Musings from the ever-changing, ever-amazing and occasionally ever-baffling Fort McMurray, Alberta.

Thursday, October 31, 2013

One Thing You Don't Know About Me - Of Prions and Science in Fort McMurray

Have you ever been at one of those conferences or sessions where they start with a "getting to know you" exercise? The usual trifecta is to give your name, occupation, and something people don't know about you. While the first two are ones I can usually answer readily (provided I have had my morning coffee), the third one often stumps me. I feel people know pretty much all there is to know, as in this blog and on various social media I have shared my obsession with shoes, my inability to keep my opinions to myself, and my tendency to cry at the drop of the hat, including at commercials (I am a marketer's dream audience). There is one thing few would know about me, though, or even suspect. I am a science junkie.

It's true, I love science. And while I have an interest in all facets of science some, like physics, leave me so confused that I feel like I've been through a blender after reading a book on them. But there are two fields of science that have always fascinated me, and that I read about, think about, and talk about - paleontology, and epidemiology.

Now, paleontology is the study of ancient life, and while many may think of it as "dinosaur science" my area of interest is the pre-dinosaur era, all the way back to the pre-Cambrian and the tiny creatures that were the beginning of life on this planet. I have a bookshelf full of books on this topic, and on evolutionary science, most by a now-deceased author named Stephen Jay Gould. My interest in this is so keen that a few years ago I climbed a mountain in BC just to touch trilobite fossils, and to sit in one of the places where life began to flourish on this planet millions of years ago. But the other science I love is a bit more relevant to day to day life: the study of disease.

I don't know why it has always fascinated me, but the study of disease, whether caused by viruses, bacteria, or other processes, has always been a passion of mine. I have read hundreds of books on the topic over the years of my life, and one particular type of disease has always intrigued me. My interest began in the 1980's. with a mysterious outbreak in England.

In that decade a veterinarian in England discovered a very sick cow on one farm. That one very sick cow went on to become hundreds of sick cows, and the outbreak of BSE, or Bovine Spongiform Encephalopathy, began. It was an interesting story even at that point, but it was with mounting horror I began to read news reports of young adults in England who began displaying signs of an until-then extremely rare disease known as CJD, or Creutzfeldt-Jakob Disease. I was a young adult then too, and what was worrisome was that this disease typically strikes rarely, one in a million, and even then in older adults. The sudden incidence in young adults, and the sheer numbers, were terrifying, as they did not know what was causing it, and if transmissible how it was being spread - until some research occurred showing that this disease, now known as vCJD, or a variant of CJD, was actually simply BSE - in humans. The new scary part was that it had come from eating beef.

Overnight the beef industry in the UK was devastated, and the consumption of meat fell to dramatic levels. Dozens of young adults fell ill and died of a disease that is invariably fatal, and that causes horrible suffering in its victims as their brains fall prey to form of neurological disease that creates rapid degeneration of their cognitive abilities. Humans showed symptoms similar to that of cows, and while hundreds of cows were killed and burned so their meat could not enter the food chain, thousands and thousands of humans realized they had been exposed to a deadly agent of disease known as a prion, a protein found in our brain that for some reason in these diseases "misfolds", causing these horrible symptoms, and a horrible demise.

The beef industry in every other country quaked in fear watching events unfold in England, and then, in 2003, a piece of bovine brain tissue was under the microscope of a scientist here in Alberta. It came from a cow that had been displaying worrisome signs, and the researcher turned on her microscope and saw something that would change the beef industry, the province, Canada, and hundreds of thousands of lives forever. She saw in her microscope a prion - and last night I met that scientist.

Last night I had the honour and privilege of attending a talk from Jay Ingram, perhaps most famous for his hosting role on Discovery Channel's science show Daily Planet as well as the author of over a dozen books, Dr. Stefanie Czub, a scientist with Canada Food Inspection Agency, Dr.Valerie Sim, a neurologist and researcher from the U of A, and Dr. Kevin Keough. the Executive Director of the Alberta Prion Research Institute. They were here at Keyano College to talk about prions, and prion research, and it was an evening I doubt either I or the Intrepid Junior Blogger will soon forget, as it was an evening which painted a picture of a tiny protein abnormality with enormous consequences.

When I asked the IJB if she wanted to attend the lecture on prions she quickly agreed, as she is currently studying genetics in school. Every night in our house has become a quiz-fest as she tries to tease out what I still remember of chromosomes and dominant traits. She too is a science junkie, although she normally avoids biology (having a bit of a squeamish side that I fortunately do not share) and prefers physics and chemistry - but she has been talking recently of how much she enjoys genetics, and how intriguing it all is. Last night she sat in the second row at the Doug Schmit Lecture Theatre in Keyano College, and she was completely transfixed as four people shared the story of prions, and their impact.

From a rare disease called Kuru found in New Guinea in the 1950's to Chronic Wasting Disease in elk and deer in Alberta, we went on a little journey around the world with an elusive science riddle that we now know as a prion. Jay Ingram, who is skilled at taking the realm of science and making it accessible, served to tell the tale, but what was perhaps most intriguing and most compelling was the way Dr. Sim and Dr Czub wove their pieces into the story. Dr. Sim spoke as a neurologist who treats these kinds of diseases (although treats is a bit of a misnomer when it comes to prion diseases, which have a fairly predictable progression and an always fatal end), and her ability to tell a tale simply shone as she shared the tragic stories of patients diagnosed with the spontaneous form of CJD (as CJD can arise on its own with no infectious cause, but extremely rarely). I was spellbound watching her, and I suspect she has either had some training in the dramatic arts or is perhaps also an actress, as she had an incredible ability to bring the stories to life. Dr. Czub shared the story of BSE in Canada, and when she revealed that she is the scientist who identified the first BSE case in Canada I think my jaw dropped, because I suspected that moment in front of her microscope was a life-changing one as she knew that the fate of an industry, a province, a country, and hundreds of thousands of people would be impacted by what she saw (I can't even quite imagine it, but it must be one of those incredibly rare moments as a professional when you realize that what you have done or discovered will change history forever). Dr. Keough was the opener and closer for all of this, sharing the incredible work being done at the Prion Research Institute, founded after the BSE crisis in Alberta began.

The IJB and I were transfixed, frankly. The intermeshing of the stories took the tale of prions to another level entirely, showing the impact on human and animal life, on industry, and on the economy and country. I recall so well when BSE was found in this province, and the "shoot, shovel, and shut up" comment from then-Premier Ralph Klein, who almost certainly wished the entire thing would just go away, knowing it would take a multi-billion dollar industry and make it worthless overnight. But Klein rose to the occasion, and his government decided to fund prion research, creating this institute and attracting scientists to do work on the causes and impact of these diseases. I am proud to say our current government continues to fund this research, despite other governments around the world beginning to eliminate similar research as the BSE crisis in their countries seems to be over (and I use the word "seems" very intently, as prion research has significant relevance to other diseases very similar to CJD and BSE, like Alzheimer's and Parkinson's, diseases which continue to devastate lives).

After the lecture was over the IJB and I took the time to chat with the panel of guests, and our conversations were of the kind that excite both my head and my heart, as the scientists are so passionate about what they do, and why they do it. We talked about how science needs to find ways to get these stories out into the world, and how this new world of the 30-second media clip and 2-minute attention span is not kind to stories that require just a bit more narration and history. The IJB was quiet during much of this, as she often is, but I could see the gleam in her eyes, especially when Dr. Czub invited her to visit the research lab to learn more about what they do. I looked over and the IJB's eyes had widened, and I knew she was utterly and completely hooked by these two women who are the kind of role models I want for my daughter.

We spoke to Dr. Keough about the work of the Prion Institute, and we spoke to Jay Ingram, sharing our appreciation of his work, and of the lecture that night. He told us that they had all been excited when I had tweeted that I was bringing my fourteen year old along to the lecture, as I don't think they get a lot of that demographic attending such lectures. He signed a book for her, and said that one day when she has her PhD in science she should look him up again to chat - and she said she would, and I suspect one day she will, as she is a creature of her word.

On the car ride home the IJB talked non-stop. She talked about BSE, CJD, and CWD, using the acronyms like a pro, and she theorized that maybe the advent of lab-grown meat was the solution to ever seeing the rise of a meat-ingestion related prion disease again. She talked about the invitation from Dr. Czub (and her eyes absolutely glowed as she did), and she talked about reading more about prions. And then, this morning, she came upstairs and said "I know what I am going to do my project-based learning on this year. I am going to do it on prions, and the impact of prion-related diseases". I knew at that moment that a lecture from 3 scientists and one adept science writer and communicator had likely impacted the trajectory of her life, as she had found a new area of science that intrigued her, excited her, and made her want to learn even more, sharing what she learned just as they shared with us last night.

Last night, you see, was science communication done right. Perhaps there are those who think researchers should spend all their time in the lab, but I believe the best people to share their passion and tell these stories is the scientists themselves, with the help of professional communicators like Jay Ingram. The story of prions, far from being some dry and dull tale, is a fascinating story of diseases that impact our world, our economy, and our very lives. The continuing tale of Chronic Wasting Disease in elk and deer unfolds daily, and it has potential significant impact if it does things like move into our caribou herds. This story is a lively one with tremendous relevance as we try to understand diseases like Alzheimer's, and it is so deserving to be told and shared - and last night it was, in a little lecture theatre in our local college.

I want to thank Jay Ingram, Dr. Keough, Dr. Sim, and Dr. Czub for coming north to share this story with us, and for being so very, very kind to my daughter and I. I don't know if they know it, but they have impacted her young life, and I am so very grateful to them. I want to thank Keyano College for presenting this lecture, and for allowing this community to attend events like this where we can learn and explore our world with individuals like those we met last night. I also want to thank our provincial government for continuing to fund this research so that scientists like these can continue to chase their passion, and pursue an understanding of a tiny protein abnormality that can create such devastation.

So there you go, folks. There is something you didn't know about me. My name is Theresa, I'm a writer - and science excites me. Damn. Now I am back to not knowing how to answer that third question again, I guess.

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