Living with Williams Syndrome, the 'opposite of autism' | Hacker News Reader

Living with Williams Syndrome, the 'opposite of autism'

bbc.co.uk

153 pointsedward65 comments

Its funny that this was just posted, because I was just at my local grocery store. This store hires handicapped people to act as baggers - so I always make sure to spend an extra moment after checkout thanking them and just generally trying to expand the goodness in the world.

Funny enough, the bagger today had such a "handicap" as depicted here. He was super outgoing, with a real infectious sense of interaction. He said to me completely sincerely, "have you been keeping your energy up and yourself positive?!?" and I stammered a bit and said I was sure trying.

Then he said, "you know, each day I try to appreciate how beautiful the sun is, it really can help you keep your energy up! So keep yours up up up! That way you can Rock On!"

There's not much you can say to that but to give him a high-five.

Having said that, I was thinking on the walk to the car how often his parents must worry about him. Precisely because you could tell he was so outgoing he would've been open to any suggestion "want to take a ride? want to go see the lake? etc etc"

Nonetheless, it feels strange calling it a "handicap", he still bringing a smile to face just thinking about how generally excited he was.

>it feels strange calling it a "handicap"

I'm not sure why. If he could perform a better job, he wouldn't have been a grocery store bagger. That he has found himself there strongly suggests that his "handicap" affects his life in such a way as we might expect.

Some people don't want to do "jobs" per se. I know I'm interested in development because it's something I like doing, and I have an unfortunate liking of expensive things. Some people are genuinely content with the company of other people, and only want to work so they can spend the rest of their time with other people, and making their days better. Would you say that someone who is a checkout operator in a store because they need money to be able to do the things that they want to do (go on holidays, or go to the beach with friends and family) has a handicap because they find themselves in what you consider a menial job? I certainly wouldn't. Sure there are a lot of people who would rather be making more money, but money isn't everything for some people.

Yea, I've seen guys literally work themself's to death. They finally get the wife, buy the always buoyed yacht; and die at 55. The wife gets over the death pretty quickly because she really never knew the work horse, with all the brilliant ways to get ahead. "He was a hard worker, and had so much ambition---too bad he passed." I guess there's a happy medium to work? I'm not sure what's considered menial these days? I heard people on this site refer to Plumbers and Mechanics as examples of menial occupations, while I bet most make more money than the average computer programmer. We are in another tech bubble. I saw this in the nineties. Guy's who threw around the word menial, and thought their cushy, exciting computer related job would always be in demand. Well, the crash hit hard. They went through their savings. Didn't take a menial job, and by the time the industry started to bubble up again they were just a little to old, or rusty. They never laughed about the menial workers of the world again. The party was over. There were no more alcohol fueled pub crawls--debating nuances of programming languages. There life just stopped. It's kind of sad this industry is so youth oriented, but then again the barriers of entry to this field are not that steep--look at Jennifer. She's a Coder!

Or perhaps he is capable of "better" jobs but faces stigma and discrimination because of his disability?

How do you define better?

I'm currently unemployed. Does that mean that if I could get a job, I'd have one right now?

I think challenged may be a better description? What I appreciate about grocery stores hiring challenged workers to bag groceries is it makes me slow down. On other occasions I might help bag them myself, but I completely change my demeanor when someone challenged is helping me and I do engage in conversation. I also have a brother who is neurologically impaired.

> If he could perform a better job, he wouldn't have been a grocery store bagger.

Or maybe he's enough at peace with himself that he doesn't mind being a grocery store bagger.

It's kind of telling that we look at jobs that are basically fundamental to the smooth operation of society (maybe not baggers specifically, but certainly clerks and stockers in the same store) and assume that the people doing them must be miserable and pitiable. What does that say about us, that we see the foundation-stones of our system as pathetic losers?

Interesting that you are getting a negative reaction here. I want to float this controversial statement with the HN community:

Downs syndrome is a mental handicap and preventing the disease should be a goal in science.

Down's is already 'prevented' in many cases, as the statistics for abortion of Down's fetuses detected in utero show. Many with Down's can live rather full lives, as some have rather mild differences in mental state than neurotypical peers. Many participate in school without the need for extensive supports, and function in work situations. There is a bit of a range in the abilities of those with Down's.

There are other diseases that can be managed in a way that minimizes the impact on quality of life. It's still preferable to prevent them.

He got downvoted for implying that only a mentally handicapped person would ever be a grocery store bagger. I used to work front line at Kroger over summer break, I know some pretty great people who still make a living that way, and I don't appreciate the insult.

When I retire at 45, I plan to work as a bagger at a whole foods, just for something interesting to do.

I got the idea from a bagger I met at my local whole foods. He was previously a developer and made a ton of money and retired early. So, he thought it was a fun gig.

Just something to think about next time you look down at the bagger. They may actually have more money in the bank than you. I know - the odds are low, but it just could be.

Is this a thing? An old neighbor was in a similar situation...

I have no idea why you're being downvoted. While it isn't my idea of an ideal retirement job, if that's what strikes your fancy then why is that a bad thing?

It's as if some people reject the possibility that one could be happy without a mansion with a 3-car garage stocked with Audis and BMWs and annual trips to the tropics on a personal yacht.

If you read the wiki article [0] they love meeting new people, but have problems forming strong relationships.

[0] http://en.wikipedia.org/wiki/Williams_syndrome

I would think that's a really hard thing to pin down with numbers. I'm guessing if my wife of 17 years is any indication, it is rather difficult for people to get past a certain point because of the high energy and then anxiety that comes up. But there will always be a core group of friends that will stick through the ups and downs because of how empathic and caring the person is.

This may not pertain directly to your story, but I feel the need to explain something, as a physically impaired individual.

We, as individuals , live with certain impairments.

These impairments only handicap us when we are put into a situation where our impairment serves to hinder us in some way.

A disability occurs when our handicaps , which were caused directly by our impairments serve to effectively eliminate an ability of ours within a given environment.

Please try your best to keep the wording straight, it can end up being contextually important.

An easy way to remember : A handicap is basically the same concept as it is applied to sports, negative points which serve to reduce the chances of conquering a goal.

Here's a helpful resource : http://www.pediatrics.emory.edu/divisions/neonatology/dpc/Im...

As a handicapped individual, I hate all this stupid word play. I'm a cripple. Unless calling me something different is going to somehow make me run, climb, or dance again, then it really doesn't matter how you refer to me. Call me whatever you want, but don't refer to me by any of this flowery language, I find that far more offensive.

Most neurological conditions are far less clear cut than physical disabilities. At some point there's a very grey area between what constitutes a 'disorder' and what is deemed to be merely an aspect of one's personality. In the end, some imbalances of neurotransmitters or differences in brain development have DSM criteria and others are written off as individual quirks.

Obviously I respect that you'd like to be referred to matter-of-factly but 'disabled' or whatever is a wider demographic than just the wheelchair-bound.

I'm the father of a child with autism and I agree with your sentiment. It's context and emphasis rather than just words that tell you whether someone is trying to be offensive. And trying to find offense where none is intended is asinine. On the other hand, being ignorant of the fact the some people find certain terms offensive is also fairly poor.

The above two comments, taken together, indicate SO MUCH. For example, they show why feminism is so problematic:

Members of the same identified group do not by necessity agree on how they wish to be treated. There is no monolithic, "correct" feminism and there is no monolithic, "correct" "handicapped person."

Brilliant. Thank you for not just sitting there and keep quiet, tensenki. Good luck with your coding, etc.

I think you're on to something there.

From my observations, feminism seems to place the woman as "damaged" or "in a state of victimhood". That viewpoint seems.. wrong. Why would I see myself as lesser and try to lower others around me so I look better?

Reading about this disorder reminds me of my sister. She has Down's Syndrome and is one of my favorite people in the world. She has basically the same strengths and weaknesses as WS -- open and friendly when not anxious, but unable to determine when it is appropriate to trust someone. It's been a blessing having her in my life. She's the linchpin of our family in many ways. I'd probably be more of a black sheep in my family's eyes if it wasn't for always being so excited to see me regardless of conflicting religious beliefs with my family, etc.

The brain is an amazing instrument and it's fascinating (and scary) how it can be wired so differently in edge cases, but deep down, no matter the abnormality, there's a "soul" waiting to connect if you can meet them on their terms.

A good friend of mine has a brother with Down Syndrom...

1 Evening, he said that he would take his brother home and then we would meet. Saying that it is okay to bring along his brother to a local bar was one of the most "rewarding" moment i ever felt...

Btw. The boy with down syndrome has a job, lives alone and even sings in french in a quior.. :-) . He is alsof very social (talks to everyone in the train, but i think he trusts people to easily, he made paintings every year for our student club because we asked him every year if he wanted to go swimming with us when we rented the pool for 30-40 people in the evening, yearly tradition ).

You could really see his joy every time... Its awesome to see that a small gesture can have such a big impact on someone

fyi, its choir or quire, not quior :)

Singing in a quire would be interesting (a small book or pamphlet; a unit of 24 or 25 sheets of paper)

When I first saw this headline I thought oh no, have we created another "syndrome"? But after reading it I realize this is probably what my wife has.

in the last 17 years I've noticed all of these traits over and over. She's extremely nice, friendly and approachable to the point where she's not only vulnerable but people have said it's "annoying". She is constantly reaching out, talking and being extremely friendly with people everywhere despite having HUGE issues with anxiety.

She has some learning disabilities and issues and works in healthcare where she is in a unique position because of her personality. She's unable to learn or retain certain things beyond a base knowledge developed over the last 10 years or so. She struggles with charts and paperwork and hasn't been able to move up beyond a certain level. She rarely makes mistakes but also doesn't take any risks with patients and doesn't do a lot of critical care.

But the reason she hasn't been fired is quite simply because there would probably be a revolt where she works. She is absolutely LOVED by the residents of the facility, her coworkers and even management. She's a "ray of light" that comes in and cheers everyone up no matter what and I honestly think if it came down to it they'd pay her just to come in talk to people if they had to.

It's one of the things I fell in love with so long ago that makes the downsides (frequent anxiety, OCD, over engagement etc) so much much worth it. It's very hard to be in a bad mood around her.

I'm glad to have found this article and it even inspired me to create an account just to post about this. It was eerie reading this article describing my wife... even the "buzzing bee" thing. Absolutely intolerable to her. Clicks, noises, things like that create great anxiety. But get her into a crowd of people and she'll be talking, relating and stealing the show.

I'm definitely going to do some more research into this.

Williams syndrome is a CHROMOSOMAL disorder which has PROFOUND effects, not just on personality but on physical appearance. It isn't all that common.

Please don't diagnose your wife with chromosomal disorders based on her being friendly and anxious and bad at charts. Perhaps she has a learning disability, like tons of other people.

It is a spectrum disorder where individuals with small deletions can be regarded as normal. Fibetera's wife may well have a mild version - the only way to know is to do a DNA test.

I would never diagnose her with anything, I am a software developer not a doctor. It just points me in the way of some research for possible things we could talk to a doctor about.

A close friend of mine has Turner's syndrome, also a chromosomal disorder with potentially severe physical effects (such as "neck webbing").

Fortunately for her, she doesn't exhibit the gross physical abnormalities. So while I do endorse the basic sentiment of your comment, you're not on 100% solid ground.

Is she a music fan? Unusually good at pitch, rhythm, instruments, or memory for music and lyrics?

Williams Syndrome is associated with unusual aptitude for music (or perhaps just unusual compared to the other more serious learning difficulties). There are even music-focused summer camps specifically for kids and adults with Williams Syndrome.

http://www.sciencedaily.com/releases/2006/10/061003191006.ht...

Interesting. AFAIK musical ability is somehow linked with mathematical ability, yet Williams Syndrome involves mathematical impairment..?

These aren't necessarily in conflict. Imagine a system,

    x = A + B
    y = A - B

If, typically, we see large ranges of A and small ranges of B, we'd say x and y tend to move together. But that doesn't preclude someone with a highly unusual B.

> When I first saw this headline I thought oh no, have we created another "syndrome"? But after reading it I realize this is probably what my wife has.

The first sentence seems to convey an annoyance with people's propensity to hastily self-diagnose/make amateur diagnosis. And then you do the same thing in the next sentence...

Fun fact: people with Williams Syndrome lack inborn racism responses -- but still make judgements based on sex.

http://www.scientificamerican.com/podcast/episode/williams-s...

"Inborn racism responses"?

Babies don't immediately start with a different reaction to different kinds of faces, but within a few months, they react differently to people who look more (or less) like their mothers. It's not like there's a clear genetic dividing line, it's just how similar people look.

Link: https://www.umass.edu/newsoffice/article/umass-amherst-psych...

This is the original paper [0].

[0] http://www.sciencedirect.com/science/article/pii/S0960982210...

It's amazing how we can identify the exact brain disorder responsible, directly or indirectly, for most British comedy of the early 20th century.

The wiki article on this syndrome is very interesting. Apparently there is a substantial number of people with this genetic deletion that are not diagnosed as they are cognitively normal. There does not seem to be a term for such 'mild' Williams syndrome like there is for autism.

There's a blurry line between 'diagnosed' and 'what kind of person you are' which is increasingly being encroached upon by modern medicine. Certainly there are lots of ways a person can be classified which only become a disorder when they go above a certain magnitude.

Progress will be made into better classifying people so that more and more regular folks can take advantage of strategies and 'treatments' to have a more successful life (whatever that may be). With this comes the risk of discrimination relying too heavily on these classifications, which, though real, will never be fair to judge a person against.

This is certainly true - we are all carrying various mutations that influence who we are. For example, I am now sitting in front of my computer drinking a chocolate milk - something I can only do as an adult because I have a mutated version of the lactase gene that causes lactase to be expressed in adulthood (lactase persistent syndrome). Until agriculture was invented this "syndrome" had negative value since unless I am drinking milk it is a waste of resources for my body to make an enzyme that won't be used.

As we learn more about each persons genome I hope we stop categorising people into people with a "syndrome" and those without.

This is awesome, I have never seen Williams Syndrome mentioned in the news before. My younger cousin has it, though, and it's interesting to hear some of the common symptoms.

She's an amazing person, but absolutely a handful! More recently, things have become awkward as she is coming of that age, and while interested in the opposite sex doesn't quite grasp those social boundaries of what is and is not acceptable.

Her personality, like the article mentions, is totally infectious. She loves music and friends and parties, and wants everyone to just be happy!

Thanks for linking this!

It seems almost as if we are afforded a certain amount of "intelligence" and it is split between "emotional" and "cognitive". Those who exhibit high ability in one seem to suffer from not enough of the other.

I really like these sorts of 'zoom out' comments, because they tend to show the similarity between disparate systems and the cross-domain value inherant in systems thinking. (Then again, HN is probably a bit of an echo-chamber for this!)

This paper [0] is really interesting from the perspective of looking at Williams syndrome as the opposite of autism. The authors found that an individual with a duplication of the Williams syndrome deletion region that was autistic. Basically they found that not enough of the genes in this region (i.e deletion) then you end up with Williams syndrome, too much and you get autism.

Autism is genetically more complex than Williams syndrome as there are many other genes that can cause autism.

[0]http://link.springer.com/article/10.1007/s10803-011-1389-4

It would be really interesting to see which genes in the section of chromosome 7 that is deleted in Williams syndrome have been under recent strong selection in humans.

We know so little of how the brain actually operates, it not surprising that simple changes in brain anatomy or chemistry can produce enormous variations in behavior. I wonder if we will ever be able to be able to correct or at least change the brain without making things worse.

Well, this sure is one neurological/chromosomal condition every hacker is immune to.

Link to reddit discussion:

http://www.reddit.com/r/science/comments/22yed5/living_with_...

Is this different from Histrionic Personality Disorder?

Does not sound at all like the opposite of autism.

The title of the article is very annoying. "Pear: The opposite of apple"

Maybe there's a spectrum, and marketing/sales folks tend to be a bit towards this pole?

Hodor.

Pinkie Pie disease?

You're not the first to suspect that Maud and Pinkie Pie were intended to represent Aspergers and Williams syndrome.

I think there are traces of a connection between dogs and williams syndrome: http://www.nytimes.com/2010/03/18/science/18dogs.html?pagewa...

Interesting, again because of the parallels to autism. My son's therapist recommended a book called All Cats Have Aspergers[0] as a way to help him relate to how he sees the world.

http://www.amazon.com/All-Cats-Have-Asperger-Syndrome/dp/184...

Hmmm, interesting.. Never heard of this before..

> People with WS are empathetic, social, friendly and endearing but they tend to have a low IQ, making tasks such as counting money difficult.

IQ is what a standardized IQ test measures. And there is no way such a person can effectively attend a standard IQ test - which is why this statement is pointless by definition.

And to connect this "low IQ" with having problems counting money is funny in multiple ways! First of all - counting anything is not what I would consider needing intelligence. Usually the opposite is correct - pertaining a boring task as counting is usually impaired by high intelligence.

And second of all - isn't the feat of "counting money" the root of most of mankind's biggest problems? Wouldn't it be more intelligent in fact trying to foster some of the conditions coming with this "disorder"?

Like approaching people open and friendly disregarding their dull and dismissive facial expressions and aura - tired from counting money very well all day long?

> And second of all - isn't the feat of "counting money" the root of most of mankind's biggest problems?

This, and long-range planning. No mass-scale killings would've ever happened if it wasn't for the human ability of long-range planning.

Out with the long-range planning!

And second of all - isn't the feat of "counting money" the root of most of mankind's biggest problems?

The answer is no, in case you are wondering.

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