Ohio boy dies of complications after transplant

BY BETH WHITEHOUSE | beth.whitehouse@newsday.com

CINCINNATI - When Keir Zangrando was alive, he created a superhero with a big "A" on his chest and named him Amphibian Man. In bright colors on spiral notebook paper, Keir drew comic books of Amphibian Man slaying enemies.

In episode three, Amphibian Man destroyed a monster Keir called EMLA. In episode six, he vanquished Dr. Snake Bite.

When Keir's father pages through the drawings today, he sees clearly that Keir used cartoons to vent his frustration with his Diamond Blackfan anemia.

EMLA is the cream Keir used to numb his thighs or abdomen before nearly nightly injections of a liver-protecting drug called Desferal. Dr. Snake Bite represented the nurses and doctors who poked him at monthly blood transfusions.

"He had a lot of anger about his medical condition and all the things he had to go through, a lot of fears that he would express through these cartoons," David Zangrando said, sitting on his living room couch, his son's drawings spread before him on the coffee table.

Keir died at the age of 12 in 2003 after undergoing a bone marrow transplant, a procedure he and his parents hoped would rid him once and for all of Diamond Blackfan. Keir's is a story of a family that reached for hope and lost a son in the process.

Keir hated the medicines he had to take, hated the anxiety he felt about his disease, hated not being like everyone else his age. When he had the opportunity to have a transplant, he begged his parents to let him.

Like the Trebings, the Zangrandos had been told a transplant would give their son greater than 90 percent odds of a cure. Like the Trebings, they were told a transplant from an exact-match sibling was the treatment of choice for young Diamond Blackfan patients. And like the Trebings, they were told the odds were better before puberty.

Sister an exact-match sibling

But the Zangrandos didn't have to go through what the Trebings did to have an exact-match sibling -- their daughter, Emma, was born a human leukocyte antigen match to Keir, meaning their DNA shared a common trait that allowed his body to accept her bone marrow. The potential solution was at home.

Even with that, the Zangrandos were initially against a transplant, scared by the chance Keir could die. Their opposition changed when Keir was 10, and they attended Camp Sunshine in Maine for a gathering of Diamond Blackfan families.

At camp, the children played together, showed each other how they did their Desferal pumps, and for a few fleeting days weren't so different.

Meanwhile, parents talked about the disease. One educational panel was made up of Diamond Blackfan patients in their twenties who talked about how in college they didn't want to be bothered with the Desferal pump, and how they had developed complications such as diabetes.

"They didn't want to keep a pump attached to them all night when they were partying," Wendy Zangrando remembered. "They didn't mean not to take it, but you know how it is, you're going out on a date . . ."

Parents opt for procedure

With those stories in their heads, the Zangrandos listened to the doctors touting transplant for younger patients.

"I thought, 'I'm so lucky I'm finding all this out when my son is still okay,' " Wendy recalled. "I knew a transplant would be tough, but I thought the odds of something going wrong were very, very remote."

She hated that the disease required Keir to have blood transfusions -- she once insisted on a vegetarian donor because she worried about mad cow disease; she constantly worried about Keir contracting AIDS. In her darkest moments, Wendy Zangrando fretted about whether a national crisis like 9/11 would prevent blood from making it to vulnerable people such as Keir.

Still, making the transplant decision was agony for the parents.

"We didn't know what the future would bring if he didn't have it," said David Zangrando, 49, an anesthesiologist. "I didn't want that to happen -- that in the future we'd say, 'Why didn't we do it?' "