MOUNT ZION - Who's the bravest kid you know?

For the University of Minnesota physician who asked the question, the answer is Chase Davis, 10-year-old son of Bev and Darrin Davis of Mount Zion.

Chase is no different from lots of other 10-year-olds. He likes to try to beat his grandmother, Pat Knox, at playing the card game Uno. He's a great fan of Sponge Bob, the St. Louis Cardinals and Illini basketball. He grins from ear to ear at the mention of a good friend's name.

But what's different about Chase is that daily he takes 11 medications. Weekly he undergoes physical, occupational and speech therapy sessions in his home, relearning and retraining muscles weakened in his battle against adrenoleukodystrophy.

After undergoing months of treatment for adrenoleukodystrophy at the University of Minnesota Children's Hospital, Fairview, Chase has been home three weeks. With assistance he can stand and take a few steps, roll over to his tummy, do arm and leg exercises and go watch his younger brother's basketball game.

It was in July 2005 that the Davises learned their son had the genetically inherited, progressive neurological disorder. According to stopald.org, adrenoleukodystrophy is a deadly disease which can be fatal or cause mental and physical handicaps due to complications of the disease itself or its treatment.

Yet, at almost the same time as Chase was diagnosed, hundreds of miles away, doctors and a medical team at the Fairview hospital were learning about the possibility of a different way to treat the Davises' nightmare.

While on a Florida vacation, the Davises and Chase, along with A.J., now almost 7, and Bailey, 4, ended up in a hospital emergency room when Chase had difficultly picking up his feet to walk.

After imaging X-rays, a doctor there told them Chase had adrenoleukodystrophy. It scared Chase so much, said his mother, he was afraid to sleep for days for fear he might not wake up the next morning.

It scared her equally after she researched the disease on the Internet.

Just weeks later at Barnes Children's Hospital in St. Louis, the diagnosis was confirmed. The Barnes' staff, said Bev Davis, suggested contacting the Fairview hospital as the best choice in the country for treatment.

That's where the Davises met Drs. Lawrence Charnas and Paul Orchard as well as a team that included geneticists, biochemists and psychologists. Charnas is an assistant professor of pediatrics and neurology at the University of Minnesota Medical School; and Orchard, an associate professor of pediatrics and the medical director of the Inherited Metabolic and Storage Disease Bone Marrow Transplantation Program.

It is Charnas who touted Chase's bravery and, on Chase's down days, frequently cajoled and teased him into smiles and laughter.

Adrenoleukodystrophy, explained the physician, describes the organs involved, adrenal glands and the white matter of the brain, and dystrophy means it's inherited and degenerative. The white matter, tubing which protects nerves, was being destroyed.

The Minnesota team offered Davises a chance for Chase to receive a cord blood cell transplant in conjunction with a drug regime untried for adrenoleukodystrophy.

The transplant process was not new and had been used from the early 1990s with some good outcomes, said Charnas. But when it was used in later stages of the disease, the survival rate lowered while the disease accelerated. Two years ago a moratorium was placed on transplants for boys under age 10.

"If they survived, their disease had accelerated too far and they were left disabled. It was awful," he said.

Charnas learned from a New York colleague about a study of adrenoleukodystrophy patients' brains where certain chemicals were identified as perhaps part of the problem. It turned out, Charnas said, an existing drug would attack those chemicals. Mucomist had already approved by the Food and Drug Administration for Tylenol overdose and for cystic fibrosis.

"It was remarkably safe, remarkably easy to use and had worked in some similar inflammatory diseases," said Charnas.

"Chase looked to be the perfect candidate to try this. His disease had progressed too far to try conventional medicines."

So Chase and the Davises were offered a chance - have a cord blood cell transplant and try a regimen of Mucomist.

"Did we know it was going to work?" asked Bev Davis. "No.

"Did we know what was going to happen if we didn't? Yes."

Chase's treatment wasn't quite experimental, explained Charnas, but it wasn't quite standard either.

To prepare for the transplant, Chase underwent 10 days of chemotherapy.

"First the chemotherapy destroyed his bone marrow," said Charnas. "That gets rid of his immune system, the immune system that in Chase's case was attacking the brain.

"The transplant puts in another immune system that in theory will not attack the white matter.

"The new system doesn't recognize the old problem."

Neither of the other Davis children was a match and do not have the disease, said Bev Davis.

Chase received a double cord blood cell transplant on Nov. 26. One transplant was from a male with A+ blood, the other from a female with B+ blood. The A+ transplant was the successful. Chase now not only has a new blood type but must retake all of his immunizations.

Chase was on a breathing tube for several weeks, said Charnas, and "became as weak as a kitten.

"We were scared about that. Was it another illness or adrenoleukodystrophy progressing?

"The first big boost was the MRI scan at 30 days. There was no change from the last one.

"That was spectacular news," Charnas said. "Then at 92 days, it was also unchanged which was really spectacular. For a child as late in the disease as he was not to worsen was spectacular."

Darrin Davis uses the word "miracle" in describing Chase's recovery.

"This was not a miracle; it's wonderful," Charnas said. "But we went into this with science and years of experience. Chase is the first truly living testament."

QUESTIONS AND ANSWERS

- Adrenoleukodystrophy is perhaps most well-known as Lorenzo's Oil disease, after a movie based on the story of Augusto Odone, the late Michaela Odone and their son Lorenzo, who though diagnosed with adrenoleukodystrophy in 1984. Lorenzo observed his 25th birthday last fall.

- Adrenoleukodystrophy manifests primarily in males.

- The nerve insulating material of a boy's brain, the white matter also known as myelin, is progressively destroyed.

- Adult onset of adrenoleukodystrophy is commonly referred to as adrenomyeloneuropathy. Bev Davis said her deceased father probably had the adult onset version but was misdiagnosed with multiple sclerosis.

ON THE NET

stopald.org

www.myelin.org/

geneclinics.org/

Arlene Mannlein can be reached at amannlein@herald-review.com or 421-6976.