This book is sooooo chocolate bar
Book raising money for UF research on rare disorder
By Nicole La Hoz
For families affected by glycogen storage disease, each night is filled with doubt. The rare liver condition, also known as GSD, leaves families like the Pournazarians on a strict schedule.
They check their son’s blood sugar levels around the clock, and 10 to 12 times a day, they feed him via a gastrostomy tube.
“You’re constantly watching alarm clocks,” said Lora Pournazarian. “You wake up in the middle of the night thinking, ‘Oh my God, am I going to miss this feeding?’”
Lora’s 7-year-old son, Jonah, was diagnosed with GSD when he was 6 months old.
Even though there’s no cure for the disease, Jonah’s best friend is collaborating with a UF professor to find a breakthrough.
Dylan Siegel’s Chocolate Bar, a 14-page book the 6-year-old wrote to raise money for GSD, is funding a cure at the UF Glycogen Storage Disease Program, the largest program of its kind in the world. Director David Weinstein, M.D., M.MSc., spearheads the research.
“There’s so much hope now because of Dylan,” Weinstein said. “It shows the power of what happens when people want to make a difference.”
Chocolate Bar, which means “awesome” to Dylan and Jonah, details what Dylan considers chocolate bar, like going to Disneyland. “The biggest chocolate bar,” he writes, is helping his friend.
Since November 2012, Chocolate Bar has raised more than $175,000 toward Dylan’s $1 million goal for GSD research.
Jonah’s father, Rabin, said the book has brought awareness and attention to a disease that has no funding because of its rarity. “GSD is an orphan disease,” he said. “Not many people know about it.”
One in 100,000 people have GSD, but Jonah’s type, Type 1B, is a one-in-a-million case. It’s the rarest of liver-related GSDs, Weinstein said, which manifests in the liver or muscles.
“It has been difficult to do work on this because no foundations in the world support research on this disease,” Weinstein said. “Dylan has raised more money for research than all fundraisers and foundations up to this point.”
With Type 1B, Jonah’s body stores sugar after meals but can’t release it. If he stops eating, he’ll have low blood sugar, which can cause seizures and brain damage.
GSD was fatal until the ’80s, Weinstein said, when doctors found that consuming cornstarch could keep children alive. The ingredient releases glucose slowly from the liver. For Jonah, that means a cornstarch treatment every four hours — including in the middle of the night.
“By getting money, we’ve been able to stabilize research,” Weinstein said. UF’s GSD program takes care of more than 90 percent of the U.S. GSD population. “We’ve made progress, but we need to do better. I’m confident that this will make a difference.”
Rabin said Weinstein has been Jonah’s doctor since 2006. The Pournazarians head to Shands from Los Angeles every year for Jonah’s checkup, which includes an ultrasound and blood test to inform them about Jonah’s liver, kidney and spleen functions.
“Dr. Weinstein is our hero,” Lora said. “I don’t know any other doctor, who, any time of day or night you need him will call you back and give you guidance.”
Rabin and Lora originally set up a fund for Jonah in 2007, but when the Siegels told the Pournazarians about Chocolate Bar in October 2012, they created the Jonah Pournazarian GSD 1B Fund at UF for GSD research.
Now, both families have appeared on The Huffington Post, ABC News, Fox News and even Australia’s TODAY.
For more information on the fund and Chocolate Bar, visit chocolatebarbook.com.
“We live with this story, and it still amazes us,” Lora said. “Chocolate Bar is just so chocolate bar.”