Living with Scleroderma

Reflections on the Messy Complexity of Chronicity

calcinosis

Outtakes

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One of the strangest aspects of my scleroderma is calcinosis—a condition that causes small bits of calcium to form under my skin and eventually work their way out. From time to time, a calcium pit will push through, leaving a small hole, like a tiny window into my finger, that fills with new skin in a day or two.

Sometimes the piece is sharp and painful as it migrates to the surface, and other times, it’s just a nuisance, a condition I’ve long learned to recognize and manage with careful hygiene to be sure an infection doesn’t settle into the resulting ulcer. The calcium tends to form at pressure points, in whichever fingertip surface I use the most often.

Over the past decade, calcinosis has also begun to occur in the bridge of my nose, where my glasses rest. I can’t wear contacts, because my eyes are too dry from Sjögren’s, another scleroderma complication. I’ve switched to very lightweight Silhouette frames, which are expensive but create barely any pressure. Still, the calcium forms. These pits need to be removed surgically, or I risk ulceration in this very delicate skin that is stretched too thin by scleroderma to heal properly. Not fun.

So it was, last Monday, that I headed in to Boston Medical Center to visit my ENT plastic surgeon, for a consultation about removing yet another calcium pit from my nose. I hadn’t seen him since 2009, when he skillfully extricated the last offender from the inside, instead of having to cut through from the outside. The time before that, I’d seen another surgeon who struggled with my fragile skin when closing the external wound.

He knew me right away—I guess my nose is pretty memorable—and made a quick assessment. The pit was very close to the surface and actually sticking to the underside of my skin. He could remove it from the outside or the inside. It would only take 15 minutes. In fact, he could do it right then. His associate also took a look and concurred. Easy enough to do. How did I want to proceed?

Now, I have never, ever, made a decision to do any kind of outpatient procedure on the spot. Especially on my face. Especially without Al along for moral support and to help me get home in case I got woozy afterwards. But as I sat there, debating and discussing pros and cons with the two specialists, I made up my mind.

It didn’t make sense to extricate the calcium pit from inside my nostril, a much more uncomfortable procedure, since it was stuck and could tear the outer skin in the process. If he cut from the outside, I’d just have a couple of stitches. And if I did it right then, I’d save time.

Yup, that was the bottom line. I really didn’t want to have to come back another day, get psyched up for the procedure, and spend yet a second morning or afternoon dealing with this. Plus, it could be weeks before I’d get into their schedule again, and the pit was stretching my skin so much it could possibly break through and not heal properly in the meantime. So, I said let’s do it.

Four shots of local anaesthetic—like sitting still for a bee sting, was his sympathetic assessment as I winced and grit my teeth—a slice, some grinding against bone and tugging on the stubborn remains of the calcium, a couple of stitches, and he was done in 15 minutes. The removed deposits, which he showed me floating in a little orange bottle that would be submitted to the lab for assessment, were like grains of sand, just like the ones that come out of my fingers. At least I’m consistent.

I felt a bit shaky as I left, but victorious. It was done. And almost routine. Has it really come to this? Even the oddest experiences with this disease are now just a matter of course? Sure, why not cut into my nose while we both have the time and take out the damn piece of calcium. Might as well be practical and just get it over with.

My nose is still healing, but it only hurt for a day. The stitches continue to absorb and will come out on their own. The worst thing that happened afterwards was getting a rotten cold, probably from the ENT waiting room, that knocked me out for the second half of the week. Two calcium pits popped out of my fingers, perhaps out of sympathy for their compatriot. Otherwise, it’s been just another week.

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.

The Waiting Game

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I’m late for my doctor’s appointment. Per usual, I tried to finish just one more thing before I left the house. Then I hit road construction on the main thoroughfare between home and the medical center. By the time I have parked and found the right office inside the cavernous hospital, it’s a good 15 minutes past my scheduled arrival. And the doctor is running on time. Uh-oh.

Usually, it’s the other way around. But I get lucky. The waiting room is empty and my appointment doesn’t get bumped. I have my Kindle along, but I get distracted by the waiting room flat screen TV. It’s an episode of The Doctors, featuring a team of attractive specialists answering studio audience questions about their health. The ER doc wears a pair of blue scrubs and the others, white lab coats. The pediatrician is responding to a young woman’s query about the birth mark on her chest when the nurse calls me into my appointment.

Hoping to shave a pound or two off the digital scale readout, I take off my coat and shoes when she weighs me. We review my meds and allergies. She takes my blood pressure and temperature. We chat about the weather. As she leaves the room, I check the magazines in the wall rack. This exam room could use some better reading material—there’s a Vermont tourist glossy, a couple of trade health publications and an ersatz women’s magazine. I flip through its pages and scan the list of recommended books, wondering why it’s such a struggle to get published when all this dreck makes it into print.

My doctor is prompt and pleasant. He’s an infectious disease specialist, and we’re reviewing the plan we made over the summer to manage any future infections in my finger ulcers. After another year of on-again-off-again antibiotics, it was time to get pro-active. We marvel at the fact that I’ve had no infections since I saw him in June. I joke that all the germs have been scared off by his presence. He laughs. “I wish it were so,” he says.

We review what to do when the next infection hits. It’s a foregone conclusion. The only question is, how soon? There’s a piece of calcium migrating toward the surface of my right thumb. It’s causing me difficulty squeezing a tube of toothpaste and picking up cups. When it finally breaks through the skin, perhaps in a few months, there’s a high chance of infection. And, as the weather gets colder, my skin breaks down and is at greater risk, anyway.

We agree that I don’t need a follow-up. I’ll just call him when the next infection hits. I have the necessary antibiotics at home and know when and how to use them. He trusts my experience and my judgment. I thank him and say good-bye, for now. As I walk out through the waiting room, Family Feud contestants cheer and clap before the commercial break.

Leaving the hospital parking lot, I wait in a line of cars. It’s almost 3:20 and the shift is changing from days to evenings. On the drive home, I ease my car around the exposed man-hole covers that have turned the street under construction into a slalom course. I get home just over an hour after I left, pretty good for any doctor’s appointment—especially when I was the late one.

At the back door, Ginger is waiting patiently for my return. The sun casts long shadows. My right thumb twinges as I set down my Kindle, little green medical notebook and cell phone on the kitchen table. It’s almost time for our walk.

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.

Making Stuff

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For as long as I can remember, I’ve had cold hands. As a kid, growing up in New York winters, I would play outside making snowmen until I was too numb to feel my fingers and toes. Summers on the Cape, I’d jump in the waves at Nauset until I was blue and my teeth chattered nonstop.

But none of this mattered. I loved to do, to plunge into the world around me. And my favorite means of engagement was through my hands, making stuff. I could do anything with my hands—draw in any medium, do Japanese brush painting, throw pots, make copper enamel jewelry, create tiny origami animals, sew doll clothes and my own, knit, crochet, embroider, needlepoint.

I could also make music, on the full range of recorders; the violin (I was first chair in my high school orchestra and worked my way up to Mendelssohn’s violin concerto); viola; alto, bass and contra-bass clarinet; tenor sax; classical guitar; piano and a little percussion.

When I was about 10 or so, I lay in bed one night, wondering what it would be like to lose one of my senses. I couldn’t decide which I valued more, sight or hearing—but the one thing I knew was that I never wanted to lose touch and the use of my hands.

I could never have imagined the strange shape of my hands today. Decades of severe Raynaud’s have rendered my hand circulation erratic. Scleroderma has bowed and shortened my fingers; the bones have resorbed so much that my hand X-rays look like someone has taken a bite out of each fingertip, and my thumbs are filled with a veritable Milky Way of calcium deposits that erupt through the skin from time to time. I bandage chronic fingertip ulcers twice daily, keep vigil against infections and am constantly reminded, when I try to do something as simple as put change in my wallet at a cash register, that I just need more time than most to do basic tasks.

But I’ve often thought that the fact that I started off with such incredible fine motor coordination has enabled me to continue creating with skill, even as the process is so much harder.

My father had a saying: “Any problem can be solved if you have the right tools.” So this has been my watchword. My drawing pencils each wear colorful triangular rubber grips to cushion my fingers. I’m never without a pair of tweezers, used for everything from picking up beads that I’m threading on jewelry wire to pulling out the bobbin from my sewing machine. I protect my bandages and ulcers with plastic gloves whenever I cook or handle material that’s wet or could be a source of infection.

I can’t play the violin or guitar any more (yes, Doctor, I really did play before), nor a wind instrument, and I find pressing the keys on a normal piano keyboard difficult (even though I still harbor a wish to someday, somehow, learn to play Gershwin).

But I do make music in a new way—through my writing. For me, writing is all about melody, rhythm, assonance and dissonance, crescendos and diminuendos of sounds tied to words tied to thoughts.

And I make visual art through video that I shoot with a simple, lightweight camera and manipulate through the magic of my iMac.

I still miss my hands, even though I can barely remember what it feels like to be normal. Then keep making stuff.

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.