Prions, not Zombies, are the Real Brain Eaters

Of all the etiologies of infectious diseases, one of the coolest has to be the prion. Prions are the smallest infectious agents and broke all the paradigm when they were definitively linked to diseases. It was almost heresy to postulate that a proteinaceous infectious particle--devoid of nucleic acid--could cause disease. But today's heresy is tomorrow's Nobel Prize, as Carleton Gajdusek and  Stanley Prusiner showed.

Prions cause disease in a unique way. Though they are definitely transmissible prion diseases they are, in essence, misfolded proteins that cause a cascade of protein misfolding leading to neurologic dysfunction. In some instances, the initial misfolding is the result of a sporadic genetic mutation and in others it is because of introduction of a misfolded protein causing the cascade. It is an impressive process.

Eventually prions, first linked to scrapie in sheep, were linked to human diseases known as the Creutzfeldt-Jakob Diseases (CJD), the most famous of which is new variant CJD (vCJD)--the human form of mad cow disease--as well as the human cannibalism-linked kuru. The story of vCJD is well documented. In short it has to do with changes in industrial farming practices allowing scrapie in sheep to get into cows and then into humans. The human outbreak, which captured headlines in the 1990s, is essentially over as farming practices have changed so as to eliminate exposure to the prion. 

Prions may have slipped from the headlines but research on them continued nonetheless and this week it was announced that a novel prion (alpha-synuclein) was linked to another neurologic disease: Multiple System Atrophy (MSA). Not surprisingly, this discovery came from Dr. Prusiner's lab. 

The biggest implication of this finding is further evidence supporting the hypothesis that other neurodegenerative diseases may have a prion component to their cause. Such an understanding may open up new avenues for treatment and screening for these dreaded diseases. 

 

Chickenpox Party in the Royals Dugout

If I ever walk through a baseball dugout, my biggest worry would be stepping in or touching chewing tobacco spit however the Kansas City Royals have much more to worry about after 2 players contracted chickenpox. It is assumed that the 2 infected players were not vaccinated and somehow escaped natural infection (possibly due to both Alex Rios and Kelvin Herrera being from the tropics where the virus spreads poorly).

Chickenpox, once a horrible rite of passage, has now been relegated to a rarity given the prowess the varicella vaccine. This vaccine that has nearly vanquished a disease that just over two decades ago infected 4 million, hospitalized over 10,000, and killed 150 Americans yearly. Now those numbers are 95% lower. Additionally because the same virus that causes chickenpox can years later reactivate and cause shingles the benefit of the vaccine will be far reaching as shingles and its horrible after-effect of post-herpetic neuralgia require billions of dollars of medical treatment costs annually.

Chickenpox is a very contagious disease and for those susceptible because they are too young to be vaccinated, not vaccinated for medical reasons, or not vaccinated for deliberate non-medical reasons can, for certain individuals, be a serious disease. As such, I oppose chickenpox parties for obvious reasons though maybe those anti-vaccine parents who fondly recall their beloved experiences with chickenpox might try to book the Royals dugout to give their poor children that same experience.

 

Alexandra Levitt's Deadly Outbreaks: An Expert Tour of Epidemiology (including aerosolized pig brains)

Reading the tales of infectious disease outbreak investigations is, to me, the equivalent of opening up Sherlock Holmes' case book and seeing a master unravel a case. The delight I get in reading books like that keeps me coming back to read each new infectious disease book with wide eyes, trying to glean whatever I can from the particular outbreak being described. 

Such was my reaction to Deadly Outbreaks: How Medical Detectives Save Lives Threatened by Killer Pandemics, Exotic Viruses, and Drug-Resistant Parasites by the CDC's Alexandra Levitt. The book is a compendium of important outbreak investigations over the past few decades that are illustrative of the power of epidemiology in elucidating causes, avoiding blind alleys, and changing policy.

Some of the included topics are: the original Legionnaire's Disease outbreak, the Four Corners Hantavirus outbreak, a multi-drug resistant malaria scare in Cambodian refugee populations, and the fascinating non-infectious pig-brain inhalation neurologic autoimmune syndrome. Being in the field of infectious disease, an added bonus for me was to read about people I know and interact with doing the investigative work.

I highly recommend the book for a concrete glimpse of what epidemiology is and how big its impact can be. Plus, who wouldn't want to read about aerosolized pig brains? 

I am a Dirty Contact Lens Wearer

I've had contact lenses for over 20 years and I can remember distinctly the first time I placed them in my eyes and how frustrating that process was. It got to the point where I threw a full tantrum when I couldn't get the contact lens to adhere to my eye.

Now it is second nature and I can do it in a car, in an elevator, or wherever I might be when one happens to fall out. I have even moistened a lens with tap water and even with saliva. It is easy to forget that your eye is a special tissue that is not nearly as resilient as one's skin and is a rather simple place for a microbe to flourish, especially when aided by prosthetic material (i.e., a contact lens) let alone poor hygiene.

None of this is good, especially not for an infectious disease physician. So, it comes as no surprise that I am not alone amongst contact lens users. Like my fellow dirty eye people, I have had numerous episodes of conjunctivitis and have had to take antibiotic eye drops and steroids several times--sometimes self-prescribed or prescribed by mom or dad. 

Thankfully my game of Russian Roulette with my contact lenses hasn't resulted in corneal ulcerations or a severe amoebic infection...yet. 

 

If You're Going to Say Something, Make it Matter

Carly Fiorina was the CEO of a major technology company whose products aimed to improve human life. In that role she was an advocate of innovation and technological process. While most people think of electronic devices and space technologies as synonymous with innovation, vaccines are indisputably on par with such conventional examples as they have transformed the life of the average human tremendously. Indeed, most advances in public health from sanitation to disease detection are the result of technological advances.

Accordingly, I was shocked to see Ms. Fiorina countenance the positions of those who seek to endanger children by prohibiting vaccination against diseases that remain clear and present dangers in this country.

While it has become commonplace to hear politicians and public figures of all political persuasions make statements that cast unwarranted doubt on vaccines, it is inexcusable for someone of Ms. Fiorina's stature and she knows it (and Governor George Pataki correctly pointed out). Hence, her nuanced position which affirms the rights of school operators to exclude the unvaccinated.

Further when Ms. Fiorina references esoteric vaccines it is clever trick that allows her to play both sides of the issue. When I hear that term "esoteric vaccines" the vaccines against yellow fever, Japanese Encephalitis, smallpox, and anthrax spring to mind. However, my context is radically different from that of the average voter she is attempting to court who may erroneously think of the vaccines against human papilloma virus, hepatitis B, and chickenpox as "esoteric."

It should be an asset to a candidate to be pro-technology and all embracing of a tool that has literally added decades to the lives of all humans, not something that requires spin. It is never a good thing pander to the primitive to be a viable candidate.

It is only through an intransigent defense of vaccines that all vaccine-preventable diseases will become esoteric.