- Associated Press - Thursday, August 4, 2016

CRESWELL, Ore. (AP) - The role of a little sister is often to be protected by an older sibling. But in the case of Cheyenne Keller, a 12-year-old Creswell girl who died last week of a rare blood disease, she may have switched roles with an older brother - and inadvertently saved his life.

Cheyenne had been struggling with hemophagocytic lymphohistiocytosis, or HLH, since Feb. 9, when her mother, Karen Keller, took her to the doctor and insisted on blood tests for her daughter.

“She just wasn’t feeling well, and that’s not like her,” Karen Keller recalled.

The blood tests were taken - and Cheyenne was whisked by air ambulance to Doernbecher Children’s Hospital in Portland, where she would spend the next six months fighting for her life.

Only one in about 1 million children is diagnosed with HLH, which makes the disease a hard one to identify and then adequately fight.

In patients with primary HLH, cells of the immune system don’t work properly to destroy infected or damaged cells, causing the immune system to become overstimulated and damaging the patient’s tissues and organs, including the bone marrow, liver and brain.

Karen Keller said that, because the disease is so rare, Cheyenne initially was misdiagnosed with aplastic anemia, another rare condition in which the body stops producing enough new blood cells. “She just kept getting sicker and sicker,” Keller said.

Doctors at Doernbecher eventually determined that Cheyenne had HLH and needed a bone marrow transplant. And that’s where her big brother - 14-year-old Joseph Keller - stepped in.

Joseph was a perfect match for the transplant and thought for a short time that he would be able to save his little sister. Instead, he was told by doctors that he also has the HLH gene - and thus would not be able to donate his bone marrow.

While the news was devastating, Cheyenne eventually got the transplant she needed from a donor in another country, and Joseph’s mutated gene of HLH was determined to be dormant.

The Keller family sees a glimmer of hope for Joseph’s future, not only because of the gene’s dormancy but because they learned early enough that he has the same rare illness as his sister. “Initially, we thought Joseph was going to be able to save (Cheyenne’s) life,” said Tom Hubbard, the children’s uncle. “And as it turns out, her path and her story are probably what’s going to save his. Her journey has allowed him to have life.”

Karen Keller said she and her husband, Michael Keller, will be monitoring their son’s health carefully to ensure that his HLH does not develop to the point that his sister’s did.

“It makes us really anxious,” she said. “We have to monitor it with blood tests and make sure he doesn’t show any signs of the gene becoming activated. The doctors truly don’t know what the chances are that it (will) become active. We just have to hope.”

With transplants of any kind, there’s always a risk that the body won’t accept the new material.

In a cruel irony, Cheyenne’s transplant was successful, and her body responded well to the new bone marrow. But by then, her immune system had been incredibly compromised because of the multiple rounds of chemotherapy she’d endured before the transplant, her mother said. “The week she died, she had beat the HLH,” Karen Keller said. “But she had gotten meningitis in her spine, which spread to her brain, and that’s what caused her to pass away. She would have beat the disease if it weren’t for her lack of immune system.”

Amid their crippling grief, Cheyenne’s family members are trying not to think about the difficult, scary and painful moments, and instead remind themselves of the beautiful little girl she was.

“She was always considerate of other people’s feelings, and she loved her brothers,” Karen Keller said. “She played music and loved to paint, and she was very outgoing and kind.

“She was also a really good student - she was smart,” Keller said of her daughter, who attended Creswell Middle School.

Hubbard said his niece was one of the sweetest girls he’s ever known.

“We gave her a little fleece jacket for her birthday in December, and you would have thought we just bought her a brand new car, she was so thankful,” Hubbard said. “She was amazing.”

To help other families who may be going through the same process, the Keller family is working to establish a foundation in Cheyenne’s name. “We want to help people who are going through this,” Karen Keller said. “We’re trying to turn this into a positive experience and make people aware of the disease.”

Cheyenne was close to both of her brothers - 7-year-old Danny and 14-year-old Joseph.

Hubbard said Cheyenne’s parents did their best to gently tell Danny that they were all going to have to let his sister go.

Danny, Hubbard said, “just kept saying, ‘Give her some medicine so she can get better!’ He didn’t really understand what was happening, but for days after she died he just kept telling us, ‘I miss her, I miss her already.’”

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From The Register-Guard: http://www.registerguard.com

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