Saturday 9 April 2011

The Family That Couldn't Sleep by Daniel Max

From The Week of May 16, 2010


It strikes me that we understand both a great deal and hardly anything at all about how organisms work. An incalculable number of hours have been spent by countless scientific minds working out the processes that underpin life. But though we're strong on the broad strokes of the thing, we are so weak on the specifics that we don't even know the function of some of the most prolific cells within our own bodies. This may say something none-too-flattering about the intelligence of humanity as a species, but it may simply be that life is by its nature the most complex thing imaginable. And that cracking that code isn't easy. Mr. Max has put forth, in The Family That Couldn't Sleep a chilling example of just how ignorant we are of ourselves and the organisms we depend on.

Mr. Max, himself the sufferer of a degenerative condition, has chronicled, here, an Italian family afflicted by an appalling curse. At some point, during middle age, each member of this family has a significant chance of waking up one morning and never again being able to return to sleep. No matter how exhausted they are, they cannot and will not rest until they die, usually within the span of a week or two. How can such a thing possibly afflict a single family? They have inherited a trait from a common 19th century ancestor in whom a deadly modification was made. This trait only activates in some of the family members which is how they've been able to survive to reproduce through the subsequent generations. And so, in a metaphorical sense, the disease has put, above the head of each member of this family, a kind of invisible guilllotine which may or may not strike them down. There's no way for them to know if the trait will activate or not. If it does, they die, painfully and horribly. If it doesn't, they live normally for the rest of their lives. Remarkable... And, to say the least, worthy of scientific investigation.

Though Mr. Max's description of and interviews with the afflicted family captivate, it's the science here which startles. For Mr. Max concludes that the sleeping sickness suffered by the family is the result of a malformed protein called a Prion. Prions, it so happens, are also the prime suspect in the cause of Mad Cow Disease for prions, once they enter the body, can convert healthy proteins into destructive prions which, in turn, pervert more healthy proteins until enough damage has been done and the organism dies. Mr. Max's vivid explanation of the history of Prion research, of the Mad Cow scare in Britain and how this all relates to our poor, Italian family is skillful and edifying.

I do not know when we will understand how life functions, but I know this. The journey to understanding is bound to be frightening if it throws up surprises even half as fearsome as Prions. Work well done... (4/5 Stars)

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