Living with Scleroderma

Reflections on the Messy Complexity of Chronicity

Body

Breakthrough

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Last Wednesday, the Scleroderma Foundation announced a research breakthrough in the search for a scleroderma cure. A team of University of Pittsburgh Medical School researchers, including the Foundation’s board vice chair Dr. Carol Feghali-Bostwick, has discovered a peptide that blocks skin and lung fibrosis.

The so-called E4 peptide was discovered serendipitously when the team was investigating agents that promote fibrosis—thickening of connective tissue. This particular peptide (a chain of amino acids that make up part of a protein) blocks fibrosis in mice. Testing the discovery further, the investigators found that E4 also reduces fibrosis in human skin samples maintained in the lab.

Exciting news, since lung fibrosis is the leading cause of death in patients with scleroderma, and excessive skin thickening is at the core of the disease process. The discovery of E4 is not a cure, but an important step forward in that quest.

I’ve been thinking about this announcement all week and wondering: Is it really possible that a cure for this disease could be found in my lifetime?

It’s an idea that I have not let myself entertain, for decades. Scleroderma is disease that affects about 300,000 people in the U.S., just under one percent of our nation’s population. It seems like a lot of people to me, but not enough to attract significant research dollars, compared to a killer like cancer.

According to language in the Scleroderma Research and Awareness Bill (HR2408 and  S1545), which has been pending in Congress since 2009, NIH funding of scleroderma research was then about $20 million annually. The Scleroderma Foundation and Scleroderma Research Foundation each raise and distribute a little more than $1 million for research each year. So, that’s around $22 million, give or take. Just about the same as Red Sox first baseman Adrian Gonzalez will earn this year.

Would that our nation made health as high a priority as sports franchises. But I digress.

For my own part, I’ve tried to help the search for a scleroderma cure by participating in studies ever since I was diagnosed 25 years ago by Dr. Virginia Steen, a leading researcher in the field, then at the University of Pittsburgh, now at Georgetown University.

Data about my blood samples are in research databases around the country. I contributed a tissue sample from my placenta when I gave birth to Emily. I’ve helped to test new drugs for Raynaud’s (I think I had the placebo) and have allowed images of my hands, pre- and post-surgery, to be published in medical journals.

I’ve also explained my case history to numerous rheumatology fellows at Boston Medical Center over the years, and I’ve talked to classes of second-year BMC med students, helping them to learn how to diagnose scleroderma.

I don’t often fit the criteria for research trials at this point, because I’ve had the disease for so long. And I don’t participate in any research that involves procedures that sound painful or too risky. I’ll leave those to someone braver. But I’m glad to help when I can. It’s the least I can do to try to find a cure and improve the chances that some new patient suffering from the disease will be diagnosed sooner, with better odds of survival.

As for the E4 peptide discovery, all I can do is hope that the progress of my disease continues slowly enough, and that the research proceeds apace, so that maybe, just maybe, as my health gets worse, there will be better treatment options available when I need them.

And when I finish this post, I’m going to make a donation to both the Scleroderma Foundation and the Scleroderma Research Foundation. I don’t really care who claims credit for the next breakthrough. I just want us to get there, already.

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.

What Did You Do to Your Hands?

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Scene One:
I’m buying a slice of pizza at a luncheonette across from Boston Medical Center before I drive back home from an appointment. As I fumble with my wallet, the middle-aged man behind the counter notices my five bandaged fingers.

“What did you do to yourself?” he asks.

“Oh, I get ulcers,” I say, giving my standard explanation.

“Does that hurt?”

“Sometimes, if they get infected.”

He nods and hands me my change.

 


Scene Two:
I’m taking my first Saori class to learn this fascinating Japanese weaving method that encourages free-form creativity and uses looms that accommodate people with disabilities. The young son of one of my new classmates comes up to greet me. He loves to run around and say a loud Hi to everyone when he’s not weaving wonderful fabric. I shake his hand, and he pulls back. Later, I offer him my hand again. My Raynaud’s has kicked in, and my palm is a mix of indigo and fuchsia from haphazard blood flow. He stares and says No, frowning and shaking his head as he steps away. I believe I’ve frightened him.

*   *   *

June is national Scleroderma Awareness Month. It’s the month for walkathons to raise money for research, ramped-up efforts to publicize scleroderma’s devastating impact and continued lobbying for NIH funding of scleroderma research to find a cure.

All of this is important, focused work. But I wonder. How do you really get anyone to care about one more way that our bodies fail us, unless they have a vested interest? This time of year, there’s a race or walkathon or bike-athon for some disease every weekend, every cause has its own colored ribbon symbol or rubbery message bracelet, and Congress is still gridlocked over cuts in domestic spending. Not to mention that nobody pays attention to anything in front of them any more, just the alternate reality of their smartphone universe.

As part of its awareness campaign this year, the Scleroderma Foundation has developed a social media strategy to encourage patients to share personal stories about scleroderma and to create a video story collage that they plan to send to Washington lawmakers in the fall. I hope it works. For me, the art of storytelling remains the most viable means of building awareness, a primal way we connect as humans. But it requires a willingness to speak up and a willingness to listen. Neither are easy to come by for a disease like scleroderma.

Before I started writing this blog in January, I had a very hard time talking about my disease. In fact, I’ve been silent about it, for the most part, except when sharing with very close friends and family, for the better part of three decades.

When asked that all-too-common question by curious cashiers (the people most likely to ask, it seems)—What did you do to your hands?—or its variants—Did you cut yourself while cooking? Did you prick your fingers with a sewing needle? Did you stick your hand in a lawnmower?—I used to demure and just say I have sores. Now I say I have ulcers, and if I think the person is genuinely interested, I’ll explain I have scleroderma.

Responses range from compassionate concern to flickering interest in freakishness—the latter, I think, veiling the true reaction so powerfully expressed by the young boy I met in my weaving class: my hands look really strange and scary.

This is the hard truth of scleroderma. It’s rare, it defies easy explanations and it’s disfiguring in a way that others find threatening to their own body image and sense of well being.

It’s hard to talk about. It’s hard to draw attention to yourself, or more attention to yourself, and say, hey, I have this weird disease that makes my hands look like claws and my face like a mask and has screwed up my lungs and digestion and God knows what else.

Honestly, I just want to be known for who I am, not for this disease I live with.

But what I’ve come to understand and am coming to embrace is that scleroderma is a part of who I am. There’s no getting around it. I have this fucking disease, and I’m stuck with it for life. Which is why I’ve begun, finally, to write about it, tell my stories and try to make some sense of it all.

I have no awareness agenda. I am aware, however, that it’s important to get past the shame and embarrassment of living with a disfiguring chronic illness and share this journey with others who want to understand. This blog is my medium for that message. And maybe the next time a stranger asks what I did to my hands, I’ll tell them my real story.

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.

Waking Up Is Hard to Do

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It takes me a long time to get going each morning. No matter when my cell alarm vibrates, I press snooze at least three times before I can fully gain consciousness and know for certain that I am here, in my bed, not hugging a newly planted tree to protect it from a group of strangers who want to rip it out of the soil.

A relief to know I’m not stuck in those early morning dreams. But then there’s the matter of getting up. My body is always stiff, my hands often a bit swollen and my mind is sluggish. In winter, as steam heat slowly rises in our radiators, all I want to do is lie there under the blankets and stay warm.

The first step is, literally, always the hardest. I know my joints will feel better once I start moving, so I roll myself up to sit on the side of the bed, let my normally low blood pressure adjust, then push up onto my feet. This entire process, from first alarm to standing upright, takes about a half-hour. I just have to plan it into my schedule.

Some of this morning sluggishness is due to my scleroderma—unless there’s some kind of emergency and my adrenaline blasts me out of bed, I simply cannot accelerate quickly from zero to even 30 mph.

Some of it also has to do with not getting quite enough sleep. I know I should get to bed earlier, but I’m hooked on the Daily Show and Colbert Report to have a good laugh before turning in. If I were wiser, I’d watch the night’s episode online the following evening. But it’s not the same, and, besides, I prefer bandaging my finger ulcers, a 20-minute process, while watching. It’s become my evening ritual.

Even when both shows are in reruns for yet another vacation hiatus, I’ll find a different reason to stay up too late, like finishing the Sunday Times crossword or watching episodes from the first season of Mad Men.

But mostly, my slow morning trajectory just is. When I used to commute every day to Boston, often an hour-and-a-half drive in morning rush hour, it was extraordinarily hard to get up early enough to beat the traffic.

Now, working for myself and being able to set my own schedule, I have more flexibility. It’s a mixed blessing—the feast-or-famine stress cycle of finding clients for my marketing consulting is offset by the freedom of knowing I can get a few more minutes’ rest in the morning if my body just isn’t ready to move. I set appointments for late morning and early afternoon to maximize my attention and alertness, and work after dinner, as needed, to put in a very full day.

Which is why I stay up until midnight to let my brain unwind, and why I have trouble getting up in the morning. Recently I read an essay by William Zinsser, one of my writing heroes, describing how he used to get to his office at the New York Herald Tribune around 10 o’clock each morning. It made me feel better. At least I’m in good company.

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.

Everyday Mother’s Day

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I’ve long felt that Mother’s Day was a contrived holiday. While it’s nice to be appreciated by your children on the second Sunday each May, do we really need modern day Mad Men (and Women) telling us when and how to be grateful to our moms?

Don’t get me wrong. I was very glad this past Sunday to connect with both our daughters long distance—one working half a world away and the other finishing her sophomore year at college one state over—and Al and I had a great afternoon at the Connecticut seashore, then discovered a wonderful Thai restaurant in Mystic. As we ate, I remembered how my mother would laugh until her eyes teared, and how hard she laughed the last time my father, sister and I had dinner with her in a restaurant, a few months before she died from a relentless tumor 13 years ago.

But I don’t need Mother’s Day for those memories or to spend quality time with the people I love. A holiday rooted in post-Civil War efforts to reunite divided families and organize women pacifists, nationalized in 1914 to honor mothers whose sons had died in war, Mother’s Day now seems like just another reason to feel obligated to buy presents—and a key part of the annual sales cycle for jewelers, florists and restaurateurs.

Anna Jarvis, who lobbied a century ago to make Mother’s Day a national holiday (in memory of her own mother’s dream to do the same), became so distraught over its commercialization that she spent the rest of her life fighting abuse of the day’s original intent. In 1948, she was arrested for disturbing the peace at a demonstration against what had become of Mother’s Day.

I’m not advocating for similar protests. But for me, Mother’s Day is not about getting a card or flowers. It’s about the sheer miracle that I became a mother at all.

When Al and I first got engaged, we talked about having a large family. Four kids, why not? When we learned I had scleroderma a few weeks after our honeymoon, all of that changed. Having children was no longer a given. I was at risk of kidney failure in the third trimester.

In April, three years into our marriage, we discovered I was pregnant and immediately shared our excitement with our families. About a week later, I miscarried. Soon after, my rheumatologist informed me that my disease was getting worse and that it was too dangerous to get pregnant. I was devastated.

That fall, Al found us a puppy. I went to see another rheumatologist at the University of Pittsburgh and began a new medication. And we decided to adopt.

The following September, our beautiful, blue-eyed baby girl, 16 days old, arrived in a pink sweater and matching bonnet, in a baby carrier on our kitchen table. She was bright, strong, funny and curious, an adventurous explorer from the moment she could squirm off my lap and crawl around the floor.

Three-and-a-half years later, after my skin had begun to loosen and I’d tapered off my meds, following the fifth infertility procedure, I was able to conceive again. It was a high risk pregnancy. At the end, I developed preeclampsia and had to be induced; in the final hours of labor, my kidneys shut down. But our second daughter, a lovely, tiny preemie, was born strong and healthy. She came home from the hospital a month later, fascinated by her colorful new surroundings, happy to be cuddled and carried.

Our daughters are now both in their 20s. I cannot imagine life without them. I don’t need a national holiday to remind me, or them, of what it means for us to be together. I’m profoundly grateful that they came into our world, each arrival deeply prayed for, precious and unique.

Each has her own way of letting me know how she values our relationship, sometimes with words, sometimes with a gift, sometimes with just a gesture. I’m often surprised, always moved. Those are the everyday Mother’s Day tributes—personal, spontaneous, heartfelt—the ones I’ll cherish long after a bouquet has faded.

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.

Skin Deep

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There are some bizarre advantages to having scleroderma. For one, I don’t have as much body hair as I used to, so it take much less effort to shave my legs, and I hardly ever need to shave under my arms, which makes summertime grooming a snap.

For another, although my facial skin has loosened with excellent medical care and time, thank God (within the first five years of my disease, my face became so tight that I was having some difficulty blinking my eyes), I still have relatively few wrinkle lines. Whereas some women pay hundreds of dollars for collagen creams that plump up their skin, I have more than enough collagen to go around.

Which isn’t to say that I don’t fret about my appearance as much as most other women my age. In a culture that values youth and physical perfection, where magazine ads feature models with Photoshopped features that defy wrinkles and flaws, I am quite conscious of the ways that scleroderma distorts my face.

There is the fact that my nose—generous, to begin with, like my father’s and his father’s—has narrowed and looks and feels pinched. There are the red, blotchy telangiectasias (dilated capillaries) that speckle my nose and dot my right cheek. There are my droopy eyelids, which have fallen over the years due to thickening. There is the asymmetry in my facial muscles, so that the right side is slightly weaker than the left, causing me to smile a bit lopsided. And there are the deep furrows around my lips, resembling a cinched purse.

Most of this I can address with some artfully applied makeup. A couple of years ago, I discovered Arbonne products, which are vegan, extremely lightweight and moisturizing, and use a wonderful color palette that complements my skin tone.

But my deep mouth creases are another story. When they first developed, around the time I was approaching menopause, I was, quite frankly, horrified. This sounds shallow, I know. There are many other, much more horrible things that can happen to you than to develop ugly mouth wrinkles. And yet, self-conscious me hated them and wanted to do something about it.

So I consulted with my rheumatologists and a couple of specialists. A plastic surgeon advised me not to frown or look down, because it exaggerates my creases, and suggested Botox. The idea of injecting a substance that would relax my face into a mask seemed ridiculous, so I passed on that (and him). A cosmetic dermatologist suggested a more promising alternative, injecting hyaluronic acid (HA), a natural filler used to smooth out “puppet mouth” (those long creases that run from either side of the nose to the chin), crow’s feet and other facial flaws.

Al was supportive, though he assured me I looked fine the way I was. He understood how I struggle with the way scleroderma has distorted my body and was willing to go along with my experiment. After doing a test of HA on the inside of my arm to be sure I wouldn’t have an allergic reaction, I went ahead and had the procedure.

This was not fun. First, the cosmetic dermatologist marked the worst creases with a felt-tip pen. Then, after numbing the skin, he stuck a very sharp needle beneath each line around my mouth to fill the furrows with HA. It hurt like hell. He gave me a stress ball to squeeze, which didn’t help much. Neither did the anaesthetic. I quipped how we’ll do anything for beauty, and he just nodded. Of course. I wondered what he really thought of me and all the other women who came to him, in vain, to try to reverse the aging process.

As expected, the skin around my mouth reddened and swelled for several days (I did this at the end of the work week, so most of the evidence would be gone by Monday morning). As the swelling receded, I checked my reflection frequently. Were the furrows gone? Did I look like my younger, healthier self again?

Alas, as weeks passed, I realized that all I’d gained were small lumps where the deep wrinkles had been. The effect wasn’t smooth and youthful. In fact, the HA caused the skin around my mouth to feel a bit tighter, because of the extra filling. When the substance fully absorbed, after about five months, I was relieved. No one ever noticed the difference (or if they did, they never commented), I could open my mouth more readily again, and we were going to save a ton of money.

And, at the risk of sounding Pollyannaish, I discovered a much wiser, free solution: My mouth furrows disappear when I smile.

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.