Category: dermatomyositis

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.

Living on the edge while working on the frontier

Posted on Posted in dermatomyositis, treatmentsTagged , , , ,

I manage and help write proposals at a university placed by the Times Higher Education in the world’s top 1 percent. Few places on earth are better for studying and advancing research in industrial, computer, electrical engineering; materials science; earth and space exploration; and biotechnology.

In doing my job, I see even before other academics and researchers how the world’s best scientists and engineers expand the frontiers of their chosen fields.

One engineer has a method for getting rid of dendrites in lithium batteries. I cannot say anymore than that, but in short, he and his partners know how to overcome one of physics and chemistry’s biggest obstacles to higher-capacity, longer-lasting lithium batteries in smartphones, drones, laptops, and electric cars.

A team of biochemists is folding nucleic acid nanostructures into geometrical shapes that can be used to reinvent biology on a nanoscale. Some day soon, they may be able to fold DNA and RNA—nature’s genetic instructions for life to grow, develop, function, and reproduce—into cures and treatments for cancer and infectious diseases.

My hope is they or someone else uses such methods to invent a cure for autoimmune diseases like dermatomyositis.

Living on the edge of what’s possible in modern medicine in a chaotic world seemingly controlled by corrupt institutions can be discouraging. Not knowing what cures and treatments await me induces as much anxiety as simply having the disease and as much frustration as dealing with hospital and insurance bureaucracies.

But it helps knowing such people are out there, looking at ways to improve humanity and society, to make our lives easier, is encouraging—especially for those of us waiting for a miracle.

The spirituality and sensitivity of broken skin

Posted on Posted in autoimmunity, dermatomyositis, existentialism, symptomsTagged , , , , , , , , ,

I look at the rashes covering my skin. I wonder if people notice, if the woman looking at me at the gym thinks I’m cute or just some monster.

Is God punishing me? Mocking me? Testing my faith? Has the devil come to collect the bill for the body I sold to keep my soul? Has Anubis weighed my heart against a feather and sentenced me to a watery, crocodilian hell?

Am I an evolutionary accident, some creature that should never have survived infancy, some being that should not be? Of the 20,000 genes in my body, should a single mutation change render me less than human?

Most people ask if I’m sunburned. I try to be polite, but how do I explain autoimmunity to the grandmother at the dermatology clinic, to the child at the store?

Before my first visit to the dermatologist two months ago, the rashes itched and burned so horribly I could not sleep—even with two antihistamines and melatonin. I could not sit in my own home with the fan on because the moving air scratched and irritated my skin. I hid under blankets to relieve the pain.

I’ve never been more thankful for prednisone.

The Hebrew Bible refers to skin diseases as tsara’at—meaning to be thrown down or humiliated. Those afflicted with these conditions were considered ritually impure; presumably, their disease was God punishing them for behaving immoral.

The Bible is not alone in its discussions of skin diseases nor are the Jews unique in their treatment of persons with them. As Philip D Shenefelt and Debrah A Shenefelt point out, across cultures, humans feel a spiritual connection with our largest organ, perhaps because it is the part of us most visible, and “persons with visible skin disorders have often been stigmatized or even treated as outcasts.”

Though we understand more than ever about skin diseases, though modern persons are not ostracized or forced to shout “unclean” as they walk down the street, I often still feel judged—even if the only one judging is the person looking back at me in the mirror.

Since being treated with corticosteroids, my symptoms have improved. My face is mostly clear, save for my Rudolph nose. The itching and burning is a fraction of what it was. I sleep better. I walk taller.

And hopefully, in time, my doctors and I will find a treatment to make my skin look new. In time, I will again feel human.

Exercise and dermatomyositis

Posted on Posted in autoimmunity, dermatomyositis, treatmentsTagged , , , ,

Exercise is important for all of us. For me, it may save my life.

The worst symptom of dermatomyositis is muscle weakness. This happens because when you have myositis, anti-inflammatory cells meant for fighting infection attack your muscle cells. Over time, without regular use, your muscles waste away faster than they would in a healthy person.

Exercise is one of the best ways to combat the muscle deterioration caused by dermatomyositis.

This, of course, means I will have to give up my dreams of becoming a fitness model for a Slavic-language bodybuilding magazine or the National Football League’s oldest rookie wide receiver. Similarly, I will no longer be moonlighting as a piano-moving, tuxedo-clad male stripper. Sorry, ladies.

But muscles are not just for opening safety-sealed jars and trying to embody all that is man. You use muscles every time you move, talk, blink, wink, beat, or breathe.

The body’s diaphragm and external intercoastal muscles operate the lungs and help expand and contract our chest cavities. If these become weak, breathing becomes tough. If they get weak enough, you can’t breathe at all.

The heart is also a muscle. If your anti-inflammatory cells begin to attack it, without treatment, your heart would eventually fail. Fortunately, heart involvement in dermatomyositis only occurs in about 10–15 percent of patients. Fortunately for me, my last electrocardiogram revealed no abnormalities.

Exercise is important for all of us.

For me, it may save my life.

To keep my lungs and heart healthy and regain the lost strength in my muscles, they need to be exerted and stressed. They need to continue to break down and rebuild themselves to combat my immune system.

My whole life I have stayed active, so working out adds nothing new to my routine. At times, I lifted weights, ran, or played touch football with friends as many as five days a week. The last several years, I added protein or creatine or other workout supplements to help build muscle and soften the jump over age 30—at which point, almost all men start to see their athletic performance decline.

So I’m ahead of the game. And doing more of the same—exercising, eating well—can only help me for now. Plus, turns out, creatine supplements are a safe, relatively inexpensive way to improve muscle performance and function in dermatomyositis patients.