Tag: lupus

Flourishing in the face of autoimmunity

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

Autoimmune diseases can leave sufferers feeling alone and vulnerable. To our friends, we sound like strangers spitting out words they have never heard. We try to get someone, anyone, to understand that every day, we walk a tightrope made taut by modern medicine over a 10,000-foot drop to oblivion. If and when we fall, doctors cast us lifelines. Sometimes, we lose our grip. Sometimes, the lines break.

The rarer the condition, the more anxious and more depressed its leaves the afflicted. We visit doctor after doctor and wander the world in search of charlatans who have answers to the darkest of questions: Why me? What caused this? Did I do this to myself?

Seeing talented sports stars, actors, singers, and musicians flourish despite their conditions inspires those of us battling autoimmunity.

We have to remind ourselves daily that diagnosis is not the end. Most autoimmune diseases have been thoroughly studied. Though few, if any have cures, many have multiple treatment options. Most treatments are safer and more effective than they have ever been. And in the twenty-first century, many autoimmune patients live long, productive, even happy lives.

Staying on top of their game: sports stars with autoimmune diseases

Pro golfer Kristy McPherson was told by many doctors she would have to give up sports after being diagnosed with juvenile idiopathic arthritis at age 11. A rheumatologist at the Medical University of South Carolina told her that even with the disease, she could still do whatever she wanted.

Pro golfer Kristy McPherson refused to give up sports after her doctors diagnosed her with juvenile rheumatoid arthritis and told her she would never be able to run or jump competitively again. [Chris McGrath | Getty Images]

“All I needed was that one doctor to tell me that,” McPherson said. “That’s when I went back to playing sports.”

Months after becoming the world’s top tennis player, Caroline Wozniacki was diagnosed with rheumatoid arthritis.

She explained: “You start asking yourself questions: What does this mean? Does it mean I can’t get in as great of shape as I was before?”

Though she initially struggled, she bounced back to win the 2018 China Open.

“[Wining in Being] meant so much to me,” Wozniacki added. “It also gave me the belief that nothing is going to set me back. I’m going to work with this and this is how it is, and I can do anything.”

Both McPherson and Wozniacki now speak with young people about living with the rheumatoid arthritis.

Having an autoimmune disease doesn’t mean you can’t be beautiful

Kim Kardashian’s makeup-free selfies have encouraged many others to acknowledge their psoriasis and helped me feel better about flare-ups on my face.

“The disease can cross all socioeconomic lines,” Randy Beranek, CEO of the National Psoriasis Foundation, told The Atlantic. “If someone as famous and visible as Kim Kardashian can have it, it doesn’t make your disease feel so isolating.”

Last December, Kardashian even polled her fans on Twitter about effective medications.

Celebrated singers and musicians have autoimmune diseases

Actress and pop singer Selena Gomez a few years ago revealed she has lupus and in 2017 informed her fans on Instagram about her life-saving kidney transplant.

Few 24-year-olds probably understand what it’s like to have their bodies threaten their lives. To deal with the psychological pain, Gomez checked into Arizona’s Meadows rehab facility. She then faced backlash from fans and from media, who thought she was being treated for drugs or alcohol addiction.

Like Kardashian, revealing her condition to the world opened up mainstream and social media discussions about lupus: What is it? What does it mean for Gomez’s career and for others living with the disease?

Singer Toni Braxton also has refused to let lupus derail her career or her commitment to her family. One of the most decorated pop singers in American history, she has won seven Grammy Awards, nine Billboard Music Awards, seven American Music Awards, among numerous other accolades.

As a guitarist, I find Shawn Lane’s story most inspiring. Widely regarded as one of the greatest guitar players ever, Lane struggled since he was twelve with psoriatic arthritis. His condition not only caused itchy, painful rashes on his skin, but also stiffness in his joints, making it increasingly difficult to play his instruments.

Shawn Lane psoriatic arthritis guitar player
Guitar player Shawn Lane played his entire career in spite of stiffening joints and skin rashes from psoriatic arthritis. His playing and compositions continue to inspire guitarists even after his untimely death in 2003.

Worse, Lane developed Cushing’s syndrome from his long-term use of cortisone to treat his skin and joints. He had to stop playing guitar and died a few years later after developing breathing problems.

Nonetheless, as readers can find more about here, Shawn Lane lived as full of a life as possible, playing in spite of the pain, and producing some of the most beautiful instrumental music of the last thirty years.

Laurence Olivier and Maria Callas: living with and dying from dermatomyositis

As I mentioned here, heralded actor Laurence Olivier and legendary soprano Maria Callas both battled and eventually died from dermatomyositis. That never stopped either of them from prolific careers.

Olivier has been called the most definitive actor of the twentieth century. He lived to be 82, dominating the British stage and starring in more than fifty films.

Callas had a vocal range just below three octaves and is still one of the best known and influential opera singers of the twentieth century. Despite her disease affecting her voice in her later years, she still performed. Forty-plus years after her death, Callas’s name still sells albums.

Hydroxychloroquine staves off dermatomyositis symptoms

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Hydroxychloroquine (Plaquenil) staves off my dermatomyositis symptoms far better than I thought.

Hydroxychloroquine is cheap, relatively safe, and controls the heart and muscle inflammation associated with dermatomyositis.
Hydroxychloroquine is cheap, relatively safe, and controls the heart and muscle inflammation associated with dermatomyositis.

Hydroxychloroquine controls heart and muscle inflammation

This past weekend, I ran out of hydroxychloroquine, an antimalarial drug used to treat autoimmune diseases. By Monday, my heart started racing and palpitating. My lungs felt constricted. Both felt like they were on fire. My throat seemed to be closing, as though I had a piece of food stuck in it.

Doctors call these symptoms myocarditis and dysphagia, respectively. Basically, along with my skin and skeletal muscles, my immune system is attacking my heart, diaphragm, and throat muscles. Oddly enough, clinical tests show nothing. My resting heart rate is a healthy 55 beats per minute. My breathing tests were normal.

I also could hardly concentrate. Much like when you have the flu or are weight lifting, all you can think about is your body’s stress and pain. Much like when you feel anxious or drink too many double-shot espressos, your racing heart makes it tough to read and write.

I forgot all of these symptoms and have not experienced most of them since I started treatment in August. Because I mismanaged how much hydroxychloroquine I had left, they returned within 24 hours of exhausting my supply.

When refilling a prescription is worse than managing a proposal

Trying to understand refill procedures with my local pharmacy is like trying to communicate with project managers building the Tower of Babel.

Speaking of the ancient world, to remedy the situation, the pharmacist sent my rheumatologist a fax for the refill.

As a Denver Broncos fan, I know all too well the dangers of faxing in the 21st century, so I sent my rheumatologist a message through his online portal Monday. No response. I called the office Tuesday. His medical assistant’s voicemail says she will call back within 24 hours. She did not.

By Tuesday evening, I gave up and phoned my dermatologist, who is always on top of things. Within 45 minutes, the pharmacy cleared my refill.

Treating autoimmune diseases with hydroxychloroquine

Today, after taking 200 milligrams of hydroxychloroquine last night and this morning, my body is returning to normal. No more heart and lung issues. My throat feels less swollen. I will not mismanage my prescription again.

First developed in 1955 for treating malaria, hydroxychloroquine is the first-line treatment for dermatomyositis and lupus. Compared to other immunosuppressants, it has few side effects and is so safe pregnant women can and do take it. Better still, it costs next to nothing; even without insurance, a month supply is less than $25.

The only downside is long-term use of hydroxychloroquine can be toxic to your eyes. To make sure nothing like this happens, I take the recommended daily maximum dose and have an ophthalmologist as part of my care team.

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?

What is dermatomyositis?

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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.

When knowing something is worse than knowing nothing

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Antinuclear antibodies. Autoimmune. I thought they were terms from contrived Hollywood procedurals to make the characters sound smart, words worth dismissing alongside the rest of the medical-school jargon I overhear in hospitals or read in news articles posted on the wall at the internist’s office.

Now, in some poststructural mockery of my own life, of my own beliefs, those words play hegemon over my mind like the Bratva over Moscow. Language has become reality. Who I am, who I was is lost.

Antinuclear antibodies. Autoimmune. I thought they were terms from contrived Hollywood procedurals to make the characters sound smart. Now, those words have become my reality.

For almost a decade, I have bowed to no god in heaven or earth, sworn allegiance to no flag but my own, obeyed no master but myself. I now supplicate to some being I cannot hear, I cannot see, wondering what a handful of numbers mean.

My last blood tests showed my antinuclear antibodies exceed thirty times the normal range. An indirect fluorescent antibody test returned a speckled pattern. My rheumatoid factor—another antibody test—is elevated.

My breaths are shallow. My chest is tight. My arms, legs, and chest are covered in rashes. Sunlight is either the cure or its burning me from the outside in, and I, like the biblical Job, cry “Violence!” to a god I do not believe in; I hear no answer—not from Jehovah, not from my doctors.

My internist suspects lupus or some other mixed connective tissue disease. My dermatologist believes I have dermatomyositis. Both say I need more tests.

Antibodies are proteins produced by the body in response to foreign substances—for example, viruses, bacteria, toxins. Antinuclear antibodies (ANAs) are produced when the body fails to adequately distinguish between what cells do and do not belong. Though healthy bodies can and do produce ANAs, more often, high concentrations of ANAs indicate an infection, most often, an autoimmune disorder.

I now wait with just enough information to make me anxious, paranoid, afraid, hopeless, depressed, but not enough knowledge to move through Kübler-Ross’s remaining stages of grief.

I spend too much time online reading about these diseases, wondering why I have them and from where they come. I am a sinner in the hands of this enigmatic twenty-first-century god, this omniscient being we call the internet, this entity with all the answers. I search website after website, looking at prognoses, forecasting my future. I see no answers.

My chest x-rays are negative for any lung disease. My electrocardiogram (ECG/EKG, depending on how much Greek you know) was completely normal. I try to focus on these positives, but I wonder if I am just bargaining. Grief stage two.

I speak with my girlfriend, with my parents, with close friends, with my therapist. I let some of my coworkers in on my news—after all, they need to know why speaking has become tough, why some days are more exhausting than others, why I keep leaving for different doctors. All are encouraging. They assure me modern medicine always has a cure (or at least a treatment).

But does it?

I try to remain positive. But at what point does optimism become denial?