A documentary featuring the work of Assistant Professor of Neurology Michelle Monje M.D. ’04 Ph.D. ’04 has been nominated for a San Francisco/Northern California Emmy Award.
The film, titled Dylan’s Gift, tells the story of Dylan Jewett, a five-year-old patient of Monje’s. In January 2009, Dylan died from diffuse intrinsic pontine glioma (DIPG), an incurable tumor that afflicts between 150 and 200 children per year.
The documentary also follows the groundbreaking development of the first mouse-model of DIPG, which Monje and her team were able to create using cells from Dylan’s autopsied tumor.
Dylan’s Gift is the ninth episode of Bench to Bedside, a television series produced by Foundation for Biomedical Research (FBR) Media that focuses on people and animals who have diseases with no known cure.
The goal [of the documentary] was to raise awareness of a horrible disease that is killing children,” said Liz Hodge, the series’ executive director. “People don’t know about brain cancer in general, let alone one [variant] as rare as DIPG. It’s always important to spread awareness of diseases that are rare.”
Although the film has only aired in California so far, Hodge expects that it will be shown worldwide by 2014. The San Francisco/Northern California Emmy awards, in which Dylan’s Gift is competing against two other entries in the informational/instructional-program/special category, will be announced on June 15.
Dylan’s donation
Monje became involved in the documentary after treating Dylan during her neuro-oncology fellowship in 2008. She had studied DIPG in a lab for five years before deciding to focus on the disease and attempt to develop experimental models that could be used to study it
“Anytime I can raise awareness about DIPG, that’s an important thing to do for the patients,” Monje said. “What I really like about this documentary is that it is focused on honoring Dylan and his family and recognizing the contribution that they made, so I was very happy to contribute in any way I could.”
DIPG typically appears in children around age six, with initial symptoms including crooked eyes, having difficulty moving muscles of the mouth and face or general incoordination and clumsiness.
The tumor is diagnosed by brain stem neuroimaging, and the median life expectancy following diagnosis is around nine months. Almost all patients die within two years.
After realizing the dire reality of Dylan’s cancer, his mother asked Monje if there was any way to help other children with the disease and subsequently decided to donate his tumor to research.
According to Monje, doctors do not normally biopsy DIPG because the tumor cells are entwined with the normal tissue of the brain stem. However, with Dylan’s donation, the researchers could conduct a postmortem harvesting of the tumor within 18 hours, and were then able to establish the first cell culture of DIPG in the world.
The doctors then injected the cells in the brain stems of mice, creating a disease that looks almost identical to human DIPG. This development enabled researchers to study the cancer more closely and investigate the effects of different treatment options.
According to Monje, Dylan’s cells have been distributed to over 35 scientists around the world, from Europe to Australia, allowing more scientists to contribute to research on the cancer.
“We share our cell lines with any researcher who is interested in studying DIPG,” Monje said. “That’s really been helpful in energizing the research on DIPG. There are a lot of people now working on it because they have the tools with which to study the disease.”
Since Monje’s initial work with Dylan’s tumor, other families have donated DIPG tumors for research, allowing six different cell lines of the disease to be established.
“Having these different cell lines is important because not every tumor is the same, and it’s important to study a group of them in order to make conclusions that are applicable to the most patients,” Monje said.
Paul Fisher ’84, head of the School of Medicine’s Division of Child Neurology, supervised Monje’s research on DIPG.
Fisher, who has diagnosed more than 100 patients with DIPG, framed Dylan’s story as particularly compelling on two levels, through both the generosity of a family who wanted to contribute to research to help save the lives of other children and Monje’s investment of her time and energy to develop the model of the tumor.
“Michelle and the family have been very generous in sharing the cells with other labs,” Fisher said. “There’s a lot of active testing right now with different drug compounds, and in the future they will be testing other types of therapy, and having this model system in place will be key to try to overcome such a horrible track record in this disease.”
Hodge also emphasized the generosity of the Jewett family, noting that the Emmy nomination is a tribute to their efforts and sacrifices.
“It speaks to their generous hearts, and the generous way that they turned a tragedy into something positive,” Hodge said.