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When the only doctor who could possibly cure her son wouldn’t return her messages, Amber Freed sent daily snack deliveries to his office. That earned her a call—but a brief one: Dr. Steven Gray, a Dallas physician who specializes in gene therapy, couldn’t talk long because he was on his way out the door to a conference in Atlanta.
So Freed, who lives in Denver, bought a plane ticket to Georgia. “I went and sat next to him in the front row,” she says.
At dinner that night, Freed told Gray her story: how she and her husband, Mark, had, in 2017, been blessed with twins, Maxwell and Riley. But soon she noticed that Maxwell wasn’t developing as quickly as his sister. After a long investigation, a pediatric geneticist at Children’s Hospital Colorado finally delivered the terrifying, mysterious diagnosis. Maxwell has an incredibly rare disease called SLC6A1, a genetic disorder that attacks his central nervous system and results in Parkinson’s disease, which can include symptoms like movement problems, debilitating epilepsy, and other development issues.
That night in Atlanta, Gray and Freed developed a plan. SLC6A1 is the product of one half of a gene malfunctioning. The technology Gray is pioneering could conceivably replace the defective gene.
But, Gray warned Freed, the research required to bring the treatment to fruition could cost upwards of $7 million. Freed would also need to begin recruiting the brightest doctors and researchers in the world to collaborate on the project. And even if the process did eventually work, he said, it might arrive too late to save Maxwell. “That opportunity cost,” Freed says, “has been hard to shake.”
Nevertheless, since then (fall of 2018), Freed has raised more than $2 million for the cause—a significant sum, and yet not enough.
While the ultimate goal is still Gray’s gene therapy, Freed has also started working with Dr. Scott Demarest, a pediatric neurologist at Children’s who focuses his research and clinical practice on genetic causes of epilepsy, to find a way to slow the development of Maxwell’s disorder.
This project also has ties to the South. At Vanderbilt University, a professor of neurology named Dr. Katty Kang used stem cells to grow a model of Maxwell’s brain that allowed her to examine the function of his neurons. Using that “brain,” Demarest was able to test the effectiveness of a compound, called 4-Phenylbutyrate, that shows promise of easing Maxwell’s symptoms.
“The hope would be it can improve some of the symptoms that we’re dealing with, improve the developmental trajectory somewhat, and limit harmful aspects of the disease, like the behavior challenges or seizures,” Demarest says.
The compound shows promise of a quick remedy because the Food and Drug Administration has already approved it—albeit for other rare genetic enzyme deficiencies. However, because it’s passed the government’s standards, physicians know it’s safe and a little about how it works: According to Demarest, 4-Phenylbutyrate could make the proteins produced by Maxwell’s malfunctioning genes behave the way they are intended.
Demarest would like to begin testing the effects of the compound (in collaboration with Dr. Zachary Grinspan of the Pediatric Epilepsy Program at Weill Cornell Medicine in New York City) in a small clinical study of 10 patients beginning this spring or early summer at Children’s. “I am so excited,” Freed says. “What Scott has pulled off is truly incredible.”
The problem, again, is money: To fund the study, Freed and Children’s need to raise $200,000. If you’re interested in donating, click here.
While Freed has a rooting interest in fighting the disease, others could benefit from this research. Since SLC6A1 was “discovered” in 2017, Freed says it’s come to be accepted as the 10th most prevalent cause of autism and the sixth most prevalent cause of epilepsy. “Rare diseases are only rare until they run into the wrong mom,” Freed says.
“Parents like Amber are saying, we’re not going to wait for other people,” Demarest says. “We’re going to try to help our kids now. And they’re a force to be reckoned with.”