down in a small notebook and go back to work. I finish typing as my cardiologist enters the room. Switch gears. This is about my health, now.

Ok, focus. The main issue of concern is a recent episode of shortness of breath. At a party in March, I had been dancing vigorously and then stopped because my knees were getting tired. As soon as I sat down, I had trouble catching my breath. This is why I have the PFT scheduled at 2:30, to get a current reading on my diffusion rate. My cardiologist reviews the details carefully. We have been working with a hypothesis of exercise-induced pulmonary hypertension, a variant of late-stage complications of scleroderma, for several years, now. It could be that, it could be something else. But the episodes are infrequent (fortunately), my echocardiogram history is consistent and my meds are all in order, so for now, he tells me, just avoid sudden, strenuous exertion, which seems to be the trigger. Keep on exercising, though. And if it happens spontaneously or more frequently, call him. He schedules a follow-up in six months. I feel reassured.

Over the next hour, I fit in lunch and search for a quiet place to work with a WIFI signal. This takes persistence. The signal is inconsistent, depending on location. But by 1:30, I’m back online, in a lobby with hardly anyone around, and am able to speak for a half-hour with my clients in NYC. I follow up with some other business, plus texts and emails with my eldest daughter. I make it to the pulmonary function lab at exactly 2:30.

More waiting. The lab tech needs to make a call, so I squeeze in another text response. Now for the tests. She reviews the procedure, which I’ve done many times, and begins instructing me to first breathe normally into the tubing attached to diagnostic equipment, then take a big breath in, push it all out and another big breathe in. It’s physically challenging for me, and requires mindful awareness of what constitutes a full breath in and a full breath out. As we’re running the test, she chats with another tech who is making a phone call.

Then a doctor—I assume, he’s wearing a white lab coat and the techs wear blue scrubs—steps into the open doorway. We’re repeating the test, the tech is waving her hand in a sine curve to indicate I should continue normal breathing, I’m trying to focus on what I’m supposed to be doing, and he’s telling her that there’s an issue with her quality scores for some research study that they’re involved in. He continues to discuss this with her as she defends herself and interjects verbal and visual cues to me—when to push out, when to breathe in.

Finally he leaves. Time for a break between tests. She realizes she forgot to set up the next test correctly and needs to recalibrate the equipment. She’s obviously flustered. I try to say something reassuring. I field another text from my daughter as we wait. We talk about our children, about texting, about staying in touch. I feel awkward for her. How humiliating, that her superior would give her critical feedback while I’m sitting there. And how uneasy it makes me feel, wondering if she knows what she’s doing, though she certainly seems to. And how ridiculous, to be conducting that conversation while we’re engaged in a diagnostic that requires concentration.

But of course, we all multitask. It’s a given, right?

Later, much later, after I’ve driven home through Friday afternoon traffic and have finished all the record-keeping, follow-up emails and return phone calls, and I can finally forget about work and relax over Shabbat dinner, I pause and notice—the pink peonies and purple irises in a blue ceramic vase, the white candles flickering, Ginger’s steady panting under the table, the smell of warm challah and sweet potatoes and baked cod. So good to slow down and just be. So good.

Photo Credit: mr.beaver via Compfight cc

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.

Fantastic Voyage

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In 1966, when movies still cost 50 cents and popcorn a quarter, I went with a friend to the Peekskill Paramount movie theatre on a Saturday afternoon to huddle in the crowded balcony with a bunch of other giggly, wise-cracking kids and watch Fantastic Voyage.

In what has become a sci-fi classic, a team of miniaturized surgeons enter the body of a scientist to zap a life-threatening, inoperable blot clot in his brain; the scientist is the only person in the world who knows how to make the miniaturization state last more than an hour, a secret essential to U.S. Cold War military strategy.

The team travels in a mini submarine through blood vessels and organs, battling antibodies along the way and a fiendish saboteur in their midst. If that isn’t enough to pique your curiosity, the movie stars a very young Rachel Welch and won two Academy Awards for some pretty neat special effects before the days of computer animation.

I’ve been thinking of this movie lately and just added it to my Netflix queue. One of the curious aspects of living with a complicated disease for so long is that I’ve seen more and more of my own internal landscape in recent years. With each new complication of my scleroderma, there are tests and more tests. And with digital imagery and optic probes the norm in medicine, and X-ray results easily viewed on an exam room computer screen, I’ve seen some pretty fantastic, albeit sometimes disturbing, sights.

There are the basics—all the many, many X-rays of my deteriorating hands, with each iteration revealing less bone at the fingertips and more starbursts of calcium floating under the skin. There are some foot X-rays, too, more recent, to confirm calcinosis in my toes.

There was the MRI of my chest a few years ago, when a CT scan to check rasping in my right lung (a possible sign of interstitial lung disease) revealed a questionable spot. It turned out, my pulmonologist explained while we toured the results on his computer screen, that the spot was nothing to worry about, just evidence that I’d contracted histoplasmosis years before, probably while spelunking one weekend near Pittsburgh when I was in grad school. As he scrolled through the MRI, the inside of my lungs revolved like the ceiling of a planetarium speckled with tiny white stars. Some scarring, yes, but so far, nothing too debilitating.

There have been regular echocardiograms to monitor signs of pulmonary hypertension, a late-stage scleroderma risk. Sometimes I close my eyes and try to doze through this non-invasive but often uncomfortable procedure, which requires the tech to press a rolling probe all over my chest and ribcage. Other times I’ll distract myself by watching the dark computer screen, with its blue and red images of blood surging through my heart, like an animated deKooning.

More tests. A CT scan of my brain and skull X-ray one Fourth of July weekend when I started to go numb on the left side of my face. Cross-sections of my very own convoluted gray matter. Yes, that’s where all those thoughts and images and feelings ping around. No stroke, thank God; rather, an inflamed trigeminal nerve was the culprit. But there was something eerie about seeing an image of my own facial skeleton, not some Halloween mask—the exact position of my eye sockets, nasal cavity, cheekbones, jaw—shades of what will remain when the rest of me turns to dust.

Of course, there have been all the routine images, too—ultrasounds of my womb when I was pregnant with Emily, the squashed elliptical pancakes of my breasts as seen on a mammogram, a slew of dental X-rays revealing how some of the roots of my teeth are resorbing—a rare scleroderma complication. Fortunately, I slept through my colonoscopy a few years ago.

I’ve seen the pink marbled walls of my bladder and the black-and-white image of a PICC line snaking into one underarm vein and then the other, when the first side was blocked by too much scarring. Not fun.

Most intriguing, once we got past the unpleasantness of inserting an optical probe through my nose, was a view of my pharynx. This took place when I saw a speech therapy specialist a few years ago to evaluate problems with swallowing. There are times that I feel like food gets stuck in the back of my throat, and I worry about choking. She handed me items to swallow—crackers, apple sauce, Jello—tinged with Kelly Green food dye, so we could see if the pathway to my windpipe closed properly as I ate. It did, a great relief, and also fascinating. I thought of a camera lens opening and closing when you squeeze the shutter.

I’m sure, as time goes on and my scleroderma does its own thing and medical technology becomes ever more sophisticated, I’ll see even more of my innards. Not the kinds of images you put in your photo album, like pregnancy ultrasounds. But miraculous, nonetheless. Even if the reason we’re digging around with probes and such is due to damage caused by an insidious disease, I’m still amazed by the view.

We take our bodies for granted, all the inner workings so hidden beneath our skin. If we could see what was really going on inside, all the intricacies of our interior universe, how the balance here affects the balance there, would we take better care?

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.