Living with Scleroderma

Reflections on the Messy Complexity of Chronicity

managing chronic disease

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.

Image: Vinzenz Lorenz M

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.

Image: Jamie Street

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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Things That Go Bump in the Night

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Returning Sunday afternoon from a four-day weekend in Raleigh, North Carolina, for a very sweet family wedding and a celebration of our elder daughter’s birthday with friends and family, I was exhausted. It was our second trip in just two weeks, and per usual, I managed all the logistics—which I enjoy doing and am good at, but there’s always a lot to track. So, it was great to get home, with no more responsibilities for anyone else, and go to bed early.

I slept for ten hours. At some point, maybe around 3:00 a.m., I suddenly woke because I thought I heard a loud musical note. Yes, I know, that sounds weird. It was. Some kind of plucking of a stringed instrument or a bell or what, I can’t recall. But it was quite distinctive. I became conscious enough to realize I’d imagined it and, thankfully, went back to sleep.

This is not the first time I’ve woken from a loud noise that wasn’t there. Occasionally I’ve roused because I’m sure the telephone rang (we still have a landline, believe it or not). Then I’ll realize it didn’t and go back to sleep.

So, after Sunday night’s weirdness, I looked online for hearing loud noises in your sleep. And, sure enough, the phenomenon is real. In fact, it has a very evocative name: Exploding Head Syndrome (EHS).

No one knows what causes EHS, but apparently it is more common among women. It doesn’t harm your health, and there is no known cure. It may be triggered by fatigue and stress. It also may be related to damage or dysfunction of the inner ear, which, in my case, seems a possibility, given that we had just flown, which affects pressure in my ears, I have occasional episodes of vertigo due to loose crystals in my inner ear, and have had tinnitus in both ears for decades.

In any case, at least now I know what’s going on. And given all the distressing craziness of our world these days, knowing that my head actually is exploding surely fits the moment.

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: David Matos

Almost Autumn

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With Labor Day behind us and schools here already in session, it’s starting to feel like fall. The maples on our street began to drop leaves, a few at a time, in mid-August. A week post our vacation, the days are noticeably shorter, with sunset at about quarter past seven.

I find this time of year bittersweet. It’s hard to let go of summer, even as it’s a relief to be out of the 90+ degree Fahrenheit heat wave and soupy humidity of the weeks before our travels. At the same time, with schools in session, everyone back from vacations, and the Jewish New Year right around the corner, fall is always about new beginnings. Even as trees go bare, they are storing sugar for the long winter ahead and forming new buds.

We have one more big family celebration coming up this weekend, and then it’s time to focus, once again, on work and writing and election season, on putting away summer clothes and getting back into layers, on birds migrating south and trees hardening off. I’ve gotten away with only my thumbs in bandages for several months, and I know that is about to change as the temperatures drop and more ulcers appear. So it goes.

To everything there is a season . . .

(Click the link, above, if you can’t see the embedded video of Turn! Turn! Turn! with Judy Collins and Pete Seeger.)

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.