KANSAS CITY, Mo. (AP) — More than five years after her death, Rebekah Grisamore is still contributing to the scientific discoveries Merlin Butler is making.
Rebekah had Prader-Willi syndrome, a genetic disorder that causes developmental disabilities and strikingly severe obesity.
Butler is an internationally recognized Prader-Willi researcher most recently associated with Children's Mercy Hospital.
When Rebekah died before her first birthday, her parents, Jeff and Mary Grisamore of Lee's Summit, donated their daughter's organs and tissues for research.
Rebekah is the youngest organ donor to Prader-Willi research, and the only infant available for study. She has become critical to scientists like Butler who are trying to understand how obesity develops in Prader-Willi.
Ultimately, that understanding could lead to insights to dealing with the nation's obesity epidemic.
"It's really exceptional that this family in their time of grief were able to make this donation," said Janalee Heinemann, director of research and medical affairs of the Prader-Willi Syndrome Association.
And, according to Heinemann, Butler was just the right scientist to receive the organs.
"He's one of the most renowned Prader-Willi researchers in the world," she said. "He's come up with some of the key things in Prader-Willi."
Butler, a lanky, soft-spoken pediatrician, has been conducting research on Prader-Willi syndrome since 1980.
He recently resigned from Children's Mercy, where he has conducted his research, but plans to continue studying Prader-Willi.
Butler calls it a bizarre condition. It affects one in 10,000 to one in 25,000 newborns.
"When these kids are first born, doctors may think that they have brain tumors," he said. "The babies are so floppy, like a rag doll."
The babies appear to have little appetite. They may suck so weakly that they must be fed through a tube. But around age 2, they gain enough strength to begin sitting up and crawling. And they undergo a great change.
"Their appetite kicks in," Butler said. "Families can remember the day. They may eat a whole loaf of bread or a gallon of ice cream. They either eat until the food is gone, or they fall asleep, or their stomach ruptures."
By the time Prader-Willi children are 5 or 6, they will forage through the house for food. Parents may literally have to padlock the refrigerator to prevent overeating.
"They're cute kids, very innocent and loving most of the time," Butler said. "But try to withhold food and they go into a rage."
People with Prader-Willi syndrome can live into adulthood and even old age.
But if their eating isn't kept under control, Prader-Willi children may weigh 200 pounds or more by the time they're ready to start school. Their weight can lead to heart failure and an early death.
Rebekah Grisamore was born on Dec. 8, 2001. She was a lovely baby with blue eyes and curly blond hair. But within a few days, Jeff and Mary Grisamore knew something was wrong. Rebekah's body was limp. She had trouble nursing.
The Grisamores took Rebekah to the doctor. From there, she went to Children's Mercy, where she was diagnosed with Prader-Willi syndrome.
She spent three weeks in intensive care and came home just before New Year's.
The house was stocked with oxygen tanks and monitors. Rebekah had to be bottle-fed very patiently. She needed as long as an hour to swallow just a few ounces of milk.
But Rebekah did well. She gained weight and stayed healthy. She regularly visited Butler's Prader-Willi clinic.
Jeff Grisamore, the proud father, looked ahead to the possibility she might one day excel in the Special Olympics.
"There is something about a baby that doesn't cry — she couldn't cry," he said. "There was a sweetness about her."
By the time she was 11 months old, she was beginning to raise her head on her own, a real accomplishment, Jeff said.
Then she became ill with flu-like symptoms. After finishing a bottle of milk, she belched and stopped breathing. Her lips were turning blue.
Rebekah was rushed to a hospital, but it was too late. She had vomited and inhaled some of what she had thrown up. Too weak to cough, she suffocated.
"It was just beyond description the shock and grief. We thought we were going to have a long, full life with her," Jeff said.
As the Grisamores pulled out of the hospital, a thought occurred to him: Could Rebekah help others through research?
He called Butler and they quickly arranged to have Rebekah taken to Children's Mercy.
In his lab at Children's Mercy, Butler grew beakers full of Rebekah's blood cells.
A computer screen glowed with hundreds of colored dots, each representing a gene in the frontal lobe of Rebekah's brain.
Butler is studying the sudden shift in Prader-Willi children from meager to voracious appetite.
"Appetite regulation between the brain and the stomach is not working," Butler said. "It makes you wonder what's going on in their brains."
The change in appetite may be caused by certain genes in the brain switching on or off from one stage to the other.
To figure out what is happening, researchers need brain tissues from both stages of Prader-Willi.
Tissues are available from many people who were in the syndrome's second stage. But Rebekah's tissues are the only ones available from Prader-Willi's first stage.
"Rebekah is key for this," Butler said. "She didn't live long enough to become obese."
A state representative from Lee's Summit, Grisamore wants to change state law to help people donate their organs and tissues for transplantation or research.
Under current Missouri law, signing the back of your driver's license to become a donor, or joining the state organ donor registry, does not guarantee that your organs will be donated. Family members can override that decision.
Grisamore backs legislation that would assure donors' wishes are honored. The measure would put Missouri in line with those of Kansas and Illinois.
For the Grisamores, knowing Rebekah is still making a contribution has been a comfort.
"We thank God for every day we had with her," Jeff Grisamore said. "But we're proud that her legacy is growing."
