Siblings sacrifice to save brother with one-in-a-million disease

Liam Smith is literally one in a million, according to his doctor. The 2-year-old from Seattle was born with an incredibly rare, potentially fatal immune disorder. But Liam’s three older brothers are doing everything they can to save their baby brother.

Doctors say Liam’s best chance at survival is a bone marrow transplant, and he is lucky to have two excellent matches in his brothers. His first transplant failed, so doctors are now preparing Liam for a second attempt while his brothers give up everything from sports to their hair to help.

“They are standing strong in this storm, but not without fear, for Liam,” said Kristina Smith, the boys' mother.

Liam has hemophagocytic lymphohistiocytosis - better known as HLH - a disorder in which his immune system goes into overdrive and attacks his body.

Dr. Paul Carpenter, Liam’s physician at Seattle Children's Hospital, said the disorder most often appears in infants and causes fevers, massive inflammation and, eventually, organ failure. It is so rare that it only affects about one out of a million children. 

Liam first became ill when he was just 28 days old. Kristina said her son developed a high fever and within 24 hours was on life support.

“It’s a really horrible, fast-moving disease," she said.

If left untreated, HLH is fatal. The only cure is a bone marrow or cord blood transplant.

Many children struggle to find one donor that is a good match. Liam is lucky to have two under his own roof – his brothers Hunter and Noa.

When doctors first discovered Liam’s condition, his 8-year-old brother Hunter enthusiastically offered to donate his bone marrow.

"He really wanted to do it," Kristina says. "My boys are so selfless. It inspires me everyday to be a better person."

Liam underwent a regimen of steroids, immunosuppressants and chemotherapies to get his disease into remission before doctors at Children’s Hospital injected him with Hunter’s bone marrow.

Liam responded well and seemed to be cured until a year and a half later when his body rejected the donated marrow and his HLH symptoms returned.

"He was so sick," Kristina says. "He had a temperature of 104 for a week straight. His spleen and liver were huge, and there was fluid in stomach."

Now, doctors are planning another transplant with Liam using bone marrow from 11-year-old Noa.

To increase the chances of success, Liam is being given a stronger drug regimen this time around. Kristina said her son is experiencing "roid rage," lack of sleep, stomach upset, hair loss, weight gain, and anxiety.

"It's kicking his butt," Kristina said. "It takes a lot out of him."

Noa has to temporarily stop playing sports so he does not get injured before donating bone marrow. And all the boys have to be careful not to get sick with infections they could pass on to Liam.

To keep Liam’s spirits up, the three brothers recently shaved their heads to match him.

"I have four superheroes," Kristina said. "I can't describe how proud I am of them."

The single mother of four said she is spending most of her days with Liam at Children’s Hospital, often showering at the Ronald McDonald House.

":I just get up every day and hope God gives me the strength to get through it.".

The family has is feeling the financial burden of Liam’s treatment. Kristina admits she can’t afford to repair the motocross bike her son Noa would love to ride again after Liam’s transplant.

"All I want to be able to do is provide for my boys," she said. "They need to so badly have something positive that isn’t surrounded by this illness."

Doctors anticipate Liam will undergo his transplant in about a month. If it is successful, he should live a normal, healthy life.

"After the transplant he won’t be nearly as sick," Carpenter said. "He should feel normal."

The Smith family is dreaming of the day when they will be out of the hospital and celebrating. The kids have suggested a trip to Disneyland.

"My boys need a much deserved vacation when this is all done and over with,” Kristina said.

In the meantime, Kristina hopes to increase awareness of HLH.

"If kids aren't diagnosed early they're gone," Kristina says. "Providers have to be aware of this disease so these kids can have a fighting chance."

Because HLH can be genetic, Carpenter urges any parents who have lost a newborn child from an unexplained illness to have their other children tested for disorder.

Readers can follow the Smith family on their facebook page "Liam's HLH Journey."