Olivia’s journey toward wellness

When your child doesn’t respond to standard treatment, a seasoned specialist makes all the difference.

Olivia Perera, 10, is much like any fifth grader. She loves to swim and skate, and sometimes her mother, Priyangani Rajapakse, has to tell her siblings — Sylvia, 16, and Riley, 14 — not to tease her. Unlike her peers, though, the young Bellerose resident has spent eight years fighting a kidney disorder called minimal change disease.

It began when she was just shy of her second birthday, and Olivia’s whole body looked swollen.

“I thought it was spring [allergies], so I took her to the Cohen Children’s Medical Center Urgi Center,” Ms. Rajapakse said. “They agreed and sent us home. Afterward, I noticed the swelling was worse and that Olivia wasn’t going to the bathroom."

Ms. Rajapakse and Olivia returned to the Urgi Center, and clinicians tested Olivia’s urine. The results showed high levels of protein, so the staff brought mother and daughter to the Emergency Department. The doctors diagnosed Olivia with nephrotic syndrome caused by minimal change disease.

“Most children with minimal change disease — more than 90 percent — respond to the standard treatment of a two-month course of steroids,” said Christine Sethna, MD, director of pediatric nephrology at Cohen Children’s. She added that Olivia initially responded well to steroids, but relapsed when Dr. Sethna began to wean her off them.

“Olivia became dependent on them, so we had to look at other treatment options to avoid complications from long-term steroid use,” Dr. Sethna said. She has prescribed several immuno-suppressants during the past eight years while Olivia maintained a steroid regimen to prevent relapse. Unfortunately, the immuno-suppressants caused side effects like high blood pressure, abdominal pain, and oral ulcers and bruising.

High blood pressure increases the risk of a cardiovascular event in children with nephrotic syndrome, although the level of risk compared to the average child is unclear. Dr. Sethna is searching for answers.

Working to reduce future risks

“My research, which Olivia participates in, is a national, 14-site study of children with nephrotic syndrome,” Dr. Sethna said. “Cardiovascular disease is the number one cause of death in adults with history of childhood kidney disease. Children usually develop it between the ages of 2 and 8. With our research, we hope to identify these patients early and intervene to reduce their risk.”

A customized medication regimen allowed Olivia to stop taking steroids last June, and she’s been in remission from minimal change disease and nephrotic syndrome since October 2016. Her doctors are hopeful she’ll outgrow the syndrome during puberty, like most children with the disease.

Now, Olivia’s enjoying life like any other tween girl, and she’s even hoping to start dance classes.

“I’m so thankful for the nurses and doctors who have helped us, and go out of their way to cheer up Olivia when we have follow-up visits,” Ms. Rajapakse said. “I’m confident her care is in good hands.”

7 tips to prevent kidney disase in children, from the National Kidney Foundation: