Lupus Nephritis: Emily's Story

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Summer was a hectic time for 8-year-old Emily. After weeks of specialty camps, she was exhausted, needing a nap nearly every day. Her mom, Angela, became concerned: this was not typical behavior for her normally active child. She called the family pediatrician. What the family learned days later, changed the course of Emily's life forever.

Emily smiling Lakshmi Hariharan, MD, FAAP, a pediatrician at CHOP Primary Care, Paoli, examined Emily soon after and agreed with Angela: Emily's body was definitely fighting something, but the mystery illness could be almost anything given the symptoms they could readily identify: excessive tiredness, low-grade fever, bruises and red marks with no known cause.

The only way to identify what was causing Emily's symptoms – and hopefully, stop them – was further testing. Dr. Hariharan recommended the family go to the Emergency Department at Children's Hospital of Philadelphia (CHOP).

On July 13, 2012, Emily went to CHOP for a series of blood and urine tests. Given the seriousness of her condition – and alarming blood test results – she was admitted to the hospital for immediate treatment of her symptoms and further testing. Doctors scheduled a bone marrow biopsy for a few days later, in the hope of discovering what was causing her symptoms.

Quick and accurate diagnosis at CHOP

But before the biopsy could be performed, the family received a visit from David D. Sherry, MD, an attending rheumatologist with more than 20 years of experience at CHOP. As a member of Emily's medical team, he'd reviewed her test results and believed he knew what was causing her symptoms.

"He said he was 99 percent sure Emily had lupus nephritis," Angela says. Dr. Sherry explained the condition to Emily and her family as a multisystem autoimmune disorder that causes inflammation in multiple parts of the body, specifically targeting the kidneys. Although there is no cure for lupus nephritis, it can be managed with medication, diet and exercise.

Further blood and urine tests confirmed Dr. Sherry's suspicion and Emily began treatment immediately with high-dose pulse steroids and a round of chemotherapy to address the inflammation in her kidneys. After 5 days, she was able to return home.

Aggressive treatment

Nearly two weeks later, Emily returned to CHOP for a kidney biopsy to determine if her kidneys had sustained any permanent damage. "The doctors were very aggressive with Emily's testing and treatment – which we appreciated," Angela says.

Emily was admitted to the hospital for the biopsy procedure to be performed under anesthesia, and a few days later received another chemotherapy infusion to further reduce inflammation.

Once home, Emily slowly began to feel better. Doctors instructed her not to run or jump for three weeks to allow her body to recover. Once Emily was feeling a bit better – but still on restricted activity – "she made a workout routine for me," says her mom, with a laugh. "It was tough, too!"

New challenges

Soon after Emily's second chemo infusion, her family took a week-long cruise. While Emily was brushing her hair one day, a clump of hair came out. "I screamed," Emily says. "I really freaked out. Losing my hair made everything real."

Over the next few weeks most of Emily's hair fell out – an uncommon occurrence during treatment for lupus, but likely due to how sick she was and chemotherapy. After her initial panic, Emily quickly accepted the new reality and began wearing an assortment of colorful headbands, bandanas and hats.

Emily continued chemotherapy monthly at CHOP until February 2013. Most months, she only missed a day of school to receive the infusion. Throughout it all, Emily continued her active lifestyle – attending school, playing competitive soccer, and performing ballet at a local dance studio.

Once chemo was complete, Emily began to regrow her hair. Over the next few months, doctors gradually decreased the amount of high-dose steroids she was prescribed to keep infections and inflammation at bay. She took low-dose steroids for another year before her family successfully lobbied her medical team to discontinue them entirely during her crucial growing years.

Getting involved

Emily and her sister Emily (R), and her sister Emily's condition has rallied her family, school and community to fight for increased lupus research and expanded treatments. Emily and her family have participated in clinical trials, served on educational panels, and continue to support children diagnosed with the disorder.

"We got involved with the Lupus Foundation of America (LFA) when Emily was first diagnosed in third grade, and were able to organize a mini-lupus walk at her school," Angela says. "The students walked laps around the playground, asked for pledges from relatives, and we ended up raising $5,000 for lupus research. We also raised an additional $16,000 at the Philadelphia Lupus Walk that year."

As Emily has grown, so have her own philanthropic interests. She decorates special pillboxes and writes cards to support newly-diagnosed lupus patients. The items are distributed by CHOP's Lupus Program and Lupus Integrated Nephritis Clinic.

"My diagnosis definitely gave me a new way of looking at things," says Emily, now 16. "It's made me more empathetic; I can understand other people's struggles better."

Ongoing care

Now in 11th grade, Emily has been extremely healthy since her initial diagnosis and treatment at CHOP. She has not had a lupus flare in nearly 8 years, and actively protects herself against infections. "I've become a bit of a germaphobe," Emily says with a laugh. "I use hand sanitizer all the time."

Emily's vaccination schedule was accelerated to better protect her against certain diseases, and she continues to take medication to strengthen her immune system and fight infections. She sees a trio of doctors at CHOP: Dr. Sherry in Rheumatology, Michelle Denburg, MD, MSCE in Nephrology, and Dr. Hariharan, in primary care.

We're so thankful for everyone at CHOP," Angela says. "Their knowledge, experience and compassion allowed Emily – and our family – to have a positive outcome. Thanks to CHOP, Emily has a bright future.

Next Steps

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