Just two months after Gov. Tom Corbett signed “Hannah’s Law” and two weeks before her second birthday, the Levittown girl the bill is named for has died.
Hannah Ginion was born with Krabbe disease, a rare genetic disorder that strips away the protective myelin coating from nerve cells.
State Rep. Angel Cruz, D-Philadelphia, said he sponsored legislation to mandate expanded testing for all newborns after learning of Hannah’s condition, making Pennsylvania the sixth state to do so. The expanded screening looks for five other rare lysosomal storage disorders along with Krabbe.
“Hannah showed us what they go through, and our hearts and prayers go out to Hannah’s family,” said Cruz Monday.
Each screening costs $8, with an annual cost to the state of roughly $600,000.
Krabbe disease strikes one in 100,000 infants. If detected immediately, stem cell treatments formulated from blood in healthy umbilical cords can help extend the baby’s life.
Babies with the genetic disorder appear healthy at birth, but usually develop symptoms within a few months, including muscle stiffness and irritability. As the disease progresses, patients often lose eyesight, suffer seizures, and stop eating.
“Think of an electric wire without the coating,” says Dr. David Wenger, neurology professor at Thomas Jefferson University Hospital. “In this disorder, you lose the insulation around the nerve.”
In his 40 years of research on Krabbe, Wenger helped develop the screening for Krabbe.
“It is terrible. It is a bad disease,” said Wenger. “I don’t pussyfoot around when I meet with the families. ‘You got a bad deal.”‘