Category: dermatomyositis

My Rudolph Nose

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My nose glows red with a dermatomyositis rash like the mythical reindeer of childhood Christmases past. The moral of that story—at least the CBS claymation version—is that what seems a flaw, what makes one different, what subjects one to ridicule, may be a blessing in disguise. But humans are not reindeer. No bearded, red-suited legend is going to ask me to guide his sleigh come some unforeseen foggy Christmas Eve.

We had a web conference with video at work today. It was all too obvious to me with my nose glowing on the screen, I am not who I was. I don’t feel attractive. I don’t feel positive. I don’t want my girlfriend to touch me. I barely feel human some days.

Doctors tell me in time my hydroxychloroquine will help. It has helped a little, but not enough, not fast enough.

Still, I wait for true relief, for what after six months seems like waiting for a miracle. Still, I taper down my steroids. Still, my nose gets redder. The skin on my jaw itches so much it wakes me in the night.

Am I supposed to find meaning in a red nose? Inspiration? Am I supposed to thank God or curse nature? Am I supposed to search for some metaphorical St. Nick for some purpose in this suffering?

As the philosopher Walter Kaufmann, paraphrasing the philosopher Frederich Nietzsche, observes, nature is too well designed to lack purpose but too ill designed to demonstrate intelligence.

Yet, as intelligent, conscious beings, we think; we feel. Aware of our own fragility in the face of near constant chaos, we agonize; we suffer. Aware of our own imperfections, we criticize; we despair. We seek symmetry, predictability, stability, security, order.

So what do we do with our imperfections? Can we fix them? Should we fix them? Or do we embrace them, stare nature in the face, and tell ourselves we are more than our faults?

I may not have a red nose forever. But right now, looking at myself in a photo or a mirror hurts. I tell myself these rashes are not who I am. But right now, I see only who I used to be. I tell nature I have had enough.

Visiting the dermatomyositis expert at Mayo Clinic

Posted on Posted in amyopathic dermatomyositis, autoimmunity, dermatomyositis, dermatomyositisTagged , , ,

Mayo Clinic Scottsdale looks like a corss between a Cold War military complex and a lost Frank Lloyd Wright building.

Mayo Clinic Arizona is nestled on the northeastern edge of Scottsdale in the desert foothills of the McDowell Mountains. Its main structure looks like a cross between an unmapped military complex and a lost Frank Lloyd Wright building. In a nod to Wright’s organic architecture, the campus blends in with the Sonoran landscape; southern Arizona staples like saguaro, pipe organ cactus, mesquite, and palo verde surround it.

My girlfriend and I drive past the sparse vegetation, then park and enter underground, reinforcing my comparisons to a Cold War military site. We take two different sets of elevators to the second floor.

Hospitals rarely, if ever inspire positivity or happiness. Those waiting next to us either have serious health problems or are accompanying someone who does. Many are in wheelchairs. Several are bald. Others, like myself, have mottled skin.

Mayo Clinic in Rochester, Minnesota is, by most measures, the world’s best hospital. U.S. News and World Report rank its sister campus, Mayo Clinic Phoenix-Scottsdale, eleventh in the United States. I remind myself as I look around, wondering how many of these people are here as a last resort.

Mayo Clinic Uber-Rheumatologist, Part I

Just like any other visit to a specialist, after being called back, a nurse measures my height and weight, and I put on a gown. I wait.

The physician’s assistant enters with a stack of paperwork—written testament to the amount of time I’ve spent over the past six months in doctor’s offices, labs, and imaging centers. Unlike many physician’s assistants, this woman’s thoroughness and knowledge of internal medicine is immediately apparent.

After reviewing my medical history, she spends another half-hour examining my muscles, rashes, and lungs. She finds no additional evidence of muscles weakness or lung involvement.

Mayo Clinic Uber-Rheumatologist, Part II

The doctor enters. In my mind, I’ve talked up his reputation and specialty as though he holds all the answers, but as I told my girlfriend that morning, he is not a miracle worker. But he does know more about dermatomyositis than all but a handful of other highly trained men and women.

The rheumatologist re-checks my muscle strength. He remarks my muscle strength is excellent, but unlike my regular rheumatologist, he notes when a dermatomyositis patient works out as much as me, it’s possible people do not notice the muscle regression.

I certainly did. I went from being able to do thirty push-ups to being able to squeeze out three. Of course, that weakness could be from all the corticosteroids I’ve needed to manage my condition.

He surveys my rashes, then says these are classic dermatomyositis rashes. It’s a relief for someone to immediately recognize what I have.

All that said, he agrees with my rheumatologist that I most likely have amyopathic dermatomyositis. He thinks my prognosis is actually quite good and likes my current treatment plan. He nonetheless orders an electromyography (EMG) test to confirm my muscles are in good health and additional imaging to make certain I do not have cancer (10 percent of dermatomyositis patients have cancer).

My girlfriend asks him if my muscles could get worse over time, if my immune system could further attack itself, if this is only the beginning of the end. She wants answers, to know I’ll be around to love her as long as she is to love me.

He cannot promise that. Neither can I. But for now, apart from the itchy, painful rashes covering my chest and arms, I still have my health.

Diagnosis confirmed: amyopathic dermatomyositis

Posted on Posted in amyopathic dermatomyositis, autoimmunity, dermatomyositis, dermatomyositis, lupus, symptoms, treatmentsTagged , , , ,

After three weeks, seven vials of blood, one vial of urine, and a computer tomography (CT) scan, my doctors have once again confirmed I have dermatomyositis.

Yet in true House episode fashion, this is an atypical presentation of dermatomyositis, called amyopathic dermatomyositis. In this instance, my skin itches, burns, and aches from the rashes on my arms, shoulders, and chest. But my muscles have not wasted enough to diagnose me with myositis.

Underneath my skin, my aldolase levels are still elevated. My breaths are sometimes shallow. I lost some muscle weakness. (But this could just as easily be all the corticosteroids I have been taking.) And my creatine kinase levels, which would indicate systematic muscle destruction, are within normal range.

Apparently, my body has decided having a very rare autoimmune condition does not make me special enough. According to Callander, Robson, Ingram, and Piguet, amyopathic dermatomyositis affects only 5‒20 percent of dermatomyositis patients. Doctors have only known about the condition since the early 1990s.

Good news at last

For the most part, this is great news. Exagen’s AVISE® test confirmed I do not have lupus, which can affect multiple organs. My urine and blood tests ruled out any kidney or liver damage. Functional muscle strength means I can maintain the quality of life I want.

However, amyopathic dermatomyositis is more likely to affect my lungs. Since my CT came back normal, for now, my doctors are not concerned. But interstitial lung disease remains a possibility. My immune system may also decide to begin attacking my muscles more severely.

My doctors and I will continue to monitor my disease. My rheumatologist has prescribed hydroxychloroquine (Plaquenil), an antimalarial drug used to combat autoimmune diseases. He has also started tapering down my steroids. And my dermatologist gave me a large tube of fluocinonide cream to keep the itching at bay.

Sometimes, being medically special is a good thing. A smile has returned to my face.

Becoming your own House episode

Posted on Posted in autoimmunity, dermatomyositis, dermatomyositis, lupus, symptomsTagged , , , , , ,

Six months have passed since scaly, irritating rashes first appeared on my elbows and knees. Based on my family’s medical history, my doctor thought it was psoriasis.

Two months later, a much larger, much more painful blistering rash appeared on my left arm. Another massive rash formed across the crest of my shoulders through my lower neck and across my chest.

This third rash—called a shawl rash by doctors—is characteristic of dermatomyositis. Add in what look exactly like all these other rashes along my joints (called Gottron signs), the results from two skin biopsies and from my blood tests, and I thought my dermatologist correctly diagnosed me.

However, yesterday’s visit to a well-regarded Phoenix rheumatologist put me right back where I was a month ago. We know I have an autoimmune disease. But after looking at the pathology reports and examining me, he hesitated to jump to a conclusion.

This is the point in an episode of House, MD, where the title character stares at his patient while his body fails him. Family demands to know what’s wrong. House replies, “I don’t know.”

Turns out, my skin biopsies only rule out psoriasis and fungal infection and confirm my immune system is attacking my skin. But as I mentioned in an earlier post, there are probably more quarterbacks capable of winning a Super Bowl than there are dermatopathologists who can differentiate dermatomyositis from lupus under a microscope.

Blood tests, at this stage, are also inconclusive. My labs show elevated aldolase—an enzyme that helps convert glucose into energy and points toward muscle damage. But all the tests for antibodies associated with myositis came back negative, except for one, which was barely detectable.

The rheumatologist also thinks my antinuclear antibodies are much too high for a typical dermatomyositis case. He also doesn’t think my muscles are weak enough.

All he is willing to conclude is I have interface dermatitis and dyspnea—that’s Greek for a complicated rash and shortness of breath.

This is the point in an episode of House, MD, where the title character stares at his patient while blood appears in the urine bag. Family members demand to know what’s wrong. House replies, “I don’t know.”

“I need answers, not tests, people,” I want to scream.

He re-assembles his team around the glass table, writes “kidney failure” on the white board and pours himself a cup of coffee.

“I need ideas, people,” he barks at his hand-selected team of doctors.

It's not lupus. Except when it could be lupus.

No, my kidneys are not failing. And yes, my doctors know much more about my condition than House’s team does 33 minutes into an episode. But like House, my rheumatologist ordered more tests. I need a CT scan of my lungs. I need my urine analyzed. And my blood has been sent to a lab specializing in detecting antibodies and other biomarkers associated with autoimmune conditions.

“I need answers, not tests, people,” I want to scream.

To make the House parallels worse, my rheumatologist has suggested lupus. Where’s a wise-cracking misanthropic diagnostician when you need him?

Surviving adolescence: lessons for a suffering seven-year-old

Posted on Posted in autoimmunity, dermatomyositis, dermatomyositis, kyphosis, Scheuermann’s disease, symptomsTagged , , , , , ,

I asked my dermatologist when he last saw a case of dermatomyositis. He said a year ago. A little kid came in with it.

Picturing a tiny human being suffering as I am suffering is enough to raise one’s blood pressure and turn one’s face red.

Most children have such sensitive skin and so little muscle to begin with, I can only imagine how he must feel—burning, itching, being teased at school.

To anyone else out there suffering: You are no more your disorder or disease than I am mine. We are humans, not monsters. And we must love ourselves as we are.

When I was twelve, my pediatrician noticed a curve in my spine. Fast forward three years: the curve became a hunch. Add in my dorky green glasses, middle school levels of self-esteem, and grades our teachers posted on the wall (“anonymously” by number, but everyone knew) other kids could only envy, and I was ripe for ridicule, the proverbial wounded gazelle on the Serengeti. Two kids nicknamed me hunchback. My own sisters called me Quasimodo.

Despite being close to six feet tall before reaching high school, my Scheuermann’s disease left me weak and deformed. I played nose tackle on one of the eighth-grade intramural football team, but I couldn’t push anyone off the line. Despite loving music and being one of the best trumpeters in school, I quit after two days of band camp because I refused to be yelled at by my section leader, assistant section leader, drum major, and the martinet of a band director for a physical deformity I never asked to have.

High school eventually improved as I grew and added muscle during cross-country practice. After all, nobody is going to pick on someone who stands half a foot above them. But scars like that never fade. Time doesn’t heal all wounds.

A kid covered in rashes from dermatomyositis looks like a leper victim. No doubt other children believe him to be contagious. Ignorant parents are equally concerned. He becomes an outcast.

We want to be angry for the suffering of innocents, but at what or whom? We blame abuse on bad parenting, but who deserves to be chastised when a seven-year-old develops an autoimmune disorder?

I imagine him still. I have not seen his face. I know not his name. But I see him now—as clearly as I see the thickened, scaly skin on my left arm, and I wonder if he sees as I see, if he feels as I feel, if kids are as cruel in 2018 as they were in 1998.

Is he, like me, tired all the time but unable to sleep? Or is that a symptom reserved for an adult hopped up on caffeine, workout supplements, steroids, and stimulants for ADHD?

Does he, a small boy, contemplate his own mortality and he waits for modern medicine’s miracles? Or is like seven-year-old me, happy and content building pirate ships from Legos as he looks forward to spring breaks, summers, and turning eight?

Childhood traumas hurt. But we can work past them. And with the right attitude (and help from friends and therapists), you see what you never saw before.

I wish thirty-something me could have told teenaged me that girls tease you when they like you. I wish thirty-something me could have told teenaged me some things you shrug off and others you just don’t have to put up with.

But would my life be the same? Would I be who I am today without those wounds, without those hurtful words? Would I be able to persevere through my latest genetic disease if I had not already conquered two?

I may never know who my doctor’s other dermatomyositis patient is. But if he’s out there, now or ten years from now, reading this, I hope he remembers as I so often remind myself: He is no more his disease than I am mine. We are humans, not monsters. And we must love ourselves as we are.

What is dermatomyositis?

Posted on Posted in autoimmunity, dermatomyositisTagged , , , , ,

My dermatologist called this week with my skin biopsy and blood test results.

Microscopes revealed my purplish rashes are thickening my skin in places, wasting it in others. Inflammatory cells meant for fighting infections have clustered around my blood vessels.

My blood shows elevated levels of creatine kinase and aldolase, the former an enzyme that breaks down the creatine commonly used by athletes and bodybuilders, the latter an enzyme that helps convert glucose into energy; these enzymes presence in my blood reveal I have muscle damage.

I have dermatomyositis.

Dermatomyositis is an inflammatory disorder in which the immune system mistakes the body’s muscle and skin cells for invaders and attacks them.

No autoimmune disease diagnosis is good. But the more common ones—lupus, psoriasis, rheumatoid arthritis—have modern treatments, including advanced immunosuppressive drugs. Over 90 percent of those afflicted with such diseases in the United States live a full life.

Dermatomyositis affects less than five in 100,000 people. Odds are my dermatologist has seen only a handful of cases in his lifetime (even in a city of five million). By contrast, over three million Americans have psoriasis. Lupus is about half as common as psoriasis, affecting 20 to 70 in 100,000.

To compare these autoimmune disorders with the more familiar, as many as 35 million Americans every year get the flu; 38.4 percent of Americans have or will have cancer.

An estimated 16,000 people in the United States have dermatomyositis. Considering the world’s third largest population is spread out over the world’s fourth largest country, the chances you ever meet one of them is very small.

None of these figures are to downplay the symptoms and suffering of other autoimmune, infectious disease, or cancer patients. These merely illustrate the rarity. If lupus is a medical-school disease, then dermatomyositis is that annoying challenge question some irritating MD-PhD-MPH snuck onto his medical student’s exams (interesting med-school fact: to the untrained eye, skin biopsies from dermatomyositis and lupus look very similar under a microscope).

Dermatomyositis is an inflammatory disorder in which the immune system mistakes the body’s muscle and skin cells for invaders and attacks them.

Major symptoms of dermatomyositis

  • Itchy, painful, bleeding purplish-reddish rashes occurring along the shoulder line like a shawl or in a V-shaped pattern above the breasts
  • Gottron’s papules, which are scaly, red rashes on the knuckles, elbows, and knees
  • Muscle weakness
dermatomyositis muscle biopsy under a microscope
In myositis patients, immune cells attack the body’s muscle cells, causing muscle damage and weakness. Here, the immune cells are purple, the muscle cells pink.

Other symptoms of dermatomyositis

  • Rashes around the eyes and on the face made worse by exposure to sunlight
  • Swollen, sometimes painful joints
  • Difficulty breathing from weakening lung muscles or interstitial lung disease
  • Difficulty swallowing
  • Calcium deposits

By modern standards, treatments for dermatomyositis are primitive. Every single drug used to combat any form of myositis was developed and used for something else like treating cancer or malaria. All of these have serious, even life-altering or life-threatening side effects. Corticosteroids are the first-line treatment, but they are not without serious risks; long-term users can develop additional muscle weakness, peptic ulcers, steroid dementia, osteoporosis, or Cushing’s syndrome.

Of course, doing nothing is not an option. Without treatment, dermatomyositis kills you.

So where does that leave me? Where does it leave others battling some form of myositis?

I don’t yet know.

Exercise and physical therapy will be very important and may one day save my lungs or my life. Stem-cell therapy may eventually work. New wonder drugs and therapies are being invented all the time. New uses for current drugs are always being explored. Meanwhile, there’s prednisone. And I’m not giving up on myself, on my disease, or on the marvels of modern medicine.