Williams Syndrome / ウィリアムス症候群

YouTube: Where Everybody Wants to Be Your Friend

This documentary gives us an idea about what Williams Syndrome is.

Williams Syndrome is a genetic condition which affects one out of 7,500 newborns. It is a condition where about 25 genes out of 25,000 are deleted from the middle 7th chromosome.

Williams Syndrome children are known to be extremely friendly. They show a great affinity towards people when they meet them. They are also known to have hyper sensitive hearing. Most of them are terrified of loud noises. Some of them have music talent, just like people with autism.

One of the 20/20 documentary directors went to a special camp for Williams Syndrome children. Many of these children were very warm and open. They all wanted to talk to him. One child asked him, "Where are you from?" He replied that he was from New York. The children nearby said "Wow!" and smiled at him. Another child asked, "Do you have children?", and he answered, "Yes, I do - two daughters and a son." Then the children said "Wow" again, and clapped their hands.

In an interview with the children, an eight year old girl, named Ashlyne, told the documentary director that she wanted to help everyone who is sick or hurt.

Terry Monkaba, the executive director of the Williams Syndrome Association, described the camp, which she started in 1994. Her 24 year old son, Ben, who also has Williams Syndrome, is now a camp counselor there. She added that in this camp, children can get together to learn to have fun and experience togetherness.

The documentary director went to the office of Dr. Helen Tager-Flusberg, a Williams Syndrome specialist who has been studying Williams Syndrome at Boston University for 20 years. Dr. Tager-Flusberg told him about an experiment she has conducted.

In the experiment, a tester and a two year old child with Williams Syndrome were put in a room. The tester would pretend to hit her knee on a table and react by crouching. Most normal two year old children would respond by doing nothing and wouldn't get involved. The child with Williams Syndrome however tried to comfort the tester after the accident.

Dr. Tager-Flusberg said, "We try to understand what is the underlying cognitive brain mechanism involved that would explain the difference between having social interest and social indifference." She added, "We are inspired when we meet people with Williams Syndrome. They make you think about how you respond to other people."

At the end of the documentary, the director spoke to the camp director, Terry. He said, "I think the rest of us have a disability. We don't know how to be as kind and open as they are." Terry answered, "It would be great if the whole world were like those people"

ウィリアム症候群は、7,500人の新生児のうちたった1人が発症する、非常に稀な神経発達障害です。7番染色体の25,000の遺伝子のうち、約25の遺伝子が欠失することによって起こります。

ウィリアム症候群の子供たちは、とてもフレンドリーなことで知られています。見知らぬ人にも恐れることなく声を掛け、友達になろうとします。

このドキュメンタリーレポートは、20/20のディレクターがウィリアム症候群の子供たちの交流キャンプを取材した様子と、専門家のインタビューによって構成されています。

ウィリアム症候群の専門家は、遺伝子欠損によって人々が社交的になることのメカニズムを解明しようとしていますが、研究の過程でウィリアム症候群の人々に会うたび、彼女が普段、他人に対しどのように接しているかを考えさせられると話し、また、ドキュメンタリーの最後のシーンでは、ドキュメンタリーのディレクターが「（ウィリアム症候群の子供たちと接すると）僕たち健常者は、“他人に対して親切にできない”という障害を持っていると感じさせられる」と語っています。

友人と一緒にこのドキュメンタリーを見たのですが、7番染色体の欠失によって人々がフレンドリーになるということは、その遺伝子は「他人に対して警戒心」を持たせる遺伝子なのではないか、という話になりました。

私は「性善説」より「性悪説」の方がなんとなく世界の真理に近いと思っています。人は本来、利己的で、善の行為は後天的に学んで行うことができるのだと思うのです。

ウィリアム症候群の人々のようなフレンドリーさは現代では歓迎されるものですが、もしこれが古代（社会（警察）システムがない時代）だったら、ここまで警戒心を持たずに他人に接したら、あっという間に騙され、搾取されてしまいます。

自分を守るために、あらかじめ“警戒心”が遺伝子に組み込まれていて、それが7番染色体の中の25の遺伝子に関係しているのではないかと思いました。