What is a “Dumbfuck?” Seriously, What is it?
Just so you know: This is a piece that STRONGLY OBJECTS to the use of the word “dumbfuck” (DF). The idea that we would use that word to describe others appals me no end — which is the point of this piece. The WS children I have tended to as a physician are a source of inspiration and happiness for me as an individual — if this guy can smile, hug and bring joy into the world like this, then certainly I can do better at that myself. My point in this piece is only this: WS children bring a robust gift to us in this life, they remind us that happiness and joy are states of mind, not the presence or absence of this or that material good or circumstance. WS children have a low IQ; so what? Their portfolio of cognitive abilities makes clear to anyone who meets them that IQ is just a number and the wild variation of abilities and perspectives we see in the world today is a gift, not something to disparage, distrust or dismiss. I want you to read this and think: “of course! Why would I use such language to describe someone different from me?”
This recent Facebook post made me wonder — other than provoking either a knowing laugh on the Left or indignance on the Right — what the hell does this mean?
Williams Syndrome (WS) occurs when the genetic code from chromosome 7 is spontaneously deleted. This happens to about 1 in every 10,000 live births. The deleted region includes more than 25 genes, including CLIP2, ELN, GTF2I, GTF2IRD1, and LIMK1 for those of you who are just deeply curious. These deletions lead to multiple problems: abnormally high calcium levels, abnormal blood vessel structures and low muscle tone with joint laxity.
The central nervous system of these patients is also affected. Abnormalities in connections between the frontal lobe and cerebellum impair their ability to decide what to do and then do it — especially with tasks that require complex and delicate movements. These kids also have different connections between parts of their parietal lobe. The lower part of the parietal lobe is essentially normal — preserving normal abilities to see and name things, such as faces. The upper part is not normal, so children with WS have trouble seeing how one large object might consist of many smaller parts or trying to get a good sense of how they fit into a room with lots of other objects.
These brain structure differences lead to measurable learning deficits — most WS kids have mild to severe learning impairment. They would have been called “retarded” back in the day. Their average IQ score is about 69, compared to the population average of 100. A score of over 140 qualifies you as a genius.
But, kids with WS are absolutely delightful to be around. They are friendly and very verbal and expressive. The “abnormalities” in the upper part of their parietal lobe decrease their social inhibition and we experience that as “never met a stranger.” Neuroimaging studies demonstrate that individuals with Williams syndrome have diminished amygdala reactivity in response to socially frightening stimuli like disapproving faces. They will treat someone looking at them with fear or disapproval with the same charm as someone who smiles and says “what up, dog?” Interestingly, a smiling face literally sets their amygdala on fire and so they warm up to approving faces at light speed.
People with WS has increased volume and activation of the left auditory cortex which is probably why these individuals have a strong sense of rhythm propensity and fondness for music. Similar sizes of the auditory cortex have been previously reported only in professional musicians
These children are delightful to be around. As a website dedicated to understanding this syndrome states:
Individuals with Williams syndrome have a very endearing personality. They have a unique strength in their expressive language skills, and are extremely polite. They are typically unafraid of strangers and show a greater interest in contact with adults than with their peers.
Here is a video of a WS kid. I have a few in my practice — they rock.