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Toddler still a ‘giggly’ girl, despite genetic illness

EMMA NICKERSON, 2, is undergoing treatment for globoid cell leukodystrophy, or Krabbe disease, at Children’s Hospital of Pittsburgh. Friends and family of Emma and her parents, Sarah and Jay Nickerson, of Carey, will hold a hog roast fundraiser Saturday at Circle P Ranch. (Photo provided to The Courier)

EMMA NICKERSON, 2, is undergoing treatment for globoid cell leukodystrophy, or Krabbe disease, at Children’s Hospital of Pittsburgh. Friends and family of Emma and her parents, Sarah and Jay Nickerson, of Carey, will hold a hog roast fundraiser Saturday at Circle P Ranch. (Photo provided to The Courier)

By SARA ARTHURS
Staff Writer
Friends and family of 2-year-old Emma Nickerson are working to raise both funds and awareness as the Carey girl undergoes treatment for Krabbe disease in Pittsburgh.
A hog roast will be held at 5 p.m. Saturday at the Circle P Ranch, 5419 County Road 139.
The family’s ordeal started shortly after Emma’s second birthday in February. Parents Sarah and Jay Nickerson noticed she was “walking a little funny,” like she was drunk, Jay said. They at first assumed it was a balance issue and took her to an ear, nose and throat specialist who determined that Emma did have some fluid on her eardrum and prescribed antibiotics, although she got another ear infection shortly afterward.
A few months after her birthday, Emma was playing on the couch and fell and hit her head. Sarah took her to Wyandot Memorial Hospital to get checked out and a CT scan revealed a mass on her brain. After some blood tests, Emma was diagnosed with leukodystrophy, and a physician at the Cleveland Clinic told them it was, specifically, globoid cell leukodystrophy, better known as Krabbe disease.
There are many different diseases all under the category of leukodystrophies, which are inherited, progressive disorders that affect the brain, spinal cord and peripheral nerves. People with leukodystrophy have a deficiency in one of the chemicals that make up myelin, the protective covering of the nerve cells which “acts like insulation surrounding an electric wire,” according to the Krabbe and leukodystrophy website HuntersHope.com.
The Nickersons learned that the foremost expert in the country in Krabbe disease was Dr. Maria Escolar at Children’s Hospital of Pittsburgh. Emma has been undergoing treatment there and her mother is staying with her while her father travels back and forth.
Emma started chemotherapy on June 11 and received a stem cell transplant on June 26. The purpose of the chemotherapy was to suppress the immune system so her body would not attack the transplant. Emma’s transplant came from cord blood from an anonymous donor.
“Emma’s been a trooper this whole time,” Jay Nickerson said.
She has had to endure a lot, including one point where she wound up in severe pain after a hole developed in a catheter carrying pain medication.
Sarah Nickerson’s mother, Kate Chidester, said her granddaughter is “easygoing, laid-back, giggly, funny” and remains so despite all that has happened.
Today Emma’s prognosis appears good. Jay said results of recent tests have been good and she has not developed graft versus host disease, which had been seen as one risk. Jay said the hope is that this transplant will stop the progression of the disease. However, some damage has been done and cannot be repaired, he said.
Emma will need physical and occupational therapy to relearn skills such as how to walk. Her parents are hopeful she will be able to do so.
Jay said Emma doesn’t understand what’s going on.
“She’s only 2 years old,” he said.
But she doesn’t like undergoing treatment in the hospital, he said.
Her brother Vincent, meanwhile, is wondering why his sister is in Pittsburgh. Jay tells him she is very sick and they are trying to get her to feel better.
Jay said he is trying to let Vincent have as normal a summer as possible before he starts first grade this fall.
Krabbe leukodystrophy is a genetic disorder and both of Emma’s parents are carriers. Vincent does not have the disease. Jay said Krabbe affects one in 100,000 births.
Emma has insurance through Medicaid, which Jay Nickerson said has been a “godsend” in paying for her care at the hospital. However, there are other expenses such as gas to and from Pittsburgh, parking fees and lodging at the Ronald McDonald House, where Sarah is staying. Lodging there is $15 a night but Sarah Nickerson will be staying there for several months. Emma is expected to be in the hospital for up to eight weeks and then an outpatient for up to six months.
Saturday’s fundraiser aims to raise money for the family’s expenses.
“Whatever’s left over is going straight to Krabbe research,” Jay said.
In addition to the hog roast, the fundraiser will include a silent auction, games, face painting for children and music by a disc jockey. The cost for the dinner is $8. Tickets will be available at the door.
The family also has established a bank account for donations at First Federal Bank in Findlay, under the name “Embracing Emma.”
Chidester said Emma has a Facebook page also called “Embracing Emma” for well-wishers to follow her journey.
The family is advocating for greater awareness of leukodystrophy, including screening of newborns.
Jim Kelly, former quarterback for the Buffalo Bills, had a son who had Krabbe disease and died at the age of 8 in 2005. Kelly and his family lobbied and got Krabbe and other leukodystrophies added to the routine newborn screenings in the state of New York. The Kelly family also started the Hunter’s Hope Foundation, named after their son.
Jay Nickerson said he hopes to have screening for leukodystrophy similarly required for newborn babies in Ohio, saying that if Emma had been screened as a newborn her illness would have been detected earlier.
Online: http://www.huntershope.org/site/PageServer Arthurs: 419-427-8494 Send an E-mail to Sara Arthurs

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