'His Body Couldn't Keep Up with His Spirit'

Even when he was getting pinned to the mat in one of his wrestling matches, Sean Fischel had a smile on his face, his two big front teeth—knocked out at the age of 2 when he tripped and fell while chasing his older brother—peeking out from behind his thin upper lip.

Admittedly, Sean was not the best wrestler.

“He didn’t have that drive to go pin somebody,” says his mother, Kim, seated on the edge of the couch in an office inside her Moorestown home. “Sean was kind of more about being happy and just having fun with it.”  

A few feet away, a trifold posterboard sits upright on the floor with photos of Sean glued to it, his smile beaming out of every one. On the other side of the room, his death certificate lays on top of a stack of papers.

He’s been gone less than a month, and the pain is still fresh for Kim and her family. She comforts herself by remembering how Sean lived, and how hard he fought, even bedridden and fragile though he was in the last months of his life, against the disease that was ravaging his 7-year-old body. Whatever warrior’s spirit he may have lacked as an athlete, Sean more than made up for in the battle against his own body. It was a fight he ultimately lost, but one that was not in vain.

“He went through a lot every single day, and was so tough about it, and he never ever complained, and he’d try to smile,” says Kim. “He died trying. It’s just his body couldn’t even keep up with his spirit.”

‘The sickest kid at CHOP’

It started so conventionally: One day at school, near the end of September 2012, Sean came down with a fever. The nurse called home and Kim went and got him.

There was nothing remarkable about it—even for a boy like Sean, who, according to his mother, was hardly ever sick. “Kids get fevers all the time,” Kim told herself.

But something was wrong. Sean was listless, unable to even get out of bed. That lasted three days before Kim took him to the pediatrician’s with a fever of 103. We’re seeing a lot of this, just a virus, the doctor told her, and sent them home.

When Sean’s temperature climbed to 105 the next day, Kim took him to the emergency room, where they were able to bring the fever down. Soon after, his condition severely worsened: the fever returned, his lips became inflamed, and he moaned he was in so much pain, unable to move.

By the time Kim rushed him back to the ER early the next morning, Sean was in septic shock—what happens when the body is fighting a serious infection and blood pressure drops to life-threatening levels—his tiny fingers and feet turning blue, and he had to be airlifted to the Children’s Hospital of Philadelphia (CHOP).

The next day, he went into cardiac arrest twice before doctors hooked him to a machine that pumped all the blood out of his heart through metal tubes in his neck, oxygenated it, then pumped it back in.

“If we hadn’t been at CHOP, that probably would have been the day that he died,” Kim says.

It took four days—“after running every single infectious disease panel there was,” Kim says—before Sean was diagnosed with HLH (hemophagocytic lymphohistiocytosis).

HLH is a relatively rare autoimmune disorder, afflicting roughly 1 in 50,000 children, involving the overproduction of infection-fighting white blood cells, which infiltrate the rest of the body and can lead to tissue damage and organ failure. There are two forms of the disorder: the primary form, known as familial HLH, in which there are identifiable genetic causes; and secondary HLH, in which there is no clear genetic basis for the disease.

Doctors believe the viral infection Sean came home from school with likely triggered his HLH, a common occurrence with the secondary form of the disorder. And once his immune system kicked in, it couldn’t shut itself off.

“In normal people, your immune system clears the virus, and once the virus is cleared, things settle back down,” explains Dr. Kim Nichols, pediatric oncologist at CHOP and the hospital’s go-to HLH expert. “But when you have HLH, the immune response can’t unregulate itself; white blood cells continue to grow and divide and they can infiltrate various organs, and patients can get very sick because they have too many immune cells in places where they shouldn’t.”

The disease wracked Sean’s body, rendering him almost unrecognizable, Kim says. She didn’t take any pictures those first few weeks because she didn’t want to remember him that way—though the images are seared into her memory: parts of Sean’s body swollen to three or four times their original size, his tongue so swollen it wouldn’t fit in his mouth.

Limbs turning black from sepsis. Giant blisters from head to toe. Shortly after he was admitted, doctors had to open Sean’s swollen belly and drain all the fluid because it was crushing the tissue.

“The nurses suggested we put pictures of him up on his door, so they could see what he looked like,” Kim says.

He should have been dead. Nichols says Sean’s symptoms were among the most severe she’d ever seen. The pediatric intensive care specialist told Kim and her husband, Brian, that Sean was “the sickest kid at CHOP that was alive.”

Kim spent every weekday there, while Brian took the weekends. They didn’t hear Sean’s voice for two months.

“They don’t call it a coma, because his brain was still active,” says Kim. “But he was so sedated, and he was on a paralytic, so he couldn’t move. He wasn’t aware of anything. It was for all intents and purposes as if he was in a coma. So during that time, there was always a huge question that, because of all the inflammation, would his brain be functional? We had no idea, for months. So we might have been doing all this stuff and not had Sean in there. But he was. After 50 days, he spoke, and that was amazing.”

This is the first of a three-part series about Sean Fischel and his family. Part two: . Part three: 'Sean's Love is Living on in Everybody'