The Family That Couldn't Sleep

This book written by D.T Max starts with a story of a Venetian family cursed over the generations by dying through the absence of sleep. Yes, that’s right, absence of sleep. And no, this is not the good old fashioned case of insomnia that occasionally keeps all of us awake at one point or another. In this poor family’s case, the victim, almost always in his 50s is suddenly unable to sleep and starts copiously sweating. More ominously, he or she becomes delirious and the situation worsens until ultimately death results. All this takes place in the space of a few months, a year if he is lucky or unlucky, depending on how one looks at it. To date they remain afflicted.
This is the frightening start of a gripping and if truth be told, scariest book I’ve read so far. It is also compulsively readable and sympathetically written. The Venetian family’s story is a counterpoint and frame for the hefty scientific and medical examination of the history of prions which are basically a kind of protein. To fully appreciate the importance of prions, the author takes pains to explain its nature [they are the building blocks and engines of the body and most importantly has only one stable form], what differentiates it from other proteins [it is able to hold two stable forms, unlike others] and why this difference is lethal to us. After long years of painstaking work, scientists have found that the prion in its second form wreaks havoc by causing several fatal brain related diseases. This includes the Mad Cow disease, the scrapie sheep disease and more ominously for humans, the Fatal Familial Insomnia of the Italian family as well as the Creutzfeldt Jakob disease which afflicts far more people than we would think. It is the medical community’s belief that it is likewise linked to Alzheimers. The most frightening thing about all this information is the fact that to date there is no cure for any of the aforementioned diseases and the fact is, the medical and scientific community still doesn’t know why or what triggers the prion to become lethal.
Despite the grimness of the topic (there are several stomach churning chapters involving sheep and cows) and the barrage of scientific information, what stands out is the sensitivity and empathy of the author for the human characters involved. The writer, himself suffering from a rare neurological disease understands that over and beyond the science of the matter, is the human story and it is an important one. Apart from the Italian family, the book narrates the stories behind the different personalities that have dominated the prion field and they are fascinating to read. We meet Carl Gajdusek who did groundbreaking studies on the cannibalistic Fore people who suffered a variant of the Fatal Familial Insomnia disease. Gajdusek had a strong penchant for young boys and along the course of his work brought back several children with him to the US. He later went to jail for his proclivities. Then there is Stanley Prusiner, Nobel prize winner, who coined the term “prion” and who dominates the field. He has a taste for the expensive life and much of the scientific community speaks about him in terms both admiring and envious.
In the end we come away enriched with scientific knowledge, not always accessible to ordinary people but more importantly knowing the people who have struggled long and hard to bring such knowledge to us all.