Living with Scleroderma

Reflections on the Messy Complexity of Chronicity

Mind

Don’t Tell Me the Odds

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Twenty-eight years ago this month, I first heard the word scleroderma. I was sitting in a rheumatologist’s exam room, a few weeks after returning from my honeymoon with Al, trying to wrap my head around the fact that I had some kind of autoimmune disease that was at the root of all the strange symptoms I’d been experiencing for the past three years.

We talked about my history of extremely cold, blue hands; the swelling fingers, migrating joint pain and photosensitive skin; the fatigue that made me feel as if someone had turned off a switch in my brain; the positive antinuclear antibodies blood test that confirmed my immune system had gone haywire and was assaulting my healthy tissue.

My rheumatologist, who has since become one of my most stalwart partners in combatting this mysterious and complex disease, thought I had one of three possible illnesses: rheumatoid arthritis, lupus or scleroderma. I went home, emotionally numb, and cried with Al for a long time.

This was before the days of the Internet. I took out a book from the library about lupus to try to learn more. Scleroderma sounded so strange, so remote, I didn’t even entertain the possibility. Plus, I couldn’t find any information.

Slowly, as the skin on my hands and face began to tighten, the S-word became more threatening. I picked the brains of our physician friends. They were supportive and grave when I told them the looming diagnosis. Back then, the mortality statistics placed the odds of survival at about seven years from onset. I chose to ignore this.

But I had to find out more. With Al, I attended several meetings of a local scleroderma support group. Here I met people with much more advanced disease—mostly women, some with fingers bent like claws; others with shining, mask-like faces, unable to close tightened lips over teeth; still others with only nubs left of their fingernails.

The conversation focused on treatment options, presentations by local rheumatologists about the many complications of the disease, shared angst. I lasted about four or five meetings and then told Al I didn’t want to go back. Everyone meant well, but the energy was so negative, I couldn’t continue. So much of the conversation focused on each person’s individual problems. I found it terrifying. I didn’t want to identify with these people. Was I really going to end up like them?

In the years since, I have come to appreciate the value of support groups, which offer a unique opportunity to commiserate, share insights and learn from fellow travelers on this strange journey. But for the newly diagnosed, any encounter with information about scleroderma—whether in person or online—can feel like drowning in a tsunami.

There is so much that can go wrong: tight skin, painful joints, ulcers and infections, kidney failure, pulmonary hypertension, numb hands and feet, lost mobility, fatigue, disfigurement. And that’s just the short list.

Today you can read for hours about the many permutations, complications, risks and challenges of scleroderma with the click of a mouse. The Internet is loaded with facts about the disease and latest treatments, commentary from doctors and patients, current research and disturbing images of worst-case scenarios, as well as plenty of bogus treatments and BS. If you don’t know how to filter it, if all you have is a diagnostic label, you can scare yourself even sicker from worry about all the bad stuff that might happen.

But scleroderma is a disease that manifests uniquely in each individual. That’s why, after three decades, I read to stay informed, but for the most part, I don’t immerse myself in scleroderma content, even as I write this blog each week. To some extent, this is because I’ve learned enough from experience and the gift of excellent physicians that I have a solid working knowledge. But it’s also a protective habit I developed years ago—to focus on what I need to know to get appropriate treatment and stay away from the long list of what-if’s.

This goes to a basic fact of living, no matter what your health: If you knew all the bad things that were going to happen to you over the course of your lifetime, you’d never get out of bed. If someone had told me 30 years ago, when I first started having symptoms, all the difficulties that were to come, I don’t know if I would have been able to cope. Time, experience, quality medical care, supportive family and friends, inner resources you didn’t know existed—all combine to enable you to manage challenges you never thought you could handle.

Years ago, my high school boyfriend’s mother said to me, “No one goes through life unscathed.” I was only 17 and had no idea how to respond, but I was deeply shaken. She was right, of course. But she didn’t tell me the other piece of that truth—when the worst hits, you learn what you’re truly capable of.

I wish I had never heard of scleroderma. And yet, I’m much stronger for it. I wish there were other ways to have come to that self-knowledge. But here I am. Some days I get scared about what still might happen, where this all will lead. I make lists and pepper my doctors with questions. Then I try to focus on the present moment, which is all we really have, and do my best to make the most of each day. I remind myself to be grateful to have made it this far. And keep ignoring the odds.

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.

Photo Credit: Lynn Friedman via Compfight cc

Outtakes

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

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

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

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

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

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

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

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

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

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

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

Evelyn Herwitz blogs weekly about living fully with chronic disease, the inside of baseballs, turtles and frogs, J.S. Bach, the meaning of life and whatever else she happens to be thinking about at livingwithscleroderma.com.

Shall We Dance?

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When I was in ninth grade, I talked my best friend into taking a modern dance class together. We met one afternoon a week in the gym of a nearby elementary school, where we wore black, short-sleeve leotards and danced to Crosby, Stills & Nash’s Wooden Ships.

The opening moves involved balancing on your right foot, with your left leg bent to the side, toe touching right knee, your arms raised and hands pressed together to form a diamond shape around your head, as you swayed back and forth. This I can barely do anymore. But I still remember the dance, leading the front row in a recital, jumping into an impromptu rendition at a friend’s pool party when the song floated into the mix, moving with ease and grace to the music.

I always wanted to take dance lessons as a kid, but our mother, like most of her generation, was not interested in playing after-school chauffeur or paying for multiple classes. I had violin lessons, and that, in her book, was enough. So, as an adult, I began to make up for lost time and took a dance class here and there–intro ballet, a little jazz, even some disco back in the early ‘80s. At some point in the ‘90s, Al and I took a few ballroom classes.

Still, I was just dabbling. Every time I’d go to a dance performance, I’d daydream about being up on stage. But my body was getting stiffer and weaker, from scleroderma and a general reluctance to exercise. I’ve never been athletic or very coordinated, and about my only exercise, for years, was trying to keep up with my growing daughters.

Things were getting so bad that I was beginning to hunch up like a little old lady, always hugging myself against the cold that causes my Raynaud’s to flair. So about five years ago, I had a talk with my rheumatologist about how I really needed to get regular exercise. He advised me to pick something I really loved doing, because I’d have an easier time sticking with it.

My first step was Pilates, which has become a weekly life saver, strengthening my core and improving my flexibility and range of motion. Then I decided to try and dance again.

I started with an evening intro jazz class at a North End Boston studio. It was a near disaster. The class was far from introductory, more like an advanced beginner level. The teacher was beautiful, very talented and rapid in her instructions. The other students memorized the floor movements and combinations easily. I could barely get my body to move the right way, let alone remember all the steps. It wasn’t just my lack of experience or weak muscles. My brain simply couldn’t hold all the information and communicate it to my arms and legs.

At the end of the first class, I went to the teacher and started crying, because I so wanted to dance but found it so confusing and difficult. She listened sympathetically and gave me some great advice: Don’t compare yourself to others. Focus on one thing each class to improve. This is about your personal best. It takes time to learn.

For the next two years, I kept at it, switching to a class run by the studio director that was a bit slower and more to my liking. But still, even as I loved the music and moves, jazz was just a bit more than I could handle. So I decided to switch to a basic modern class at a different studio, this time in Cambridge. This worked well for several sessions. Whereas jazz, like ballet, embodies flight, modern is all about gravity. Fewer leaps. More feeling your weight, connecting to the floor.

And yet, after a couple more years, I found that I still couldn’t keep up. Too much balancing on toes and one foot. As my feet have deteriorated from scleroderma, the fat pads have thinned significantly, and I just can’t put that kind of pressure on the balls of my feet. Even slipping a pair of soft orthotics into an old pair of jazz shoes wasn’t enough to help me balance.

So for the past six months, on Thursday nights in Cambridge, I’ve been taking Middle Eastern belly dancing. Now, it took me a while to try. I’d been contemplating the idea for at least a year. There are so many lewd stereotypes about belly dancing that I had to get past. But I was drawn to the form by the basic fact that, at an intro level, it doesn’t involve balancing on your toes. And I liked the music. And it sounded like fun.

My expectations have been met, and then some. The music is great. My teacher is a wonderful dancer and performer, very experienced, excellent at breaking down the many isolation movements that are part of this most graceful and sensual art form. By the end of the hour, I’m usually sweating from the workout. It dovetails well with Pilates to strengthen my core muscles.

Best of all, I’m beginning to be able to move my body in ways I could have never imagined were still possible. I can snake my arms and wrists. I can swirl a veil. I can do figure-eights and hip bumps and shimmies. I can feel the music and move with the rhythm. And I feel good about my full figure (belly dancing is the one form of dance that rewards you for those extra inches).

In fact, it’s that last point that is perhaps the most important. Scleroderma is a disease that locks up your body, distorts your face and hands, and makes you feel ugly. Belly dancing, reconnecting with your sensuality, is about as far from that as you can get. I can’t think of a better antidote.

Evelyn Herwitz blogs weekly about living fully with chronic disease, the inside of baseballs, turtles and frogs, J.S. Bach, the meaning of life and whatever else she happens to be thinking about at livingwithscleroderma.com.

The White Glove Test

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Years ago there was a commercial—at least, I think so, because I can’t find it on YouTube, which is, of course, the repository of all things ephemera—that involved a nosey neighbor who wore white gloves. She would come to visit, and while her hostess wasn’t looking, check to see if there was any dust on the furniture by swiping a tabletop with one of her gloved fingers. Heaven forbid if she found a smudge and you failed The White Glove Test. Your reputation as a proficient homemaker would be ruined.

That was, of course, back in the day when women wore white cotton gloves to go shopping and white kid gloves to a cocktail party. My mother used to outfit me and my sister in white cotton gloves, black patent leather shoes and roll brim hats with grosgrain ribbons that dangled down our backs when we went into New York City for an outing. You could walk into any department store and find a variety of fine gloves for every occasion, and a saleswoman who knew how to fit you.

No more. So when I recently had the brainstorm to use white cotton gloves at night to relieve my ulcer-ridden fingertips from the bandages that have begun to shred my skin like cellophane—an insidious problem that seems to have arisen from some kind of reaction to the adhesive in the only bandages I can tolerate otherwise—I went online.

This is why I love the Internet. Type in a phrase like “white cotton gloves,” and you discover a variety of options plus an anthropological snapshot of how our culture has evolved from the formality of the early ‘60s to our denim-casual style of 2013. No dress gloves in the top ten. But you can find white cotton costume gloves from party stores and white cotton gloves from parade uniform suppliers.

Dress-up resources aside, the best option, for my purposes, were white cotton gloves from a photography supply site for handling archival film and other materials that shouldn’t get smudged by the natural oils in your skin. The kind of oils I don’t have enough of to keep my fingers lubricated and my skin elastic.

These lightweight gloves cost about eight bucks for a dozen pair. They are so thin, I can text with them on my iPhone. And they’re hand-washable.

Best of all, they seem to be helping my skin to heal. My new routine at night, after I shower and bandage any ulcers that are open wounds, is to slather my other fingers with a really good moisturizer, dip the most delicate tips in Aquaphor ointment (which I’d normally bandage for protection from overnight cracking) and pull on the gloves.

It feels absolutely luxurious to get out of those bandages, even while I sleep. And the treatment is working. In just a few days, I went from seven heavily bandaged fingers to four. This is a major accomplishment. Especially in January in New England.

If I’m not going out of the house, I can even cut back to two bandaged fingers, and either wear hand lotion under a pair of the white cotton gloves to protect my fingers while I type (I cut down the fingers on one pair and stitched them on my sewing machine to fit my stubby fingers for day use), or swaddle the most sensitive tips in white cotton-polyester finger cots—like little white socks for your fingers, which I also found online.

Now, you may ask, why didn’t I think of this before? I’ve spent a small fortune on bandages, ointments and dressings to protect my fingers over the last 30 years. Honestly, I don’t know. Probably because I’ve been so focused on finding the right bandage that it didn’t occur to me. Also, my finger skin is extraordinarily fragile because my circulation is lousy, even with medication. And I’ve had far too many infections. So I’ve always erred on the side of overprotection at the first sign of damage. What spurred this latest experiment was a conversation with a wound care specialist. When I showed him my shredding skin, he said you need to get out of the bandages. His ruminating about silicon finger protectors got me thinking about low tech solutions, and here I am.

The system is not foolproof. Of course, nothing with scleroderma can ever be that simple. I have to continue to be vigilant, to catch any skin cracks or scaling, and just keep moisturizing during the day. The finger cots really help with this, because I can moisturize any damaged skin, slip on the cot, and keep going.

Right now I’m typing with four bare fingers on my right hand. One, my ring finger, has had an ulcer that has refused to heal for at least six years. I’ve gone without a bandage or open sore on that fingertip for over a week. In January. In New England.

So, miracles can happen. I’ll continue to bandage up against dirt, bacteria and the elements when I go beyond my doorstep. And I know that my ulcers will continue to wax and wane. But at least I can get some relief at night. And who knows. Maybe white cotton gloves will come back in style someday.

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.

Grateful

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One year ago, on New Year’s Day, I was sitting with friends in their cabin out in Western Massachusetts, talking about the idea for this blog.

Writers are always advised, “Write what you know.” Well, with three decades’ experience, I certainly know about scleroderma. For several months, I’d toyed with the notion of writing about this disease, possibly as a freelance project. Those plans didn’t materialize, but the idea lingered.

Even still, I was unsure about going public online. It was scary. For many years, I’ve remained mostly silent about having scleroderma, sharing only with Al, our daughters and a few close friends. The disease is relatively rare, hard to explain, and just plain strange. I’ve had trouble accepting my health challenges, even as I’ve lived with the daily consequences for so long. Like everyone else, I just want to be healthy.

But, reality is, I have this disease and a gift for words.

So, with encouragement from friends and family, I decided to go ahead and blog about what it’s like to live, day in and day out, with this most complex and mysterious illness.

I didn’t want this blog to be a harangue about my travails, or a litany of symptoms and doctor’s appointments. Rather, my goal was to explore the experience of living with a chronic illness, how it shapes your daily life and relationships, as well as how you perceive yourself.

It’s been quite a journey, writing every week. I’ve become more comfortable and confident, sharing with all of you, my loyal subscribers and readers, what it’s like to live with scleroderma. I’m no longer embarrassed when a stranger asks about my hands. I’ve found greater self-acceptance.

I’ve also been deeply moved when readers have shared their thoughts and taken inspiration from my words. And I’m so appreciative of both the Scleroderma Foundation and the Scleroderma Research Foundation, for their wonderful work toward finding a cure, and for their encouragement and help in sharing some of the content of this blog.

Some of you have asked if I will turn this blog into a book. My answer: I’m working on it. Will let you know as I explore concepts, formats and options. Meanwhile, I plan to keep posting for another year, and see where it leads.

To all of you, my profound thanks for reading, commenting and sharing this blog. Most of all, for caring. My best wishes for a healthy, healing, prosperous and peaceful 2013.

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.