The monster

Bilateral perisylvian polymicrogyria (also called congenital bilateral perisylvian syndrome) is an extremely rare neurological disorder caused by a malformation of the brain.  BPP manifests itself in a variety of ways.  It can affect the patient’s speech and fine motor control, can cause partial paralysis of the facial muscles, tongue, jaws and throat, difficulties in speaking, chewing and swallowing, and sudden episodes of uncontrolled electrical activity in the brain, resulting in grand mal seizures.  In many cases, mild to severe mental retardation is also present.  The effects of BPP can range from mild learning disabilities to infant death.  Diagnosis of polymicrogyria is difficult, only made possible through an MRI or CT scan of the patient’s brain.

In the summer of 2003, when Schuyler was diagnosed with BPP, there were only about forty cases identified worldwide, according to Yale University’s Medical School.  By the time Dr. William Dobyns at the University of Chicago Hospital’s Department of Human Genetics saw her a year and a half later, that number had grown to several hundred cases.  (According to a review of the Atlanta-based registry of the National Center on Birth Defects and Developmental Disorders of the Centers for Disease Control and Prevention from 1994-1999, polymicrogyria occurred at a rate of 0.1 per 1000 livebirths.)  In the past, such a small number of cases would have meant a kind of isolation for the parents of a child with BPP.  The chances of making contact with another parent in a similar situation would have been remote at best.

Schuyler’s parents were fortunate, however, to have the power of the internet at their disposal.  When Schuyler was diagnosed with BPP (known as CBPS at the time), Robert Rummel-Hudson had already been writing about her life and difficulties online for a long time.  At that time, his online journal was getting thousands of hits every month and had a large subscriber list of regular readers.  Word spread quickly about her diagnosis, and before long he was hearing from other parents who were in the same situation in which he and his wife found themselves.  Eventually, his online presence led the Rummel-Hudsons to Dr. Dobyns himself, one of the world’s leading researchers in polymicrogyria and its related disorders, and resulted in his meeting with Schuyler in January of 2005.

In the spring of 2005, the Rummel-Hudsons began exploring Assistive Technology as a way to give Schuyler a voice by the use of an electronic device that would speak for her.  The device that could open up her world was expensive, however, and their insurance did not cover Assistive Technology.  Schuyler’s school was not able to afford the device, and applying for help from the government involved a labyrinthine appeals process that could take a year or more.  As they explored options for third party funding, Robert put together an online fundraiser, creating a page where readers could contribute to a fund to help defray some of the costs of the device.  Robert and Julie never anticipated the response they’d receive, or what was going to happen next.

Five weeks later, the Rummel-Hudsons shut down the fundraising web page.  They had received over ten thousand dollars in contributions, almost all of it from people who had never met Schuyler and only knew her from Robert’s online journal.  It was an extraordinary outpouring of generosity from a virtual community, the proverbial village it takes to raise a child.  The story of her communication device begins a whole new phase of Schuyler’s life, one in which she will finally have a voice.