Living with Scleroderma

Reflections on the Messy Complexity of Chronicity

Scleroderma Foundation

It’s Up to Us

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There’s a lot going on in scleroderma research, particularly regarding efforts to understand the causes of fibrosis—what makes skin get too thick—as well as possible treatments. Just this October, a study published in Arthritis Research & Therapy found that “two approved treatments for pulmonary arterial hypertension— Tracleer (bosentan) and Opsumit (macitentan)—can block a molecular pathway that promotes fibrosis in systemic sclerosis (SSc), and may be a potential therapy for these patients as well, according to an early study.” You can find a summary of the study here, on the Scleroderma News website.

I’m particularly interested in this study, because I have taken Tracleer for years, and I noticed within a few months of starting this medication that skin on my face and backs of my hands had loosened a bit. I’ve discussed this with my rheumatologists, who agreed with my suspicion that the Tracleer may have been responsible. One note of caution: this is a very expensive drug. You need good health insurance to pay for it. But the study results are certainly encouraging. Not my imagination. Not by a long shot.

Path-breaking research into the causes of scleroderma, as well as the ongoing search for a cure, depends on all of us to contribute in any way we can—with financial donations as well as participation in research studies. As December 31 draws near, I hope you’ll join me in making a gift to the scleroderma organization of your choice. Here are some links:

We may not see a cure for this cruel disease in our lifetimes, but progress is being made. Each year brings more insight and increasing hope that we will get the answers, sooner than later. Thank you for your help.

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: Connor McSheffrey

Plant a Seed

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In Australia, the sunflower has become the symbol of scleroderma awareness—because those of us with the disease feel better in the warmth of the sun. I like this idea a lot. Enough, in fact, to consider trying my hand at planting some sunflowers, if not this summer, then next.

1428247_41776650Yesterday, on June 29, scleroderma associations around the globe marked World Scleroderma Day. In nearly two dozen countries in North America, Europe and Oceania, a month of awareness-building culminated with a host of activities, from hospital presentations and meetings of scientists to art gallery exhibitions and a social media series of 25 second videos, in recognition of the estimated 2.5 million people diagnosed with scleroderma worldwide. Today, June 30, the need for a cure will be presented to the European Parliament in Brussels.

June 29 is significant because it marks the death in 1940 of the brilliant artist Paul Klee, who suffered from systemic scleroderma. Klee’s later work was strongly influenced by his disease, even as his last few years were among his most prolific.

Paul Klee’s legacy helped to inspire the formation of the World Scleroderma Foundation, which is based in Basel, Switzerland, and draws together world experts in scleroderma research and patient groups in an effort to support research for a cure. The WSF’s lead patron is Klee’s grandson, Alexander, and his family.

While 2.5 million individuals with scleroderma represents just a sliver of the world’s 7.3 billion people, The WSF makes a compelling argument for the importance of scleroderma research on its website: Not only is the search for a cure needed for the millions who suffer from the disease—most of whom are women of childbearing age—but also scleroderma is a “linchpin disorder”: 

A breakthrough in understanding scleroderma would doubtless have a strong impact on many other diseases involving abnormal scarring and blood vessel injury. This might include conditions as diverse as interstitial lung disease, intestinal hypomotility disorders and many illnesses with blood vessel injury as their basis.

Which brings me back to sunflowers. Each sunflower head can contain as many as 2,000 seeds, each of which, in turn, with the right soil, sun and moisture, becomes another sunflower. If we could find a cure for scleroderma, imagine how many other millions of people who struggle with related diseases could be helped, as well.

Plant a seed to find a cure for scleroderma. Visit wolrdsclerodermaday.org to see what’s going on around the globe in support of research for a cure, or make a donation here in the U.S. to the Scleroderma Research Foundation or the Scleroderma Foundation. We’re all in this fight together.

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.

Imagine

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Passover this past weekend was extra special. A few hours before we were scheduled to begin our seder on Friday night, we learned that one of our cousins received the all-clear on her lymphoma, following six months of chemotherapy. There were big hugs all around when she and her family arrived for dinner.

A central theme of the seder is retelling the story of the Israelites’ Exodus from Egypt, as if you, personally, were escaping from slavery to freedom. The word in Hebrew for Egypt is Mitzrayim, which means “a narrow place.” So the metaphor had exceptional resonance at our table that evening, and again Saturday night, when we repeated the seder at our cousins’.

Then, on Sunday, I learned that the beloved husband of a childhood friend had died the day before of ALS. They were married only a few short years. The words of comfort I shared with her seemed so shallow compared to her loss.

Our bodies can betray us in so many ways.

There are never any guarantees that a treatment will work for a particular disease for any given individual. I am profoundly grateful that our cousin has responded so well to chemo and is on the path to full recovery from cancer.

My friend’s husband, however, had no such options. ALS has no cure, although research is progressing to identify the genetic underpinnings of the disease and treatments that may slow the deterioration of nerve cells.

According to the ALS Association, about 30,000 Americans may have the disease at any point in time. By contrast, figures from the American Cancer Society project more than 1.6 million Americans will be diagnosed with one of the four major forms of cancer this year—colon/rectal, lung, breast and prostate. And that’s not counting the myriad of other ways cancer can attack our bodies. No wonder a preponderance of research dollars go to finding a cure for “the emperor of all maladies.”

Scleroderma research for a cure faces similar hurdles as ALS research. With Congress deadlocked over basic federal spending issues, let alone medical research for rare diseases, the need to find other resources to support this important work has never been greater.

Where could it come from?

Here are some mind-blowing figures:

It’s not that we as a nation don’t have enough money to support medical research for rare diseases. It’s just a matter of priorities and the need to make a commitment, as a society, to be responsible for each other’s well being and not only for ourselves.

Imagine, for a moment, what it would be like to live in a country where we spent more on finding a cure for scleroderma or ALS or any number of horrible, painful, debilitating diseases than we do on all those half-eaten snacks that get tossed in the garbage.

Imagine.

Then please consider donating to the Scleroderma Research Foundation or the Scleroderma Foundation—or to the ALS Association.

Thanks for listening.

Photo Credit: a.s.ya 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

May I Have Your Attention?

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If you spend any time on social media, watching TV or reading the newspaper, by now you’ve undoubtedly heard of the extraordinarily successful “Ice Bucket Challenge” that has raised record-breaking donations for ALS research.

It goes like this: You pour a bucket of ice water on your head while someone makes a video of your antics, upload it to social media, then challenge friends to do the same or make a $100 contribution to the ALS Foundation, which funds research to find a cure for amyotrophic lateral sclerosis, also known as Lou Gehrig’s disease. ALS afflicts about 30,000 Americans, attacking the nerve cells and eventually causing total paralysis, most often within two to five years of diagnosis. It is debilitating, terrifying and, ultimately, fatal.

Though dismissed by some critics as “slacktivism”—an all too easy, narcissistic and short-lived way to feel good about yourself for giving to a good cause, with no lasting impact—the Ice Bucket Challenge, as of this writing, has attracted support from celebrities ranging from the New England Patriots to Jimmy Fallon and raised more than $15.6 million for ALS this August, compared to $1.8 million during the same period last year. You read that correctly—a more than seven-fold increase. And that, my friends, is nothing to sneer at.

I’m thrilled for the ALS Foundation that they’ve been so successful. I have known good souls who died of this scourge, and a friend’s husband was just recently diagnosed. I hope and pray that all this money may somehow buy him and others like him some time and better quality of life.

But, of course, now I wonder . . . what would it take to draw that kind of attention and donations to scleroderma research for a cure?

A few folks in the online scleroderma community have been wondering the same thing. Suggestions range from sticking your hand in a bucket of ice water for a few hours to simulate severe Raynaud’s, to wearing a scuba diving suit that’s a few sizes too small to sample tight skin.

What about something more playful—a Mummy Wrap Challenge—you know, that silly game where you wrap up your partner in rolls and rolls of toilet paper to look like a mummy? At least it might get people to laugh, even as they developed some glimmer of what it feels like to be trapped in your own skin with this rotten disease. Maybe Scott Brands could be a corporate sponsor. (I’m kidding. Really.)

All of this copycat daydreaming begs the broader questions: How do you break through the clamor of fundraising demands for so many good causes to build lasting awareness and a solid donor base for scleroderma research? And how do you do it in a way that doesn’t trivialize the cause itself?

I can just imagine, on the heels of the Ice Bucket Challenge’s success, that we’ll soon be inundated with all kinds of attempts to be clever and cash in on self-perpetuating viral social media campaigns for various diseases. As it is, every single organization trying to raise disease awareness has a walk of some kind, including for scleroderma. These walks are successful, up to a point, raising needed funds. But they have also become so ubiquitous that they begin to merge into the white noise of our over-programmed lives, attracting mostly those who already know about the disease in question.

There are just so many, many ways that our bodies can break down. And there is a limit to our attention spans and compassion for so many illnesses. Which, ultimately, is why there’s so little government funding, relative to the need, for research into the so-called “orphan diseases” that afflict smaller numbers, compared to, say, cancer or diabetes.

If all of the foundations devoted to finding cures for obscure auto-immune diseases, including scleroderma, banded together, would we have a better chance of raising awareness and donations for cures? Is there research that cuts across all auto-immune diseases that could benefit those who suffer from the all-too-many variants? Would there be more cooperation and knowledge sharing among medical scientists? Would we have more clout in Congress?

I wonder. One thing is certain, though. The Ice Bucket Challenge proves that there are plenty of people out there with good intentions and money to give to find cures for terrible diseases. It’s just a matter of getting their attention and holding it long enough to dump out a bucket of ice water on a hot summer’s day.

In the meantime, please consider supporting one of these fine organizations that are working toward a cure for scleroderma:

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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.

Ray of Hope

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Nothing like starting the new year with some good news about scleroderma research. In my email box last week, I found this item in the Scleroderma Research Foundation’s monthly eNewsletter (12-30-13):

Researchers Prevent and Reverse Mice Fibrosis in a Mouse Model of Stiff Skin Syndrome; Study Shows Promise for Scleroderma

I read on, heart quickening:

Dr. Hal Dietz and his team at The Johns Hopkins University have made a key discovery that may have broad implications for future scleroderma therapy. In a report in the November 7, 2013, print issue of the premier scientific journal, Nature, the researchers demonstrated that integrin-modulating agents (integrins are molecular receptors that mediate the attachment between a cell and its surroundings) can stop fibrosis in a genetic mouse model of scleroderma-like skin and, more strikingly, that established fibrosis can actually be reversed by the same agents. The Scleroderma Research Foundation has supported the work for the past six years.

In other words, it may be possible, some day in the future, to reverse skin stiffening that is the cursed hallmark of scleroderma.

Here’s a bit more explanation from the same piece:

In the SSS mouse model and, seemingly, in scleroderma, there is a fundamental process that goes awry: cells in the skin lose the ability to attach to the extracellular matrix and to sense their surroundings. Those cells then become activated and stimulate an immune response that causes the surrounding cells to produce excessive amounts of collagen, resulting in fibrotic skin. What’s most exciting is that the Dietz lab discovered a strategy to suppress the abnormal activation of the immune cells. In doing so, they also found that they could not only prevent, but also reverse established skin fibrosis.

You can read the full news release here.

There is so much we still do not know about the causes of scleroderma and potential cures. But the Johns Hopkins study gives real hope that somehow, someday, this disease will have a cure. Maybe not in my lifetime, but, then, who knows?

Both the Scleroderma Research Foundation in San Francisco and the Scleroderma Foundation in Danvers, Massachusetts, raise and distribute millions to find a cure. But they need help. There is just not enough funding through the National Institutes of Health for the research that remains to be done. Scleroderma is simply not up there on the top ten list of diseases-that-affect-enough-people-to-get-more-funding.

So, it’s up to all of us who are affected by this mysterious and disabling disease, friends and loved ones, to help out. Please, even though it’s past December 31, consider making a contribution.

Thank you, and profound thanks to everyone at both Foundations for all you do to help those of us who continue to fight our daily battles, living with scleroderma.

Photo Credit: Kuzeytac (Hopefully Back) 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.