Living with Scleroderma

Reflections on the Messy Complexity of Chronicity

scleroderma research

Chopin to the Rescue

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Last Thursday, I drove two hours in heavy traffic to Beth Israel Deaconess Medical Center in Boston to participate in a three-hour clinical study. As I wrote back in November, not long after I had a heart catheterization stress test, one of the cardiologists asked if I’d be willing to participate in a study to find a non-invasive alternative. I agreed, because the test was very unpleasant. If I could help to spare someone else that ordeal, I was willing.

So, after ignoring my GPS, which led me to the wrong side of the hospital, I finally found the parking garage and headed inside. (If you’ve ever been to the Longwood Avenue complex of medical centers in Boston, you’ll appreciate that this was no easy feat.) A pleasant research associate greeted me and reviewed the study protocol, which I had read in advance, so I knew, approximately, what I was in for: a six-minute walking test to establish my baseline, followed by an ECG, an IV insertion, a blood draw, then being hooked up again to an ECG for a 20 minute MRI, followed by up to 10 minutes peddling a recumbent bicycle, followed by a contrast dye infusion and another 30 minutes in the MRI. Not a cakewalk, but still better than the invasive procedure.

Now, I’m no fan of MRIs, which are loud and claustrophobic, and I was trying not to get anxious, anticipating THE BIKE. Last time I did this, I lasted three minutes before I felt really awful, because my pulmonary pressures skyrocketed. I was hoping that my new medication, more exercise in recent months, and better diaphragmatic breathing would all help.

So I really appreciated it when one of the researchers kindly asked if I’d like some music while in the MRI. I requested classical. “What kind of classical?” she asked. Really? You get a choice? I went for Chopin piano etudes, a favorite, and some of the most soothing music I could think of on the spot.

The walking test was easy. They set up two cones in a hallway of the research patients’ floor, and I kept a steady pace, back and forth, for the full six minutes with no issues. Ever the A student, I was pleased to know I was among the fastest walkers in the study, so far.

Then came the MRI. Lots of equipment to attach and adjustments to make as I lay on the bed that slides into the maw of that noisy monster. And, of course, it took two sticks to get a working IV in my arm, which is always the case. The final step was a set of earplugs to lessen the bangs and beeps, plus the headphones, and adjusting the volume so I could still hear Chopin. I hung onto every note of the beautiful melody as they slid me into the MRI and the study began.

The piano etudes were interrupted every few minutes by a recorded voice that instructed me when to breathe in, breathe out, then hold until I could breathe normally again. Beeeeep-bang-bang-bang-rumble-bang-bang-beep-beeeeep-bang. Ahh, Chopin.

I was glad when they rolled me out of the MRI, until the research tech told me that we weren’t done, yet, because the research software had crashed. Help was on the way. Fortunately, rebooting the computer solved the problem—and we were able to pick up where we left off. “Three million for the research software, but we’re still on a Microsoft platform,” he quipped.

Finally the first phase of MRI scans was completed and they rolled me out again, this time for THE BIKE. No headphones for this phase. I was on my own. I peddled up to the tempo they needed to boost my heart rate and made it through the first two minutes of resistance without a problem. “You’re like a metronome!” said the research tech. “Most people slow down and speed up.” “We aim to please,” I said, focusing on my breathing.

“On a scale of 1 to 10, how difficult is this?” asked another member of the team. I had trouble answering the question as she raised the resistance to the next level. “A 4?” I answered. Honestly, it was hard to assess while I was trying to manage my breathing. After about a minute at that resistance level, I began to feel some mild chest pressure, which I reported. I was able to finish another minute of peddling, and then they ended that part of the process. A good thing, because I could sense that I was going to start tanking soon.

Headphones back on, first dose of contrast dye infused, Chopin playing in my ears, I began to relax again—until the banging started up. At one point, there was some brrp-brrp-brrping that almost drowned out the music for what seemed like an eternity. I began to feel a bit claustrophobic, but at least could feel my legs outside the machine and even, sort-of, see them. The piano notes that I could catch were my buoy.

Finally, after a second infusion of dye and more banging and clanging, I was done. I felt a little shaky when I sat up, with help, but was soon able to walk back to the changing room and get dressed. They got the data they needed, and I survived without that awful shortness-of-breath feeling. I did my bit for medical science, and, I hope, for someone else down the line who can avoid having a mask with a breathing tub clamped to their face and a heart catheter threaded down their neck while peddling THE BIKE.

On my way out of the hospital, I rewarded myself with a glazed doughnut for the drive home. And just as I got back on the Mass Pike, what should be playing on my Sirius XM station? Chopin, of course.

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. Please view Privacy Policy here.

Image: Accuray

Guinea Pig

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Over the four decades I’ve had scleroderma, I have occasionally participated in research. One of the first studies I signed up for was in the mid-’90s, a trial of medications for Raynaud’s at Boston Medical Center. It was a randomized double-blind study that involved taking a daily pill, recording my experience with Raynaud’s in a journal, and coming to BMC every so often for a check-up with the lead investigator, the late Dr. Joseph Korn. Dr. Korn was responsible for BMC becoming a research center for scleroderma, and his successor, Dr. Robert Simms, became my rheumatologist until his retirement a few years ago.

Which is to say that, even though I’m pretty sure I got the placebo in the Raynaud’s study (no improvement), the long-term benefit was that I ended up being treated by one of the top scleroderma rheumatologists in the U.S. as a result of my participation. I also realized, after driving into Boston on a semi-regular basis, that I could expand my options for work to include that city. Indeed, within about a year, I landed a job as marketing director at a small college in a Boston suburb, a position I held for a dozen years.

Even before the Raynaud’s study, I contributed tissue samples from my placenta after my younger daughter was born to researchers at the University of Pittsburgh. My hands have been photographed and written up in medical journals. For several years, I participated in Grand Rounds at BMC, to help educate young medical students about scleroderma. And I’ve served in a focus group to test intake forms for patients with scleroderma.

I’ve also given blood work for various studies over the years, though I draw the line when it comes to tissue samples from my hands. Given my history with ulcers and long healing times, I don’t want to aggravate my hands more than necessary, even for science.

Most recently, last week I received a call from the cardiology fellow who helped administer my right heart catheter stress test for pulmonary hypertension, to ask if I’d be interested in participating in a study of a non-invasive version of that test. The investigators want to know if a stress test that takes measurements using an MRI would be as accurate as the invasive version that I did. I said I’d be willing to do it, but in a few months. I just need a break from all the measuring. But I do want to help, especially if it means sparing others from the heart cath version, which, as I’ve written here, is no fun.

The other study I’m participating in currently is about cognition (related to aging, as opposed to scleroderma). This one involves playing a video game on an iPad at least once a month for a year. You have to do a variety of tasks that require you to navigate an obstacle course while capturing certain shapes. Conceptually, it’s straightforward, and I do okay. No decline, at least, in my scores. But the problem with the game itself is that it requires manual dexterity that I do not have. So it’s not really measuring my cognition as much as my ability to manipulate my fingers. I’ve mentioned this to the researchers, and they’re aware of the issue. But I’ll continue, anyway.

I write this not to pat myself on the back, but to encourage all of you who are able to take the time, to consider participating in scleroderma research. We’re a relatively small cohort, and whatever information researchers can glean from our experiences will help move us closer to a cure. It’s often easy, and the personal benefits—as I found with the Raynaud’s study— can be significant. If you are not being seen at a research center, as I am, you can find more about studies looking for participants on the Scleroderma Research Foundation website.

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. Please view Privacy Policy here.

Image: Bonnie Kittle

If I Had a Billion Dollars

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Al and I were sitting at the dinner table Sunday night, finishing our meal of salad plus macaroni-cheese-and-tomato-sauce casserole. “The Power Ball is worth over a billion dollars,” he said ($1.4 billion, to be exact—but who’s counting).

rainbow-1393626-639x391“Really?” I said. “What would you do with a billion dollars? I mean, whatever was left after taxes?” (Even if it was less than half, that would still be a whole lot of money.)

We agreed that our first priority would be to pay off all debts—our mortgage, the equity line, the girls’ college and grad school loans. We’d make sure their futures were financially secure. And we agreed that our second priority would be to set up a charitable foundation.

“Who would you give the money to?” Al asked.

I began enumerating—certainly I’d donate to scleroderma research, also our favorite charities and our synagogue. I’d give a lot of money to climate change research, like how to desalinate sea water, alternative methods of farming during droughts, better ways to control coastal flooding. I’d fund interfaith dialogue and Middle East peace initiatives. Grants to fledgling artists and writers. For starters.

“What else?” he asked.

“Well, I’d fix up the house, do all the projects we’ve put off. Maybe I’d want to sell it and downsize.”

“Downsize? With all that money?”

“Sure. Then we could buy vacation places around the world. Except they’d be a lot of upkeep.” (So much for downsizing.)

“It wouldn’t matter,” said Al, caught up in the game. “We’d have enough to pay for a home on Block Island.”

“Okay, then we could also have an apartment in Tel Aviv and a place on the French Riviera. And I’d want to travel. First class. Go to Antarctica.” (I could certainly afford to buy whatever gear I needed to stay warm, there or here, for that matter.)

Al smiled. He began to clear the dishes.

I imagined buying custom-made shoes for my hard-to-please feet and any clothes I wanted.

“You could get a personal trainer,” he said, picking up my empty dinner plate.

“Yeah,” I said, “and I’d want to put aside enough money so we’d be able to afford in-home care, 24/7, for when we get older, so we’d never have to live in a nursing home.” (Might as well be a little practical about the future with all that imaginary money. Certainly no more worries about medical expenses!)

What about work? We agreed we’d have our hands full managing our foundation, even if we hired staff to run it for us. We’d also be busy traveling. The rest of the time, I’d want to write my novel.

“Better buy some tickets,” I told him. (I can dream, can’t I?)

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: Yarik Mishin

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

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.