Reviewed by Anna Brinkmann

No one will be happier to read this review than my boyfriend, because it means I will finally stop talking incessantly about this book – and about prions: the tiny bits of protein that do nasty things to brains, most famously through “Mad Cow” disease. In my defense, I challenge anybody to read The Family That Couldn’t Sleep and not talk about it, or at least think about it whenever they eat a hamburger or mutton, or whenever they contemplate the various ways a person can die. Author D.T. Max approaches the topic of prion diseases from a personal point of view – his own immune disorder – and his interest is infectious.

“The Family” has been painstakingly researched and footnoted. However, far from being clinical and dry, it’s a tale that crosses continents and includes laughing cannibals, government cover-ups, pedophiles, glamour-hounds, sleepless nights in 18th century Venice – and at its core it is a medical mystery still in the early stages of being unraveled.

“The Family” deals with a particular subset of neurological diseases, and they are the final frontier in medicine. As Max points out, based on his own experience and the extensive research he did for this book: for a neurologist, the year is still approximately 1860. Neurologists can diagnose a problem, but they can’t cure it.

Short on cheeriness, the book provides mystery in gothic proportions. The sleepless family of the title lives just outside Venice, and they have been, in a very real way, haunted by an inherited prion disease since the 18th century. The family battles the bizarre  disease which causes them, usually when they turn 50 or 60, to lose the ability to sleep. They lose neuromuscular function, their heart rate becomes elevated and for months, they exist in a twilight state between being fully awake and asleep.  Within 10-15 months, the person dies – exhausted, but still able to recognize their loved ones and fully cognizant of what is happening to them.

Max unfolds the entire known world of prion diseases through the lens of this family’s experience over the last 5 generations – from the first ancestor they’ve been able to deduce died from the disease, through frustrating hospital appointments and stumped doctors, to their modern struggle for some kind of treatment – any kind of treatment – for their horrific and inexorable ailment. 

Indeed, the book reads at times like science fiction. Zombies of the micro-world, prions are not alive. They are simply bits of protein that sidle up to other proteins, and behave normally – they connect to and influence them.  Actually, their molecular bonds do the influencing of neighboring proteins, causing them to misfold the way they are misfolded. The misfolding snowballs, and eventually enough proteins are folded badly to create a problem in the host’s biological machinery. Unfortunately for us hosts, the machinery usually affected is the brain.

Because they are not alive, prions are very hard to “kill.” Bleach, radiation, boiling, and formaldehyde are all rendered useless. (Prions actually enjoy formaldehyde and it makes them stronger.) They bond to metal, and they have been shown to endure in soil for at least a decade. Prions violate the underlying, Darwinian presumption of modern science that infectious agents are in competition with us, and that we can therefore hope to understand them – and thwart them – based on that understanding. Max’s book is a compelling compilation of the knowledge about prions that scientists have managed to glean so far, and of the strange way these zombie proteins are impacting our world.