Williams syndrome (also Williams-Beuren syndrome) is a rare genetic disorder, occurring in fewer than 1 in 7,500 live births. It is characterized by a distinctive, "elfin" facial appearance, along with a low nasal bridge; an unusually cheerful demeanor and ease with strangers, coupled with unpredictably occurring negative outbursts; mental retardation coupled with unusual (for persons who are diagnosed as mentally retarded) language skills; a love for music; and cardiovascular problems, such as supravalvular aortic stenosis and transient hypercalcaemia. The syndrome was first identified in 1961 by Dr. J. C. P. Williams of New Zealand.
The beautiful photo of my baby above was taken by my mother last weekend while Brian and I were away. Brian and I were talking last night, and we both seem to be on the same page regarding Erik's rapidly changing behavior and his frightening angry outbursts that seemed to begin overnight. For example, if I ask Erik to do almost anything at all, there is often a fierce battle that follows. Yesterday I was kicked in the stomach while I tried to brush his teeth and then later (twice) while changing his diaper. He looks directly into my eyes and almost smiles at me when he strikes at me and these outbursts occur. This behavior is very definitely not Erik-like, and I find it extremely upsetting. I have adopted a deliberately cool demeanor when it occurs and simply put him in his room for five minutes when it happens, but I'm not sure if this is the right thing to do or not. Yesterday when he was asked to find the car at the stables, he went face down in the parking lot and refused to move in front of everyone. Unfortunately, he becoming too heavy to carry very far! Luckily, most of that particular incident came out of being stubborn, not angry, and had a very normal (albeit frustrating) feel to it. His behavior became more aggressive after I carried him to the car. I worry about days when he suddenly seems very angry at me and the world without any warning at all. You may ask why I need to sort out what behaviors are related to my child's syndrome and what are of the normal 2-year-old variety. My answer to this question would be that I need to confirm that these outbursts are WS related because they are heartbreaking. I need to know my own child doesn't really hate my guts. I need to confirm that these are common WS issues that can be successfully dealt with and are not the result of something I did or didn't do. I need hope and reassurance. Pretty simple.
We agreed we need some new techniques and tools to help us cope with this worsening problem, and I found the book Understanding Williams Syndrome: Behavioral Patterns and Interventions by Eleanor Semel and Sue R. Rosner on line. Hopefully, it will answer most of our questions and promises solutions to behavioral problems common in children with WS. I'll post my input on it when I'm finished. The following is taken from what Ursula Bellugi had to say in the foreword in the book. That was the most compelling selling point for me, as I have great respect for the woman.
...the first comprehensive source book on the behavioral patterns of individuals with Williams syndrome. Not only does it summarize and analyze the research literature, it...provides problem-specific interventions, general guidelines for addressing problems...and innovative techniques for developing the potential of many individuals with Williams syndrome....combining research findings with real-life examples, clinical observations, and anecdotal reports...[it] goes beyond generalities by describing variation among individuals with the syndrome...as well as subgroups...[The authors]...are the ideal people to pull these strands together, both with respect to research and to intervention...
Cognitive Neuroscience Laboratory, The Salk Institute