Broken on the inside

Craig youth, family gutting out a difficult fight with rare disease

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Meet Willie.

That's not his given name, just a nickname.

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He's 5 years old, has blue eyes, pale skin and lives with his parents on Barclay Street. He's like any kid -- he likes toy cars, the Cartoon Network and Marvel Comics superheroes.

He likes playing T-ball and wrestling.

Interviewer: Why do you like T-ball?

Willie: "Cause."

Interviewer: Why do like to wrestle?

Willie: "Cause."

He loves, absolutely loves, pickles. Loves to eat them in his room while watching the Cartoon Network, surrounded by toy cars, with a Marvel Comics superhero poster as a backdrop.

Interviewer: Why do you like pickles? Just because?

Willie: "Cause ... they're good."

But, while the preceding words describe a simple child with simple tastes leading a somewhat simple existence, they don't do Willie justice.

He's not simple, or ordinary or anything of the like. He's the needle in the haystack, the one in a billion.

Something inside Willie is broken. There is a fancy name for it, X-linked inhibitor apoptosis deficiency, or XIAP-deficiency. It's extremely rare -- Willie's doctors say there are less than 10 cases worldwide.

So, sometimes Willie gets real sick and can't do all those things he likes to. Can't play T-ball, can't wrestle, can't play with his buddies at school.

Has to stay inside, usually under the watchful eye of his mom, so he doesn't get hurt or sicker.

So he won't have to go to the hospital, or to see the doctor.

They stick you with needles, the hospital and the doctor do. They've stuck Willie many times through the years.

So many times he doesn't cry anymore. So many times he actually likes watching it happen.

David R. "Willie" Lewis was born Aug. 9, 2001, the fourth child of Craig residents Nathan and Sarah Lewis. He's been sick a lot, his parents said, so much the doctors have "told us he's had everything under the sun."

But, two months ago, the XIAP diagnosis was made.

In layman's terms, it depletes Willie's immune system, and causes overactive, but ineffective cells. It makes his liver, spleen and lymph nodes swell, causes fevers of up to 104, 105 degrees, and makes Willie susceptible to cancer or leukemia.

"Never," said Dr. Sheila Fountain, Willie's pediatrician, on whether she's seen a patient with a similar affliction, "and I've seen some weird stuff."

"It's very rare," said Dr. Erwin Gelfand, chairman of the department of pediatrics at the National Jewish Medical and Research Center in Denver. "There are maybe two patients in the U.S. at this time. ... The whole disease is just being genetically identified."

Gelfand said only nine similar cases have been documented worldwide.

Sarah Lewis said Gelfand left the United States this week for France, where he is scheduled to consult with doctors who have treated the condition.

The Lewis' have questioned Willie's doctors about the disease.

What treatments have worked before? Can a bone marrow transplant be curative? How serious is this condition?

How long can Willie live?

The queries have revealed few answers. In April, shortly after the XIAP diagnosis, National Jewish doctors wrote "the understanding of this disease is still largely unknown."

Fountain, who practices in Craig and Steamboat Springs, said doctors are just as frustrated with the uncertainty.

"We just don't know everything there is to know about it," she said.

The disease has been hard on Willie. It's been equally difficult for Sarah and Nathan.

They face the prospect of not knowing his fate.

"The future is a huge question mark at this point," Sarah said.

"When he gets sick, it's overwhelming. Can't sleep, can't concentrate ... you're just scared," Nathan said.

They face the prospect of having a child, who doesn't fully understand what's happening to him, become angry and sometimes violent when he's held back from doing things as a medical precaution.

"He gets mad, sad," Sarah said. "He wants to play. He gets mad and lashes out."

They face financial difficulties. Tests, doctor's visits, hospital stays, more tests, doctor's visits and hospital stays. It adds up and has drained the couple's funds.

"It's like we can lose everything," Sarah said, holding a stack of unpaid bills about a foot thick. "If we can get (bills) paid off, hopefully they'll stop calling."

Sarah, who quit nursing school to look after Willie, works when she can as a waitress at Applejack's restaurant. Nathan works in production at Twentymile Coal Co.

Still, they are about $25,000 in debt from medical expenses, and out-of-town doctor's visits, appointments and consultations mean taking time off from work. That means missing hours, means losing money they can't afford to lose.

"It takes a lot not to cry when talking to people," Sarah said.

They are asking for community help.

They have established donation funds at Moffat County National Bank and Steamboat Springs National Bank. There also are donation jars at Rob's Grocery, Applejack's, Spirit Pass and Sheer Envy.

Money donated to either the fund or the jars will be used to pay the family's mounting medical bills.

Friday morning.

Willie is at home with his brothers and sisters. He's not feeling all that well, but he's being a trooper, bouncing around the house with his siblings, paling around with his dad, swiping his mom's cell phone and quietly hoping he'll later get a chance at the backyard trampoline.

But, he probably won't.

He's sick. His liver and spleen are enlarged, his lymph nodes swollen and he has ulcers in the back of his throat.

When he's like this, sports/activities are out of the question. He could rupture a spleen or liver, Sarah said, and the risk is too great.

So, after a while, he trots off to his room, can of pickles (the best thing about pickles is, of course, "the pickle juice") in hand for an episode of "The Grim Adventures of Billy and Mandy."

The door is closed and the shades drawn.

His doctors are hopeful, though short of certain, that a bone marrow transplant can make a dent in Willie's XIAP. But, like XIAP itself, this is uncharted territory, and it's anyone's best guess what will happen.

His parents want a cure, or at least a research program Willie can enter to help defray costs and restore hope. They're searching for any good news to come before their biggest fear is realized.

"We're just hoping to find something before it manifests into leukemia or cancer," Sarah said. "We're willing to try anything as long as it does not mean losing him."

And while questions float out in the world, and others work hard to answer them, young Willie sits tucked away at home, another sunny day spent behind closed doors and drawn shades.

Joshua Roberts can be reached at 824-7031, ext. 210, or jroberts@craigdailypress.com.

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