Monday, March 11, 2019

People in Bubbles

You've probably heard of the Bubble Boy.  It's one of those weird shared cultural memories we all have without ever really knowing where it came from.  We've heard it referenced on shows like Seinfeld and the Simpsons, and never really questioned the concept, though the words conjure, for most of us, the same image: a person inside of a round, clear hamster ball, similar to the type you'd see on a Japanese game show.

The bear represents infectious disease

Today I'd like to talk about the Bubble Boy.  There was, in fact, a real-life Bubble Boy, and his story is as fascinating as a Japanese game show.

Let me take a step back, however, and explain that germ theory is a rather recent discovery, and that the whole concept of a "bubble boy" is only possible because of it.

Bubble boy's bubble was not truly a bubble but a sterile environment not dissimilar to the type lived in by axenic (germfree) animals.  If you'd like an in-depth look at how these environments are created, look no further than my post explaining how they are constructed, complete with pictures.  I have seen many debates online discussing what would happen if all bacteria in the world died.  Most people posit that all life would also cease to exist, but I can say with confidence that we wouldn't all perish.  Yes, cows and termites would be goners, but plenty of animals (humans included) can live without bacteria.  Although, is it really living if you can't have a mindful kombucha to go with your morning chakra alignment?

Sterile environments and the practice of raising axenic animals came about less than 100 years ago, in the 1940s, but wasn't perfected until much later.  In fact, in 1963, Julian Huxley declared that "a germ-free world is an ecological absurdity, just as a perpetual motion machine is a mechanical absurdity, [and] it is just nonsense to talk of [it]."

Fortunately for David Vetter, Huxley was wrong.

David Vetter was born in 1971, less than ten years after Huxley's statement regarding germfree environments.  His was a normal pregnancy and birth by all accounts, with one caveat: David had SCID, or Severe Combined Immunodeficiency, a rare genetic condition in which your immune system doesn't work because your cells don't activate correctly, and your T cell count is low.  (Your B cells produce antibodies that fight antigens, or foreign invaders such as bacteria; your T cells regulate immune responses and are involved with acquired immunity.)  SCID's symptoms are similar to that of AIDS; without any immune system, your body is basically a bacteria buffet, and pathogens run rampant, resulting in chronic infections and rare cancers caused by viruses that can reproduce unchecked in your body.  (The AIDS virus destroys T cells; people with SCID either lack T cells or have non-functional T cells.)

David Vetter could be said to be both lucky and unlucky.  Certainly, having SCID is unlucky... the exact opposite of winning the genetic lottery.  But he was lucky in that his condition was known.  His parents had had another child with SCID already, who had died after seven months.  (Most people with SCID die within a year.)  Knowing that they were carriers for this recessive genetic condition, they opted for prenatal testing and discovered David would have the disease.  This allowed them to prepare for his birth; David was moved from his mother's womb into a sterile bubble, where all items were sterilized to prevent infection.  The air passed through filters; food, water, diapers, toys, blankets, and all other essentials had to be germfree to enter his bubble.

David lived in his bubble for twelve years before getting a bone marrow transplant, which subsequently killed him.

Bone marrow transplants, alongside gene editing, are the current methods of treatment.  Your bone marrow produces your blood and its products... including lymphocytes such as B and T cells.  Giving a person with defective bone marrow new, healthy marrow can, in theory, functionally "cure" their condition.

While David Vetter is often hailed as "The" Bubble Boy (SCID is now called "bubble boy disease"), he wasn't the only child to live in isolation.  Ted DeVita had a completely separate condition but he, too, required a germfree containment unit to survive infancy.  Ted's condition, aplastic anemia, is a result of having bone marrow that doesn't work.  Terrifyingly, aplastic anemia often has no known cause; you can develop it abruptly for no discernible reason.  In normal, healthy people, about half of your bone marrow is made up of hematopoietic stem cells, which produce the products of your blood: red blood cells, white blood cells (including the B and T cells of your immune system), and platelets.  If you develop aplastic anemia, those stem cells disappear, replaced by nonfunctional fatty tissue.

People who claim they're not fat, just "big-boned," are still therefore unhealthy.

Because his body was unable to produce B and T cells, Ted, like David, suffered terrible immune problems.  As his condition worsened, it was decided he should be placed into a sterile environment.  The sterile environment in question was a "laminar air flow" room, which the hospital already had set up for immunocompromised leukemia patients.  Ted spent the last eight years of his life in a "bubble," dying in 1980 due to iron toxicity.

The attention given to the "bubble boys" had both positive and negative effects.  In 1976, John Travolta starred in a movie called The Boy in the Plastic Bubble.  If you're paying attention to dates, you'll notice that this movie came out during Ted's lifetime, when he was 14 years old.  The cheesy melodrama borrowed heavily from David's and Ted's real lives.  Fortunately for Ted and David, they were dead by the time Bubble Boy, a 2001 comedy starring Jake Gyllenhaal, came out; the movie later prompted a musical in 2008 by the same name.

While such media portrayals were at times tasteless, they did spark public interest and awareness, leading to pubic financial support of the "bubble boys" and driving forward research.  Today, over 80% of people with aplastic anemia can be treated using procedures such as bone marrow transplants.

(Note: one of the charities I highly support is Be the Match.  You can sign up to a bone marrow registry and potentially save a life; keep in mind that your bone marrow, like your blood, grows back, so live donations have no permanent effect.  I am already in the registry myself.  Sign up here today!)

As with many diseases, the major treatments and cures began with thought experiments that were turned into realities.  (Suck it, Julian Huxley!)  The widespread interest and education about the "bubble boys" prompted a leap forward in the medical community, and while it was too late for David and Ted, for thousands of bubble boys today, it's nothing short of a miracle.  Take, for example, Ayaan Ahmed Isaacs, who is currently receiving gene therapy and may be one of the first to be truly "cured" of SCID.

If you clicked on that link, you might have seen what I saw on the sidebar.  Under "recommended stories," three out of four are about measles.

Measles is a highly contagious virus that was eradicated in the United States in 2000.  In 2015, we had 188 cases.  In 2018, we had 349.  This should scare you, regardless of whether or not you are immunocompromised.

The anti-vax movement took off the same year that we eradicated measles.  In the year 2000, Cindy Crawford appeared on Good Morning America to discuss vaccines and how she had decided to delay vaccinating her baby, due in large part because of Andrew Wakefield's recently published (and now debunked) "study" linking them to autism.  Later, Jenny McCarthy hopped on board, and suddenly, a movement exploded.

This post isn't about the anti-vax movement.  You could write a book on it, and a single blog post can't do it justice.  It's a damaging, dangerous, lethal anti-science movement that targets society's most vulnerable citizens: the young, the elderly, and the sick. No, this post is merely touching the tip of the anti-science, anti-authoritative iceberg, which happens to include the anti-vax movement, and its purpose is to beg, rally, cajole, and otherwise convince you that we really, really need to take anti-vax shit seriously and push back against it, instead of saying, "Oh, that's just Aunt Karen, who cares, Facebook isn't real life, anyway."

Perhaps the biggest question I have about the anti-vax movement is, why?  How?  How could we watch polio and measles disappear before our very eyes and then decide vaccines are somehow a bad thing?

In part because of social media.  The anti-vax movement is rooted in privilege, and privileged people tend to self-segregate themselves into echo chambers.

A brief word on privilege and anti-vax: the thing about vaccines is that, if you're living in a third-world country, you will work your ass off to get a measles vaccine for your children.  Half a million children die every year from the measles according to the World Health Organization, and most of those are in underdeveloped countries in Africa and Asia.  As the article I linked above mentioned, "the fear of measles disease waned from the collective memory of a new generation of parents who were fortunate enough to have been vaccinated as children. The mild side-effects of the vaccine, such as fever or pain, are now perceived by some people as a greater disturbance than the disease itself."  And if you're in a developed country, the chances of getting measles is low to begin with, thanks to herd immunity; your unvaccinated child might never come in contact with the virus, so why bother vaccinating?  And if they get measles, well, you've got money, you've got healthcare... you'll be fine.  In theory, anyway.

But in reality, measles is bouncing back, and with it, other diseases we should have gotten rid of by now.  And it won't be long before we see a "trickle down" effect from the privileged to the less privileged, who are unable to fight against it.  We need to stop it before it gets to that point.  Currently, the anti-vaccine movement is spreading like the viruses its helping, and it's aided in a very large part by casual misinformation.

I saw a great example of this recently; someone on Facebook claimed that the recent measles outbreak in New Zealand mostly hit vaccinated people.  (They were trying to argue that vaccines are not effective.)  A cursory Google search got me the real news: of 28 measles cases in Canterbury, only 4 were fully vaccinated.  But when I posted the link to the article and the correction, I was blocked.

Why?  Because the anti-vax movement cannot exist outside of its bubble.  It relies on an elaborate system of denial, misinformation, and wilful ignorance.

Bubbles can protect; they can keep bad things out.  (Iceland recently banned unvaccinated visitors.)  But bubbles can also injure by keeping good things out.  The best weapon we have against disease of any sort is public knowledge and interest.  It was public knowledge and interest that led to the creation of sterile environments of SCID patients, environments that scientists themselves thought might not even be possible.  (Suck it, Julian Huxley.)  It was public knowledge and interest that led to the eventual push for research and treatment of these disorders.  A cure looms on the horizon.

But as measles has shown up, a cure isn't enough.  Because if a cure exists and people refuse to use it, well, then, what's the point?

I know I post the G.I. Joe "Knowing Is Half the Battle" meme a lot, but in this case, it's much more than half.  The anti-vaccine conspiracy isn't a harmless, silly fad.  It's a life-threatening, dangerous movement, and it's not enough to simply ignore it.  We need to fight it with knowledge; we need to educate, to look up sources, to publicly butt heads any time anti-vaccine rhetoric rears its ugly head.  We cannot ignore or turn a blind eye to this; we need to learn their arguments and their language and find ways to change their minds.  It doesn't matter how we do it, so long as it's effective.

It won't be easy.  But it's every citizen's duty to do so, because this is a movement that has far-reaching consequences for the most vulnerable members of society.  (Don't worry; if this sounds hard, here's a source on how to fight anti-vaxxers using their preferred methods of communication: memes.)

The parents of David Vetter and Ted DeVita would probably have a few choice words for the parents of unvaccinated children, children who have normal, robust immune systems and whose only bubble is the informational one that their parents have put them into.  People who say that it's their own, personal choice not to get themselves or their children vaccinated are forgetting people like David, and Ted, whose unlucky circumstances led them to a life of isolation and immunological isolation.

The reality is that unvaccinated members of society are everyone's problem, because their "personal choice" is putting others at risk.  Measles is not going to get tired or take a break, so neither can we.  Every person needs to be ready, willing, and able to argue against anti-vaccine rhetoric whenever they see it, because anti-vaccine rhetoric can only exist inside of a bubble.  It's a fragile, tenuous position that exists in a vacuum of any real scientific evidence, and it can be cured.

But that cure requires us to pop the bubble.

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