"172. The Sad Condition of Happy Puppet Syndrome Presenter: Eliza Short Faculty Mentor: Elizabeth Ehrenfeld Department: Southern Maine Community College, Biology/Genetics Abstract: Angelman Syndrome is a neurological disorder that causes slight mental retardation, seizures, and impaired cognitive development. This disorder is most commonly diagnosed in children who show excessive and uncontrollable flailing or waving of their hands and arms. They often appear happy or excited even when sadness, frustration, or another emotional response would be more appropriate. This is why Angelman Syndrome was originally called “Happy Puppet Syndrome.” There is currently no treatment or cure for this disorder. However, it has been found that all these children show a mutation or deletion of a particular gene, Ube3a, located on the maternal copy of chromosome 15. The gene, Ube3a, codes for the enzyme ubiquitin ligase. Recent studies by Paul Greer have shown a correlation between Ube3a transcription and the formation of the ubiquitinating arc. This poster addresses how the deletion 34 thinkingmatters of this gene leads to the mental retardation state of the person. Understanding the role and function of the ubiquinating arc could improve the understanding of the patients’ neurological impairments."
A couple of years before she was born the Windscale accident occurred close by us. This is similar to your 3 mile island accident and although not quite a bad a Chernobyl, given our geographic location I have never been able to get this out of my mind.
We are called urgently now to her bedside - her traumatic life is almost over.
Best wishes, Briggy. I lost my sister to cancer. It was very difficult. And yes, radiation is linked to many bad things, particularly cancer and also mutations, though it's hard to show direct links sometimes.
And my sister was a downwinder, but in NW Colorado. People aren't aware that some of the 50s and 60s testing affected us, just like St. George.
Edited 2 time(s). Last edit at 12/26/2011 04:49PM by lostinutah.
With all the different isotopes released into the immediate area there is a chance that these two events may be related.
Of particular concern at the time was the radioactive isotope iodine-131, which may lead to cancer of the thyroid, and it has been estimated that the incident caused 240 additional cancer cases.
It's hard to know if the Windscale accident caused Angelman's in your sister, or the cancer, but it's possible.
I have found myself wondering about cause and effect regarding illness in the past. There's not anything that can change the outcome as it stands, so I try to leave it alone, especially if it is something that has happened far in the past. But I do understand that it can play over and over in your head and it's hard not to wonder.
Be comforted. People here care about you and yours.
The church however, have a totally different explanation - special spirits et al. That's how the church got their claws into mum. We all know what they really think though don't we?
It was tough growing up with all that stuff going on - we had to dish out a bloody nose or two as a result of insensitive comments but we love them both dearly and don't think it is fair - there is no god.
Briggy, my older brother had muscular dystrophy and died when he was 17. Having someone in your family who isn't "normal" definitely changes things. And when it's caused by human stupidity, as in a nuclear accident, then you know for sure there's nobody up there looking out for you or yours.
I am so saddened to hear you are going through such tragic times. It seems you have had no time to go through the grief process from Greek Islander. Please know, you are in my heart.
My ex-husband's dad was also a downwinder who eventually died of cancer, but the type of cancer he had could also have been caused by him being a heavy smoker as well. He died a few years before I met my ex, so I never met him.
Damn... I had no idea Selafield... They still have a mox plant on the site and greenpeace had someone in there a few years ago that found some scary stuff.
I've seen alot of info on it under the name of selafield rather than windscale but from what i've seen oh yes quite easely it could have caused both problems.
The mother walking out into a garden contaminated with radioactive dust on the ground and in the soil is enough to give her a birth defect and as for the cancer that could be a single radioactive particle that could have gone though the mother from her eating it or could have been from food she ate.
The really nastsy bit is that down the genorations the damage gets worse. We still may not know the full extent of the genetic damage from that desaster.
If there was an environmental cause, I would look more at the Nuclear Testing of the 50s and 60s as a more likely suspect.
I will also warn you, that it is easy to want to blame environmental factors for genetic ones. I am not saying this is a genetic condition, but don't rule out the possibility until you know for sure. It's kind of like the whole vaccines cause Autism crowd. In that particular case, it has been overwhelmingly shown that autism is caused by genes, and that the one test that showed a possible correlation with vaccines was seriously flawed. However, we tend to internalize guilt when we should not, and so people feel guilty that it is their genes that "did this" to their child. If that is the case, don't feel guilty, because it wasn't your fault, anymore then it was your fault that you choose to live in an area that was contaminated. (You probably did not know that was a risk either) Just do what you can to help the sick, and take precautions for future children.