January 9, 2013
School kids help give their friend sweet support
Jonah Pournazarian is a bright, playful 7-year-old at Stephen S. Wise Temple Elementary School. He has a best friend, Dylan Siegel, loving parents, devoted teachers and an extremely rare genetic disorder.
This is a story of how Jonah’s classmates, family and friends, as well as a doctor in Florida are standing with Jonah in his battle against glycogen storage disease (GSD). Among their efforts, Jonah’s fellow first-graders got together at the school’s Mitzvah Day to sell a “book,” written and illustrated by his friend Dylan, to raise money for GSD research.
There are 14 known categories of GSD, and Jonah’s is identified as type 1b, one of seven types that primarily affect the liver. Fewer than 100 cases of type 1b have been identified in the United States; some of these cases are in descendants of Sephardi Jews.
Glycogen is the stored form of glucose, or sugar, which the body’s metabolism breaks down and converts into energy. Enzymes play a crucial part in the process, and when they malfunction, as in GSD, the metabolic process slows or shuts down completely.
GSD, predominantly a children’s affliction, is inherited through the genetic makeup of both parents and targets different parts of the body, such as the liver, muscles, heart, kidneys and others. In Jonah’s case, his doctor said, glucose is stored in his liver and “can’t get out.”
The disease was almost always fatal until 1971, when researchers developed the first effective therapy for GSD. A major breakthrough came a decade later with the discovery of a simple “medication” in the form of cornstarch, inexpensive and readily available.
However, the prescribed doses of cornstarch, administered through a surgically implanted feeding tube, have to be given every three to four hours, day and night, without fail.
“We live by the clock,” said Lora Pournazarian, mother of Jonah and his two healthy siblings — twin brother, Eli, and older sister, Rachel, 9.
Lora and her husband, attorney Rabin Pournazarian, keep two alarm clocks in their bedroom, both set to 3 a.m., to wake them to give Jonah his early-morning dose.
Missing the feeding is unthinkable, because the consequences could range from a short hospital stay to death.
After Jonah was born, weighing 4 pounds, he was constantly sick. With low or barely detectable sugar and white cell counts, his body could not fight infections, he suffered from massive diarrhea and at times woke up drenched in sweat.
After Jonah’s disease was initially diagnosed at Cedars-Sinai Medical Center, his parents sought out Dr. David A. Weinstein, a pediatric endocrinologist at the University of Florida, Gainesville, who was devoting his professional life to treatment and research of GSD. Since then, each medical consultation for Jonah requires a trip to Florida.
During an evening visit to the Pournazarian home, Jonah was challenging a friend to a Monopoly game upstairs. His parents encourage him to lead as normal a life as possible.
“We never say that Jonah is ‘sick’ or ‘not normal,’ ” his mother noted.
The boy’s 20 classmates have quickly adjusted to the full-time presence of his medical aide, who administers frequent blood tests. And Dylan has become a particularly close friend.
Dylan’s parents, Debra and David Siegel, are also friends of the Pournazarians, and have been actively involved in the Jonah Pournazarian Fund to Support Glycogen Storage Disease 1B research, which Jonah’s parents established in 2007. Since then, the fund has raised $410,000.
About two months ago, 6-year-old Dylan Siegel told his mother that he wanted to get involved, too.
“I suggested that he set up a lemonade stand,” Debra Siegel recalled, “but he said he wanted to write a book.” She took her son’s plan as a quickly forgotten fantasy, but the next day Dylan presented his mother with the finished product.
The cover of the richly illustrated, 14-page booklet reads “Chocolate Bar by Dylan Siegel” and the tone is set in the first entry, which reads, “I like to go to Disney Land. That is so Chocolate Bar.”
Other “Chocolate Bar” (read: “awesome”) experiences by the author and illustrator include going to the swimming pool, aquarium, bowling alley and so forth, ending with, “I like to help my friends, that is the biggest Chocolate Bar.”
As every first-time author knows, writing the book was just the beginning, but Dylan kept pushing the project. He drafted his father — a Disney film marketing executive — for the production phase of the project; he ordered an initial print run of 200 copies of the book.
The evening before the Stephen S. Wise Temple Mitzvah Day last Nov. 4, the Siegel and Pournazarian families both worked feverishly to package chocolate bars in colorful wrappers duplicating the book’s cover.
With Dylan and Jonah manning the Mitzvah Day booth, they sold enough autographed books at $20 each and chocolate bars ($5) to raise about $7,000 for GSD research.
Since then, there has been a second printing of 300 copies, and a third of 500 copies. Spurred by exposure at a Barnes & Noble book fair and through TV news reports, 750 copies have been sold so far.
Dylan and his parents found a valuable ally in Wendy Camacho, marketing manager of the Sherman Oaks West Whole Foods Market, which, over time, has donated 300 chocolate bars, valued at $750.
Total Chocolate Bar sales, combining the book and edible versions, have yielded some $30,000 for GSD research.
Meanwhile, their Facebook page, facebook.com/chocolatebarbook, has drawn more than 1,000 “likes” from all over the world.
Dylan told the Jewish Journal that he is now planning a second book. “It will be about two characters, two slices of french toast competing with each other,” he said. “I like writing.”
Orlee Raymond and Kimberly Snyder are Jonah’s and Dylan’s teachers, and they have gotten into the spirit of things by sporting two-of-a-kind T-shirts with the legend “First Grade Is So Chocolate Bar!”
(Full disclosure: Raymond is my daughter and was the catalyst in making this story public.)
At this year’s Back-to-School Night in September, Rabin Pournazarian made an emotional plea to the parents of Jonah’s classmates, Snyder recalled. He asked the parents to please keep their children at home if they had a routine cold or minor illness, because if transferred to Jonah, they could mean his instant hospitalization.
Alternatively, the father asked parents of sniffling kids to call him the night before, to allow him the option of keeping Jonah at home.
Jonah’s mother gave a similar talk to the school’s teachers.
All parents have voluntarily accepted these strictures, Raymond said.
Both teachers characterize Jonah in such terms as “the sweetest little boy,” “caring” and “very bright but doesn’t show off.” The only time Raymond heard Jonah mention his own illness was on the day students were asked to bring in $5 each to find a cure for breast cancer.
After listening to a very basic description of the disease, Jonah mentioned wistfully that he hoped for a cure for GSD, so that he could get rid of the gastro-intestinal tube he needs to absorb his cornstarch portions.
Snyder told of her great admiration for the way Jonah and his parents are coping with their challenges. The mother of two children, 11 and 14, Snyder observed, “If this should happen in my family, I would hope that I could handle the situation with equal grace.”
Her view is echoed by Debra Siegel, who describes the Pournazarian parents as “wonderful, unbelievable parents … they’re not victims, they are an inspiration to us.”
In return, Jonah’s mother says of him, “He is such a happy boy. He is our life … we are really blessed.”
To learn more about GSD, visit glycogenstoragedisease.com. To donate to the Jonah Pournazarian GSD fund, visit facebook.com/chocolatebarbook. The project’s Web site is chocolatebarbook.com. For those not on Facebook, donate to the fund through the University of Florida: http://bit.ly/YjO2PZ.