The Family That Couldn't Sleep

Note: I will be traveling this weekend, so although this post hasn't been officially assigned yet, I am going to write something that reflects my understanding of the assignment as discussed in class. I may edit this post once we get the "official" assignment notice.

Readers will have a variety of stakes in this book. I am going to start with "Abstract and Immediate" because I feel that it is one of the most important.

Abstract & Immediate: for those wondering what else is wrong

A major theme of this book is the failure of our health care system when faced with novel problems (now and throughout the ages). By health care system, I mean both the doctors who are supposed to cure us and the government which is supposed to protect us. We are all conditioned to trust systems: our public school system was originally designed to turn us into obedient factory workers, and its design hasn't changed much since the Industrial Revolution (See: http://www.spinninglobe.net/gattopage.htm). Systems are useful and necessary for societies with large populations to function. They allow us to quickly and consistently fix well-understood problems. Although novel problems, by definition, resist conventional classification and treatment, systems will always try to treat the FFI patients of the world for alcoholism.

Treatment for microbial infection was pretty useless until the discovery of antibiotics and sterilization. Countless people died from now-preventable viral diseases before vaccines were invented. We are still in the dark ages when it comes to prion disesases. When a public health crisis as poorly understood as a prion disease emerges, it becomes almost impossible for a system as rigid as a law-making body to get a handle on it. I am a big believer in conspiracy theories, or, more accurately, government cover ups--but not because I think that Pinky and the Brain are really out there trying to take over the world.

Systems are comprised of people and paperwork. My general experience with people as system cogs is that they are mainly concerned with covering their own asses, they know much less than they say they do (and are generally content with their ignorance), and they are highly influenced by groupthink. I think we all have enough experience with paperwork that I need not comment about that. Once a public health crisis is discovered, the first thought of most organizations is "let's not alarm the public." The second thought is, "let's make up a committee to investigate this." After the committee spends awhile arguing about something they don't really understand, the issue might get passed along to the politicians.

Now, my general understanding of politicians is that they need to be practiced in the following arts to succeed:

1. Putting on a dog and pony show for their voters (i.e. special interest lobbyists), which mainly entails pretending to have "moral values"
   
2. Financially appeasing their bosses (i.e. corporate lobbyists and party organizers)
3. Circumlocution
   
4. Preventing their sex scandals from leaking onto the Internet

I know a lot of highly educated people who have very sound and logical political opinions, but none of them would touch career politics with a 50 foot pole. The fact that these kinds of people are smart enough to avoid the job might explain why the politicians we DO have are so fundamentally ineffectual.

Most politicians aren't doctors, scientists, or IT specialists, so passing on issues to them is like playing a game of telephone where the receiving party has bad reception. However, politicians also understand that they need to appear to know what they are talking about (See: http://en.wikipedia.org/wiki/Series_of_tubes). Eventually, the issue is bound to be mangled. The worst thing that can happen to an issue is for it to get spun into the religious or corporate agenda of some politician. Once a public health crisis runs the risk of being a red mark on some politician's report card (politicians must perform an economic balancing act of lowering risk to the public while maintaining friendships with the industries who paid for their campaigns), the issue gets buried in a slurry of committees, sham investigations, red vs. blue, bureaucracy, theocracy, idiocracy, whatever.

As an aside, the scariest part about "government cover ups" is that even if the issue isn't a big secret, most people aren't likely to care. Or, even if they do care, it will only be until Britney Spears dies tragically young. A big problem is the way the ad-sponsored TV news (or, as I prefer to call it, non-news) decides to do its entertainment non-journalism (See: http://www.technologyreview.com/Infotech/19845/page1/).

So, what's my point? I think everyone knows that politicians are crooks and liars, and that Fox News (Fair and Balanced!) just wants your Nielsen ratings. My point is that systems are fallible, and if a system has always failed you in the past, you really have no reason to trust it in the future. We shouldn't find it so strange that the Italian family with FFI from mistrusts doctors and the media. We shouldn't laugh at the "Creutzfeldt-Jakobians"--actually, I found this term rather derogatory--for their skepticism.

If you want safety, it is your personal responsibility to educate yourself about the best practices for safety. If you want privacy, it is your personal responsibility not to share personal information with those who might expose it to the public. Relying on clunky government machinery or the questionable opinion of a single doctor increases your risk of personal injury. This isn't to say that doctors and the government should be exculpated for any potential harm they directly or inadvertently cause you, but wouldn't most people prefer to avoid injury or illness in the first place?

I think there are a lot of potentially dangerous things out there that have been "covered up": phthalates in our plastics, hormones and chemicals in our food, mercury preservatives, lead in our cosmetics, toys imported from China. Some people pay attention to these things and try to change their behavior to lower their risk of exposure. That leads me to concrete and long term stakes:

Concrete & Long Term Stakes: for those interested in observing and/or modifying their behavior

Readers may become more conscious of their intake of certain types of animal proteins after reading about how mad cow disease probably exists in some form, under the radar, in the U.S.. Readers may be less likely to eat hamburger, venison, or T-bone steak. Beef brains are also a specialty item sold in some parts of the U.S.

The story of the vegetarian who died of mad cow disease was particularly interesting to me. I read this story in the news when it came out, and I have never forgotten it. Although I have been a vegetarian for most of my life, I still ingest (or risk ingesting) some animal proteins:

• Most pills are made with gelatin, which is extracted from animal bones by boiling them. Although I don't eat Jello or gelatin marshmellows, I have occasionally needed to take antibiotics with gelatin capsules.
  
• Many restaurants add meat in unexpected places. For example, most Italian restaurants add beef stock to their maranara sauce to give it a richer, fuller taste. I probably eat some small amount of meat every year without ever realizing it.
  
• I share pots, pans, dishes, and utensils with others who do eat meat. I know that my roommates sometimes use our shared pots and pans to cook venison or ground beef. Prions survive heat and stick to metal, so if I were really, really unlucky, I could potentially contract prion disease from one of these pans.
  
• I eat eggs and dairy. Prions might be passed on in these forms.
  
• My boyfriend sometimes eats meat and then kisses me.
  
• Some cosmetics and toiletries are make with animal proteins.
  
• I know that at one point, it was possible to get prion diseases from blood transfusions. It also might be possible to get prion diseases from surgical implements that are reused. I'm not sure what the current policy is regarding these things.
  

Why care? Why worry about these seemingly insignificant risks? I can't really avoid them, so I just have to accept them...but death by prion certainly seems like quite a terrible death.

Concrete & Immediate Stakes: for those touched by prion and similar diseases

Some readers will have concrete stakes in this book. Naturally, the book will be very important to the Italian family suffering from FFI. Other readers may be suffering from another prion disease (or know someone who is). They may also belong to families that suffer from other genetic diseases that strike later in life, such as Huntington's.

Most readers will be able to empathize with the members of the family with FFI on some level. Although very few people will ever understand the full extent of Fatal Familial Insomia, there are many terrible diseases about which we know very little and for which we have no cure. As a child, I saw one of my great aunts wither away from Alzheimer's disease and eventually die. The changes in her thought processes and behavior were very disturbing to witness. As I was reading, I could imagine myself as the young relative of one of the FFI patients.

Along with most of the population, I've also experienced insomnia (if only for a few hours at a time), so I know how it can impact the daily life and thought processes of a person.

Some readers will be doctors or medical researchers studying prion diseases. They may know or know of some of the researchers mentioned in the article.

Other readers will just be interested in the theory of prion disease. I grew up in Wisconsin and Minnesota, and I knew that chronic wasting disease was a big problem for the deer population. I was interested in the theories about how it originated and the hunting policy changes meant to curtail it.

Abstract & Long Term Stakes:

After reading this book, the reader is left with many questions to ponder, such as:

• How did we come to know the medical knowledge that we now have? Textbooks often present scientific history in a simple, linear fashion, bleaching out details like Carleton Gajdusek's pedophilia. However, studying the motivations and personalities of scientists provides an important context for understanding why we know what we do.
  
• What else don't we know about mechanisms of disease? No one thought that proteins without viruses could spread scrapie or vCJD. Maybe scientists will discover the mechanims behind almost-related diseases, like Alzheimer's and Huntington's. Maybe we will discover something even stranger than prions, and there will be another paradigm shift..
  
• Will we find a cause of or cure for our obscure diseases? Must we rely on scientists who hunger for a Nobel prize to care?
  

Another important theme of this book is that human intervention in natural processes (breeding, animal growth, the food chain) can have serious unforeseen consequences.