Living with Scleroderma

Reflections on the Messy Complexity of Chronicity

Mind

Hula Finger

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Decades ago, when scleroderma first attacked my hands, my right index finger began to droop. Over the course of several years, it stiffened into a hooked flexion contracture, bent to an immobile 100-degree angle.

To me, it looked like a claw. It was ugly, hag-like. I hated it. I was constantly smashing my bent knuckle into drawers and cabinets whenever I reached for something. It hurt when I shook hands. It was an embarrassing deformity.

The hardest piece was that it had been my favorite finger, enabling me to maintain deft control of pencils when I drew, of needles when I sewed, anything that involved fine motor coordination. I had talented hands with great kinesthetic feel, and my right index finger was the most talented of all.

As the risk of permanent knuckle ulcers and infection grew, I decided to look into corrective hand surgery. I went to one of the best orthopedic hand surgeons in Boston for an assessment. The surgery was doable, but I was scared. What if it didn’t work? What if I lost my finger in the process?

While I debated what to do, our youngest, Emily, expressed a very different view. For her, my digital hook was her favorite. Born a premie and always petite, she had tiny, slender fingers, and she loved to hold my bent index finger like the handle on a tea cup whenever we would snuggle. Her gentle touch was always a salve.

It took me a full year to gather up the courage to have the surgery. All went well, no complications or infections—though my hand surgeon commented that the operation was challenging, my skin as fragile as an onion’s. He shortened the finger so I could still pinch the tip of my resorbed thumb and stabilized the joint with two steel pins.

Emily was disappointed that she couldn’t hold it the same way anymore, but old enough to understand why the surgery was necessary. Gripping the finger, with its internal pins, was painful. So we found other ways to hold hands, as hers grew.

Then the bones in my finger began to resorb. Gradually, the pins poked their way out of the bone and stabbed my knuckle from the inside. I saw another hand surgeon, and we agreed he would remove the pins. As my finger had healed well from the initial surgery, it had formed what he called a “false joint,” and would still be useable.

So, the pins came out. My finger looked a bit squashed and quite stubby, but this time, I was much less concerned about aesthetics and just grateful to have a working finger without pain.

And there was one new attribute that delighted Emily: Since the joint was now more tendon than bone, I could rotate the tip of the finger in a circle, like a hula dancer. I’d hum a tune and make it jiggle, and she would giggle. Another saving grace.

I had my first hand surgery when Emily was five. This week, she turned 20. We still occasionally joke about my hula finger. And we still hold hands when we visit.

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.

Smelling in the Present Moment

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Our golden retriever, Ginger, may be going on 14, but her sense of smell is still in tact. Whenever we go for a walk, she leads with her snout close to the ground, sniffing every leaf and lamppost, inhaling dank secrets.

Which has me musing: How fascinating that we take in smells with every noseful of air. If we breathe through our mouths, we simply breathe. But through our noses, we get more than just essential oxygen—we get a world of sensation that resonates deep in our psyches. Smell, as any writer knows, is the strongest way to evoke memory. For a dog, olfactory memory is all the more powerful, I suppose.

And musing: How it is that our aging Ginger, who is nearly a centenarian in dog years, can still smell a whole lot better than I can? She has, of course, the canine advantage. But I’m also gradually losing my sense of smell.

Some of this is due to genetics. Both my parents began to lose olfactory acuity as they aged. This almost caused a calamity once, when my dad decided to cook sorbet from scratch and left the pan of boiling sugar on the stove unattended. The pan caught fire, igniting the cabinet above the stove. Fortunately, my parents called the fire department in time and their house didn’t go up in flames.

But some of the problem is due to scleroderma, as well. My nose has narrowed and tightened. In addition, Sjögren’s has significantly dried up my nasal passages. I irrigate my nose and sinuses twice daily with a saline solution, which helps my breathing and limits nosebleeds, a real problem in winter. But I still can’t smell as well as I once could.

Very frustrating. I used to have a really acute sense of smell—to the point that I would tell Al about some odd odor and he’d look at me as if I were crazy. This skill saved us some major kitchen disasters of our own. Years ago, in our first home, I began to notice a sour smell. After some investigation, we discovered that either mice or chipmunks had been storing dog food kibbles in the grid behind our refrigerator. Condensation had leaked over the kibbles, which began to mold. Not pleasant.

A few years ago, a plastic surgeon told me he could widen my nostrils to their original dimensions and “take a little off the top” of my rather prominent Herwitz nose to make it easier to breathe. I gave this some serious thought for a while. But so far, I’ve decided not to go the extreme makeover route. My nose may be big and narrow, but it’s mine, and I’m pretty attached to it.

Just recently, I’ve discovered another, much simpler strategy that appears to improve both my breathing and ability to smell—meditating. If I can settle into that quiet, still, contemplative place in my mind (not easy, but I’m working on it), slow down my breathing and just be present, my nasal passages widen, I can breathe deeper and smell more of the world around me again.

Ginger seems to know this, intuitively. Like all dogs, she lives in the present moment all the time. For me, walking her is a form of meditation itself (provided no other dog passes us in the opposite direction). So, I need to take my cue from her: Slow down, pay attention and smell the roses—or whatever else may line my path.

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.

Cutting Bandages

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Every morning and every night, when I get dressed and before I go to bed, I cut bandages for my ulcers. I divide them lengthwise to layer over my fingertips, then wrap a whole bandage around each finger to secure the half bandages in place. It’s become a ritual, this hand management, a routine essential to avoiding infection, a pit stop for damage control, a meditation.

For the past two weeks, as I traveled in Tel Aviv, Jerusalem and London, this ritual-of-necessity anchored me. No matter where Al and I were staying or who we were with, twice a day I had to stop and take care of my hands.

I cut bandages on my fold-out tray in a British Air Boeing 777 en route to Tel Aviv, on a bed in Al’s cousins’ apartment in Ra’anana before sundown on the Jewish Sabbath, at the kitchen table in our friends’ Tel Aviv pied-à-terre over a 1:00 a.m. heart-to-heart about letting go of your adult children, at an old oak table in our cousins’ London flat after our late night arrival from Israel, wanting only to go to bed and knowing I couldn’t, yet.

I was in the midst of cutting bandages when Mindi came to greet us at our friend’s apartment in Tel Aviv, the morning after we’d first arrived. I hadn’t seen her for nearly six months, since she’d left to make a life for herself in Israel, so I jumped up from the table, fingers half-done, to give her a big hug.

And I was cutting bandages last night, sitting on our own bed once again, relieved to have peeled off the day’s grubby dressings, blackened by twelve hours of travel. Were we really at the Tate Museum in London that morning?

Sometimes, the bandaging ritual during our journey was a damn nuisance, the last thing I felt like doing before leaving the house for the day’s adventures or when all I wanted to do was go to bed.

But at other times, it was peaceful, a time to collect my thoughts when everyone else was either asleep or away, an island of quiet to sort out what I’d seen and done and learned that day. As I’d cut the bandages, I’d listen to the familiar sounds of an unfamiliar setting—a wall clock’s tick, a dog’s bark, the click of heels on the floor above, the subterranean rumble of nearby Tube trains—and feel grounded.

I needed that stillness. Travel is so packed with newness, the unpredictable, the need to process so much information quickly and make snap decisions based on estimates of how your experience of your own world approximates this one, even though the two may be only tangentially related. Much as you’re constantly on the go, to fully appreciate the experience, it’s essential to slow down and just be.

So, I guess I have my bandages to thank for that.

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.

Dropping the F-bomb

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Whenever I smash one of my fingertip ulcers—reaching for a faucet, reaching for the gear shift, reaching for a doorknob, any time I’m rushing and not paying attention—I curse.

And the only curse that works is the f-bomb. It’s short, explosive and foul. It’s the best way I know to discharge my anger and diffuse the intense, sharp pain.

My fingertip bones are severely resorbed, so the tips are jagged. When I hit an overlaying ulcer, it’s a double whammy of banging a deep sore on the outside and stabbing it from the inside. Even with my bandages, which provide a little cushioning, it hurts like hell.

But oh hell doesn’t cut it.

I also curse when my hands won’t do what I want.

A spoon slips through my grasp and hits the floor. F-bomb.

Coins slide out of my palm and scatter. F-bomb.

It takes five tries to pick the coins off the floor. F-bomb.

I can’t grab a knife from the flatware tray and have to pry it out with another utensil. F-bomb.

It takes ten minutes to align the zipper pull and zip up my winter coat. F-bomb.

I have a battle with shrink-wrap and the shrink-wrap wins. F-bomb.

I drop my cell phone getting out of the car and the back falls off into the sewer (yes, this really happened once). F-bomb.

Sometimes, when I’m cooking a big meal for company and getting tired and things start slipping out of my hands—like a potato I’m peeling or an onion I’m slicing—I don’t simply drop the f-bomb, I start throwing utensils into the sink and slamming drawers and yelling about how the counters are too cluttered and there’s no place to put anything. My family knows enough to stay out of the way.

I try my best not to curse when others are around or within earshot. I don’t want the f-bomb to creep into my everyday conversation and contribute to the decline of civil discourse.

But I hate this disease. Even though I’ve been living with scleroderma for three decades, and most of the time I can manage quite well, it really gets to me some days. I hate the way it’s wrecking my body. I hate how it’s robbed me of activities I love. I hate all the bandages, the ulcers and infections. I hate all the trips to various doctors and all the waiting in waiting rooms and all the medications. I hate discovering yet one more bizarre complication, like the fact that the roots of my molars are resorbing or the time the left side of my face went numb and I thought I was having a stroke and had to go to the ER and learned that I had an inflamed trigeminal nerve that the ER doc diagnosed as trigeminal neuralgia, which fortunately turned out not to be the case. It was “just” a rare neurological issue associated with scleroderma.

There’s no polite way to put this: When your body craps out on you, it sucks. And with a disease like this, you’re stuck knowing there’s no cure, for now, probably not in your lifetime, and even if, God-willing, there is a cure, your body’s too damaged for it to make a difference, and your health is only going to get worse.

Like aging.

When my sister and I were kids, our family used to travel by overnight train from New York to Cincinnati to visit my grandparents every December. Inevitably at the big family gatherings, all the adults would start complaining about their latest physical ailments. We’d sit on the side and snicker to each other, “When are they going to start passing around their X-rays?”

Fifty years later, I find myself engaged in those same conversations with my friends all too often. The older we get, the more stuff malfunctions, breaks and hurts. It’s shocking when it starts. We all know that our bodies are going to give out and we’re going to die someday, but we don’t really want to believe it until our mortality slaps us in the face. I’ve just been battling the inevitable much longer than most of my peers.

So I guess I have something of an advantage in the how-to-cope sweepstakes. But that doesn’t help on days when my house keys slip through my fingers and I drop the package I’m carrying as I try to pick them up and have to take off my glove to grasp the keyring but it’s too cold and my fingers go into a Raynaud’s spasm.

Then the only thing that works is dropping the f-bomb.

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.

Whatever You Do, Don’t Treat Me Like an Invalid

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Years ago, when I first learned that I had one of three possible auto-immune diseases—either rheumatoid arthritis, lupus or scleroderma, pick your poison—I was terrified. I knew RA could be crippling, and I knew that lupus had killed one of my literary heroines, Flannery O’Connor, at 39. I’d never heard of scleroderma, but the more I read, the more overwhelmed  I felt.

Al and I were newly married, and we were both in shock by my emerging diagnosis and how my body was changing. For a wedding present, knowing I wanted to get back to playing music after a decade of dormancy, Al had my violin repaired—the fallen sound post repositioned, the bridge straightened and the strings replaced. But the first time I picked it up, I realized that I could no longer wrap my fingers around the neck or press on the strings, let alone flex my wrist for bowing.

As my hands got stiffer, my joints became more inflamed and I felt more and more exhausted, the simplest tasks—like pulling on a pair of socks or filling a pot with water and placing it on the stove—became difficult and painful. One weekend, out of the blue, I  experienced a sharp pain with each breath; a trip to urgent care revealed pleurisy. Sensitive to cold from childhood, my fingers and toes now flickered numb throughout the day. I still didn’t have a definitive diagnosis, but my rheumatologist correctly suspected scleroderma.

Neither of us knew how to talk about it. When we first started dating, Al’s mother had warned him that I seemed too fragile. Now it appeared that her words were a harbinger of disaster. We sought help in couples therapy, which opened the lines of communication. But we were both still scared.

When we got together with friends, I would inevitably pick the brains of any health care professional in the crowd (of which there were many, all of them supportive and very concerned). This was before the days of the Internet and before there was much detailed information about scleroderma available to the general public. Al went with me to a scleroderma support group, but I found it depressing rather than uplifting, so I quit after several sessions.

Friends and family were concerned and tried to be helpful. But the reality was that scleroderma is so strange and obscure, that no one had a point of reference. Our physician friends were aware of the severe risks, but they couldn’t predict the course, any more than my rheumatologist.

I soon discovered that, despite everyone’s best intentions, most people really didn’t want to hear about it. We were all in our thirties, friends were having children and building their careers, and most were quite healthy. I was a newlywed the second time around, desperately wanting this marriage to work, facing my mortality. Not the stuff of social banter.

If I did start to share, I would typically get one of several reactions:

People would listen but soon get distracted or change the topic because they couldn’t deal with it. Or they’d listen and then tell me about someone else they knew with an even worse condition, perhaps as a way to relate my predicament to their own experience, but it always made me feel unheard. So I’d shut down.

People would try to offer advice. This was always well-meaning, but most of the time was of no help, since they didn’t really want to take the time to understand what I was dealing with because it was so threatening. So, again, I’d shut down.

People would feel sorry for me and try to mother me with their concern. Of all the reactions, this was the worst. If there is one thing I can’t tolerate, it’s being treated as an invalid.

While I appreciate the underlying compassion, I never want to be handled as if I can’t do for myself. Ultimately, that level of smothering concern is more about the helper’s need for control over the unknown than the helpee’s need to be cared for. It completely undermines the fighting spirit that’s so essential to managing this and any other chronic disease.

There’s a fine line to be tread here, of course, because I also have had to learn to ask for help. There are many things that I can’t do easily anymore, and I’m no longer ashamed to be assertive in requesting assistance.

But my family and closest friends all know by now how important it is to me to continue pushing back, managing this complicating disease, outsmarting it, accepting it, running with it, on my own two feet.

Al, to his great credit, has never babied me, even when sometimes, in weaker moments, I wished he would. For the 27 years we’ve been married, with scleroderma as our third partner, he has always helped me when needed, but never expected less of me than I do, myself. And that has been one of my most powerful medications.

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.