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The Case of a 13-Year-Old Girl with Life-Threatening Lupus Onset

Charles Radis, DO  |  Issue: October 2018  |  October 18, 2018

That morning, Dr. Parker and I met with Amanda’s parents in the family room off to the side of the ICU. They were still shell-shocked. Amanda had gone from a goofy, hypercritical teen to a critically ill, unstable lupus patient, seemingly overnight. But at least she was off the ventilator. Dr. Parker explained that just as rapidly dividing cancer cells are sensitive to chemotherapy, the over­active immune system cells responsible for Amanda’s lupus kidney damage were susceptible to chemotherapy as well.

“What’s more,” I added, “we now have the ability to protect other organs from the effects of chemotherapy. An injectable drug called Lupron can shield the ovaries from the side effects of chemotherapy and preserve fertility.”

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Dr. Parker and I believed in her recovery, and pregnancy could still be in Amanda’s future if her lupus came under good control.

After a few minutes, Amanda’s parents gave their consent.

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With her system bathed in the immuno­­suppressive effects of high-dose steroids and an infusion of cyclo­phosphamide, Amanda’s kidneys stabilized just short of requiring dialysis. The cyclo­phosphamide and high-dose steroids slashed the production of antibodies directed against her blood cells, and the bone marrow responded by pouring out new red and white cells and platelets. Her kidney numbers steadily improved, the pericardial drain was pulled, her supplemental oxygen discontinued.

Ten days after she was admitted, Amanda was able to get to the bathroom without assistance. The constricting pain of pericarditis and the tortuous pain in her muscles and joints slowly dissipated. It was as if a malignant finger were lifted from every organ system.

Lupus is primarily a disease of women in the child-bearing years. For every 10 cases of lupus, nine are women.

Life with Lupus

Through her teenage years, Amanda’s care remained a challenge. Her parents separated and subsequently divorced. She sulked, became depressed and, for a while, probably didn’t take her medications at all. She was a no-show at four consecutive office visits.

Following a night of binge drinking, she suffered another seizure and was admitted in kidney failure. This triggered another six-month course of cyclophosphamide and high-dose steroids. Miraculously, the disease was beaten back and the kidneys recovered.

Then, a few days before her high school prom, she decided to go to a tanning booth—with predictable results: dozens of blisters erupted over her back and upper chest. The open sores became secondarily infected and, a few days later, she was found by her father lying on the kitchen floor, unconscious, in septic shock. Somehow, she survived.

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Filed under:ConditionsSystemic Lupus Erythematosus Tagged with:cyclophosphamidekidney failurePericarditisSteroids

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