Living with Scleroderma

Reflections on the Messy Complexity of Chronicity

managing chronic disease

Do Not Use If Allergic

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I am one of those people who still watch the news on cable, rather than solely on social media, streaming services, and other internet sources. And because I still watch the news on cable, I am part of the aging demographic that is bombarded with ads from drug companies.

There are ads for diabetes drugs that lower your A1C level, ads for drugs that supposedly prolong your life if you have advanced cancer, ads for drugs to relieve symptoms of autoimmune diseases like Crohn’s, ads for drugs to help manage asthma, ad nauseum. Perhaps all these drugs have their place, and if so, I hope they actually do some good for those who need them.

But I find the ads ridiculous. Inevitably, as images of happy folks living wonderful lives in loving relationships flash across the screen, accompanied by up-beat music, the voice-over ends the sales pitch with a laundry list (because they must, under FDA regulations for marketing directly to patients) of all the dire side effects that could happen. Why would you want to take a drug to manage bipolar disorder, to name just one example, if it could potentially make you feel suicidal? Isn’t the cure worse than the medical condition?

Of all the required warnings, however, the one that strikes me as most inane cautions the viewer not to take Drug X if you’re allergic to it. So, how do you know if you’re allergic to Drug X if you’ve never taken it?

Apparently only in America—and in New Zealand, it turns out—can drug companies peddle prescription drugs directly to easy marks, excuse me, potential patients. How fortunate for us. I suppose the strategy is if you push brand recognition, a patient would, of course, ask her physician to prescribe the particular miracle cure. Unless, of course, she listened to that long list of negative side effects.

The goal of this information overload is undoubtedly truth in advertising. A good thing. But when we only hear a litany of side effects, absent the evidence-based, actual risk of having a particular bad reaction to the drug, the information is basically useless.

I have a better idea. Just cut all drug ads for good, like we did years ago for tobacco products. The money that goes into making and placing these ad campaigns only hikes the cost of prescription drugs for the people who actually need them. It’s high time for Big Pharma’s marketing strategy, priming patient demand to push their pills to physicians, to go the way of the Marlboro Man and ride off into the sunset.

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.

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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.

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Getting a Boost

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I got my Covid bivalent booster vaccination last Thursday—Pfizer this time, as opposed to Moderna up until now. I scheduled the shot for mid-afternoon, knowing I could set aside Thursday evening and Friday for down time if I got sick, as expected from past experience.

While I did get draggy and had some achy joints, I was very pleasantly surprised that, this time, the aftermath was not debilitating. No rash at the injection site. No real brain fog (other than my normal age-and-scleroderma baseline). I was able to sleep through the night. I modified my morning exercises to accommodate my sore left arm, but otherwise went about my day, editing a blog for a client, writing more in Novel 2. It was only by late Friday afternoon and evening that I ran out of energy. But by Saturday morning, most of the aches were gone.

I don’t know if this is because I went with Pfizer. From what I’ve read, the two versions are effectively equivalent and highly successful in reducing risk of severe disease from both earlier Covid variants and Omicron BA.5. Maybe there has been something in the Moderna vaccine chemistry that wallops me. In any case, the FDA says it’s fine to mix-and-match the vaccines, so I decided to try Pfizer and see if I could tolerate it better. That seems to be the case.

I chose not to pair the booster with my annual flu shot, because I wanted to get over whatever side effects I’d have from the former before adding in the latter. Now that I’m over 65, I get the super-duper flu shot (a friend called it “the old geezer shot”), and I need to pace myself. So that’s scheduled for this coming Thursday.

Fortunately, it is super easy to get vaccinated. Both shots are free and readily available at many local pharmacies. One piece of advice: don’t count on a walk-in. I thought that would be possible, given all the reports that there has been no run on the bivalent booster, but found out when I arrived without an appointment at my local CVS that I definitely needed one. The online appointment scheduling is simple and takes only about five minutes.

I hope your experience with the bivalent booster goes well. Please don’t wait. Flu season is upon us here in the Northeast, already intensifying in the southern U.S., and new, wily Omicron variants have also hit our shores. We have the tools and a lot more experience than we did two-and-a-half years ago.

Stay safe out there.

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.

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The Heart of the Matter

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So, things have gotten a bit more complicated health-wise. For more than a decade, maybe 15 years, I’ve occasionally had an espisode when I’ve pushed myself to exert physically—running through Penn Station in New York to catch a train, hustling through a huge international airport to make a flight connection—and have gotten weirdly out of breath. Not the normal huffing and puffing from running, but actually finding it hard to breathe. And for all these years, the working hypothesis has been that I have exercise/stress-induced pulmonary hypertension (PH).

PH is a known late stage complication of scleroderma, and my cardiologist has monitored me for a long time via regular echocardiograms, which have shown normal pulmonary pressures, for the most part. I’ve also had many, many pulmonary function tests over the years to check my lung capacity and diffusion rate. But over the past year, in particular, these episodes have happened more frequently, with the added bonus of feeling like I’m going to pass out. Fortunately, that has never happened. But it’s become quite worrisome. I’ve also had a heart arrhythmia for many years that has gotten more pronounced.

So, I’ve had a series of heart diagnostics, including a Holter monitor study, a heart MRI, an echocardiogram stress test, and a couple of weeks ago, the gold standard for PH, a right heart catheter stress test. This was not fun. It involves having a catheter inserted into the vein on the right side of your neck, which is then threaded into your heart and the pulmonary artery to the lungs. Your mouth and nose are covered with a tight mask and breathing tube, which collects data on carbon dioxide exhalation, and you have to pedal a recumbent bicycle until you can’t anymore. I lasted about four minutes before my breathing became difficult.

After three conversations with three of my specialists (PH pulmonologist, rheumatologist, cardiologist), the diagnosis is clear. I have Type 2 exercise/stress-induced pulmonary hypertension. Unlike Type 1, which involves changes to the pulmonary artery, Type 2 involves stiffening of muscle on the heart’s left side. Basically, as I understand it (and it’s taken some time to wrap my head around all this), when I exert too fast or get really stressed, my blood pressure spikes, my heart rate increases, and although my heart does a great job of contracting to pump blood to my lungs, it cannot relax readily—kind of like a clenched fist that can’t easily release—which then causes my pulmonary pressures to spike, some fluids to leak into my lungs, and the shortness of breath and near fainting.

It’s a lot. The standard treatment is to go on a diuretic, which is what my new pulmonologist prescribed, with follow-up blood work and appointments to monitor potassium levels and how I’m doing. However, and here is one of the big lessons of this experience: while very knowledgeable, my new pulmonologist doesn’t know me, yet, and we didn’t have enough time to talk, due to her schedule getting backed up, so I never got to ask a basic question. If I have Sjogren’s, which causes severe dry eyes and mouth, how would a diuretic affect me? Also, she only read the most recent stress test results, and not the rest of my heart work-up.

So when I saw my long-time cardiologist the next day, I asked him, and he nixed that plan and put me on a calcium channel blocker. He said my other heart diagnostics had shown I am not retaining fluids in my heart, and a diuretic like Lasix, which she had prescribed, would probably leave me feeling pretty crummy and dehydrated. The calcium channel blocker is supposed to relax my heart, lower my blood pressure (which has been all over the place) and slow my heart beat, also possibly ease some of the arrhythmia.

Even on the first day of taking my new medication, which is time-released over 24 hours, I began to feel better. Only had a slight headache, and by day two, that was not noticeable. By weekend’s end, I was marveling at the fact that my heartbeat seems to have faded into the background, for the most part, and is not demanding attention as it has been for months now. So, off to a promising start.

I’ve chosen not to write about this until I had a clear diagnosis and some answers. There is no way to know, without a heart biopsy, which I have no intention of doing since the information wouldn’t change my treatment plan, whether scleroderma is the cause of the stiffening of my heart. But it’s likely a culprit. No cure. My approach is to work with my team on the right balance of medication, increase my aerobic exercise as tolerable, and also try yoga to see if it helps me with meditative breathing, strength and flexibility.

I’d be lying if I didn’t admit it’s been scary. The diagnosis last week was quite sobering. I’m grateful for my medical team, and that I have a very experienced and knowledgeable cardiologist who really respects me and knows my history. He also has the calmest voice and manner, which was incredibly soothing when we met on Friday. Scleroderma is complicated. You have to advocate for yourself and build a trusting relationship with medical professionals. And while there is no cure, yet, there is medication that certainly helps.

There is also a lot I can do to give myself the best odds of managing this evolving disease process. That’s been my strategy for the past four decades—living with scleroderma, not letting it run my life. And that’s what I intend to continue doing.

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.

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Beautiful

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One of the scariest aspects of a scleroderma diagnosis is to realize how deforming this disease can be. Everyone is different, and how your body changes will be unique to you. Early on in my progression, the skin on my face became so tight that I began to have discomfort blinking. For some, this facial tightening can make it impossible to close lips over teeth. It can reduce your hands to look clawed. At its most virulent, it can make obvious the skeleton beneath.

For all those who live with scleroderma, this is a terrifying prospect. For women, especially, among whom the disease is four times more prevalent, and especially for young women, it can be a harsh sentence in a culture that puts such a premium on youth and physical perfection, narrowly defined.

I have been extremely fortunate that, over the forty-plus years I’ve lived with scleroderma, my skin loosened. I credit the use of D-penicillamine, with which I was initially treated. Six months after I started taking the medication, I began to once again have face wrinkles. Therapies have advanced significantly since then.

Nonetheless, my skin is still not normal on my face, particularly around my mouth and eyelids, and in my fingertips. It has been a long adjustment to aging prematurely. That is why I found this interview with Chloé Cooper Jones, author of the recent memoir Easy Beauty, to be so apt and powerful.

Cooper Jones, who was born with a rare congenital condition called sacral agenisis, has spent her life living with reactions to her visibly disabled body. A writer and philosopher, she explains the difference between the kind of beauty that seems obvious (a sunset, a Monet painting) and that which is more complex and difficult. Her conversation with sociologist and writer Tressie McMillan Cottom delves into the ways we define beauty, what makes beauty intrinsic, and how we view and live with disability.

It is insightful and inspiring. It’s given me some needed perspective as my body continues to age and I contend with my own scleroderma. I hope it does for you, too.

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.

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