Living with Scleroderma

Reflections on the Messy Complexity of Chronicity

Mind

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.

#19

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Friday afternoon, Greenwich Village. Al and I squeeze into two remaining back row seats of a tiny, darkened theatre at the IFC Center just as the previews end. The acclaimed documentary is Manakamana, a mesmerizing character study of pilgrims traveling to and from a Hindu temple in Nepal via cable car. This is first on my list of things to do on our big weekend celebration of my 60th birthday and Mother’s Day. I’ve been looking forward to this trip for weeks. And I have a toothache.

Central Park Bass 5-11-14I have a rare complication of scleroderma: The roots of some of my teeth are resorbing. So far, I’ve had a couple of back molars extracted and a front molar replaced with an implant. My dentist and periodontist have been monitoring the relentless deterioration of several other molars, since. The worst one, lower left, has been hanging on for five years, occasionally oversensitive to cold, but manageable. If it had a name, it would be Grumpy. But it only has a number, 19.

A few days before our trip, 19 was acting up. I assumed it would calm down with careful tending, per usual. But as we drove closer to NYC Friday afternoon, the twinges were becoming more persistent. I tried to ignore it.

A couple sits shoulder to shoulder in the cable car. He wears a traditional peaked cap, shirt and vest, and carries a live rooster. She wears a red blouse and necklace of green beads. Her face is shriveled. She leans with her arm over the back of the seat, exhausted. He checks his watch. As the car rides higher and higher above terraced corn fields and sal forests, she brightens. It’s fun to go to the temple, she says. It’s good to go out when you can.

As we leave the theatre, I realize that not only is 19 aching, but the pain is also traveling into my left ear. I can’t believe this is happening. I’ve come prepared with my pharmacopia of meds, but I don’t want to deal with a rotten tooth on my birthday weekend. It’s drizzling. We sit on a bench outside a bakery to sort out options. I don’t want to ruin everything we have planned, and I certainly don’t want to waste time in an ER or try to find a dentist who may not take our insurance. So we agree that I’ll try to manage the pain with my meds, wait and see.

Later that night, after a great meal (despite 19) of wine and risotto, enhanced by Al’s magical ability to find interesting people (across from the cafe, at an artist’s opening in a church gallery, we shared Shabbat candle lighting and kiddush), I lay awake, unable to sleep in strange surroundings. My mind travels back to the film.

Three young long-haired men, all dressed in black, joke and fiddle with their digital cameras and cellphones as the cable car travels up to the temple. It feels like we’re going up steps, says the one in the middle. My ears keep popping. They pose for each other’s selfies and play with a scrawny kitten. People ski on hills like this in other countries, says another. What if the cable broke and we fell, laughs the third. 

Despite a fitful night, I get just enough sleep to go ahead with our plans for the day. So far, 19 is achey but manageable. It’s warm and the sun is shining. We attend Shabbat services at B’nai Jeshurun on the Upper West Side, then take a long stroll through Central Park, watch turtles sunning by a pond and wander through the Shakespeare Garden. I lie down on a bench while Al explores. People row on the lake, others play softball. Horse-drawn carriages clop along the road. The skies open up and we take refuge in the Museum of Modern Art–Al’s first-ever visit. I’m weary but elated to view these stunning works once again and watch Al’s enthusiastic response.

It’s still raining when MoMA closes, but we find a great restaurant right next to the Broadway theatre that is our final stop for the evening. I’m revived by the meal and we finish just in time to pick up our tickets for After Midnight, a revue of Cotton Club jazz from the ‘30s, starring Vanessa Williams. The music, singing, tap dancing, costumes are spectacular. Even from the very last row of the rear mezzanine, we can see everything perfectly. Exhausted, I sleep through the night, grateful that 19 has not gained the upper hand.

Three women sit in the cable car, dressed in bright colors and beads, their hair white, their faces gnarled like the bark of ancient trees. They talk about how their husband couldn’t come because he twisted his ankle while carrying a bucket of water he had drawn. They nod and look out the glass windows of the cable car, admiring the view. Life is so much easier than it used to be, one says. We had to struggle to survive.

Sunday is warm, beautiful. We enjoy a hearty breakfast in a little cafe, a stroll to the East River, and then head to the Brooklyn Botanical Garden for a glorious walk beneath blossoming cherry trees. Next door, at the Brooklyn Museum, we immerse in the works of dissident Chinese artist Ai Weiwei and so much more. I make sure to take my pain meds on time, to keep 19 in check. We reluctantly depart for home when the Museum closes its doors at six.

On Monday, I call my dentist and get a late afternoon appointment. The x-ray tells the story: There is a round gray shadow over the molar’s left-branching nerve. The resorption has exposed it. Nineteen has to go. It will take six to nine months after the extraction to complete the implant. Not surprised, I accept the bad news reluctantly, rub my achey jaw and drive home. At least the procedure will get split between this year’s remaining dental insurance and the next.

Two men sit in the cable car, each holding a stringed instrument and a bow. The older one looks out the window and recalls walking over the hills below to get to the temple, before there were even paths. We should tune up, he says to his younger companion. As the cable car descends, they watch treetops pass while playing a rhythmic folk melody, round and round and round.

 

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.

Logistics

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12:15, Thursday morning. I should be asleep by now. I have to rise at 5:10 to get ready and leave the house by 7:00, to drive to New Haven in time to catch the 9:28, so I can arrive in Manhattan in time for an afternoon of meetings, starting at Noon.

This is an experiment. I want to see if I can manage a one-day trip to NYC on business without wearing myself out. But, of course, I can’t sleep, too preoccupied with whether I’ve selected the right outfit for the predicted mix of rain and possible thunderstorms and 70-degree temperatures. If I dress too warmly to ward off dampness, I’ll end up sweating and getting chilled. And if I wear something too lightweight, I’ll freeze, especially if the office has turned on the AC.

When I wake after a meager four hours of sleep, I review the fiber content of the outfit I’ve chosen—a white sweater top that is a mix of silk and cotton, an ivory crocheted cotton cardigan and black wool crepe pants—okay, I’m good. Comfortable and professional, made from natural fibers that won’t trap perspiration, with loose layers to allow plenty of air circulation.

Next step, hands. The night before, I cut all my bandages and dressings to be sure I could take care of my finger ulcers in five minutes instead of the usual twenty. It’s absolutely essential to cover every possible skin crack when I travel, but if I feel pressured by time, I can get sloppy and have to redo the dressings. With everything ready, I relax and neatly prepare my fingers for the long day ahead.

As I do my stretching exercises and get dressed, I rethink my plan for my laptop and decide to shift it from a carrying case to the center zipped compartment of my large purse. The laptop is lightweight, and I don’t want to fumble with extra stuff to carry when I make purchases. I test the arrangement. The purse is roomy enough for easy wallet retrieval, even with the laptop, and remains securely over my shoulder. Check.

After a breakfast of scrambled eggs and toast—need to be sure I have enough protein in my system to stay awake for the two-hour drive ahead—I head out the door. Only 10 minutes behind schedule. Okay so far.

That is, until it starts raining. Not just raining, pouring. And there’s fog, too. I pull over at a rest stop on the Mass Pike to check traffic around Hartford on my cell phone. I have to drive around the city during rush hour on my way to New Haven. There are two ways to go, and I select the one with the least congestion.

This plan works until I get closer to Hartford and find myself crawling at 11 mph on Route 84. Should have left earlier, but no use getting upset now. Nothing to do but sit out the traffic jam. I decide to catch the next train, if need be, and let my clients know I’ll be a half-hour late. I am not going to run through the station and risk getting winded from what my physicians think is exercise-induced pulmonary hypertension. I had a recent, unnerving episode, and I don’t want to push it.

Once the traffic eases and I get onto 91S, I drive as fast as I can without exceeding the speed limit by too wide a margin. More traffic back-up on the exit ramp to New Haven’s Union Station, but, miraculously, I find a space in the parking garage just one level up. There’s a covered walkway to the station, and the restroom is right down the hall, conveniently located for a quick pit stop. Time, 9:12.

Okay, now I just have to buy my ticket and find the train. Only a few people in line at the counter. Ticket in wallet, I walk briskly to the gate, up a long flight of stairs. The train is waiting. Plenty of seats, still. I settle down, take off my raincoat and catch my breath. Made it! Seven minutes later, we pull away from the station.

By the time we reach Grand Central, all traces of rain are gone. I switch to sunglasses as I climb out of the Union Square subway station. People stroll and hustle in shirtsleeves, shorts, flip-flops. Pink crabapples abound. Somewhere, someone is making a loud May Day speech about workers’ rights. Delivery trucks battle for curbside parking. A siren wails down another block. The air smells of car exhaust and felafel and fresh doughnuts. I unzip my raincoat, slip my collapsable umbrella into my purse and head toward West 17th Street. It’s going to be a good day.

Photo Credit: JefferyTurner 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.