Living with Scleroderma

Reflections on the Messy Complexity of Chronicity

Smell

My Everest

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Much has happened in the weeks since I last wrote. I had hoped to share a simple, upbeat travelogue about our wonderful August vacation to Iceland and Norway upon our return a week ago, Sunday. The trip was, indeed, magnificent. But life is not simple, especially with scleroderma. My severe ulcers that I have written about for months took a serious turn for the worse. As I write on Sunday afternoon, I am facing major hand surgery Monday morning. Here is what happened:

For more than three decades, I have been managing digital ulcers on multiple fingers. I have gone through infections requiring IV infusions, more rounds of antibiotics than I can remember, and months — sometimes years — of waiting for ulcers to heal. I have learned how to compensate and find creative solutions for handling daily tasks. I have learned to live with people’s reactions to my oddly shaped, bandaged fingers. But this spring, something went terribly wrong. My ulcers on five fingers formed large scabs, or escars. They were hard and thick and dark; when tapped, they felt like turtle shells.

As I have been documenting in this blog, for the past three months I’ve been trying to find a way to get them to heal. As the escars have receded, part of the natural healing process, I have sought out state-of-the-art wound dressings and consulted with wound specialists, with varying degrees of success. When I didn’t understand, however, was the real risk for the ulcers to work their way down to bone.

The Wednesday night before we were set to leave on our trip, I was changing my dressings as usual. But suddenly, the escar on my right pinky (the ulcer that had been infected a few months ago) lifted up to expose the knuckle bone beneath. I was terrified. We called the hospital’s Wound Center and spoke to a nurse practitioner who was covering for the night. She suggested that unless I started running a fever, it was safe to wait until morning to get medical attention. This was good advice, because the last thing I wanted to do was spend five hours in the ER.

On Thursday, Al came with me to see, first, a nurse practitioner in the Wound Center (no surgeons were on that day), and later in the afternoon, an excellent orthopedic hand surgeon/plastic surgeon. He looked at my pinky with its exposed knuckle bone and gave me the news straight up: because of my scleroderma, options such as skin grafts would not work, and the only solution was amputation.

I was in shock, as was Al. Never in my wildest dreams had I anticipated this path. He said the escars on my other fingers were “classic scleroderma,” and that they were covering dead skin. I do not know if there had ever been any hope for real healing once the damage had been done. I asked him if it was still possible to travel. He said yes, so long as I kept the wound clean, moist and protected, and was on appropriate antibiotics. It would not change the prognosis nor make things worse.

By the time we got home, I had made up my mind to take the risk and go ahead with the trip. Even though it was insured (this time, I had met the deadline for getting travel insurance that would cover us for pre-existing conditions), we had been planning this wonderful trip for months, and I just wanted to go. But then I changed my dressings that evening. Suddenly, the escar on my left index finger began to recede to expose the back of the knuckle. I was in hysterics. How could this be happening? I told Al that I couldn’t imagine traveling now. He said he would go with whatever I decided. He went to sleep, and I went downstairs to sit on the living room couch and try to think.

I emailed a couple of friends for help to sort it out. I realized after a few hours of agonizing that I was channeling my mother, who was a very anxious woman who never took risks.  She came to this country from Germany in 1936 at age 14, escaping the trauma of the Holocaust, but never free of what might have been. Her fears of danger kept me from exploring the world when I was young, with the exception of a two-week tour of Europe that my mother’s mother paid for when my sister and I were in college — my grandmother’s way of exposing us to the world that she missed so much.

Facing the inevitability of losing at least one finger and maybe more, I knew that I needed the inspiration of beautiful landscape in order to face what was to come. I wrote an email to the hand surgeon and asked him if the risk of exposed bone was additive with more fingers involved, and if the prognosis would change if we went ahead with the trip. I received a thoughtful email back in the morning that explained that each finger had to be considered independently, and that the risks and prognosis remained the same. I also checked with my ID specialist about antibiotics, and he said that what I had already planned to take with me was appropriate. I told Al that I wanted to go. He gave me a big thumbs-up and a big hug.

And so, we went, first to Reykjavík for two days, and then on to Norway. We spent five days in Bergen, on the southwest coast, home to some of the country’s most famous fjords, then took a scenic 7 1/2 hour train ride to Oslo, stayed overnight, and flew to Tromsø in the Arctic Circle. There we stayed for three days, and then wrapped up our trip in Oslo for our final weekend.

The scenery was everything I’d hoped for and so much more. Iceland is in constant formation, with active volcanoes, geysers, thundering waterfalls and visible tectonic plates. The mountains are sharp, craggy and snowcapped, a visible reminder of the earth’s power to force rock skyward. We saw puffins and glaciers, smelled the sulfur of hot springs, watched Icelandic horses and sheep cropping emerald grass, marveled at moss reclaiming lava fields.

In Norway, we immersed ourselves in beauty, from towering green and rock mountains bordering calm saltwater fjords to the art of Edvard Munch (MOOnk). We drove through the world’s longest tunnel (25 km) with its sapphire blue lighting, stared slack-jawed at thousand-foot waterfalls nearly everywhere we turned, rode the scenic Flam railroad up and down a mountain. Staying in wonderful Air B&B flats, we ate many of our meals at home to save some money (restaurants are very expensive in Norway), but treated ourselves to four exquisite dinners out.

For all this, however, travel was very strenuous for me. It took about two hours in the morning and the same at night to change my dressings. Our supplies included 700 cloth bandages, 32 sheets of silver alginate dressing, a cream I had discovered online that includes hyaluronic acid and is intended for radiation burns, lidocaine gel, Q-tips and more. Along the way, three more ulcers receded to expose bone. It was as if all my ulcers had hit a tipping point within the same week. Sometimes changing the dressings was so painful, it reduced me to tears — and I am not one who cries easily. Al was my rock, so attentive and supportive. He would read to me of Norse mythology as I went through the tedious process of tending to my fingers, help me get dressed, take my arm to make sure I didn’t fall, comfort me when fears overwhelmed me.

Throughout the first week, as my ulcers deteriorated, I agonized over whether I had made the wrong decision. Al said let’s take one day at a time. Even though we often didn’t get out until afternoon, we made the most of each day. New friends in Bergen gave us a grand tour of the fjords and invited us to their home for Shabbat dinner. When I dropped my wallet without realizing it, in the pouring rain as we were getting on a bus, a young man tapped me on the shoulder and returned it to me.

My self-doubts finally dissipated when we reached Tromsø. The idea to go there had been mine, a major challenge with my Reynaud’s. The Gulf Stream keeps temperatures in the 50s Fahrenheit in the summer — not the warmest climate for me, but still an opportunity to get as close as I ever will to the North Pole. Our first full day there, the rain that punctuated our entire trip cleared as we were riding a cable car to the top of a small mountain that overlooked the city, which is on an island. As I walked out on the top of the mountain, with its spectacular view of huge, jagged, snowcapped peaks in the distance, I was suddenly overwhelmed with emotion. I had made it, here to the Arctic Circle, with my aversion to cold and my crazy hands. This was my Everest. In that moment, I regained my courage. I knew I could face what was coming next.

The following day, the sun was bright and the temperatures in the 60s. We had signed up for a five-hour tour of a neighboring island, and with luck, ended up as the only passengers on the trip. Our tour guide, Pedro, who had come from Lisbon to Tromsø in search of the Northern Lights (only visible in late fall and winter), proved to be a wonderful companion and conversationalist. We covered everything from Norwegian geography to European views and fears of the Trump presidency. The highlight of our day was eating lunch on a cream-colored coral sand beach (there is a coral reef in the Arctic — who knew?), talking politics, drinking rice milk hot cocoa and eating delicate Finnish homemade cookies.

As the end of our travels drew near, my apprehension began to mount again. Fortunately, our seven-hour flight back from Gatwick, London, was on a Boeing 787 Dreamliner, the newest and most advanced jet in the sky. Better humidity, improved air pressure changes and even comfortable seats in economy made it a much easier flight than I had anticipated.

This week, reality hit home hard. We saw the hand surgeon late Monday afternoon, and his assessment was that I would need what’s called a hand “revision” that would involve trimming five of my fingers. His goal was to leave as much length as possible. But, in all probability, my right pinky and left index finger would be reduced to one digit stumps. The other three — left middle finger, right middle finger and right ring finger — would involve trimming exposed bone and leaving open wounds in the hopes of preserving length as skin regenerated. As it turned out, however, his OR schedule was booked through September. He referred me to an experienced colleague with similar credentials, who had an opening on Monday.

We met the second hand surgeon on Thursday. He is meticulous, thoughtful and thorough. With a very sober face, he told me that this would be the first of “many surgeries” because of the complexity of the damage. He described my situation as “serious scleroderma.” He is concerned about healing with my poor circulation and suggested the possibility of having a sympathectomy done at a later point to increase blood flow to my hands. He agreed with the first hand surgeon that I should have hyperbaric chamber treatment in the wound center to help improve oxygenation with my blood and speed healing. On Monday, he will debride all the ulcers, trim back exposed bone, and determine the best way to deal with my two broken knuckles. He will also take pictures and share them on a list-serve for hand surgeons to get more input about next steps.

I was very frightened and distressed after this meeting, even as I feel fortunate to have such an expert taking care of my hands. He has  an excellent reputation, especially for follow-through, and is in high demand. The last few days have been an emotional roller coaster ride, softened by love and support from family and good friends.  I wax and wane between fear and grief over losing part of five fingers and just wanting to be rid of these painful digits that no longer work.

My surgery is scheduled for first thing Monday morning. By the time you’re reading this, it will all be over. I do not know if I will be up for writing what happened by next week, but I will certainly share the next chapter when I am able. Let the healing begin.

Post-op P.S.:  Thank goodness, the procedure went better than expected. My circulation exceeded the hand surgeon’s expectations. Still have all 10 fingers, for now. He put temporary pins in my right pinky and left index finger to stabilize them and buy me some time. He is sharing pictures of my fingers on an international list serve for hand surgeons, so we will have input from the best of the best for next steps. Pain management will be the next challenge, but I am so grateful and relieved to have made it through this procedure. Thank you for sharing my odyssey, and thanks to Al for being my post-op scribe and life’s partner.

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.

While the Soup Simmers

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I’m writing on Sunday night, as the Egyptian potato soup simmers on the stove and our community radio station plays a Middle Eastern mix. I’ve been cooking all day for our Monday night Passover seder, and I’m feeling good. A lot better than I anticipated this morning, when I woke with pain in my ulcers, an aching foot and one thought: How am I going to get through the cooking marathon today?

I groused at Al. I rubbed my temples. I studied the long list of fruits and vegetables that I needed to buy before lunch and realized I’d forgotten to ask Al to pick up one key ingredient from the kosher market in Brookline (an hour’s drive from home) several weeks ago.

He suggested checking the Passover aisle at our local supermarket, just in case they had those kosher-for-Passover hearts of palm. I agreed, then thought of an alternative in case they didn’t. I knew Al stood ready to serve as sous-chef, as need, for all the chopping and peeling ahead. Time to dive in.

To my astonishment, when I got to the store, the Passover aisle was still well-stocked, including hearts of palm—three cans, even. I moved on to the second supermarket and filled my cart with fresh strawberries, blueberries, blackberries, bananas, a mango, avocados, cauliflowers, leeks, romaine lettuce, potatoes, beets, onions, garlic, celery, parsley, asparagus, baby spinach, eggplants. At the check-out, the cashier admired my choices and told me how much he loves vegetables (except eggplants). I told him how to enjoy beets in a salad (add gorgonzola and toasted walnuts).

By the time I got back home, Al had switched over our kitchen to all of our Passover dishes—the culmination of several days of cleaning and preparation. We went out for a quick lunch, and then I began cooking in earnest. The night before, I’d already started the pickled salmon, which marinates for a couple of days. Next up was curried eggplant. I was able to do all the peeling and chopping myself while Al worked on the yard.

Then came the Egyptian haroset, a mixture of dates, golden raisins, ground almonds and sugar syrup. Only one problem: when I placed the mixture in my little Passover food processor, it wouldn’t turn on. I tried another electrical outlet. No go. I asked Al to try it. Maybe I hadn’t aligned it properly. Zip. Four o’clock in the afternoon, and it was time for another run to Target.

I opted for an immersion blender and picked up a few other cooking items to make life easier for the rest of the week. Before we left for dinner, the haroset was well blended, cooked to perfection and chilling in the refrigerator.

By 7:30, I was back in the kitchen, separating nine eggs for the apricot sponge cake and cursing at the little pieces of eggshell that had dropped into the whites. But I persisted. Al helped me fold the meringue into the batter, the one part of the recipe I can no longer do.

Now the sponge cake rests upside down in its tube pan, cooling overnight. The asparagus are happily plumped with water, standing tall in their pan until it’s time to steam them tomorrow afternoon. The potatoes and leeks and celery and garlic and turmeric, salt, pepper, bay leaf and water have finished simmering in the time it took me to write, and the lovely mix is now cooling in the 70-year-old white enamelware that was once my mother-in-law’s Passover soup pot. Just need to add the fresh lemon juice before serving.

All that’s left for tomorrow are the spinach-cheese patties, the avocado-tomato-hearts-of-palm-pesto salad, the roasted cauliflower, the boiled eggs and the seder plate. That’s the easy stuff.

The prospect of cooking for Passover, with my once-a-year set of dishes, the crazy schedule, and the inevitable stuff that goes wrong, always overwhelms—especially because the holiday falls in the spring, when my ulcers are at their worst. But somehow, it always works out. And tastes great. And provides a beautiful setting for our seder. This year, more than ever, I am grateful that I can still make a splendid feast for family and friends, and focus on what really matters: what it means to be free.

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.

Spring Tease

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I bought a bouquet of Irish daffodils on Friday, three bunches of slender stalks with buds barely open. By Sunday, they had bloomed, a vase of sunshine in our dining room. Outside, snow still covered the ground. I bundled up in my long winter coat, wool hat, scarf and mittens to brave the damp chill for a half-hour walk around the neighborhood. Winter is clinging, white-knuckled, to Central New England. It’s high time to let go.

In some ways, the spring-masquerading-like-February makes me feel like a bear that is groggy, awakening from a long winter’s hybernation. My finger ulcers are simply not healing, and they smart when I change bandages twice a day. My metabolism feels sluggish from the cold. It’s hard for me to get going in the morning, when the sunlight spells spring but the temperature remains in denial. I really had to force myself out the door on Sunday, but I was glad for the reward of a cleared mind.

But winter cannot supress spring forever. As I walked, I noticed a misting of pale green about some trees. The Callery pear in front of our house has white buds, too. Near the melting edges of snow, tender green blades of grass poke skyward. The earth smells muddy and ripe.

There is birdsong, too. On Sunday, beneath overcast skies, the crows dominated. But the day before, as I walked up the street, dozens of melodies filled the air. Exuberant birds trilled, tweeted, cooed. I wondered what they were saying to one another, and I was glad for their company.

So, I await warmer weather with impatience, yet reassured that nature’s rhythms prevail. Until the snow melts, I’ll fill my vases with daffodils and let the sunshine in.

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.

Guilty Pleasures

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That old saw about New England weather—just wait a minute, it will change—holds true now more than ever. After what seemed like an onslaught of snow and ice, we suddenly were treated to a major thaw. Temperatures last week rose to the high 60’s. Only lumps and clumps of snow remain, blackened by car exhaust and grit. Friday afternoon, I went food shopping wearing just a sweater to keep me warm.

We’re back to seasonable 40’s for a few days, then more balmy temperatures. Some find this anxiety-provoking. Record-breaking warmth is more evidence that climate change is real. I worry about this, too. News reports are frightening: severe drought in some parts of the world versus severe flooding elsewhere, melting ice caps, reduced ocean oxygen levels, bleached coral reefs, declining biodiversity, extreme storms—how will our precious planet survive?

But I must admit, on a purely personal, very selfish level—I really enjoy the warmer weather. I can’t help it. I just feel so much better when the temperature goes above 60 degrees Fahrenheit. My whole body relaxes. My ulcers heal. And I don’t have to go anywhere beyond my front door.

Don’t get me wrong. I believe we must do everything possible to slow the trend of global warming. There is far too much reliable scientific evidence that without serious efforts to reduce human production of greenhouse gasses, the ice caps will continue to melt, ocean levels will continue to rise, too many species will die before they can adapt to rapid climate change, food production will be disrupted . . . the list of dislocation and natural disasters goes on and on.

Knowing all that, doing my best to recycle and reduce my carbon footprint and support public policy that promotes responsible environmental stewardship . . . I still can’t help it. I won’t go so far as to wish for oceanfront property in Central Massachusetts. But I’ll take a warm day in February over ice and snow, any time.

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.

Image Credit: Miriams-Fotos

In 3-2-1

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I stay up way too late most nights watching late night comedians. Since we live on the East Coast, this means I’m getting to bed around midnight. My evening routine is prolonged by tending to all the bandages on my fingers—up to six ulcers at present, plus one on my left ankle—so my excuse is that the shows keep me company while I’m taking care of my hands. But in all honesty, I rely on satire to keep my sanity.

My favorite is Stephen Colbert. Al’s, too. So when Al suggested that we mark our anniversary this year by a trip to NYC to see a live taping of The Late Show, I readily agreed. We were married in December 32 years ago, but due to scheduling conflicts, our first opportunity to go was last week.

And go, we did. We decided to make a mini vacation of it, booking a four star hotel on Park Avenue at a January discount, scoring half-price tickets to a Sunday afternoon off-Broadway show, enjoying great food and wonderful art museums on Monday and Tuesday. But the highlight of the trip was our pilgrimage to the Ed Sullivan Theatre for Colbert on Monday afternoon.

Now, as children of the ’60s, it was exciting enough to be at the very spot where the Beatles made their American debut. The theatre features architectural filagree that gives it a period flare. It’s located on Broadway between West 53rd and 54th Streets–the latter also designated as Señor Wences Way, a throwback to that wonderful, corny feature act on the Ed Sullivan Show that we loved as kids.

But it was also fun just to be with other Colbert fans as we waited outside, joking and speculating about the program as we stamped our feet and huddled against the cold. The priority ticket line formed at 3:00 p.m. We arrived shortly after and quickly made our way through the check-in, staffed by friendly red-jacketed twenty-somethings armed with iPads and headphones, who made occasional announcements about what to do and where to go. A nice couple offered to take our picture in front of the marquee, and we returned the favor.

By 3:45, rehearsal was over and we were finally allowed to enter the warm theatre lobby. More waiting and waiting in a long, snaking line beneath large TV monitors playing excerpts from previous shows, though the sound was muted. From time to time, one of the staff would fill us in on next steps and rules: turn off all cell phones, no food allowed in the studio theatre, and—around 4:30—now’s the time to use the bathroom, because once you’re seated, there are no bathroom breaks.

Wait, what? I’d figured that, if I had to go, I’d be able to do so during a commercial. But, no, only if it was an emergency—and no guarantee you could be reseated. Now, this was potentially a major issue for me. I can no longer go long stretches without a trip to the bathroom. My bladder just doesn’t empty efficiently. So I joined a line of other women, waiting for a stall in the Ladies’ Room, and hoped I could squeeze out the last drop. Fortunately, we’d eaten lunch about two hours earlier, and I hadn’t had much to drink.

Back in line, I distracted myself by chatting with some of our neighbors, flexing my ankles and feeling grateful that I was wearing compression knee socks, so that my feet wouldn’t swell from all the standing around. Finally, shortly after 5:00, it was time to be seated. The red jackets were very experienced at crowd control, and we efficiently filed into the main floor. Lo and behold, the center section was full, so Al and I found ourselves guided toward the third row of the right-hand section, directly in front of Jon Batiste’s Steinway concert grand piano. Al was in heaven.

Here we were, with a great view of the Late Show set, so familiar from our TV at home. We gawked and chatted with our seat mates (mine was a Lutheran pastor from Saskatchewan, here with friends for her first visit to NYC), listened to more instructions about our role as audience (enthusiasm and energy are essential for the performers as well as the 2.5 million folks watching later tonight), practiced standing and cheering, warmed up to the warm-up comic, clapped and bopped to the outstanding jazz of Jon Batiste and Stay Human, and then, finally, screamed our heads off, just like those Beatles fans fifty years ago, when Stephen Colbert ran out on stage to greet us.

He was genuinely warm in person, very down-to-earth, as he fielded a few questions from the audience with his quick, dry wit. Then it was time for the taping to begin. We could watch the cold open on the video monitors, then Colbert ran out on stage again, this time as part of the show. The boom camera swept the audience, and we were off to the races.

Time zipped by. There was a surreal quality to the experience, watching Colbert perform for the four cameras that surrounded him in his opening monologue, even as he fed off our energy. There was a pause for him to switch from his suit jacket to a Dad sweater for a skit with guest Leslie Mann, another pause because one of the lights wasn’t working properly, casting a shadow on the couch where they were to sit. “The Russians must have hacked our set,” he quipped.

The band played on during commercial breaks (how I wish that were the case when you watch on television–they are such amazing talents). Lewis Black and Dan Levy rounded out the program. We stood and cheered on cue (when the stage manager waved his rolled-up script in the air). Colbert’s wife made a surprise appearance to roars of approval.

And then it was over. I’d been so absorbed, I’d forgotten all about any bathroom jitters. Al and I looked at each other. We didn’t want it to end. Despite the cold, we walked all the way from Times Square back to a little Italian restaurant near our hotel, where we enjoyed a fine dinner and live piano music. Later, we watched the show again in our hotel room, to see how it was edited and, of course, to see if we made it onto the tape. And there we stood, cheering in the crowd after one of the early commercial breaks! I finished bandaging my fingers, and we went soundly to sleep.

All in all, it was a wonderful anniversary celebration—a great break, a much-needed chance to recharge, a gift of resilience. And, oh, did I mention? The Colbert tickets were free.

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.