GLUT1---DO YOUR RESEARCH

          Courtesy of Family First Magazine—Letters To The Editor

I enjoyed the story “Oily Blessings.” The writer describes a rare genetic disorder, Glut1 Deficiency Syndrome (G1-DS), in which the blood-brain barrier does not allow enough glucose to reach the brain, causing severe seizures and often mental retardation.

In the story, the writer’s son is successfully treated by drinking MCT oil with his food. I’d like to clarify a point about the efficacy of MCT oil in treating this disorder, to ensure that nobody reading this story gets the wrong information. The standard treatment when treating G1-DS, and the only treatment that has been proven to work, is the unmodified ketogenic diet initially mentioned in the story. It’s a difficult diet to follow, but consistently manages symptoms. There are a number of other therapies available for G1-DS, including the modified ketogenic diet (modified Atkins diet and using MCT oil, both mentioned in the story) and the use of triheptanoin oil.

I’m currently working at Columbia University Medical Center, where Dr. . Darryl De Vivo, who first described the syndrome in 1991, advocates only the ketogenic diet as a treatment. Studies being run on the use of alternative therapies are so far inconclusive, and more research needs to be done before they can be considered an effective treatment. While anecdotally the author of “Oily Blessings” reports that the use of MCT oil worked for her son, anybody considering treatment options for Glut1 DS should thoroughly research the literature before making a decision.

Peryl Agishtein, PhD