In this issue:
Awareness Month 2017
It's a Wrap! May's formal campaign is complete, but the need for awareness continues.
We have "officially" wrapped up 2017's awareness month campaign, and it was successful in every way - 35+ events nationwide; 11,000+ participants; more than $500,000 was raised for WSA programming and family support; local media attention... As parents, relatives and friends of someone with Williams syndrome, we are all extremely grateful for the individual and group efforts that are necessary to make each awareness month campaign successful - Thank you! Your creativity and generosity as you worked to donate, spread the word, attend or plan an event, design shirts, bake specialized cookies and cakes, create photo frames, bows and headbands, poster boards and videos...the list goes on, and the effect was amazing. Please be assured that whatever you did was recognized and appreciated by each of us.
Awareness month would not happen without event coordinators and their committees. Special thanks to our coordinators and to each and every person who sold tickets, set up tables, provided entertainment, promoted events, helped with food, cleaned up and much more. Nearly 8,000 of you were able to join us at the events - thank you for forming teams, gathering friends and attending the walks, golf outings, family fun days, baseball games and more.
The events are only one portion of our awareness campaign. Some of you shared our daily posts on the traits of WS. Many of you educated your networks, reaching people with compelling personal stories. Others donned the t-shirts and answered questions when people asked "What is Williams syndrome"? Some of you spread the word about Williams syndrome at your work place and involved your coworkers, or provided an opportunity for local retail volunteers to help with a project and learn about Williams syndrome. A large group of "Kohl's Cares" volunteers created wonderful note cards, and Kohl's provided a donation in honor of their employee volunteers.
Some of you worked to get your city or state to proclaim May as WS awareness month. Others were interviewed for radio, television and print news stories in markets big and small, sharing your family stories and promoting the walks. There was wonderful art work created, with artists selling and donating a portion of the proceeds to the WSA. Creative graphics were designed and shared with the community for people to use for their social media posts. One grandmother ran three miles each day for the month of May and encouraged her friends/coworkers to be involved with pledges to raise money and awareness. The generosity was remarkable, and with each activity, and many more learned about Williams syndrome.
And then there was our shared mission and efforts to uphold the legacy of increased awareness for Jonathan Martinez and honor the Martinez family. Thank you to all who made boards, posters and other visuals to honor Jonathan at the walks, and for the many special messages of condolence and support that were sent to his parents.
There are so many things to be gained from our awareness month activities. I'm sure that each of you has a favorite aspect. For me, the best part of awareness month is the camaraderie - the nationwide (even worldwide!) feeling of overwhelming support and celebration. Williams syndrome presents each of our children (and us!) with a unique mix of challenges and gifts - we talk about them all the time. For many of us, the challenges always seem to be at the forefront of our lives as we attend appointments, fight for services and deal with the frustrations of not having all the answers about Williams syndrome that we want and need. But during the events of Awareness month, as we create teams in the name of our sons and daughters, we take a moment to focus primarily on the "good stuff." We join together to celebrate our kids with Williams syndrome and the tremendous love we all have for them is so evident on the team posters, and the special team shirts and the smiles of every individual's "teammate" as they gather together. I love that, and I hope you do too. We need to do that more often - not just during the month of May! In my view, it is through celebrating our children's gifts that we gain the strength and the dedication needed to wage the battles against the challenges of Williams syndrome.
There is a broader impact as well. If we take every opportunity we have to share our kids with the world, door will begin to open - our kids will work the magic with their special personalities and then we will have the opportunity to follow up with all the other characteristics - both celebratory and challenging (even devastating!) of Williams syndrome. Our story will be told! Our efforts over the last several years have made an impact - gradually, awareness outside of our own communities has been growing. That is great but it's not good enough - it is only through year-round efforts that we will be able to pick up the pace and gather the national support we need to help us find the answers!
Intellectual Assessment of Students with Williams syndrome: Update for 2017
By Carolyn Mervis, Ph.D., University of Louisville.
Different tests provide different information, and some tests are better than others for documenting the intellectual strengths and weaknesses of children with Williams syndrome (WS).
Several tests that are commonly used when assessing children with WS are described below. The "right" test will vary for students with WS at different ages, and the recommended tests for students with WS may be different from the tests that are typically given by school psychologists. Therefore it is very important for families to ask what tests will be given and provide school districts with the following information to help ensure that your child's team has the best possible information.
A Quick Primer on Intellectual Assessment Test Scores Intellectual ability tests measure the abilities of an individual child relative to the abilities of children in the general population. (For well-normed tests intended for children in the United States, this means that the group of children used to norm the assessment was matched to the U.S. census proportions in terms of characteristics such as race, ethnicity, parents' level of education, rural vs. suburban vs. urban residence, and geographical region of the U.S. in which the child lived. Children with disabilities are usually represented in the norming sample in the same proportions as for children in the U.S. population.) In order to compare an individual child's performance on a test to the test's norms (that is, relative to children in the general population), standard scores (with an average score of 100 and a standard deviation of 15), T-scores (with an average score of 50 and a standard deviation of 10), and/or scaled scores (with an average score of 10 and a standard deviation of 3) are used.
All of the tests that are discussed below provide an overall IQ score that is expressed as a standard score. However, performance on the separate components of the test is often expressed as either a T-score or a scaled score. The likelihood that a particular test will accurately capture the abilities of a child with a disability is related in part to what the lowest possible score on each subtest is. For example, a test that reports T-scores for the subtests will be better able to capture the performance of a child who has significant difficulty in the area the subtest measures if the lowest possible T-score is 10 than if the lowest possible Tscore is 20. If the lowest possible T score is 20, then this score will be assigned to everyone who would have earned T-scores of 10 - 19, in addition to children who would have earned a T-score of 20. At the present time, the lowest scaled score on tests of intellectual ability that use this type of measure rather than T-scores is 1, which corresponds to a T-score of 20. Why does it matter what the lowest possible score on a subtest is? First, it may affect the services that a child is offered. A T-score of 20 corresponds to mildmoderate disability, whereas a T-score of 10 corresponds to moderate-severe disability. Children with more severe disabilities are often provided more intensive services than children with mild disabilities. Second, if T-scores as low as 10 are available on a test, then it is more likely that an individual child's pattern of strengths and weaknesses will be made clear than if the lowest possible T score is 20.
For whom is this likely to matter? In general, this will matter most for children with WS who have a moderate or severe disability. It will also matter for many children who have a mild disability or are in the borderline range (the range between mild disability and low average ability), especially if they have a clear area of weakness (most likely visuospatial construction). It is less likely to matter for children whose intellectual abilities are in the low average or average range. Sometimes parents are given the results of standardized assessments in the form of age equivalents (e.g., "5 years 6 months"). These scores appear to offer a lot of information, but in fact they are quite problematic, for several reasons.
First, age equivalent scores do not mean what they appear to mean. That is, you should not automatically consider your child to be functioning at the age that corresponds to the age equivalent score.
Second, age equivalent scores do not tell parents whether or not their child's performance is in the range expected for children his or her age. A given child's age equivalent score indicates the median (middle) age at which the children in the group used to norm the test obtained the same number of points as that child did. Depending on the particular test or subtest, a very wide range of age equivalents may be within the average range for that child's chronological age. For example, on a well-regarded academic achievement test, age equivalents between 7 years 8 months and 11 years 0 months are considered to be in the average range for a child aged 9 years 0 months on the subtest measuring ability to read English words. For a child aged 12 years 0 months given the same subtest, age equivalents between 9 years 4 months and >19 years 11 months (the highest possible age equivalent on this test) would be considered to be in the average range.
Third, age equivalent scores cannot be compared across different tests or even across subtests included in the same test. That is, if your child received a higher age equivalent score on one subtest than on another, that does not necessarily indicate that he or she actually performed better on the subtest with the higher age equivalent score than on the subtest with the lower age equivalent score.
The only accurate way to compare a child's ability on one subtest or test to his or her ability on a different subtest or test is to compare standard scores, T-scores, scaled scores, or percentile scores. Assessments for Children - Infant through Preschool In the very early years, the Mullen Scales of Early Learning (MSEL) is a good choice. It is normed for children from 1 month to 5 years 8 months. For children with WS, it is best used for 18 - 48-month-olds. Advantages for children with WS: The MSEL yields scores for 4 separate ability areas (nonverbal reasoning - called Visual Reception on the MSEL - receptive language, expressive language, and visuospatial construction - called Fine Motor on the MSEL).
For this reason, the MSEL can show a child's patterns of strength and weakness, rather than simply providing a single score averaging different types of abilities together. Children with WS often have the most difficulty with visuospatial construction, and the MSEL Fine Motor scale highlights this need area. Goals for the OT and classroom teacher will often come to light as well as challenges that will have to be overcome. Disadvantages: The MSEL norms do not have a large enough range to show differences for children who are having considerable difficulty in a particular ability area or overall. This is because the lowest possible Tscore on each scale is 20. A very large group of children with WS earn the lowest possible scaled score (T-score), referred to as the "floor," on one or more of the four scales. For children who are having considerable difficulty in more than one ability area, relative strengths and weaknesses will be camouflaged. Note that this problem occurs with all tests that are currently available for toddlers and young preschoolers. An additional problem with the MSEL is that small differences in the age of the child at the time he or she is tested can create artificially large differences in scores.
Assessments for Preschool and School Age Children For children aged 4 - 17 years, the Differential Ability Scales-II (DAS-II) is an excellent test. There are two versions of the DAS-II, the Early Years version for ages 2 ½ years to 8 years 11 months (not recommended for children with WS who are younger than 4 years), and the School Age version for children 5 years to 17 years 11 months. The Early Years version should be used for children with WS aged 4 years - 8 years 11 months. Extended norms are also available for older children to allow for the use of the Early Years version for children aged 9 - 17 years who have moderate or severe disabilities. Advantages for children with WS: The lowest T-score available for the DAS-II is 10, which means that the DASII is able to accurately characterized the abilities of children with WS even if they have a severe disability. Therefore, the uneven pattern of strengths and weaknesses typical for children with WS can be clearly identified by comparing standard scores for the 3 core clusters of this test - the Verbal Reasoning, the Nonverbal Reasoning and the Spatial clusters.
In addition, three diagnostic clusters that are not included in the IQ score also are available: School Readiness (for ages 5 years 0 months - 8 years 11 months), Working Memory (recommended for ages 7 years 0 months - 17 years 11 months), and Processing Speed (also recommended for ages 7 years 0 months - 17 years 11 months). Determining an IQ score from the DAS-II The DAS-II GCA, which is like an IQ score, is meaningful if the 3 core cluster scores do not differ significantly. However, this is the case for < 10% of children with WS. About 90% of children with WS show a clear pattern of higher scores on the Verbal cluster and/or the Nonverbal Reasoning cluster but substantially lower scores on the Spatial cluster. Only 2% showed substantially higher scores on the Spatial cluster than the Verbal cluster. When children's cluster standard scores are uneven (differ significantly), which is true for about 90% of children with WS, a single score such as an IQ or GCA is not the best estimate of their intellectual abilities. Instead, each cluster standard score should be considered separately.
Other Commonly Used Tests School psychologists and private psychologists usually use Wechsler tests to measure children's intellectual abilities. The most commonly used versions are described below. Wechsler Preschool & Primary Scale of Intelligence (WPPSI-IV) The WPPSI-IV is normed for ages 2 years 6 months - 7 years 7 months (not recommended for children less than 4 years old who have developmental delay). It is completely redesigned and is more similar to the DAS-II than the WPPSI-III was. In particular, instead of a single Index that includes both spatial and nonverbal reasoning subtests, the WPPSI-IV has a Visual Spatial Index (similar to the DAS-II Spatial cluster) and a Fluid Reasoning Index (similar to the DAS-II Nonverbal Reasoning Index). However, the WPPSI-IV is not normed as low as the DAS-II is.
In particular, the lowest possible scaled score on the WPPSI-IV subtests is 1, which corresponds to a T-score of 20. This means that the WPPSI-IV is not normed low enough to accurately capture the abilities of children who have moderate to severe disability. It also is likely not normed low enough to accurately capture significant differences between performance on the Visual Spatial Index and performance on the Fluid Reasoning Index or the Verbal Comprehension Index for children with mild disability. Wechsler Intelligence Scale for Children - IV (WISC-IV) The WISC-V, which is normed for children aged 6 years 0 months - 16 years 11 months, was released in 2016. For children aged 6 years 0 months - 7 years 7 months, either the WPPSI-IV or the WISC-V may be administered. It is recommended that the WPPSI-IV rather than the WISCV be administered to children with WS in this age interval. The structure of the WISC-V is the same as that of the WPPSI-IV. Thus, unlike the WISC-IV, the WISC-V includes a Visual Spatial Index (similar to the DAS-II Spatial cluster) and a Fluid Reasoning Index (similar to the DAS-II Nonverbal Reasoning cluster). For children with WS, this is a considerable improvement over the WISC-IV Perceptual Reasoning Index, which included both spatial and nonverbal reasoning subtests. The lowest possible scaled score on the WISC-V is 1, corresponding to a T-score of 20. Thus, the limitations described for the WPPSI-IV with regard to testing children with WS also apply to the WISC-V. Some schools or private psychologists may still be administering the WISC-IV. If the WISC is to be administered to a child with WS, it is important that the WISC-V rather than the WISCIV be used. There has been no research published on the performance of children with WS on either the WPPSI-IV or the WISC-V
The WSA's website contains comprehensive resources for families, researchers and medical professionals. Many detailed articles are contained in our online resource library, which has been recently updated, including the Intellectual Assessment: Common Profiles and Testing Tools and Scoring Figures.
WSA Increases Research Funding for 2017 - Supports Critical Research Efforts into Cardiovascular and Anesthesia Issues
The WSA has awarded research grants to Boston Children's Hospital and Columbia University Medical Center (NYC) for important research into cardiovascular and anesthesia issues in Williams syndrome.
The Cardiac Anesthesia team at Morgan Stanley Children's Hospital at Columbia is conducting a study aimed at "Improving Clinical Outcome in Children with Williams-Beuren Syndrome." The proposed study will summarize an extensive clinical experience in children with Williams syndrome who had anesthesia care over a ten-year period at Columbia University Medical Center. The results will provide the necessary framework to design a robust clinical database for the anesthesia/sedation care for these children, which will be critically important to inform future practices and improve outcomes.
The study at Boston Children's Hospital involves four separate but integrated projects over a three year period. Project 1 aims to create a simulation-based program to train surgical teams around the successful management of anesthesia-based emergencies for the patient with Williams syndrome.
Project 2 will utilize intravascular ultrasound imaging to understand the arterial wall and its response to catheter intervention. The vascular abnormalities that create obstruction (stenoses) in the pulmonary arteries of patients with Williams syndrome are poorly characterized. An improved understanding of the underlying nature of the vessel wall, and its response to intervention could improve the safety profile of balloon angioplasty for peripheral pulmonary artery stenosis.
Project 3 will examine the current state of healthcare delivery for patients with Williams syndrome and use this information to develop optimal models of care deliveries. The goal of these analyses will be to have an enhanced understanding of the current state of healthcare delivery, and the healthcare needs of people with Williams Syndrome across the lifespan, as well as to identify populations of patients with Williams syndrome with lower access to critical services.
Project 4 will perform a cross-sectional retrospective study to assess the relationship of medical and family factors to health-related quality of life, adaptive functioning, and neurodevelopmental function in children with Williams syndrome followed at Boston Children's Hospital. BCH will work with the WS Registry to allow for inclusion of comparable data, facilitate coordinated future studies and enhance general awareness, specifically focusing on cardiovascular aspects of WS.
Following the retrospective study, a prospective analysis and study of DNA samples will be used to determine if genetic modifiers of the varied vascular complications of WS can be identified based on general knowledge of the genetic controls of vascular development that are either predictive of severe vascular disease or protective from severe vascular disease.
WSA has also extended funding for the behavioral study by Dr. Bonnie Klein-Tasman and her team at the University of Wisconsin, and the Longitudinal study by Dr. Carolyn Mervis and her team at the University of Louisville. Future website blog posts from each research team will provide more detail on each study.
New Books about Williams syndrome: Available Now
The Boy Who Loved Too Much (Simon & Schuster): the poignant story of a boy's coming-of-age complicated by Williams syndrome. Journalist Jennifer Latson follows Eli over three critical years of his life as his mother, Gayle, must decide whether to shield Eli entirely from the world and its dangers or give him the freedom to find his own way and become his own person. (from Amazon).
"[Latson's] book provides a thorough overview of Williams syndrome and its thought provoking paradox. No doubt life for people with Williams (and those who love them has its difficulties. But given the state of the world today - the hate attacks, the divisiveness, the vitriol - it's hard not to wish that we all had more kindheartedness and openness even if our embrace of other humans is only metaphorical." (NY TIMES Book Review)
Throughout the research and writing of her new book, Jennifer Latson became a friend to many with Williams syndrome - attending Whispering Trails Therapeutic Camp. WSA Conventions and local events along the way.
The Stars In His Eyes
Written by 13-year old Joey's parents in their son's honor
Joseph and Jill Tantillo have written a novel inspired by their 13-year-old son Joey, who has Williams syndrome. Based on a dream Joseph had that Joey was cured, the story raises the question: "Would we change our children if given the opportunity?"
Proceeds from the book (self-published by the Tantillos) will support the WSA and Joey's parents have started a fan club for those who want to follow him.
Melissa Murphy and Jenny Knox receive "Special Impact Volunteer" award
The Williams Syndrome Association is all about connections - we work hard to connect families to each other, and to the professionals who can help. The ability to connect with each other for the emotional support we need throughout our journey with a child who has Williams syndrome is tremendously helpful. However, those aren't the only connections we need. It's also important for us to have the opportunity to connect with all types of professionals - medical, therapeutic, educational, research (for most of us there are too many to count) to help us find the answers about Williams syndrome that we are all searching for.
None of us can personally make all the connections we need on our own - we need help. That's one of the reasons the WSA was established - to help us make the connections we will need throughout our child's lifetime. Just like you, the WSA office cannot make all the connections you need on our own. And that is why we treasure our volunteers. Without them we wouldn't be able to hold the social events, fundraisers, conferences or national convention -without them our list of excellent professional resources would not continue to grow and improve as it does. Many of our volunteers have a "niche" - a special social event they hold annually, a fundraiser they like to organize, or they are someone who loves research and surfing the net, so they keep a look out for great resources and pass along that information to us. Thank you ALL for everything you do for the WSA and our member families!
In this new segment of the eNewsletter we will spotlight our amazing volunteers - special individuals who just keep finding more time for us, and more ways that they can assist.
Melissa Murphy and Jenny Knox are two volunteers who have made a huge impact on the WSA and local families in the Philadelphia area. They are volunteers who always find a way to do more -helping local families get together; creating one of the WSA's premier walks; helping on major fundraising event committees; opening doors to development opportunities for the WSA, regularly posting terrific resources for families, sharing personal and professional expertise on the WSA Board of Trustees and Board committees.
The list goes on...but it's not even the length of the list that is so remarkable, it's that Jenny and Melissa are making time to do all of these things for the WSA in the midst of very busy lives, as career women and young Moms. Jenny and Melissa, are incredible volunteers for the WSA, and it gives us great pleasure to recognize them as the WSA's first recipients of our "Special Impact Volunteer" award and to thank them both for, as the special award states, "Leading with Their Heart" for the Williams Syndrome Association.
Enrollment is complete for the 2017 season. We look forward to providing a great experience for participants and providing pictures of all the fun! The Berkshire Hills Music Academy has a few spots left in their summer program for teens and young adults. Information here.
Camp Blue Skies, which had its inaugural session for adults with WS this March in Georgia, will be offered again in March 2018!
2018 registration information will be available in late 2017/early 2018 depending on camp dates.
To view the great time participants had in March 2017 at Camp Blue Skies, check out our album here.
Save the date! July 11-14, 2018
Baltimore Marriott Waterfront Hotel
Click for printable PDF version of newsletter here