Girl Survives Deadly Medicine-Resistant Fungal Infection

Nov. 18, 2010— -- Sierra King, 16, of Kansas City, Mo., finished the book "My Sister's Keeper" in a single week in August 2009 and jotted it down as one of her favorites.

The bestselling fiction book by Jodi Picoult -- which later became a hit movie -- follows a young girl who battles leukemia and the difficult decisions her family had to make, and sometimes had no choice in making, to keep her alive.

Just a week after finishing the book, Sierra herself was diagnosed with leukemia.

"It was so weird to me," said Sierra.

Sierra's father, Walter King, 59, took her to the hospital because they thought she was coming down with the flu. But hospital tests revealed Sierra's white blood cell count was dangerously low and doctors diagnosed Sierra with acute lymphoblastic leukemia.

"It was right out of the blue," said Walter King, who was so overtaken by news of the diagnosis, he quit his job to stay with his daughter.

Acute lymphoblastic leukemia is the most common type of blood cancer in children and has more than a 90 percent survival rate if treated early, according to the Leukemia and Lymphoma Society. So doctors immediately began chemotherapy.

"We thought she could definitely beat it," said Walter King. "We were optimistic."

Within three weeks, Sierra was in remission. But, her fever persisted and she noticed abnormal bleeding.

"I had some spots on my legs too, so they [the doctors] did biopsies on that," said Sierra.

While Sierra made strides to treat the leukemia, she contracted a fungal infection that spread to her blood system and sinuses.

"We kind of thought that cancer was bad, but we knew it treatable," said Sierra. "But the fungal infection, it was so much more than we ever expected to deal with."

Infections are common among many leukemia patients, because chemotherapy weakens the immune system. But Sierra contracted a rare form of fungus called fusarium, which was resistant to all forms of medications normally used to treat an infection.

Sierra's doctor, Dr. Kathleen Neville, a pediatric oncologist and clinical pharmacologist at Children's Mercy Hospitals and Clinics in Kansas City, Mo., said she was stuck between continuing her chemotherapy and risk lowering her immune system -- which would put her at risk of dying from the infection -- or treating the infection but risk falling back on her leukemia treatment.

Surgeons tried to remove parts of her sinus tissue where the infection built up, but found they could not keep up with how fast the infection spread.

"We both [my wife and I] sat on the floor [of the hospital] crying, and we thought our girl just had enough," said Walter King.

Meanwhile, the pain of chemotherapy treatments mixed with the cocktail of medications sent Sierra in a month-long state of delirium.

"I remember people visiting," said Sierra. "That's one of the only things I remember, a lot of people supporting and praying for me."

But Neville said she consulted other oncologists across the nation who specialized in fungal infections or alternative treatments.

"Because there are so few people who survive this and the infection was everywhere, there was no right answer," Neville said.

Neville decided to stop parts of her leukemia treatment to allow for Sierra's white blood cells to build back up. Neville said it was a risk, but Sierra's immune system was the last weapon they had.

"She was so sick at that point that we had no choice but to let her white blood cells fight that infection," said Neville.

Neville said she expected the rush of white blood cells would cause certain parts of Sierra's body to become inflamed. And she anticipated Sierra's condition to get worse.

In fact, she thought it would be safe to tell the Kings that Sierra may not survive. She said she told the Kings to "say their goodbyes."

"I remember we told her that we loved her and if she needed to go she should go, but she's always welcome to stay," said Walter King.

But Sierra did make it through the night.

As each day went by, the infection subsided, while the leukemia continued to be kept at bay.

"You just take your best expertise and make what you think is the best possible decision," said Neville. "Did I know it was the best at the time? At the time, I don't think anyone could've known. ... It was the best possible decision in the worst situation."

Sierra was only one of four in the nation to overcome this type of infection, Walter King said doctors told them.

"You just don't survive it, they said. But she did, that little stinker," said Walter King.

Sierra has remained in remission for a year. The leg braces she now wears as a result of the nerve damage caused by the chemotherapy treatments are the only remnants of her disease and near-fatal infection, she said.

"I always felt very loved, so I never felt alone fighting through it," said Sierra. "I never wanted to give up, and I think that's because of all the love and support that I had."

Indeed, Neville said there were many times during Sierra's illness where conventional means of treating Sierra would not have worked. She said the choice Sierra's family made to stick by Neville's treatment decisions contributed to Sierra's survival.

"I've seen so many cases, and the fact that she has battled back from it all is remarkable," said Neville. "I think there's an unquantifiable piece of the human spirit that makes this possible."