S1 Bonus 2 - The Santa Clause
Podcast episode show notes
This is an extra EXTRA special holiday bonus episode where I talk about SANTA, Siberian Arctic Northern Thyroid Autoimmunity, the diseases that causes you to turn into Santa Claus.
Sources
Progeria: A rare genetic premature ageing disorder: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4140030/
HGPS is autosomal dominant: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3221230/
Werner Syndrome: Clinical Features, Pathogenesis and Potential Therapeutic Interventions: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5025328/
Hutchinson-Gilford Progeria Syndrome: a premature aging disease caused by LMNA gene mutations: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5195863/
Our bodies replace our cells regularly: https://www.scientificamerican.com/article/our-bodies-replace-billions-of-cells-every-day/
Hayflick, his limit, and cellular aging: https://www.nature.com/articles/35036093
Progeria incidence as of 2022: https://my.clevelandclinic.org/health/diseases/17850-progeria
DNA replication errors: https://www.nature.com/scitable/topicpage/dna-replication-and-causes-of-mutation-409/
Scleroderma: https://en.wikipedia.org/wiki/Scleroderma
Marie Antoinette Syndrome: https://www.healthline.com/health/marie-antoinette-syndrome
Sudden whitening of the hair: an historical fiction?: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2625383/
Elie Metchnikoff’s phagocytosis discovery: http://scs.carleton.ca/~soma/biosec/readings/sharkimmu-sciam-Nov1996.pdf
Candy pulling causes it to turn white due to air: https://vm.tiktok.com/ZM6239tM9/
Factors Associated with Premature Hair Graying in a Young Indian Population: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4830165/
Dietary causes of prematurely grey hair: https://www.healthline.com/health/beauty-skin-care/reverse-gray-hair#diet
Vitiligo: Mechanisms of Pathogenesis and Treatment: https://www.annualreviews.org/doi/10.1146/annurev-immunol-100919-023531?url_ver=Z39.88-2003&rfr_id=ori%3Arid%3Acrossref.org&rfr_dat=cr_pub++0pubmed
Did Michael Jackson have vitiligo: https://www.umassmed.edu/vitiligo/blog/blog-posts1/2016/01/did-michael-jackson-have-vitiligo/
Human immunodeficiency virus—associated vitiligo: Expression of autoimmunity with immunodeficiency?: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7134483/
The Prevalence of Thyroid Disorders in Patients With Vitiligo: A Systematic Review and Meta-Analysis: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6340922/
Historical mass hysteria cases: https://en.wikipedia.org/wiki/List_of_mass_hysteria_cases
Folie a deux: https://en.wikipedia.org/wiki/Folie_%C3%A0_deux
7 Mysterious Mass Illnesses That Defied Explanation: https://www.history.com/news/mysterious-illnesses-mass-hysteria
How to Stop Time by Matt Haig: https://www.goodreads.com/en/book/show/45152372
Transcript
MUSIC INTRO
Welcome listeners to an extra special bonus episode of Tierah Ruins Things With Science!
This is the podcast where I talk about the science of infectious diseases in pop culture, and critique what they did right, and ruin what they did wrong.
It’s still the holiday season as I record this, and so I’m going to talk about something I thought up over the lead up to Christmas, and I’ve just now in the post-Christmas limbo had the time to research and record
I don’t know about you folks, but I have a list of holiday films I like to watch every year to help me with the Christmas spirit, and two of them are The Santa Clause and Ernest Saves Christmas
This year as I was watching, I realized that being Santa is a transmissible condition! And then I started wondering what kind of condition it is, how it transmits, who can be infected, what the symptoms and signs are, all of that
So now you get to to come with me as I tell you what I found out
Both of these movies, The Santa Clause and Ernest, are currently streaming on Disney+ in Canada at the time of recording, but you can also rent them from all the regular services.
[CLIP, T=1:19:50, Name, Sinterklaus, Name, Pere Noel, Babbo Natale, Peres Nicole, Popo Gigio]
I am going to be focusing mainly on The Santa Clause, but I’ll dip into Ernest Saves Christmas a little towards the last half of the show.
If you haven’t seen The Santa Clause, Scott Calvin startles Santa on Christmas Eve, Santa falls off Scott’s roof and disappears, so Scott puts on the Santa suit to appease his son and finish delivering gifts so Christmas isn’t ruined
Scott doesn’t know that by doing this, he has unknowingly contracted SANTA, Siberian Arctic Northern Thyroid Autoimmunity
SANTA is spread by fomites
A fomite can be anything, really, but I usually think of it as a portable object, something you can pick up, as opposed to what we would call a surface like a table or door handle
It’s things like your phone, a debit machine, maybe even the handle of a gas pump, which are gross and are the reason I always have hand sani with me all the time
The fomite here would be the Santa suit itself, having been worn immediately previously by another infected patient
Later in the movie we find out from Bernard that this is indeed the case
[CLIP T=33:35, “It means you put on the suit, you’re the big guy”]
While Scott was infected immediately upon putting on the Santa suit, there is a few months incubation period before symptoms begin to appear.
Sometime in summer the year following Scott’s infection, he wakes up one morning having had longer-than-normal beard grow overnight, and having gained a jolly amount of extra Santa weight
Eventually as it turns to autumn, his hair also turns white as the syndrome takes full effect, and when he shaves his beard and dyes his hair, it immediately changes back to white and fluffy
There are a few different syndromes that we already know about that can cause some of these signs of premature aging, they’re all very rare, meaning only a few hundred people are known to have them
The most studied one in children is called Hutchinson-Gilford Progeria Syndrome, or HGPS
Hutchinson and Gilford were the first two guys who described the syndrome
and progeria, pro meaning premature or early and geria from the Greek geron, meaning aged
Same root word that gave us gerontology, the medical discipline that focuses on senior citizens
HGPS occurs in only one out of every 4-8 million births, so in Canada, if you generously round our population to 40 million, that means we might only have 5 - 10 people in the whole country with this
As of 2022 there were only about 400 known children and young adults living with the syndrome worldwide
So actually if you calculate that out, with 8 billion people on the planet, that’s about one in every 20 million people, although given that many people live in remote areas or places that don’t report openly to the world the health status of their citizens, people die of quote unknown causes without finding out why, there might be more than 400 people, but we can’t know for sure
It’s estimated that children with HGPS age 10 times as fast as an average child, but it’s usually just their body, not their mind, so this is not an M. Night Shyamalan movie
The average lifespan of a person with HGPS is anywhere from 8 to 21 years, usually about 14, before they pass away from something typical of old age, like a heart attack, cancer, or stroke
HGPS is completely random that could happen to anyone, there’s nothing that makes it more or less likely to happen, it’s really just a result of the fact that the enzymes that replicate our DNA make mistakes sometimes
HGPS is a dominant genetic condition
I’m sure a lot of you know this, but some may not, so generally you have two alleles or copies of every gene because you have two sets of 23 chromosomes, one from each parent, same genes on each set, just slightly different versions
For a dominant gene, you only need 1 copy for it to be expressed because it’s so strong it overpowers the gene from the other set of chromosomes
Whereas for recessive traits, you need your two copies of the gene to be the same for the result to show up
With HGPS, one of your two genes for a protein called a Lamin has a mistake in it
Lamins are the proteins that help hold your nucleus together and form the membrane, sort of like the membrane that keeps an egg yolk separate from the egg white
In HGPS, the lamin proteins don’t have the right shape, so your nuclear membrane breaks apart more easily, which causes your cell to die sooner than it normally would
Like, I don’t know, if you could pick up an egg and shake it a bit and get scrambled egg because the yolk broke apart inside the shell from the agitation
There are at least I think 7 different kinds of lamins, and they each have a different shape, and so if one is a little wonky, it can probably hold together for a little while, but it would be delicate and break down at the slightest damage very easily
Like trying to shove the wrong piece of a jigsaw puzzle into a space
Most cells in your body don’t live forever, and eventually almost every cell in your body die and will be replaced
Cells in your intestines replace themselves about once a week
Most of your blood cells last a couple months, some muscles last several years as long as you’re not a bodybuilder working out all the time
But some cells like in your heart, your eyes, or your brain, they don’t get replaced and once you’re fully grown, that’s all you get and if they’re damaged, they can’t replace themselves
Even the ones that are replaced, cannot do so forever unfortunately, like lobsters can
There is a limit to how many times the stem cells in our body can divide and replace a cell that has died
There’s a name for this limit, you may have heard of it
It’s called the Hayflick limit, after the guy, Leonard Hayflick, who first published on the idea back in 1961
The Hayflick limit is different for different tissues and types of cells, but it’s usually between 50 to 70 divisions
Hayflick actually got a lot of shit for his ideas because it was opposite to what everyone else thought at the time, they thought stem cells were forever, and they’re not
Aging comes down to the fact that every time a cell divides, it has to make a copy of your chromosomes, and when it does that, the tips of your chromosomes get a little shorter because of the way the replication enzymes have to attach
You can think of it like the enzyme can only replicate what’s in front of it, and it has to attach to the end of the DNA by at least a few base pairs, right, so when it uses those few base pairs to attach and hold on, it can’t replicate them, so little by little, the chromosome gets shorter every time it’s replicated
We have a lot of extra DNA at the end of our chromosomes to account for this clipping, but once they get short enough that it would start to endanger actual genes and DNA we need, then the cell just refuses to go any further and won’t divide
It’s more complicated than that, but that’s basically the root of aging - cells that can’t divide anymore
There is an equivalent to HGPS in adults which is called Werner Syndrome
It involves a different gene, and this one is inheritable, since people frequently survive to adulthood and middle age, with average lifespan being in the 50s
And this one is more common in some areas than others, with about 80% of the known worldwide cases being in Japan, and another cluster on the island of Sardinia
In Werner Syndrome, there is a mutation in a gene that codes for a DNA helicase which is an enzyme that has a big job in guiding DNA repair, replication, and transcription in preparation for making proteins
There’s a science nerd pickup line that mentions DNA helicase, it goes, I wish I were DNA helicase so I could unzip your genes
You have a few different DNA helicases that guide replication and those processes, but this one in Werner Syndrome from what I read is mainly focused on DNA repair
Our regular DNA replication makes mistakes about one in every 100,000 nucleotides, most or all of which get repaired by the repair helicases
Sometimes these mistakes are critical and a cell can’t survive, or it gets repaired, or it doesn’t get repaired and doesn’t make a difference, or maybe the change proves more beneficial and the cell survives better
That’s basically evolution from first principles
Werner Syndrome was originally described in 1904 by Otto Werner, a German medical student who was treating a group of 4 siblings who all had premature aging
This is a recessive disorder, meaning you have to inherit the non-functional gene from both parents for there to be a problem
People who have this generally develop normally until they’re about to hit puberty, but then they don’t get their pre-pubertal growth spurt and remain smaller than their peers
That’s almost the only sign until the late 20s when they start to develop old people things like cataracts and so the lack of growth thing is really something that is only noticed in hindsight
When you hit your 30s, your skin begins to change and get thinner, have trouble healing, but also patches where it’s thicker and fibrous
The thickening part is called scleroderma, and it can affect some of your internal tissues as well, not just your epidermis, your outer skin
It can cause stiffness in the joints, poor blood flow to the fingers and toes, acid reflux disease, and a ton of other issues caused by thick skin and tissue where it shouldn’t be thick
Also in the early 30s, there will be typical middle age conditions, like an increase in Type 2 diabetes, osteoporosis which is brittle bones, atherosclerosis which is thickened and hardened arteries, an increase risk of cancer, and loss of fertility for everyone
This is also when you start to get thinning and greying hair and hair loss
That stiffness in the joints can apparently lead to atrophy of the Achilles tendon or tendons in the elbows, which can cause these limbs to require amputation, which, you know, I feel like that escalated quickly
Interestingly, dementia and loss of mental faculties that is more common with age are not things that people with Werner Syndrome or HGPS appear to have increased risk of
It seems that their minds tend to be perfectly normal for their chronological age
There are a few other reasons why a person might appear to exhibit the symptoms of premature aging and weight gain that Scott Calvin goes through
Sudden hair whitening is actually common enough in folklore that it’s been given the name Marie Antoinette Syndrome, because the former French queen was said to have had all her hair turn white on her way to her execution, and also Sir Thomas More, all his hair turned white the night before his execution for refusing to acknowledge Henry VIII as being more powerful than the Pope
You might also know him because he wrote Utopia, which, movie connection side note, was mentioned in Ever After, the cinderella movie with Drew Barrymore and Angelica Houston
An old timey French dermatologist in the 1800s said that emotional triggers can cause sudden immediate hair whitening
Quote ‘Paroxysms of rage, unexpected and unwelcome news, habitual headache, overindulgence in sexual appetite and anxiety have been known to blanch the hair prematurely’
I think that’s a debatable use of the word ‘known’ in that sentence, but who knows what we know today that will be laughed at in 100 years
Also in the late 1800s and early 1900s, there was a scientist called Elie Metchnikoff
He was and is a very famous immunologist, he’s the one who discovered that phagocytosis plays an important role in the innate immune system
Phagocytosis being where your white blood cells engulf or eat foreign bodies like bacteria to destroy them, it’s a very important process that keeps you healthy before infections can get out of control
He’s called the Father of Innate Immunity for a reason, he is foundational to early discoveries about the body’s innate defence systems.
I heard about him a lot during my undergrad, especially how he stabbed thorns into a see-through starfish larva and then watched the larva’s white blood cells try to eat the thorn
Bit cruel, but so was a lot of science in the 1900s and before
He figured that out and did all that so yay, but he did also hypothesize that the reason why hair turns white as we age is because of what he called pigmentophages, which he thought were phagocytes that gobbled up the pigment in your hair and then traveled down your hair strand to your skin and deposited the pigment there
Pigmentophages sound cute, but they of course don’t exist
Another guy called Landois in 1866 thought that hair went white because bubbles got trapped under the surface of the hair and altered the way light traveled through it
Not the craziest idea, this is what happens to egg whites when you whip them, or to sugar when candy making, but it doesn’t happen to hair
I’ve got a cool video from a candy maker that I follow on tiktok in the notes that shows this
In terms of explaining how hair could go white overnight, there is one paper that I will post that posits what I think is a perfectly reasonable actual explanation
And that is that permanent hair dye wasn’t invented until 1907
Before that, dark rinses or washable dyes were used, things apparently like chamomile, lead, silver, tea made from walnuts and walnut shells, that kind of stuff, things that would wash out right away
If someone has been sentenced to death and held in a cell, even a short time, they wouldn’t have access to their normal hair dyes which probably had to be applied every day
In the case of someone rich and famous like Marie Antoinette, they may even have been bathed and washed before their execution so they could meet their fate pure and innocent as a sacrifice or some bullshit like that
I’m not a historian so take that with a grain of salt, but the bit about permanent hair dye is true
Nothing magical or whimsical, no fear or sudden piety, just a lack of access to hair dye
Now, again for realsies, there are a few vitamin deficiencies that can over time cause an increase in white hairs
Namely, low ferritin which is the protein in your blood that stores iron, and low vitamin B12
So I need to keep taking my iron if I want to hold off on any more grey hairs I guess
A diet low in protein can cause you to not produce enough keratin which partly makes up hair follicles, and if the keratin in your hair breaks down, pigment loss could occur if there’s no fresh keratin to replace it
Being low in folic acid can cause hair, skin, and nail pigment changes…
So basically, it’s important to eat a balanced diet, or take multivitamins to make sure you’re not deficient in any of these things as they have a whole host of other effects that are important for living, not just having pigmented hair
[CLIP T=58:58, “What’s your diet like” “Milk and cookies, but I don’t finish all the milk”]
Another option is an autoimmune condition called vitiligo
In vitiligo, your T cells, which are supposed to target and kill cells that have been infected with a virus or bacteria, begin to target the melanocytes that give your skin its colour
It shows up as very stark light patches of the skin and hair and has been described as far back as 3500 years ago in Egypt
It affects about 1% of the world and it has no further health implications beyond just being white
People with vitiligo are otherwise healthy, they can’t spread it to people by touching them, but it can cause a lot of anxiety people are assholes and make mean comments
Unlike a lot of autoimmune conditions, vitiligo is now completely reversible with continued treatment for the inflammation, because your body produces new melanocytes to replace the ones that get destroyed, but treatment for most people is generally lifelong since it’s not a cure, it’s a treatment
But I don’t think people should have to get treatment for it, just stop being assholes to them because they have pigment loss
The reason I bring vitiligo up is because there has been a noted association of people with HIV that is progressing to AIDS, and an increase of vitiligo causing white patches on the skin and hair.
I am going to do HIV and AIDS I think next season, because there are many movies that have portrayed it, but that is not this episode so that’s all I’m gonna mention
I found it interesting, though, because of the association of vitiligo with a virus, something that is transmissible, while most of the time autoimmune conditions are not infectious
Not everyone who gets HIV is going to develop vitiligo since autoimmune conditions only develop in people with the genetic predisposition towards them, but HIV seems to trigger something that causes the autoimmunity to begin
Not only that, there’s another association, this time between people with vitiligo and with thyroid disorders, specifically there is an increased incidence of people with vitiligo having very mild hypothyroidism
Hypo meaning low, thyroidism being thyroid activity, and your thyroid basically controls your metabolism
You can have hyperthyroidism and be very skinny, gaunt, and have a very active metabolism, or you can have hypothyroidism and have very low thyroid activity and low metabolism
People with vitiligo are more likely to have hypothyroidism
One of the main symptoms of hypothyroidism is Un. Explained. Weight. Gain.
[CLIP, T=58:35, “I don’t know, Scott, you’re as healthy as a horse” “yeah, a clydesdale”]
So now I bring it all back together
SANTA, Siberian Arctic Northern Thyroid Autoimmunity, the disease that I have invented and that I posit is responsible for transmitting the qualities of being Santa from one Santa to another
Transmitted how? I suggest that it’s a retrovirus of some kind, one that doesn’t infect your white blood cells and destroy your immune system as in HIV, but one that brings about a sudden onset of vitiligo and hypothyroidism causing the hair to turn white and some jolly extra weight gain around the midsection
In order to extend Santa’s life because Santa could be 100s of years old, the virus probably brings along a few extra enzymes that help function in cellular repair and DNA replication, all to keep Santa actually young and vigorous inside while the vitiligo keeps his hair white and looking old outside
Oh, but it doesn’t end there folks, because there’s still one important aspect of being Santa that I haven’t mentioned
It’s Santa’s most important job, in fact, and that is knowing who has been naughty and who has been nice
[CLIP, T=34:20, “Bernard: he’s making a list, Charlie: Checking it twice! Elves: Gonna find out who's naughty or nice”]
Before I talk about that, I want to mention another thing that this could be
Rather than an infectious disease, it could be an infectious belief
Beliefs and ideas can spread through society just like infectious diseases, often a great deal faster and better
My partner actually brought this up when I mentioned that I wanted to do this episode
The widespread belief in Santa could be one of these things, which are otherwise known as an incidence of mass hysteria, or something else called Folie a Deux.
I am not a social scientist to talk about these with any great authority, but there have been a number of things, including unexplained diseases, throughout history that we have no idea exactly why they happened and which some people blame on mass hysteria
Some of these are the Salem Witch Trials - at times blamed on ergot poisoning and other times as mass hysteria, the Dancing plague of 1518 France, the Trembling Disease in 1905 Germany, a laughter epidemic that spread across villages in Tanzania in 1962… there are a lot, I’ve got links in the notes
Folie a deux is a psychiatric condition where delusional or manic beliefs, and even hallucinations, are shared by two individuals
There are other ‘folies’ that involve more people, including ‘folie a plusieurs’, meaning folly of several, so belief in Santa could be that, maybe
In fact, Ernest Saves Christmas exhibits this quite well
At the start of that movie, the original Santa is traveling to Florida so he can find his successor who he has already identified as Joe Carutthers, a children’s tv show host
Santa spends the whole movie trying to convince Joe that he’s now Santa, that he has to take over this job before Christmas Eve or Christmas will be ruined
Santa has to convince everyone else, too, because if people don’t believe in Santa, he doesn’t have any magic or power
You also see that in Elf
Santa is a social construct like countries, religion, time, or gender
They don’t exist if we don’t believe in them
At the end of Ernest Saves Christmas, Joe finally accepts that he is the new Santa and in that moment, he shakes Santa’s hand and transforms, everyone believes in him, and he magically has power to control the weather
As an autoimmune condition, SANTA wouldn’t be able to take effect immediately like this so I’ll say my theory still needs some research and testing, but like any hypothesis, you gotta start somewhere
Now, back to Santa’s ability to know who is naughty and who is nice
I recently read a book called How to Stop Time by Matt Haig
It is a delightful book about people that exist within our society that age one tenth as fast as average
I am going to say right here on the record that I think Matt Haig read about Progeria and Werner’s Syndrome, or found out about it somehow, saw that it’s about a condition that strikes just at puberty and ages people 10 times as fast, and thought, ‘what if that, but in reverse?’
The book follows Tom, a guy who appears to be in his 40s, but was actually born in 1581, and describes his experiences having performed with Shakespeare and known F. Scott Fitzgerald, but also spent his life trying not to become an experimental subject
In the book he even describes progeria as a syndrome, and has Tom name his condition as anageria, as the opposite of progeria
Tom meets another guy with the same syndrome, a dude named Hendrich, who is nearing a millenium old
Hendrich describes that after around the 6th or 7th century of life, people with anageria gain a certain enhanced sense of detecting people’s moral character
Hendrich says that when he meets someone he can instantly tell whether they’re a good person or if they’ve got bad ulterior motives
So I think that my hypothesis is sound, that SANTA the condition is a transmissible retrovirus that enhances DNA replication and repair by integrating into the host’s genome
This has the consequence of activating latent autoimmunity genes that cause vitiligo and hypothyroidism, and due to slow aging, gives the patient a heightened sense of perception about people’s moral character
It is only transmissible very rarely, likely under conditions of stress, which is a known factor in having flare-ups of autoimmune conditions, and holidays and Christmas time, and maybe having been in one’s job for several hundred years and wanting to find a replacement and retire
So as a man you’ve got to be careful in this and future holiday seasons, don’t put on any Santa suits you see lying about on Christmas Eve, and don’t shake hands with anyone trying to convince you that you’re Santa, unless you want to become Santa, that is, then by all means, knock yourself out!
MUSIC OUTRO
That is the end of this special holiday episode of Tierah Ruins Things with Science.
Thank you so much for listening and for being here
I am a researcher, not a doctor, so please don’t take any of this as medical advice, as particularly this episode is a little tongue-in-cheek and meant for entertainment only
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Music was written and provided by James Stratton-Crawley, who also helps with editing and occasional snarky comments, and there’s a link to his channels in the show notes
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See you all next season which isn’t too far away!