If you’ve ever spent hours lying in bed, waiting in vain for sleep to come, you’ll know that insomnia can be a terrifying thing. Now imagine that it goes on night after night until eventually you do not sleep at all. Imagine knowing that this sleeplessness will never end, that you will eventually start having hallucinations, followed by weight loss, and then death. Imagine knowing that there’s nothing you can do to stop it and that there’s a 50-50 chance that your children will someday suffer the same fate.
Members of the Italian family that D.T. Max writes about in The Family That Couldn’t Sleep have suffered this fate for centuries, but because of shortened life spans and inaccurate records, it’s only in the last 30 years that doctors, scientists, and family members have begun to understand that this fatal insomnia is in fact a rare genetic disease. The disease, fatal familial insomnia, is linked to several other, equally strange diseases. Scrapie causes sheep to behave as is they have an uncontrollable itch. Kuru causes people to laugh uncontrollably. Creutzfeldt–Jakob disease brings with it dementia and involuntary movements. And then there’s mad cow disease.
All of these diseases involve prions, or infectious proteins create holes in the brain and other nervous system tissue. Prion diseases are rare, but they’re still terrifying because they are always fatal, and the means of transmission are unclear and subject to some controversy.
Max writes about various prion diseases and how they were first discovered and how scientists eventually uncovered the links between them. Because prion diseases behave completely differently from other infectious or hereditary diseases, doctors had a difficult time tracking down the cause, and they still haven’t found a cure. The descriptions of the diseases themselves are terrifying. I found the discussion of mad cow particularly troubling because it touched on so many of my concerns about the food supply and how easy it is for something nasty to get into it.
The science is explained in a way that is easy enough for a non-scientist reader like me to understand. However, because I listened to this on audio, a lot of the facts and explanations regarding proteins and cells and whatnot went in one ear and out the other. It’s a problem with listening to nonfiction that’s even slightly technical. I may be able to follow it well enough to enjoy it, but not well enough to retain to retain more than a few general facts. For my purposes, that’s OK. I have no intention to go out and become a prion researcher, and I don’t know anyone with a prion disease. However, if I really wanted to understand this stuff, I’d get a print copy.