As neurologists, we often have to relate bad news to patients and families about a loved one's diagnosis. Too often, physicians fall back on textbook descriptions of prognosis for specific disorders, rather than taking each patient as an individual. A classic example of this is described in Schuyler's Monster: A Father's Journey with His Wordless Daughter, a poignant book by Robert Rummel-Hudson.
The author chronicles the true story of his daughter Schuyler and how she came to be diagnosed with bilateral perisylvian polymicrogyria (BPP), discovered as a result of her workup for profound language delay.
BPP is an extremely rare condition that occurs as a result of a migration defect in the brain early in embryonic development. The brain shows smaller and more numerous gyri in the perisylvian areas than are seen in a normal brain. Symptoms may be present at birth or may not become apparent until early childhood. Common symptoms include bilateral facial weakness, difficulty feeding, and seizures. Children often have some degree of global developmental delay, but the prognosis is variable. The diagnosis is made by performing a MRI of the brain.
AN ‘EMOTIONAL ROLLER COASTER’
From the beginning we were captured by the author's irreverent humor — particularly describing his wife's pregnancy and delivery, which we one can only hope she was allowed to edit prior to publication. The author's humor is a constant throughout the story, and that is required to balance the sadness and devastation so clearly apparent later when the diagnosis is first presented. Quite frankly, the book is an emotional rollercoaster, which in our experience as clinicians, accurately reflects the mental state of both parent and physician faced with a diagnosis as catastrophic as BPP.
As physicians, we have all experienced the horrible sinking sensation and ensuing sadness when discovering that we have a bad diagnosis to impart, particularly malformations of the brain. For most of us we are not usually in the position of experiencing this situation from the parent's perspective. Rummel-Hudson offers a heartrending glimpse of that — with brutal honesty.
Upon first learning of Schuyler's diagnosis, he writes: “I cried for the future, for the life I had always imagined for my little girl, a life that would never ever be what we'd imagined it to be…I cried at the word ‘retardation’ and the atom bomb of low expectations it carried with it. I cried at the idea of Schuyler growing old in our care and never knowing a life on her own terms.”
Particularly disturbing were the obstacles created by both Schuyler's school and insurance company when her parents were trying to obtain a communication board for her. The school wanted her to use a simpler version of a communication board and disagreed with the parents about which model should be ordered, because they doubted she would be able to handle a more complex device. The insurance company put up its own obstacle in advocating for a cheaper, simpler version. This story is not shocking to any of us in clinical practice. However, the author used his blog — available at www.schuylersmonsterblog.com — to raise $10,000 (in three weeks) to buy Schuyler the expensive and more complicated model, and her language production flourished.
This reaffirmed our general observation that parents really do best know their children and their abilities. Unfortunately, from the father's account, the parents were not provided with much hope or support in their efforts from the health care professionals involved in Schuyler's evaluation and care.
We have a busy child neurology clinical practice that focuses on neuro-developmental disorders, including language impairment and autistic disorders, as well as many children with structural brain malformations. Since reading Schuyler's Monster, we have recommended it to parents, particularly parents of children with brain malformations. We believe that it reinforces fundamental lessons, the most important being that the parent (not the physician, not the teachers) will always be the strongest advocate for their child.
The Rummel-Hudson family was constantly on the alert for and questioning physicians and teachers they thought were underestimating Schuyler's abilities. For example, they were told after detailed developmental and psychological assessments that their child had pervasive developmental disorder and developmental delay.
The Rummel-Hudson family were told the “good” news that Schuyler wasn't autistic. The family moved to a different city to provide a better education for her, seeking out schools that would provide an educational plan more suited to her needs. Schuyler is currently attending a special school that focuses on augmentative communication devices.
An important message for readers is that each patient should be considered as an individual, not a diagnosis, with individual variations despite having a “generic” disorder.
For doctors, this book reminds us that children with developmental disabilities also need to be challenged, just as “neurotypical” children do, so that they can maximize their potential.
Schuyler's Monster: A Father's Journey with His Wordless Daughter | By Robert Rummel-Hudson | 288 Pages | St. Martin's Press 2008