Living with Scleroderma

Reflections on the Messy Complexity of Chronicity

calcinosis

Outlier

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photoI am typing with three fingers on my right hand—middle, ring and pinky—and three on my left—ring, pinky and thumb. Usually, I use my right thumb as well. I actually had to stop and take attendance to figure this out just now, as I’m so used to compensating for digital ulcers that I automatically adjust how I type to which fingers are most healthy.

But my right thumb is out of commission for a few weeks, and my right ring finger, while occasionally useful at the moment, is also in need of a rest. Last Thursday, I finally had hand surgery to remove excess calcium deposits from both fingers that were interfering with my grip. Overall, I think it went well. I’ll know for certain next week, when I’ve healed more and the sutures are removed. I’m glad it’s over. I don’t want to repeat the experience any time soon.

The actual procedure went smoothly enough (despite our arriving nearly a half-hour late to the hospital’s ambulatory surgical suite, due to excessive rush hour traffic, which did not help my anxiety level that morning). Everyone was pleasant, informative and reassuring as they prepped me for surgery. I was glad to see my hand surgeon, who chatted with me as he marked my fingers with a purple felt tipped pen (“Yes” with an arrow pointing to the incision spot on each digit).

But there is a routine, auto-pilot aspect to the process that’s easy to succumb to (they must know what they’re doing, right?). Being assigned the role of patient—stripped down to a johnny and rubber grip socks, lying on a gurney, with your hair in a paper surgical cap and your glasses taken away—renders you more compliant. And vulnerable. In retrospect, I realize, from many years of dealing with the medical profession, regardless of setting or situation—I needed to be more assertive.

The IV nurse’s first attempt to insert a cannula in the back of my left hand was doomed to failure. I had warned her of my small, rolling veins—far too many experiences with IV antibiotics in past decades have rendered them hard to tap. But I didn’t think to stop her from trying, which I should have, because it really hurt and it didn’t work. Per normal, whenever I have blood drawn, the vein in the crook of my left arm was the right spot.

Next, the anesthesiologist came in to speak with me and asked me a bunch of questions about prior surgical experiences and my recent echocardiogram. But when I proceeded to give him a more detailed summary of the report, he dismissed me with the fact that he’d read it already (I certainly hope so, but then why ask the question?). At least he seemed to hear me when I said it takes me a very long time to metabolize anesthesia of any kind. “We’ll go on the lighter side,” he assured me.

The anesthesiology nurse was a bit more approachable and reassuring as he began the light sedative cocktail infusion that would help me relax during the procedure. As he pushed me into the OR, I was already starting to sink into a featherbed of valium. This was a good thing, and the addition of fentanyl made me quite comfortable (as comfortable as you can feel, under an extra layer of blankets because the OR is so cold—to keep the surgeons from overheating under all their gear, according to the anesthesiology nurse—when your head is being covered with a blue paper surgical drape and your hand that you can no longer see is being placed in some kind of protective sleeve and doused in chilly disinfectant). There was rock music playing—nothing I recognized, but good music, all the same.

Then came the local shots, which hurt like hell for far too many seconds—one in the fat pad beneath my thumb and the other, beneath my ring finger. The anesthesiology nurse was kind and comforting, standing by my left ear. Soon I felt nothing in my hand except odd pressure. One of the surgical team hummed to the music. I alternately closed my eyes and stared up at the surgical drape, which was perforated in the shape of tiny stars, as the team scraped out intractable globs of calcium, surrounded, my surgeon explained, by some abnormal cells that were essentially trying to encase the crud—like a tree closes off a wound. No wonder the stuff wouldn’t come out on its own.

Samples were sent off to pathology, and I was wheeled back to my berth. The whole procedure took about a half hour. Al was surprised to see me sitting up and drinking ginger ale when he was called back to my side. I was glad he was there.

I had hoped to see my surgeon again before leaving, but he was busy with other patients. His resident came by, instead. And here is where, once again, I found myself struggling to get my points across. I am allergic to oxycodone (Percoset). If I need a heavier pain killer, I take hydrocodone, the active ingredient in Vicodin. I thought I had some at home from a prior surgery, but didn’t recall. But he would not write the prescription. It wasn’t in their protocol. I don’t know if this is a reaction to the tighter restrictions on opiodes, but it made no sense. He wouldn’t budge.

In addition, I asked for a prescription of my most effective antibiotic. I had discussed this with my surgeon, who agreed it would be a “good idea” to start it when I got home as a preventive measure, given my propensity for infections. I had some left over from a previous infection, but not a full bottle. The resident informed me that research has shown antibiotics as a prophylactic neither help nor hurt, so he wouldn’t write the scrip.

“If it makes you sleep better at night, you can take what you have,” he said. In essence, he was telling me I was taking a placebo. I was really frustrated, but I was also exhausted and just wanted to go home. So I didn’t insist on seeing my surgeon and dropped it, knowing I could call my other docs and take care of it. The resident confidently told me that I would have minimal discomfort from the procedure and left.

As it turned out, it was a good thing I had some Vicodin that had not expired on hand. It took 18 hours for the local anesthesia to work its way out of my body—I could not assess the pain level before I went to bed that night, except for a glimmer of a warning of a problem in my thumb. I started the antibiotics and took one Vicodin before going to sleep.

By 5:00 a.m., I awoke with significant pain in my thumb. On a scale of 1 to 10, it was a 7. A second Vicodin didn’t really make much difference, because the pain train had already left the station. It took the entire day of alternating Tylenol and Ibuprofin, plus distracting myself, to get it fully under control without making myself too queasy from more Vicodin, even as the initial doses were essential to the whole mix. Al stayed home with me again, taking another day off from work, because I was so uncomfortable from the pain and woozy that I was afraid of falling. I missed the follow-up call from the hospital and decided not to return it. What were they going to tell me that I didn’t already know, better?

By Saturday, I was up and about, and a couple of days later, I’m back to almost normal, just managing the wounds. In a few weeks, I hope my right hand will be more functional. I’ve written this long entry, which is good progress.

But my experience reminds me that I have to be assertive, whatever medical setting I find myself in. There are times to listen and learn, and times to speak up and educate. Scleroderma does not fit neatly into a protocol. I am an outlier on the bell curve. The sooner those who seek to attend to my medical needs understand this, the better we’ll all be.

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.

The Nose Knows

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One of the odd and frustrating complexities of my scleroderma is the production of calcium deposits. Typically these grow under my finger tips, at pressure points. And every so often, they rise to the surface—sometimes painfully, sometimes not—until they break through. Their consistency ranges from toothpaste to a pebble. The calcium can ooze out or poke like a pin beneath the skin.

No fun.

maya-1214706-639x424Especially when the calcium forms on the bridge of my nose. This has become a chronic issue for the past dozen years. About every two to three years, I’ll develop a lump of calcium large enough that I have to have it surgically removed in order to avoid the risk of a skin break and infection.

And so it was, a week ago Monday, that I went back to my ENT plastic surgeon to have a lump of calcium removed, once again, from my nose. I used to have a theory that the condition was exacerbated by my glasses (my eyes are far too dry from Sjogren’s Syndrome for contact lenses), and I have gone to great lengths and expense to get only the very lightest weight frames. Certainly these are more comfortable. But after this fourth excision, I’m beginning to think that the bridge of my nose is a little calcium deposit factory, and this is just an inevitable maintenance issue that I’ll face again.

I had felt the calcium lump growing for about a year—not huge, just large enough to poke up and stretch the skin. But our insurance policy was not great, and my specialist was in the second tier, with a big deductible. We couldn’t afford the procedure. Then, thank goodness, Al’s health coverage at work changed for the better (not in small part due to a lot of employee complaints and talk of unionizing). So the first week of January, I called for an appointment. Even though I knew I’d have to heal in cold weather, I couldn’t let it grow any larger without risking a skin break.

Al was able to take the day off and go with me to Boston. After about a half hour wait (during which time a woman sat near us in the busy waiting room and began to play her smartphone music without earphones—and no one in charge said a word), I was called in.

Despite the fact that I had specified what I needed done when I made the appointment (with a referral from my rheumatologist), however, no one seemed to know that I wanted my doc to take the thing out that morning. I made my intentions clear, and we got the ball rolling.

My specialist stepped in to see me, recognizing me right away, even though I haven’t seen him in three years. He checked my nose and started explaining the options, but I stopped him and said I wanted to take care of it now. Then he said, “All right, I’ll have one of my associates take care of you.” I looked at him, a bit stunned, straight in the eye, and said, “I really want you to do it.” So he agreed. I don’t know what was going on with communications there, but thank goodness he’s a good guy and a brilliant surgeon. I absolutely would not trust anyone else.

His resident took care of the local anesthesia. One shot, right where the lump lay. I was actually surprised, because the last time I had this done, I think it took at least two shots, and one was in the nostril, itself. Not a pleasant memory.

Only one problem. As soon as she gave me the shot, I began to shake. My heart started racing. Then I felt a pain in my lower gut, which quickly moved to my lower back. When I closed my eyes and tried to steady my breathing, I could see the light behind my eyelids pulsing with my very rapid heartbeat. When the resident came back in the room (she had stepped out for a few minutes), I asked what was in the shot.

Turns out it was Lidocaine plus epinephrine, a combination I have never had before. (The two are combined to make the Lidocaine “stay in place” and work more effectively.) Fortunately, the side effects passed as quickly as they came—but it definitely threw me. Some people are sensitive, my specialist commented. Yup.

Five minutes later, he had popped out the calcium, a pebble about five millimeters in diameter, and stitched me up. “You have a soft nose again,” he said. I was relieved. With a piece of surgical tape over the incision, I walked out into the waiting room and found Al. I was really glad he was there, because the aftereffects of that shot were still making me feel off kilter.

Anesthesia works through my system very slowly. It took about seven hours before it wore off completely, during which time I tried to sleep, couldn’t, even with a Vicodin, I’m sure because of the epinephrine lingering somehow. I did get to sleep, finally, that night, and was nearly back to normal the next day. By Wednesday, I felt like myself again.

The small incision is almost completely healed, a week later. The stitches are absorbing. I have been able to go without any bandage for 24 hours, even as the temperature has dropped into the teens and we’ve gone from springlike conditions to snow storms over the past seven days. It’s great to have that damn calcium pebble out of my nose.

I made notes so I’ll remember what happened and what to watch out for the next time I deal with this. And whenever I require a local anesthetic, you can be sure I’m going to find out what’s in it, ahead of time. Living with chronic disease means being vigilant, informed and a strong advocate for exactly what you need, when you need it. Nothing less will do.

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: Tamer TATLICI

Milestone

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It’s maybe seven years ago, February, midday. The pea-soup-green classroom, a science lab of some sort, is packed with second year medical students. They fill every seat behind the rows of black benches and crowd onto window ledges in the back.

I sit before them on a metal stool, dressed in my favorite red wool pencil skirt, a black and beige tweed jacket, black cashmere sweater and rainbow scarf, stockings, black pumps. I want to look my best, not like a suffering patient with scleroderma.

I have come here, to Boston Medical School, to help would-be physicians learn about this rare and complicated disease, at the request of my rheumatologist’s research fellow. I’ve helped out several years in a row, so I know the drill: The fellow asks questions and I describe my symptoms. Then the students have to figure out which auto-immune disease I’m describing—rheumatoid arthritis, lupus or scleroderma.

I summarize the disease’s onset—fatigue, puffy fingers, fleeting joint pain in my late ‘20s; pleurisy, thickened skin that migrated from my fingers to the backs of my hands halfway up my forearms in my early ‘30s, facial skin tightening that made it uncomfortable to blink, problems swallowing. I tick off more details. Severe Raynaud’s. Calcium deposits, digital ulcers and infections. Friction rubs in my wrists. A miscarriage at 6 weeks. Pre-eclampsia and premature birth of my younger daughter.

The skin tightening, of course, is the giveaway, and several suggest the correct diagnosis of scleroderma—to be specific, limited systemic sclerosis, explains the fellow. Then it’s time for the med students to ask their own questions.

I am always surprised by how tentative they are. I’m one of the first real patients they have met in their medical training, and they stick to the technical details, nothing really personal. Do I get short of breath when I go up stairs? (Sometimes.) What triggers numbness in my hands? (Cold weather, but also a change in relative temperature, like going from 80 degrees outside to 72 degrees inside with air conditioning.) Have I experienced any skin changes on my torso? (No. That’s a sign of diffuse systemic sclerosis, which tends to be much more severe. My skin has actually loosened somewhat with time, thanks to medications, excellent health care and good luck.)

Our session flies by. At the end, I let the students feel the backs of my hands. Their fingers flutter over my skin like butterflies. They are most appreciative. I leave with a sense of accomplishment, that scleroderma will no longer be just another diagnosis to memorize from their textbooks, but something tangible. Maybe, just maybe, after they’ve completed their training, one of these young physicians will be able to diagnose this disease early on and save her patient at least some irreversible harm.

I am also exhausted. There is something about sitting in front of that group, good as it is to teach, that makes me feel like a bug under a microscope. The discussion among the students and the fellow, as they explore my symptoms, is both theoretical and specific. I am reminded of all the scary things that could go wrong—kidney failure, interstitial lung disease, pulmonary hypertension, GI problems, heart issues, on and on.

I understand this discussion—it’s a necessary piece of the students’ medical education. But it depresses and angers me, too. I am not a litany of symptoms and would-be symptoms. I’m a whole woman who has been living with this disease for far too long.

That’s why I dress up. I want to make it clear to the students that I am much more than my scleroderma. Yes, it affects every aspect of my life. But it does not define me.

This is my 100th blog post. Though I haven’t taught second year med students about scleroderma in a long time, I have chosen to share my life with this disease in the blogosphere for some of the same reasons. I want to educate—about not only what it means to live with scleroderma, but also what it means to live with chronic illness.

The more I have written over nearly two years, the more I find myself wanting to share what I’m learning about living fully. We are a society obsessed with categorizing, labeling, one-upping. Health, wealth and beauty guarantee high social status. Those qualities are compromised by chronic disease, especially scleroderma.

The older I get, the longer I beat the odds on this disease, the less I care about those status markers. What I value is my ability to make the most of each God-given day, to nurture loving relationships, to put my talents to good use. And that’s what I’ll be writing about more in the weeks and months ahead.

To all of you who have subscribed to this blog since Post #1 and stayed with me, my profound thanks for your support and continuing enthusiasm. To those who have joined along the way, I’m so glad you’re here.

Photo Credit: A.M. Kuchling 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.

Surfacing

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At the inside crease in the first joint of my right middle finger, a charcoal gray chip of calcium is working its way to the surface. This has been going on for months. But now the tip of the chip is visible, and if I try to budge it, the sharp edges pinch.

So I need to wait it out. Kind of like a tiny submarine that isn’t quite ready to emerge. If only it contained miniaturized scientists, à la Fantastic Voyage, on a mission to repair my immune system.

I’ve discovered over decades of managing calcinosis that it’s better to let nature take its course than try to pry these odd calcium stones from my fingers. For one thing, I can’t really grasp a pair of tweezers tightly enough to dig them out. For another, it really hurts to do this. And disturbing the skin increases the risk of infection. So I use a combination of Aquafor and antibiotic ointments, dressings and bandages to soak them out, gradually.

Most of the time, this works. Once, several years ago, I had to have a clump of calcium surgically removed from my left thumb because it was too painful and wouldn’t come out on its own. Turned out it was attached to bone. But that’s been the exception.

Patience. It takes a lot of patience to let your body heal itself. For scleroderma, there are no quick fixes or easy cures. And there are many aspects of this disease that won’t heal unaided, if at all.

But one of the things that continually amazes me is how my skin, abnormal as it is, still knows how to heal itself. It just has a much longer timetable than normal.

Many of my finger ulcers take months to heal; some have taken years. Some of them form because of hidden calcium deposits that begin to surface; others, at pressure points. And yet, they do eventually heal. Sometimes the skin grows back thicker and sometimes it retains flexibility. The ulcers may reopen, but at least for a while I’ll get a respite.

This week, I was surprised and very pleased to realize that two intransigent ulcers finally closed up—in fall, of all seasons. So I’m down to three bandaged fingers from five. This is a major improvement.

Whenever a piece of calcium finally pops out, I’ll roll it around between my fingertips, just to explore it. How does my body make these strange, pointy crystals? Some can be as large as an eighth of an inch in diameter and leave a small crater in my finger.

But once I’ve cleaned out the hole with peroxide and dabbed on antibacterial ointment and clean dressing, within 24 hours, my skin has begun to repair itself and filled in. It’s really quite remarkable. For all the strangeness of this disease, my skin cells still can repair some of the damage. This is comforting.

Of course, nature can use a bit of help. I take medications to improve my skin circulation, which is critical to healing. I’m vigilant and meticulous about skin care to minimize risk of infection. I change my bandages every morning and use ointment, moisturizer and white cotton gloves at night to aide the healing process.

I also try to be mindful of how I move my hands and grasp things so I don’t bang myself. I take extra precautions, like wearing cotton work gloves when cleaning or moving cumbersome objects, to protect my bandages and skin. And I dress carefully, often in layers, to keep my hands warm.

All of this takes patience, too. After 30-plus years of living with scleroderma, I’ve adapted to the rhythm of my body’s long healing process. Some days, I’m far less patient and rant. But as long as I’m not in any significant pain, I’m able to ride out the frustration and regain my inner balance.

Today, checking the calcium chip’s long journey outward, I’m just glad it’s located in a spot that’s mostly out of the way. Maybe in a month or so, it will slip free and my skin will heal over, once again. Until the next time.

Photo Credit: Derek Lyons 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.

Harbingers

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I think it’s spring. At least, it’s supposed to be, according to the calendar. But we should fire the groundhog.

This has been one long, cold winter here in Massachusetts. Our lavender crocuses just sprouted last week, but they’re hiding from freezing night temperatures and sharp winds, petals clasped against the cold, praying for the warmth that’s supposed to come with longer daylight hours. When the sun’s rays trickle into their corner of our backyard garden for a few hours in the afternoon, they gape as if astonished, exposing fuzzy stamens the color of flame, welcoming bees.

I’m slowly exposing my hands, too. Spring in New England has always been my toughest season, a tease of warmth to come, but mostly chilly and damp with harsh, sharp breezes that stir the sandy dregs of road salt, stinging eyes of those unwary.

The cycling from cold to warm to cold again exacerbates my Raynaud’s with frequent episodes of icy lavender fingers and numbness, ulcers that sting as if singed, new sores appearing weekly. The sensation is captured precisely by poet Elizabeth Bishop’s description of frigid seawater in At the Fishhouses:

If you should dip your hand in,
your wrist would ache immediately,
your bones would begin to ache and your hand would burn
as if the water were a transmutation of fire
that feeds on stones and burns with a dark gray flame.

Last year at this time, I had nine ulcers and a bout of cellulitis that took several blasts of antibiotics to cure. But no significant ulcers this season, so far. This is quite extraordinary. I’ve been very vigilant since I discovered a few months ago that I could heal my ulcers and reduce the number of ever-present bandages by wearing white cotton gloves and paraffin hand cream at night—this, a serendipitous solution to the fact that my skin started shredding in reaction to bandage adhesive.

Today, I have only my right thumb in a bandage, mainly because a grain of calcium is slowly emerging through a cracked ulcer. That’s it. I’ve been out and about for the past four days with no bandages at all. Truly amazing. I tote my moisturizer and apply it strategically throughout the day, type at my computer using cotton gloves to protect my skin and generally try to pay attention to what I’m doing so as not to cause any collateral damage to my fingers.

Our new heat pumps have helped, too, maintaining a much more even temperature throughout the house than our old steam radiators ever could. I still feel the cold all too readily, but at least I can quickly adjust the heat for the room I’m in and sense warmth within minutes. This, I’m certain, has aided my hands’ miraculous recovery.

So, even as my fingers are in happy denial, I guess it’s fair to assume that spring is on its way, at last. The weather forecast predicts temperatures in the ‘60s and low ‘70s this week. Slender blades of grass tinge lawns green. Buds mist the maples that line our street with the barest hint of chartreuse and crimson. Children’s bikes and basketballs litter front yards. Long-limbed girls from the nearby Catholic high school’s track team run down the street in shorts and tees, gleaming ponytails abounce. As Al rakes away winter’s detritus, the turned earth smells pungent with promise. Time to switch out my snow tires and at least consider bringing my down coat to the cleaner’s. But maybe not ’til April’s end, just to be certain.

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.