Erik Quinn: The Heart of a Family: Understanding

Saturday, July 15, 2006

Understanding

This is an interesting article that might provide a little insight as to what we are expecting in terms of future challenges. Erik will probably seem perfectly fine to the people around him most of the time. There is absolutely no doubt he is going to be a delightful person, but my desire is to bring a little understanding of what WS means to the handful of people who dimiss or minimize what we are going through by stating that Erik is perfectly "fine." I'm just going to come out and say it. The truth is that is he is NOT just fine. My son's diagnosis was absolutely devastating to both of our families. I had to actually mourn the death of the man I thought my son would be starting from the very first moment the ultrasound technician told us we were having a boy. It was as painful as any death in the family would have been. I now stare at children's clothing in stores emblazoned with "Football Star" and "Future President," knowing that my son will most likely never be these things. I am having to face the possibilities that our son will never drive, live on his own, or marry. We may never be grandparents because of this diagnosis. He may never even learn to tie his shoes. We do not focus solely on Erik's diagnosis, but it is important to us to acknowledge it because it affects how he sees the world. We generally do not treat him any differently than we would any other child. I am just asking for some quiet respect. Take a look around at some of the comments other mothers have left to read on this blog and take some understanding of what Williams syndrome means to our lives with you. Saying the words "birth defect," "disability," and "mental retardation" is not comfortable, but it is the reality we face daily. These words are hard to say, but they are simply words used by professionals to determine how best to help kids like Erik. Brian and I will not let these words put limits on what Erik is able to accomplish. He is ultimately the only one who has the power to determine what he is capable of, and he will show us in good time. However, please understand when a parent hears these words, all of their hopes and expectations change dramatically. All we can do is love our son, give him the tools to succeed in ways never thought possible, and hope to bring some understanding to those around us to make his journey easier.

From: University of Delaware (1998)
Williams Syndrome: New UD Study May Shed Light On Rare Genetic Disorder-And Normal Development

Children with Williams Syndrome are delightful and engaging, with elfin-like features and often-extraordinary verbal skills but severe spatial deficits, and a new University of Delaware study may reveal the cognitive impacts of the rare genetic disorder.

"Understanding the details of the cognitive profile in this syndrome will likely be extremely complex," says Barbara Landau, a professor of psychology and director of UD's Language and Cognition Laboratory. "But ultimately, it will shed light on how brain and cognitive development become compromised by small genetic defects. This, in turn, will enhance our understanding of how normal development occurs."

The musical and verbal skills of children with Williams Syndrome are extraordinary. But when they see a circle that is half red and half green, they are at a loss to replicate it. They may correctly select a red crayon and a green one, but their drawings will not even remotely resemble the original two-tone circle.

A recent report on "60 Minutes" described a similar grown-up who can sing nearly 2,000 songs memorized in more than 20 foreign languages, yet is unable to solve simple mathematical problems.

Such are the mystifying intellectual discrepancies of those diagnosed with Williams Syndrome. First recognized as a separate syndrome in 1961, it has only been in the last 30 years that persons with Williams have been recognized as a group with a unique cognitive profile.

In particular, individuals with Williams Syndrome have very large discrepancies across their cognitive abilities. One striking discrepancy is that between language and spatial skills: Their language is, in many ways, quite normal, but they show profound deficiencies in certain spatial skills. Landau, an expert in the field of spatial cognition, was intrigued by this riddle.

With her colleague James Hoffman, a professor of psychology, and her team of graduate and undergraduate researchers, Landau recently received a $59,208 grant from the National Office of the March of Dimes to study spatial language and spatial congnition in Williams Syndrome. Further funding from the National Institutes of Health and the National Science Foundation is pending, she says.

"We look at space and language and see what goes wrong," Landau explains. "We're looking at the relationship between the children's spatial abilities and language learning--how they talk about space."

It's quite possible, Landau says, "that you could carry on a conversation with a child with Williams Syndrome and not realize that anything is wrong. When they are just chatting, their normal interactions using language are very good. They are so personable, sweet and friendly--and so competent in many ways--that it often covers up the fact that they have a very uneven profile of cognitive abilities."

When tasks involving spatial relationships come into play, however, the limitations of Williams Syndrome become evident.

"When we ask the children to describe direction and motion, they have problems," Landau says. "For example, if a normal child watches a doll jump into a bowl (an animated video event), they might say, 'The doll jumped into the bowl.' But, when a Williams Syndrome child views the same event, they do not describe the event using the same complex verbs and prepositions. Instead, they might say, 'The doll went down.' This simplification may be due to faulty spatial perception (that is, they might not have perceived the event in the same way as the normal child), or it might be faulty language (that is, they might have difficulty learning rich spatial language)."

Most likely, Landau says, "it is some combination of the two. It might be hard to learn to talk about space if you have difficulty conceptualizing it."

Similarly, those with Williams Syndrome have difficulty describing the location of a dot, relative to a square. Explaining that the dot is above or below the square isn't easy for them, and they often make errors, unlike normally developing children.

And, while moving a mouse on a computer seems simple for children with Williams Syndrome, when they try to replicate block patterns on a computer screen they fail.

"This is very interesting, as it suggests that certain spatial skills (e.g. coordinating a mouse and a computer image) are intact, but that other skills (e.g. copying a pattern) are profoundly impaired," Laudau says.

"The children are very persistent and can tell you what they've done is not right, but they don't know exactly what's wrong," she added.

Another interesting contrast can be found by examining how the children search space for hidden objects. Although they are impaired when asked to copy patterns, they do not seem to become disoriented in space. When asked to find a coin hidden under one of several cups on a table, the children do so quite easily--even if they have moved from one place to another between the hiding event and the finding event.

The two activities "suggest real differences in the kinds of intellectual abilities that are compromised and the kinds that are spared," Landau says. "Obviously, not every spatial capacity shows a deficit.

"This is not just retardation, this is something unusual," Landau asserts. Landau and her research team have used some of the March of Dimes funding to purchase a special eye-tracker that records the children's eye movements as they perform spatial tasks. The tiny camera is hidden in a cap that the children wear, and this allows them to freely move their head, body and eyes. The purchase of this special piece of equipment also was supported by matching funds from UD.

The children in the study will wear the cap when trying to replicate the block patterns on the computer. Tracking their eye movements may yield some clue as to what is happening in their brains as they try and fail the simple exercises.

By understanding the nature of the spatial deficits, the researchers hope to understand what parts of language go uncompromised in Williams Syndrome. Such research could lead to a better understanding of how to educate people who have the syndrome.

Forty-five families who have children with Williams Syndrome, ages 7-14, responded to a letter Landau sent asking for volunteers. Names were provided by the Williams Syndrome Association in Connecticut.

Landau says the work is still in the preliminary stages, and will likely continue over a period of years.

4 Comments:

Blogger Lisa R said...

WOW! I think I am just going to post on my blog read your if you don't already. I never in my life would have expected to be able to relate to what you wrote. SURPRISE "life moves fast if you blink you may miss something". You are truly amazing at expression through writting. I was also wondering...are you a mind reader :)

The reading was great too. I wonder if there is a follow-up to the research in publication....

Love, Lisa

8:41 AM  
Blogger John A. Frye said...

This may seem very different from your perspective, but to me Erik seems to be growing so fast! I read every day, but this week I have barely been able to eat I've been so busy and now on the weekend catching up to read about Erik clapping for the first time and to see the new pictures of him- it is just so amazing to see since we saw you last. I hope Erik continues to suprise you.

1:34 PM  
Blogger Kerry said...

Oh my, Nance! I read your blog and couldn't comment... I was speechless. It was a great article and what you wrote was great too. I big dose of reality! As you know by now, I'm a bit of an optimist, so seeing the words you wrote and knowing they were true hit a nerve I suppose. Don't you know it's much for fun in the dark? haha Just kidding!! Something I'll work out... I was having a similiar conversation with my girlfriend the other day about Brady's future, but I think I gloss over has "can nots" and "won'ts".

I did come across a scrapbooking layout about a boy who has Down's syndrome and the mother's journaling went something like this: "We've reflected a lot on Joseph and his Down Syndrome since his birth (8th kid for this couple) we've come to one conclusion we believe with our hearts: Joseph's condition is a gift. For Joseph it means he will be free from so many cares in the world. He will live out his life with no guile, with no unkind thoughts. Our gift is that we have the joy of this little angel in our midst. It makes all of us want to be a little better, to reach a little higher, to love a little deeper, to be a little more grateful."

1:47 PM  
Anonymous Nancy GM said...

WOW ~ Nancy, you are truly amazing to me. Amazing Mom, Wife, Friend...an inspiration.
I'm pretty much addicted to reading whatever you write. I can hear your voice as I am reading it - makes me laugh ALOT and cry a little. I TOTALLY relate to your last statement - "All we can do is love our son, give him the tools to succeed in ways never thought possible, and hope to bring some understanding to those around us to make his journey easier." I feel that exact same way about parenting my two, beautiful daugters! I want them to discover their strengths (and understand their weaknesses) so they can succeed in ways never thought possible. And a parent I want to make their journey as smooth as possible by teaching them to be understanding of others as well as themselves.

6:22 PM  

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