For the last several years, one of my favorite bands has been Cloud Cult. Their upbeat lyrics and peppy melodies belie a darker past, one characterized by the sudden death of the lead singer's very young son. Since that time, much of what Cloud Cult has sung about is this loss and what comes after. As I read Emily Rapp's shattering new memoir, The Still Point of the Turning World
, I was reminded of Cloud Cult, and marveled again at the desire of so many artists - bordering on compulsion - to record this experience of grief and loss, not to capitalize on it but rather because they have no choice.
When Rapp's son Ronan began missing his developmental milestones shortly after his six-month birthday, Rapp and her husband took him to an ophthalmologist who confirmed their worst fears - Ronan had Tay-Sachs disease, a genetic enzyme deficiency with no known cure and the...
Beyond the Book
At the age of nine months, Emily Rapp's son Ronan was diagnosed with a deadly disease called Tay-Sachs. The disease is caused by the lack of a vital enzyme called hexosaminidase-A (Hex-A); the result is a progressive buildup of a fatty substance in nerve cells that causes destructive neurological decline and eventually death. There is no cure. A baby with Tay-Sachs can appear "normal" up until six months, although the disease is present even in the fetal stage; at that half year point, however, any developmental progress begins to decline, and the child who could once crawl or babble is eventually unable to see, move, or even hold his or her head upright.
Tay-Sachs is a genetic disease, named for Warren Tay, a British opthamologist who, in 1881, first identified a...