Harrison child fighting rare brain disease By LIZ SADLER April 24, 2006 Jordana Holovach stood at her kitchen counter, grinding up medicine with a mortar and pestle. In the next room, her 10-year-old son, Jacob Sontag, sat in a wheelchair, his gaze fixed on the television as a saber-toothed tiger from the animated movie "Ice Age" danced across the screen. "We just saw 'Ice Age 2,' so we thought we should watch the first one," Holovach said. Although Jacob stared intently at the TV as his mother mixed his medications, nobody knows for sure how much of the movie he could understand. Jacob has Canavan disease, a rare genetic disorder that causes the brain to degenerate. He can't walk, talk, crawl or sit up. And though his mother and doctor say he understands much of what he is told, he can only express himself through smiles and subtle noises. "He's really just trapped," Holovach said. Only about 1,000 children worldwide have Canavan disease, which is caused by a gene most commonly found in Ashkenazi Jews, who are of Eastern European descent. As the disease progresses, children may have seizures or become paralyzed, mentally retarded, deaf or blind. Most live less than 10 years. But Jacob belongs to a group of about 45 Canavan children who have undergone cutting-edge, experimental treatments in the hope of prolonging and improving their lives. Thanks to his mother's persistence and diligent fundraising efforts, he has had three gene therapies and is awaiting further study and approval of a neural stem-cell transplant that Holovach hopes will help revive some of his aural and motor skills. "If he could just be able to verbally communicate, then I would be happy with that," said Holovach, who shares custody of Jacob with the boy's father and has remarried. Jacob is her only child. Since Jacob's diagnosis in 1996, Holovach has testified before Congress to request federal funding for Canavan research and founded a nonprofit foundation, Jacob's Cure, that has raised more than $3 million. Between shuttling Jacob to doctor appointments, school at Purchase Elementary and various therapy sessions, she runs the foundation from a home office. "When you have a rare disease or a child with a rare disease, you have to fend for yourself," Holovach said. "There's no ifs, ands or buts." Jacob's Cure helps fund the research of Dr. Paola Leone, a neuroscientist and director of the Cell & Gene Therapy Center at the Robert Wood Johnson Medical School and Cooper University Hospital in Camden, N.J. "Jordana is an amazing woman that has received the worst news that any person would ever want to hear — the diagnosis of a child with a terminal disease," said Leone, who oversaw Jacob's gene therapies. "She was able to find the strength to go beyond everything she was told and start her own fundraising." Leone said she is now testing stem cells in animals and rodents with Canavan disease and seeing improvement. With enough data and federal funding, the treatment could be ready for a clinical trial in two years, Leone said. To raise enough money to continue to support Leone's research, Holovach is gearing up for another round of congressional hearings and has plans to step up her fundraising efforts in Hollywood. She is also seeking a celebrity spokesperson, a crucial component to fundraising. "We're in unchartered waters and I don't have the answers," Holovach said, "but I'm prepared for the battle and I'm prepared for the fight."