Making gains against lupus kidney disease
Brian Kaplan was a 14-year-old high school freshman when he was diagnosed with lupus kidney disease. His aunt had died from lupus years before, so the diagnosis felt decidedly grave. Kaplan was prescribed a course of cyclophosphamide, a chemotherapy drug developed for treating various types of cancer that is used off-label to try to halt the progress of lupus. And with the treatment came another piece of grim news : He might never become a father, because cyclophosphamide is known to cause infertility in both men and women. « At that point, my dad even suggested going to a bank and freezing » sperm, says Kaplan. « I didn’t want to do it, but it was always something in the back of my mind. But then, i was also worried about not even living long enough to be a father. « Twenty-six years later, the Washington, DC, resident is not only surviving, but also chasing around a gleeful 3-year-old son. The cyclophosphamide didn’t cause infertility, and after a few major flares of the disease-most notably in college, when his kidneys nearly failed, doctors have told him the disease is active, but low-grade and not progressing. « I wish it were a full-on remission, » he says, « but this is a close second ».
Los Angeles resident Dawn Snow also experienced her first symptoms of kidney disease due to lupus at a Young age, in her 20s
But in two big ways, Kaplan, now 40, and Snow, now 33, have had vastly different expériences. For one, Kaplan had the advantage of a mother who recognized his aches and red face as lupus and wouldn’t give up until she persuaded his doctors to perform a kidney biopsy. The biopsy showed kidney involvement. Within 10 days of showing symptoms, he received his first dose of cyclophosphamide.
Snow, meanwhile, wrote off first her hair loss, then her fatigue, and later her aching joints as « superwoman syndrome » from caring for her mother, fostering her newborn nephew, and working two jobs. She wasn’t diagnosed until her markers for the disease were triple what her mother’s were when she diagnosed with lupus four years before.
And second, Kaplan’s family had health insurance and access to National Institutes of Health (NIH) doctors to treat his lupus kidney disease, while Snow had no insurance at all. Even after she was diagnosed, Snow didn’t get a second opinion when a doctor told her not to worry about her kidneys. As a conséquences, Kaplan is healthy enough to work at a full-time job for the Department of Homeland Security, and Snow receives Social Security disability insurance payments and must endure dialysis treatments to remove toxins from her blood three times a week.
Kaplan is Lucky his disease was caught as early as it was, since early diagnosis meant his kidneys were not as damaged as they might have been. Still, in the 1990s, doctors had few médications, cyclophosphamide, azathioprine, and corticosteroids, to treat lupus kidney disease. Today, though Snow’s kidneys have suffered more damage, her doctors have more treatment options to choose from, as well as the benefit of several Landmark studies and improved treatment guidelines. If there’s a chance Snow can recover form her debilitating lupus kidney disease, it will be because of the expanded base of knowledge about lupus.
How we got to the point where doctors have a greater arsenal of drugs and information for managing lupus kidney disease is a story of diligent researchers, persistent patients, determined physicians, and increasing interest from pharmaceutical companies. Together, these factors have opened up a whole new world of lupus kidney disease care that doctors say is just the beginning.
« Anyone who lived through the decade before this one and then this decade can see that all of a sudden, we’re getting a world of more options, » says Gerald Appel, M.D., professor of clinical médicine and director of the clinical nephrology division at Columbia University College of Physicians and Surgeons. « One decade has just changed everything. »
Countdown to Remission
Kidney involvement occurs in as many as 60 percent of those with lupus. For people like Snow, the clock is ticking. Dialysis has made her feel better, but whether her body can hold out until she gets a kidney transplant is an open question. The good news is that a lot more people being treated for lupus kidney disease are surviving than in decades past : Fifty years ago, the five-year survival rate, the period of time researchers studied patient records was close to zero. Today, it’s more than 85 percent, according to the NIH.
That’s because in the past decade, a series of research studies and new medications have created options. When Appel started treating lupus kidney disease 35 years ago, the only medication available was cyclophosphamide. The plan of attack then was to hit hard, using high drug doses that would suppress the overactive immune system. The drug worked well, but it also caused infertility, hair loss, and an increased risk of opportunistic infection. You couldn’t stay on it too long, because it increased the risk of cancerous tumors, too.
For young adults in their 20s or 30s, the drug often killed their chance of having a family and could ruin their self-esteem with its physical side effects. And while cyclophosphamide did beat the disease into something resembling remission, it was too toxic for people to stay on it as a « maintenance » therapy. You were either on cyclophosphamide, or you weren’t on anything, leaving people subject to lupus flares.
Then, in 2002, a study out of Europe called the Euro-Lupus Nephritis Trial found that lower doses of cyclophosphamide were as effective as the traditional high doses, with reduced toxicity. This offered hope to physicians and patients alike that side effects could be minimized somewhat.
This encouraging news was followed in 2005 by a crucial study, for which Appel was a co-author, published in The New England Journal of Médicine. It showed that a newer drug called mycophenolate mofetil, or MMF (trade name : CellCept), could be as effective as cyclophosphamide,without the hair loss, with much lower risks of infertility and cancer, and with fewer lupus flares later.