The Family That Couldn’t Sleep

There’s nothing I love more than sleeping, so I was particularly horrified to recently find out about a rare, genetic disease called Fatal Familial Insomnia.  It’s sufferers will live normal lives until they hit middle-age, and then suddenly, they’ll develop insomnia.  A number of other nasty symptoms manifest themselves, and the infected person will eventually die, their brain filled with holes caused by an invasion of prions.  FFI is just one of many diseases caused by prions, and it’s the basis for a book I just finished, The Family That Couldn’t Sleep.  The book is a history of prions (aka, “misfolded proteins"), and it traces a pretty fascinating path as it meanders through discussions of “scrapie” in sheep, “mad cow” disease, Creutzfeldt-Jakob disease, a disease called “Kuru” which only occurs on one South Pacific Island, and FFI.  The author ties all the different aspects of the book together by putting a human face on prion disorders with an Italian family who can trace instances of FFI in their family tree back to the 1700s.  The family had always known that there was some sort of medical issue in their family tree, and each generation lived in fear of developing it, yet the disorder was never diagnosed or truly identified until about twenty-five years ago.

I was really glued to the book, and the author did a great job of making it informative yet a bit “gossipy”.  The politics and ego of the scientific research community will horrify you, as will an in-depth look at how the “mad cow” problem has been bungled in both the UK and the US, mainly by greedy bureaucrats.  There’s a fair amount of information about chemistry and genetics included, but it’s all very well-explained and easy to understand.

The book should leave you a little bit scared, and rightly so.  Prions can just spontaneously start reproducing themselves to infect you, as in cases of spontaneous Creutzfeldt-Jakob.  You can be infected by prions by ingesting them, which leads to a great anthropological discussion of how our ancestors were probably all cannibals until a prion disease wiped out entire populations and made cannibalism a taboo.  Prions can jump species, which is how sheep with scrapie led to cows with bovine spongiform encephalopathy, which led to people with infected with “mad cow”.  Sheep with scrapie are also believed to have been the cause of Chronic Wasting Disease, which affects so many elk and deer herds in North America.

I finished the book just as beef producer Creekstone Farms was making the news headlines again.  They’re the fairly small ranch which announced a few years ago that they were going to test every one of their cows for BSE.  The USDA stepped in and put a stop to it, complaining that if Creekstone Farms tested every cow, the USDA would be expected to test every cow, and it would be too expensive (boo hoo!).  The case went to court, Creekstone won the right to test their cows, and they were going to start doing that as of June 1st until the USDA stepped in and got an injunction to stop them.  The arguments the USDA is using are ridiculous, and I can’t imagine any citizen thinking it’s acceptable for a government agency to prevent a private business from ensuring their products are safe.  It’s also ridiculous that YOUR TAX DOLLARS are paying the USDA’s legal bills, to help them do something which isn’t in your best interests!  It’s extremely likely that BSE exists in some cows in the US, and if they aren’t ending up on your plate, they’re ending up in your pet’s food dish, so we all need to speak up and tell the government to put safety before profits.  I’ve been trying to eat less meat lately, yet I’m willing to buy from Creekstone just to make a point. Here’s a little news story about the issue:  Legal fight over BSE testing drags on

Getting back to the whole point of this entry, The Family That Couldn’t Sleep was a great read, and I highly recommend it.  I’m not sure what I’ll tackle next in my book pile… I’ve gotten so far behind in my reading since I started to work for a living.  Working is for suckers, man.

Posted by Leigh-Ann on 05/31 at 04:49 AM

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