Living with Scleroderma

Reflections on the Messy Complexity of Chronicity

Mind

Red Sandals

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Right before Passover this past April, I went through my closet and gave away about a dozen pairs of shoes and sandals that I could no longer wear. I’d accumulated them over decades, and each set was a favorite.

But it was simply time to face the fact that the fat pads on my feet have thinned so much from scleroderma that I need a lot of cushioning, and my old favorites hurt. Most of them I hadn’t even considered wearing for years. I had just kept them because I liked them so much.

Hard to part with the shoes, and the idea they represented—that it’s still possible for me to walk with style. Recently, the only shoes I’ve been able to tolerate are two pairs of lightweight, fabric mesh Merrell clogs, navy and black, in which I can insert custom, full-sole orthotics. I’m grateful that these are so comfortable, but they really don’t go well with skirts and dresses.

Of all the shoes I gave away, the ones I parted with most reluctantly were a pair of red sandals with two-inch heels. Nothing like red sandals. They always used to give me a boost, height-wise and mood-wise.

So now, mid-June, it’s finally feeling summery for more than a day here in Central Massachusetts, and no red sandals, no walking sandals, no sandals I could count on for casual wear or work appointments.

I had scoured online shoe sites without seeing anything that seemed worth trying. So hard to tell, and with sandals, the foot sole is key because you obviously can’t insert orthotics.

The only real solution: Go to a shoe store where the staff still know how to fit your feet. This is not easy to find. But there is such a store about a 40 minute drive from home. I haven’t been there in years.

So, with an hour to spare between two appointments last week that took me in the right direction, I made a pilgrimage. The selection hadn’t changed much since my last visit. The show window and displays were full of all the predictable comfort brands, some attractive, some downright clunky.

One would think, with all of us baby-boomer women now at the age of sore feet, that someone out there would approach the question of how to design comfortable, stylish shoes with a bit more imagination. But apparently not.

Round and round the store I walked, picking up possible choices and pressing the foot beds with my thumb. Per usual, the nice-looking sandals didn’t have enough arch support or cushioning. The most comfortable walking sandals were $225 and really, really ugly—like a pair of shovels.

I was about ready to give up and leave when I circled around one more time. There, on the wall, was a pair of raspberry red Dansko sandals—two wide straps of faux snakeskin with silvery buckles on a cushioned, rubbery platform that was styled to look like carved wood, but much more shock-absorbent. Now, I had given away a similar, well-worn black pair, not as attractive, right before Passover, because the cushioning was just not thick enough and they were too loose and caused blisters (probably because my feet are much thinner than when I had purchased them at least five years ago, so they didn’t fit properly anymore, and the footbed was worn out).

But, on a whim, I tried on the sample. It fit. Perfectly. The salesclerk found the mate in the store window, and I took a walk up and down the aisle. No pain. The shoes rolled easily from heel to toe. Excellent arch support. Good cushioning. They even made me stand up straighter, something about the balance of the shoe.

And they were red. On sale.

So I bought them. The salesclerk assured me that I could bring them back within two weeks and get a refund if, after wearing them around the house (not outside), I had any problems.

Over the next few days, I tried them on at different times. Still comfortable. I could do stairs. I could walk on our wooden kitchen floor and on the concrete in the basement.

On Sunday, sunny, full of summer promise, I decided to commit. Out the door, with Ginger on her leash, around the block. Success! Then in the car, over to the art museum, on my feet walking around for an hour to view my favorite works. A little foot fatigue, but still good. No real soreness.

There are probably no ready-made sandals in the world that will ever solve all my issues, but this pair sure gets at thumbs-up for darn near perfect.

Oh, and did I mention? They’re red.

Image: June, 1975—Hydrangea by a Pond, Stencil-dyed paper calendar by Keizuke Serizawa (1895-1984), Worcester Art Museum

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.

Pillar of Salt

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Surrounding Israel’s Dead Sea, spires of salt tower like sentinels, guarding barren hillsides. In bright, humid heat, they shimmer, casting craggy shadows on salt-infused sand. These ancient formations no doubt inspired the biblical story of Lot’s wife, who, fleeing from God’s destruction of Sodom and Gemorrah, made the fatal error of pausing to look back and was frozen into a pillar of salt. Indeed, a salt formation near the Dead Sea is named for her.

I once read somewhere that Lot’s wife’s plight was a metaphor for scleroderma. In its most virulent form, this deforming disease locks the body inside hardened skin, from which there is no escape. But scleroderma takes many forms, unique to each individual. And those of us who live with this disease in no way brought it upon ourselves. So the metaphor, while dramatic, breaks down for me.

Nonetheless, scleroderma has been known since ancient times. The Greek physician Hippocrates first described it as thickened skin, around 400 BCE. Carlo Curzio, an 18th century Italian physician and dermatologist, gave the first detailed description of the disease in 1753. His monograph, published in Naples, presented the case of a 17-year-old woman named Patrizia Galiera, suffering from hardened skin that varied in thickness, tightening around her mouth and neck. He noted that her skin lacked warmth, but that her pulse, respiration and digestion appeared normal.

Over the course of 11 months, her treatments consisted of warm milk, vapor baths, leeched blood and small doses of quicksilver. Miraculously, at least according to Curzio’s report, Patrizia’s skin softened and she fully recovered. (However, we will never know if she eventually experienced symptoms of mercury poisoning from the quicksilver.)

During the 19th century, physicians began to differentiate the variations within the broad disease category, including localized scleroderma and en coup de sabre (morphea), the latter so-named because it dents the forehead like a sword blow and then deforms the skin on only one side of the face. Scleroderma also found its way into fiction, afflicting a character in a Sherlock Holmes story by Sir Arthur Conan Doyle, “The Case of the Blanched Soldier.”

The connection between scleroderma and abnormal vasoconstriction was documented by the French physician Maurice Raynaud in 1863. He described the case of a farmer whose arms became numb in winter, and whose hands gradually hardened and darkened. Well-known to many of us who suffer from numb, tri-colored hands, this condition is named for him—Raynaud’s Syndrome or Disease, depending on whether it accompanies a disease like scleroderma or is free standing.

Six years after Raynaud’s discovery, the American Journal of Medicine published the first American report of scleroderma, with a history of three patients. More about the history of scleroderma can be found here.

Today, knowledge of the many variants of scleroderma is deeper and broader than at any other time in history. Research into all aspects of the disease progresses, although a cure has yet to be found. But the rarity of scleroderma—afflicting a median of only 15 individuals per million each year, worldwide—makes it all the more elusive.

Last week, a post I wrote two years ago about a recent breakthrough in scleroderma research found its way around the Internet. Several readers shared poignant stories about experiences with the disease and the loss of loved ones to the most deadly variant. All shared hopes that a cure will soon be found. I was struck, however, by another common thread: Scleroderma is so rare, and so varied in its presentation, that a number of patients suffered all the more because they were misdiagnosed at early stages of the disease. We do not all present as obvious scleroderma patients, with hardened, mask-like faces and clawed hands.

As important as finding a cure, it is essential that physicians in the field, especially those who live and work far from urban centers, with less access to current research, learn to recognize scleroderma and know about available treatments. I applaud the work of the Scleroderma Foundation, which has begun to offer online CME/CNE training for physicians and nurses, in tandem with the Postgraduate Institute for Medicine, to reach out in this regard.

Every step helps—whether in the lab or in medical school classrooms—to ease the path of those with scleroderma throughout the world. We are not pillars of salt. We are not responsible for our illness, helpless or hopeless. We need to support those who are working for a cure, and those who are educating physicians to recognize and properly treat scleroderma. June 29 is World Scleroderma Day. Make your mark.

You can find more information about current research and education efforts here:

Photo Credit: M Disdero 25/02/2007, Pilastro di sale sul Mar Morto, indicato dalle guide turistiche come la moglie di Lot trasformata in statua di sale durante la distruzione di Sodoma. Wikimedia Commons

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.

Commencing

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Polliwogs dart in the reflecting pool like animated apostrophes, their slender tails whipping water, propelling bulbous black heads. Within a few weeks, they will sprout legs and become tadpoles, then absorb their tails to grow into froglets, and, finally, full-fledged frogs.

I watch them zig-zag in their search for algae, blissful, I imagine, ignored by the goldfish who share the pool and travel in schools. A red-winged blackbird lands at the water’s edge and splashes, cleansing its wings, then flies to a nearby tree to dry in the sun. A boy skips rocks across the water, but the polliwogs seem undisturbed, flitting beneath mirrored clouds.

Al and I are sitting by the pool on Sunday afternoon, waiting for Emily, who is waiting for the residents of her dorm to pack up all their belongings and leave campus. This is her final resident adviser responsibility on the very last day of her senior year of college. This time yesterday, she graduated, walking proudly in black cap and gown with her classmates up the hill, in the welcomed sun, following the path to the huge, white commencement tent—a tent as large, one parent quipped, as an airplane hanger.

I study the pool’s inhabitants and replay Saturday’s ceremony. The class of 2014 leaves this bucolic campus for a troubled world. The Commencement Speaker urges graduates to employ their proven imagination to help solve the seemingly intractable conflicts and challenges facing our nation and planet. The College President provides the context: This August marks the 100th anniversary of the outbreak of World War I. So much progress, so much bloodshed, so much discovery and retrenchment in the century that has followed. Creativity, optimism in defiance of cynicism, the power of the individual to make a difference, reasoned debate to resolve conflict, empathy for the other rather than obsession with material success—these are the values he charges the graduates to take with them.

The greatest ovation comes for a tall man who walks jauntily across the dais after receiving his diploma—a former prison inmate who served time earning his bachelor’s from this rigorous college. Recidivism rates for graduates of the college’s prison outreach program are very low, proof of the power of serious education to enable a fresh start, with promise. We all stand, cheer and cheer, our hope for the future renewed.

Hugs, photos, receptions. The sun defies rainy predictions, and the light breeze refreshes. Mindi, our eldest, serves as family photographer and Facebook chronicler, so much more adept than I, and her real-time posts prompt kudos from relatives and friends around the country.

The celebration ends with a barbecue overlooking the Hudson River and spectacular fireworks that echo off hillsides. I have looked forward to this event since Emily first arrived on campus. Her college education, formal and informal, has exceeded all our expectations, and this day is a glorious conclusion.

By the reflecting pool, shadows grow longer, and a breeze stirs the water on a clockwise course. With staccato rhythm, a pond skater on delicate, hinged legs hops across the surface. Polliwogs swim beneath it, oblivious, but when they become frogs, they will consider the insect a delicacy. I marvel at its amazing ability to walk on water. What inventions will someone yet discover, adapting its evolved mastery of surface tension to maneuver in new realms? And what predators will swoop down and gobble up the polliwogs before they are full grown and able to devour the pond skater?

The pool surface ripples from the boy’s skipped stones, briefly shattering the reflection of cumulous clouds above trees in full leaf. Then all is stillness, apparent stillness, though the water is always moving, the goldfish and polliwogs and pond skaters in their endless dance, searching for sustenance.

Emily is home for only a week, than returns to her alma mater for the summer, to help with preparations for the intensive orientation program that will greet the incoming class of 2018 in August. This makes our leave-taking a bit easier. She will deeply miss it here, even as she knows her next move—on to graduate school, to prepare for a career in higher education administration. She has blossomed in so many ways, found her voice, found her direction, made lasting friendships. But new challenges await. The next transition begins.

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.

Lucky

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Sitting in the hallway of Mass Eye & Ear’s Cornea Service, as doctors, fellows and technicians in white lab coats stride back and forth, back and forth. Before, all the seats that line the wall were full. Now I’m by myself. A cranky teen sitting next to me earlier complained to her mother about prisoners being seen here. “Why do they have to go to the good hospitals?” she griped. “Why do they have to take my spot when they’ve committed a heinous crime?”

Doors open and shut with a swish and ca-chunk. I’ve had my medical history taken, my vision checked, my tear production dip-sticked, my eye pressure tested for glaucoma, my cornea thickness measured. I’ve been here for nearly three hours, having made it through all the preliminaries, and now I await the big cornea specialist.

This is the first time I’ve ever had my Sjogren’s (another autoimmune disease that can accompany scleroderma, which damages moisture producing glands) carefully assessed, because the dryness in my eyes is causing light sensitivity and blurring toward day’s end. After my travel episode with conjunctivitis in March and resultant corneal abrasions from irritating eye drops, I am wary of putting any new medications in my eyes without a thorough evaluation.

The cranky teen told me her first appointment here took five hours. I have made the mistake of parking in the Mass General Hospital garage, which does not accept validation from Mass Eye & Ear, even though the institutions are right next to each other, and I realize this visit is going to cost a small fortune.

I wonder if they’ve forgotten about me.

I check my email on my laptop. At least I had the sense to bring it along so I could get some work done. Could I have gotten so absorbed in responding to a client that I missed hearing my name?

“Evelyn?” The man’s voice is loud, authoritative. Finally, the specialist has called me in. He is tall, trim, graying. No glasses, maybe wears contacts. Two women fellows accompany him into the exam room. He apologizes: my chart was put in the wrong place and he’d been looking for me for the past half-hour. Sigh.

More drops, more lights, more gentle prodding to see if my eyes exude any tears. After he checks, he has the fellows look, too. It’s a teaching institution, after all, and I am a teachable moment. He types on the computer for what seems endless minutes as the fellows, now three of them, watch in silence. Occasionally he asks a question to clarify a detail. I feel like I am being tested. Have I given the right answer? Is he ever going to talk to me?

Finally he finishes his notes and swivels around in his chair. He knows his stuff. This man teaches ophthalmology at Harvard Medical School. We discuss options to relieve the dryness from Sjogren’s, which include blocking the ducts that drain tears from my eyes with tiny silicone plugs to increase the moisture level—like stopping up your bathtub, he explains—but apparently my ducts are “pouting” because they are too irritated and wouldn’t hold the plugs well. The whole idea is so new to me that I’m actually relieved not to have little stoppers inserted in my eyelids. It’s hard enough for me to let anyone touch my eyes at all or put in drops.

So, next best option: steroid drops to heal the inflammation and then a round of Restasis, which quiets the autoimmune process that has tightened the spigot. He’s forthright—the Restasis may not work to increase my tears, it takes four to six weeks to kick in, and it can be irritating. He recommends refrigerating the drops before inserting them to minimize side effects, also going a week on the steroid drops, first, to ease the inflammation. “And don’t rub your eyes,” he cautions. “It’s not your fault that you do, but it doesn’t work to produce more tears and it makes the irritation worse.”

Four hours have passed since I first arrived. I am exhausted from the long appointment and complicated prognosis, even as I appreciate the expert consultation, and overwhelmed by the prospect of yet another very expensive medication which may or may not be effective, just as our health insurance coverage is about to change to a higher prescription co-pay in July.

I find the cashier at the garage and catch my breath. Anything over three hours will cost a whopping $48. But by a miracle, the cashier assumes I’m a Mass General patient and only charges me $10, even without a stamped validation. Okay, thank you.

I take the elevator to the floor where I think I left my car. Then I get confused. Did I walk up an incline or down to get to the elevator after I parked? I’m watching the signs and not my feet. Whomp! I trip over a curb and smack wrists, elbows and knees on concrete. It hurts. A lot. But by another miracle, I’m able to get up without any severe pain that would indicate fractures or broken bones. Even as I feel tears welling that won’t come, I’m also amazed and relieved. I find my car and check all the points of contact. My gloves, pants and coat have protected me. No cuts, no scrapes, no torn clothes, just achey. All I want to do now is get home.

I turn on the ignition and the tire pressure gauge glows yellow. I first noticed it this morning, as I was leaving to drive into Boston. A quick visual check revealed no soft tires, and I didn’t want to miss the appointment, which had taken weeks to get. So I risked the drive in, through two hours of heavy traffic, and just made it to my appointment on time. Now the car rides evenly down the ramp to the exit, and I assume it’s just a finicky sensor, so I keep driving.

An hour later, closer to home, I call our tire dealer on my cell to see if she can do a quick check. No problem. And a good thing, too, because it turns out that I have a screw in the left passenger tire that is causing a slow leak, but the tire could have blown any time. It can’t be patched. I pull out my credit card to pay for the new one. “You were lucky,” she says.

I guess so.

Image Credit: Illustration from “God’s Providence,” from the 1705 English edition of Orbis Sensualium Pictus by John Comenius, considered to be the first child’s picture book, originally published in 1658 in Latin and German; Boston Public Library. Thanks to www.publicdomainreview.org.

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.