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FIFTY YEARS IS A LONG WAIT: ISN'T IT ABOUT TIME FOR LUPUS?
AS I SEE IT
By MARIANNE CLAY
from Sunday, January 11, 2009, Harrisburg Patriot

Eight autumns ago in her senior year of high school, my then 17-year-old daughter began struggling to get her backpack on and off. Moving her arms and shoulders hurt, she explained, because she was folding so many jeans at her job at The Gap. Or maybe, she added, she needed a new mattress.

A few weeks later, when her long hair began to fall in piles of yellow silk in the bathroom sink and dark circles deepened under her eyes, I dragged her to the family doctor for a mono test. But she didn't have mono.

By Thanksgiving, she'd lost her enthusiasm for play rehearsals, for being yearbook editor, and for hosting exchange students. She didn't stop, but instead of grinning and chatting about her activities, she was soldiering through. And what a tired soldier she was.

During Christmas vacation, when her fingertips turned as black as India ink, and crushing fatigue brought her life to a halt, we stopped looking for possible explanations in everyday life. Something was so wrong.

She didn't have cancer, and she wasn't infected with a contagious disease. In fact, she didn't even have a rare condition, but she had a serious problem that wasn't going away. Turned out, after all the tests were in, she had lupus.

In lupus, the immune system, instead of protecting the body from foreign invaders, becomes a misguided missile that hurls its weaponry at healthy tissue and organs. What a betrayal. The body's protector becomes its destroyer. While a healthy immune system produces antibodies to fight and destroy viruses, bacteria and other foreigners, the immune system with lupus produces autoantibodies -- antibodies against the body's healthy cells and tissues.

These autoantibodies inflame the body causing exhaustion, skin rashes and joint aches. With more time and intensity, these autoantibodies can damage and alter the function of organs and tissue in every part of the body. Why all this happens and how to stop it are mysteries.

"You have," the rheumatologist told my daughter shortly after New Year's Day 2001, "systemic lupus erythematosus, SLE, a chronic, lifelong, autoimmune disease that can affect your entire body. We will treat it by quieting down your immune system, and you will begin to feel better. But you will have to carefully choose from the array of things you want to do, because you will need to parcel out your energy carefully. And you will have to be monitored, always."

Our minds reeled, but she was not alone then, and she is not alone now.

At 17, she had just joined the 66,000 Pennsylvanians and the more than 1.5 million Americans who live with lupus. According to the Lupus Research Institute, more Americans have lupus than cerebral palsy, multiple sclerosis, sickle-cell anemia, cystic fibrosis or AIDS, making it one of this country's most prevalent medical problems.

And it's hardly new. Lupus was first described in 1828.

Though so many Americans have lupus, lupus keeps a low profile. But its low profile shouldn't minimize its seriousness. A small, but significant minority die each year in the U.S. from lupus. Most lupus patients have, thankfully, more manageable cases, but even the lucky ones lead lives altered from what they planned for themselves. I say planned because most are diagnosed between ages 20 and 40.

They are often, like my daughter, just beginning their lives, and nine out of 10 lupus patients are, like my daughter, female. Besides being a predominantly women's disease, lupus also is more prevalent among African-Americans, Latinos, American Indians and Asians. Yet another lupus mystery.

Even when lupus is mild, it is ever-changing, a disease characterized by periods of illness or flares, when activities such as going up the stairs, getting to work and shopping for groceries become enormous challenges. These flares are made worse because lupus is so unpredictable. A stiff knee one day becomes swollen feet the next, and utter exhaustion the day after that.
Keeping a demanding job during a flare takes a special perseverance, but lupus patients seem to have perseverance in abundance.

The flares alternate with periods of wellness or remission, and, of course, the whole goal, until a cure is found, is to make the remissions last far longer than the flares. All lupus patients, even those with mild cases, must be ever vigilant, protecting themselves as much as they can from fatigue, stress, crowds and even sunlight, because sunshine is a known trigger for a flare.
Fortunately, my daughter's diagnosis came swiftly, thanks to the dramatic black fingertips. (While many people with Raynaud's phenomenon do not have lupus, some people with lupus develop this inflammatory response that shuts down circulation in their fingertips and toes.) Her speedy diagnosis meant earlier treatment and more opportunity to protect her body from damage, an opportunity I wish for all lupus patients.

Far more often, according to the Lupus Foundation of Pennsylvania, patients average four years between onset and diagnosis. That's not because they're not seeing good doctors. It's because lupus is a complex disease.

Known as "The Great Imitator," it symptoms mimic many other health problems that often send diagnosticians racing down the wrong paths. In addition, no definite diagnostic test exists for lupus. Finally, because the worst of the disease activity happens silently within the body, lupus patients often look well, and friends, families and the patients themselves minimize and explain away their health problems. That's why many do not seek help, until after they've suffered organ damage.

Less than a year after my daughter's diagnosis, she went into remission and stayed there for most of her college years until flaring again. This flare has been far more stubborn than the first, and she has stayed, despite all the meds she takes each morning to get her feet on the floor and out the door to work, in high disease activity.

Meanwhile, she and her doctors continue to work to find ways to turn off her immune system's attacks. Her up-and-down, variable situation is a common lupus scenario and, so once again, she is soldiering through. And for now, that's all anyone can do.

For now, researchers cannot point to an exact cause for the development of lupus, though they know genetics and the environment play roles. Nor can they offer a cure. But things are happening.

Thankfully, after decades when lupus got little notice, its study is attracting the best and the brightest. The National Institute of Health recently awarded $60 million for lupus research in addition to its own studies and, for the last six years in Pennsylvania, the state has been helping, too, with money for lupus research and education.

Lupus patients are grateful, as they need every helping hand they can get. After all, no major new treatments for lupus have been approved in 50 years.

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