You may think you can huff and puff and blow my door down but I’m ready for you to get the hell out of my way. I know that I’m not alone. Get out of their way, too – all 1.5 million Americans with lupus have had it with you. So Mr. Wolf, take your fancy Latin name (lupus), your mean, fiery eyes; ferocious growl and vicious bite and turn it on your own body, the way you make us turn on ours.lupus

Howling Back

We’ve tried it your way; we’ve tried to let your sleeping dog lie, but you insist on waking up and picking a fight. We’ve tried to tame you, pet you, and coddle you. We’ve fed you what you what craved – sleep, the dark, the corticosteroids, yet you’re still ravenous.

the lupus wolfWe quiver at the sight of you out there sniffing in our yards, inhaling the scent of our vulnerabilities as you do. We bolt our doors, draw the shades and hide from the sunlight, but still you find a crack in the window, a forgotten ladder to the upstairs, a back door not locked. The alarms sound: you’re hungry – our hair falls into our hands, mouth ulcers erupt, temperatures flare, joints creak and swell; migraines explode, fingers stiffen, and infections flourish. If you’re especially starved, you’ll go for the good stuff: heart, lung, kidneys, brain, gut. Carnivorous bastard. Is there any part your nasty teeth don’t devour? Flannery O’Conner was right. “The wolf is tearing up the place.”

The body cops arrive armed with their steroids, the only acute weapon in their arsenal, non-discriminating as they are. They start firing in every direction. Now cower. Lick your wounds. Hobble away. Friendly fire explodes. Is that you limping back Mr. Big Bad Wolf, or is it I? And others like me, or God forbid, much worse.

Go back to your den. Curl up with your pack. I’ve got a pack too – my family, my friends, my doctor, myself. And another thing – get near my cub and I’ll tear you apart.genetic lupus

Aujourd’hui, le lupus

What will it be today? Will you take our breath, our strength, our mental clarity? Will you simply light upon our skin, a visible outward threat to the inward inflammation? These are some of the questions asked by people living with systemic lupus erythematosus (SLE), ninety percent of whom are women. The disease most often strikes women of childbearing age. For an unknown reason lupus is all too often more physically devastating for women of color. Research is finally focusing on this group after a CDC study identified the highest lupus-related death rates to be among black women. (Another study published in “Disease and Ethnicity” predicted that the lowest SLE disease severity was among Caucasian females with PPO/POS insurance.)

With lupus, the body overreacts to an unknown stimulus, making too many antibodies directed against otherwise healthy body tissue. In other words, the body turns on itself, often igniting an inflammatory or infectious process, hence its classification as an autoimmune disease.

sites for lupusCapricious by nature, lupus is cruel in its unpredictability. Severe, organ-threatening disease can cause such complications as lupus nephritis, or other kidney involvement (The Lupus Foundation estimates that approximately 40% of lupus patients have kidney involvement); pericarditis, (an inflammation of the pericardium, a sac lining the heart); and premature atherosclerosis (the third most common cause of death in lupus patients, following kidney disease and infection). Even the non-organ threatening manifestations such as arthritis, recurrent infections and more can be chronic and painful, forcing lifestyle changes and limitations of one’s capabilities. Patients experience acute stages of the disease, known as “flare-ups,” periodically throughout their lives.

Lupus is a disease that is simultaneously under-diagnosed and over-diagnosed, either of which can have devastating implications for people in the prime of their lives. A highly elusive disease, lupus frequently dodges diagnosis for many years. Surveys show that most patients have suffered symptoms for at least three years, and commonly, as many as ten before lupus is identified. In the absence of a diagnosis and treatment, patients may sustain permanent damage to vital organs. At the same time, tenacious patients in pursuit of answers all too often find themselves hearing the humiliating words, “that it’s all in your head.”

On the other hand, an inaccurate diagnosis may render costly and toxic treatment. Corticosteroids (such as Prednisone) are often among the first line strategies of treating the disease, particularly during an acute episode. These medications, while offering relief and results for lupus patients, are not without a potentially dangerous, disfiguring,  and disheartening price. A mistaken diagnosis may also influence a patient’s ability to obtain insurance, significantly raise their premiums if they have insurance, or potentially interfere with successful employment. (A health insurance broker once told me that insurance companies really dislike lupus patients as “so many don’t die and none get well.”)

In “The Lupus Book,” Daniel J. Wallace MD, a world-renowned rheumatologist and Clinical Chief of Rheumatology at Cedars-Sinai Medical Center, wrote, “lupus is a difficult diagnosis.  A complex diagnostic workup is often necessary, and few physicians are equipped to interpret the necessary battery of tests. In these instances, most physicians will consult a board-certified rheumatologist or recommend that their patients visit such a specialist.”

My own story. This is not a pity-party. This is for other lupus patients, or those lost in diagnosis land.

I began suffering symptoms at the age of thirty, a year after the birth of my daughter. Stabbing pain in my chest and center of my back prompted me to see my doctor. The diagnosis was pleurisy, an inflammation of the lining of the lungs that is not unique to patients with lupus and can strike virtually anyone. Ten years later when I was diagnosed with SLE, I was told that pleurisy is often one of the first manifestations of the disease.

Profound fatigue followed. By ever-increasing measure, the fatigue was soon accompanied by muscle and joint pain. I had always been an energetic and active person, described by some friends as actually hyperactive. A reduction in my stamina was glaringly apparent to me and them.

Another bout with pleurisy followed. After much frustration and fear, I sought a rheumatologist who diagnosed my condition as fibromyalgia, which is defined as “a pain amplification syndrome characterized by fatigue, a sleep disorder, and tender points in the soft tissues.” This was in the 80’s and fibromyalgia naysayers abounded. I was among them, now thoroughly convinced it must be in my head.

Within a few years other symptoms presented, yet my efforts to find successful treatment remained elusive. In time, a series of “episodes” occurred in which I began to have memory loss, acute speech difficulties, dizziness and loss of balance. (Close friends should be advised that these episodes are not be confused with my general klutziness. Moreover, no wine was involved.) Migraine headaches held me hostage in the dark and quiet. Next came a rare form of migraine that sent me to the emergency room several times with acute and bizarre symptoms in which I could suddenly only see half of an image – as in half of someone’s face. At the same time, the room I was in might turn upside down in my vision. Trust me, that is scary. I never did LSD in my youth, but I’ve wondered if those experiences couldn’t be likened to a bad trip. At least one of these episodes may have been a stroke, or TIA as a later MRI showed evidence of such. I was 42.

A chronic and mysterious swelling in various lymph glands alarmed me and became more painful and more swollen every day, particularly when I was more fatigued than usual. My hands, which ached night and day, began to look rather patriotic: red, white and blue. I had chronic kidney and bladder infections and was treated with prophylactic sulfa, which turns out to be an absolute “no-no” for lupus patients.

Again, I made the rounds of physicians including neurologists, cardiologists and even a psychiatrist. Each offered an explanation, diagnosis and treatment of the symptoms related to their specialty. None could link the symptoms to a bigger picture. I soon found myself on a wide variety of expensive and potentially harmful medications.

I was defeated and ready to surrender in my quest for an answer when I became acutely ill with a rare form of inflammatory colitis. Oddly, what I recall most about that time was the distant sound of a train making its way toward Atlanta around 5AM during those sleepless nights. To this day, the sound of a train makes me feel alone and afraid. After three weeks and fourteen pounds of weight loss, I was admitted to the hospital, dangerously dehydrated and with life threateningly low blood pressure. The situation led me to a gastroenterologist, one of the few physician specialties I had not previously sought. A colonoscopy identified lymphocytic colitis (also known as collagenous colitis), which, like lupus, is an autoimmune condition. My joints were swollen and painful. The gastroenterologist brought in a rheumatologist to consult. After another extensive battery of tests, I was diagnosed with SLE.

My husband and I were somewhat relieved. On the one hand, I had begun to fear the likelihood of lupus, so much so that I had dreams about the disease, yet I was relieved to at last comprehend the relationship between the vast array of seemingly unrelated symptoms and be able to begin a treatment regimen.

Everyday living with lupus

Since my diagnosis fifteen years ago, I have experienced many other manifestations of the disease: pericarditis, costochondritis (inflammation where the sternum meets the rib); osteoarthritis, hip bursitis, uveitis (inflammation of the uvea – the eye’s middle layer); anemia and malabsorption; interstitial cystitis, repeated opportunistic infections, shingles, and  today’s diagnosis of an inflammatory eye condition associated with a staph infection and more lupus du jour expressions. No matter – I have lupus-lite! I’ve included this list not as pity-party favors, but rather to demonstrate the myriad of ways in which this wolf can bite, even among those of us who have dodged some of the scariest and life-threatening afflictions that can lead to renal failure, dialysis, kidney transplant, pulmonary embolisms and death.

Tell Congress to continue biomedical research and lessen the disparities in care for people with lupus.

(to be continued: other patient stories, treatment, research and the future)

Terri Evans

Terri Evans

Terri Evans is 25+year marketing communications professional, a partner at LeslieEvansCreative and Bcauz marketing (cause-related). She has been a food columnist for Atlanta Intown and Atlanta Buckhead newspapers, and a contributing writer for Georgia Magazine, the Atlanta Business Chronicle and other publications. Evans was also a finalist in a Southern Living cooking competition. She is (and has long been) at work on a novel set in the South (of Georgia) and the South (of France). She's always cookin' up somethin'.