Longing to sleep

Abstract

Imagine spending 15 months without sleep. Then imagine spending those same 15 sleepless months feeling feverish, being so neurologically disabled that you cannot walk, and knowing that your impending death will be your first and last opportunity to rest. The Family that Couldn't Sleep is the haunting tale of a Venetian family afflicted by fatal familial insomnia (FFI): an autosomal dominant inherited prion disease that strikes in middle age with this unusual clinical picture of insomnia and neurological degeneration. This poignant and intriguing medical mystery follows the Italian family's story from their first remembered relative with FFI, who was a Venetian doctor in the 18th century, to the present generation, as they seek to understand the disease and its cause. D T Max details the trials and tribulations as the family finally discover that the likely cause is a prion disease. The book touches upon the stigma that this previously unknown, misdiagnosed, and misunderstood disease brought to the Venetian family—in the past, some members of the family were viewed with suspicion and thought to be “possessed” or suffering from alcoholism. Unravelling the pathogenic mystery of FFI is just one part of this highly instructive, meticulous, and frank scientific narrative. Max also explores prion biology, discussing the first cases of scrapie in the UK in the 18th century, the Kuru outbreak in Papua New Guinea in the 1950s, the UK controversy of bovine spongiform encephalopathy (BSE) and variant Creutzfeldt-Jacob Disease (vCJD) in the early 1990s, and findings in the USA of chronic wasting disease and the first US cases of BSE and vCJD. From diverse sources that include interviews and letters, Max outlines not only the causes of these outbreaks, but the scientific dispute that ensued. The research into prion diseases, which are caused by infectious proteins, has been an area riddled with controversy ever since the new theories underlying their transmission and progression were first proposed. Max cajoles the reader into reflecting on whether the emergence of prion diseases was self-inflicted by human nature—perhaps through cannibalism, or by misdirected efforts to improve meat and milk production by in-breeding, or through the practice of feeding previously herbivorous livestock unnatural animal-protein supplements. He also questions whether the BSE epidemic and transmission to human beings as vCJD could have been prevented or perhaps better handled. This book is an extraordinary accomplishment. It would be a challenge to read it without becoming fascinated by the underlying science, experimental controversy, and personal stories of the emotional challenges faced by the sufferers of FFI. And, be warned, this book may well keep you up at night while you wait for the mystery to be revealed.