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### Mathematical difficulties in Williams syndrome and how to provide support

By Admin, on 6 May 2024

By Professor Jo Van Herwegen & Professor Victoria Simms

Our research has focused on mathematical skills in children and adults with Williams Syndrome (WS) and compared their skills to typically developing children and adults as well as those with Down Syndrome (DS). In addition, we have reviewed existing studies that have previously examined mathematical skills in WS. For more information about our work, see details below. This blog is based on our own research as well as the findings of our review.

What do we mean by mathematics?

First of all, mathematics is a really complex subject with many components. Mathematics is made up of many different types of concepts and skills. To be successful in mathematics we must know things like being able to recognize and name digits, know the meaning of number words, understand the meaning of counting, knowing how numbers fit together in a system and realising the relationship between operations, such as that addition is the reverse of subtraction. We must also have good skills, such as being able to count accurately, carrying out addition and subtraction procedures and tackle word problem solving.

What do people with WS find difficult?

Our own research has shown that all people with WS struggle with mathematics. All verbal children and adults do learn the counting names and are able to count to 20. However, few are able to count onwards from a number different to 1 or to count backwards, due to poor working memory skills. Therefore, people with WS may “get lost” in the number word list when being asked to do something that they are not used to doing or when they get distracted. So, although we sometimes hear that people with WS are good at counting, we should put that in context: Yes, the knowledge of counting names in WS tends to be better than most children with DS, but they do struggle with numbers beyond 100 as well as counting tasks that really push working memory skills.

Secondly, many children with WS struggle to understand the meaning of counting or what counting is for: When you count items in a set, it is so that you know what size that set is. Yet, many people with WS make errors when counting. Partly, these errors can be explained by a lack of pointing when counting the items and a lack of keeping track of items that have already been counted compared to those items that have not yet been counted. However, even when asked to give a number of objects (a task that does not involve pointing or working memory), many people with WS give a random number of items showing that they struggle to understand what counting is for.

People with WS usually display poor visuo-spatial skills, this means that they struggle with orientation of numbers and letters and therefore digit recognition can be tricky for some, especially when double (e.g. 21) or triple digits are involved (e.g. 341). These poor visuo- spatial skills may also impact on counting skills and being able divide up counted and not- yet-counted objects.

People with WS are quite good at estimating small numbers and are able to recognize the number of objects (up to 5) quite quickly. However, their estimation of larger numbers is poor and a number of studies have now shown that their ability does not exceed that of 8-9 year old typically developing children. For example, when being shown 9 red dots and 14 blue dots, people with WS find it very difficult to say which set has more without counting. Yet, this is a task that young typically developing children find quite easy. In addition, people with WS find it difficult to understand how numbers relate to each other, or to estimate where numbers go on a number line. This all suggests that their organisation of how numbers and quantities are organized in their brain is different and might cause mathematical difficulties.

When it comes to addition and subtraction, many children and adults with WS manage to solve simple sums that use single digits and do not exceed the number 16. However, we have no research about more complex sums. As we know that people with WS struggle with double digit recognition, these larger sums might be a challenge for them.

Overall, across all aspects of mathematics, except for small number estimation, people with WS may show difficulties throughout their lives.

What can you do about it?

Our review of existing research has shown that there are no intervention studies that have assessed how to improve mathematical skills in people with WS. There are no studies that have evaluated what works in the classroom in terms of mathematical practice for children with WS. So we can only make an informed guess based on what we know about mathematical abilities in typically developing children and studies that have examined what works for other children that have mathematical difficulties.

1. 1)  Improving number names: Although the knowledge of number names is delayed, children with WS are able to learn the number names and songs can particularly help children with WS. Actually there are a number of studies that suggest that the verbal abilities might, to a certain extent, help children with WS compensate for their non- verbal mathematical difficulties.
2. 2)  Pointing: Children with WS rarely point, especially younger ones. So try and find opportunities for children to point at things (by putting objects out of reach for example or by showing them two things one of which they really like and ask them to point to what they want). Also praise them and respond when they point. Pointing can help them when counting things later on.
3. 3)  Number line skills: Understanding how number relate to each other is very important for solving mathematical problems. There is good evidence to suggest that playing very simple board games- like snakes and ladders, helps children understand the number line (i.e. that numbers go from 1-10, with small numbers on the left and large numbers on the right). Playing games in which children have to put numbers in right order or amounts/sets of objects from small to large may also help.
4. 4)  Set sizes: Practicing comparing small sets of objects may help estimation skills. Share out two small sets of sweets and ask them to guess who has more! This will be motivating but also useful.
5. 5)  Cardinality: Task a bag of toys and ask children with WS to take out a specific number of items and give them a sticker each time they give the correct number of items. As children find this very difficult it is OK to start with numbers 1 and 2.

6) Digit recognition: As digit recognition difficulties are partly caused by visuo-spatial issues using larger font size, more space between sums (and even better just to have 1 per work sheet) as well as using colour to show how the digits go together (in 12 both numbers are blue but in 1 and 2 each number is a different colour) can help people with WS.

Also children with WS have working memory difficulties and will find it hard to keep track of items counted or to manipulate information in their minds only. So teaching them strategies of how to break sums down, how to keep track of items counted (line items to be counted up!) and encourage the use of fingers might really help them.

As with all learning in WS, it can take a very long time for people with WS to show true understanding and repetition is key. In addition, we know that some days people with WS can show good understanding of something and the next time it seems like they have forgotten everything (due to anxiety, lack of motivation, lack of concentration, sleep, who knows?!). Therefore, what works one day may not work on another day but luckily maths is all around us and thus, it should be easy to implement some maths activities in your daily routines. But also, motivation is key so using their favourite topic to cover some maths questions will really help them (e.g., How many people fit in that plane/train? Is that more than on the other plane/ train? How long it is the extension of that hoover? Is that longer than the other one?).

Some examples of maths in daily life:

• When driving in the car there are a lot of digit recognition games you can play: speedlimits, road numbers, car registrations, they all contain different digits!
• When shopping you can talk about the amount of items in your trolley, how thatcompares to people around you and how much you think you need to pay. This

game is good for large number estimation.

• When a child or adult with WS asks for something (sweets, money, anything to eatlike three fishfingers, four beans, one potato) ask them to count out the number of

items.

• If your child likes to line up toys, you could for example stick stickers with numbersor quantities (a number of dots) on these toys and use these and your child’s fondness of lining up toys to improve their understanding of how numbers relate to each other.

There are many more examples of how you can incorporate maths teaching in daily life, without the need to sit at a table and complete some worksheets. If you have any good examples, do get in touch!

References:

1. Van Herwegen, J., & Simms, V. (2020). Mathematical development inWilliams syndrome: A systematic review. Research in Developmental Disabilities, 100, 103609. doi: 10.1016/j.ridd.2020.103609
2. Van Herwegen, J., Ranzato, E., Karmiloff-Smith, A., & Simms, V. (2020). Foundationsof mathematical development in Williams syndrome and Down syndrome. Journal of Applied Research in Intellectual Disabilities 33(5), 1080-1089.
1. Simms, V., Ranzato, E., Karmiloff-Smith, A., & Van Herwegen, J. (2020). Understanding number line estimation in Williams Syndrome and Down Syndrome. Journal of Autism and Developmental Disabilities 50(2), 583-591.
2. Van Herwegen, J., Ranzato, E., Karmiloff-Smith, A., & Simms, V. (2019). Eye movement patterns and approximate number sense task performance in Williams syndrome and Down syndrome: a developmental perspective. Journal of Autism and Developmental Disabilities. Advance online. doi: 10.1007/s10803-019-04110-0
3. Van Herwegen, J., Costa, H.M., Nicholson, B., & Donlan, C. (2018). Improving number abilities in low performing pre-schoolers: symbolic versus non-symbolic training programmes. Research in Developmental Disorders, 77, 1-11.
4. Costa, H.M., Nicholson, B., Donlan, C., & Van Herwegen, J. (2018). Low performance on mathematical tasks in preschoolers : the importance of domain-general and domain-specific abilities. Journal of Intellectual Disability Research, 62(4), 292-302.
5. Van Herwegen, J., Costa, H. M., & Passolunghi, M. C. (2017). Improving approximate number sense abilities in preschoolers: PLUS games. School Psychology Quarterly, 32(4), 497-508.
6. Karmiloff-Smith, A., D’Souza, D., Dekker, T., Van Herwegen, J., Xu, F., Rodic, M., & Ansari, D. (2012). Genetic and environmental vulnerabilities: The importance of cross-syndrome comparisons. PNAS 190(2), 17261-17265.
7. Van Herwegen, J., Ansari, D., Xu, F., & Karmiloff-Smith, A. (2008). Small and large number processing in infants and toddlers with Williams syndrome. Developmental Science, 11(5), 637-643.

### Mathematical abilities in Williams Syndrome and future directions

By Admin, on 11 May 2023

Blog by Unta Taiwo and Stella Xu

Mathematical skills are an essential part of everyday living. From preparing a meal, setting a table, planning a daily schedule, or embarking on your weekly shop. We need to understand the quantities we need when we’re hosting dinner with friends or deciding how many apples to buy for a weekly supply. Other calculations come in handy when we are working out how much to pay for a bus fare, or a birthday present. Numerical skills, also referred to as mathematical abilities, prepare us for independence in our adult lives. Research has shown that, generally speaking, individuals with Williams syndrome can recognise and count smaller numbers. However, when it comes to comparing quantities and estimating the size of a set of objects, individuals with Williams syndrome are behind typically developing children (Van Herwegen & Simms, 2020). For example, children and adults with WS find it difficult to identify where numbers go along a number line (Simms et al., 2020) or understanding how sets of objects relate to each other (which one is larger/ has fewer) (Van Herwegen et al., 2020). Understanding how numbers relate to each other or what they mean forms the basis for more complex maths skills like arithmetic and can help identify mistakes or check answers when solving mathematical problems. For example, have you ever used a calculator to add large numbers and then decided that you must have made a mistake? It is your understanding of how numbers relate and what they mean, i.e. your Number Sense abilities, that help you estimate that the result on the calculator was wrong. Current evidence suggests that Number Sense abilities are delayed and may develop atypically in children with WS from infancy onwards.

It is important to understand the specific difficulties related to the development of mathematical abilities in individuals with Williams syndrome so that we can think about targeted interventions and guidance for parents and practitioners to improve learning outcomes. In the Child Development and Learning Difficulties (CDLD) lab we have a number of studies with the aim to better understand the specific strengths and difficulties associated with mathematical development in Williams syndrome:

HoME intervention study:

###### Photo provided by Unsplash

This intervention involves parents to deliver very short foundational maths games in the home environment over two 5-week blocks. Evidence from previous research (Van Herwegen et al., 2018) has shown that these games improved maths outcomes for children with mathematical learning difficulties. Each game is designed to be flexible to family dynamics, so games might be adapted to include siblings or other relatives. These carefully designed activities can be played in and around the house, including around the dinner table, whilst having a bath, walking to school, or getting ready for bed. Each programme will involve a degree of multi-sensory activities for engagement that tap into tactile, auditory and visual senses.

Recruitment:

We are looking for parents of children with Down syndrome or Williams syndrome aged between 5-11 years to take part in this intervention study. This intervention will be starting in the new year. If you are interested taking part in this study and would like more information, please get in touch with Unta (unta.taiwo.14@ucl.ac.uk).

Study on development of mathematical abilities

###### Photo provided by Unsplash

Currently very little is known about the predictors and the longitudinal development of mathematics in individuals with Williams syndrome. This study is therefore focusing on investigating which cognitive abilities at infancy may affect later math performance at school age. The results of this study will inform parents and practitioners on what areas to work on to improve mathematical achievement in school, as well as everyday life experiences. Part of this research will be pulling together findings from different UK labs that focuses on Williams syndrome, so we can better understand the long-term development of mathematics.

Recruitment

We are looking for parents of children with Williams syndrome aged between 5-18 years to take part of this study. This study will begin in June. If you are interested in taking part and would like to know more, please get in touch with Stella (stella.xu.21@ucl.ac.uk).

### Extracting the voice of young people with intellectual disabilities: Top tips

By Admin, on 11 July 2022

We recently co-produced a workbook with young people with Williams syndrome (WS) aged 5 to 15 years old about their education, named “My Own Williams Learning”. As part of this co-production we wanted to obtain the voice and opinion of young people with Williams syndrome about what Williams syndrome is, what children might be good at or struggling with and what support or good practice in school looks like. The booklet contains advice and activities to help children with WS understand their own learning but can also be used by parents and teachers. This booklet will be available soon via the Williams Syndrome Foundation website (https://williams-syndrome.org.uk/clinical-guidelines/).

As part of the co-production process of this booklet we reflected on best practice to elicit the voice of young people who have intellectual disabilities. Although most young people with Williams syndrome are very talkative, they have mild to moderate intellectual disabilities and their language abilities are rarely age appropriate. In addition, they often have difficulties with language comprehension, working memory difficulties and maintaining conversations. As such, the voice of children with WS has rarely been captured before and often parents are asked to report on their child’s behalf. In our latest research, we showed that only 7% of the Education Health and Care plans of 33 children with WS explicitly reported the child’s voice in section A. Most of the EHCP’s used third person discourse to describe the child’s wishes, strengths and difficulties (Palikara et al. in press).

Photo by Jason Rosewell on Unsplash

We worked with the children either one-to-one or with small groups of up to 3 children at one time (like a focus group). All of the sessions took place online due to COVID-19.

Below are some tips that can be used by teachers and parents (and researchers!) to elicit the voice of young people with WS.

• Reduce working memory load by asking simple short questions and use visual reminders. It often helps to repeat the question in the same way (not rephrase it).
• Provide waiting time of up to 10 seconds to allow them to process the question and to formulate answers.
• Use images to introduce topics. So for example, when asking what children might be good at or need help with, it might be helpful to have pictures of topics and issues at school: maths, reading, listening, writing but also toilet, hand washing, PE, going on the bus. There are existing pictures from visual time tables that might be handy here. If there are a lot of pictures to go through, it might help to do several rounds of sorting these with the child into piles of ‘I need help with this’, ‘ I am good at this’. So introduce the pictures one by one and ask the child to sort them.
• If working with a group of children, it helps to ask the questions in the same sequence each time to support predictability of their turn.
• Finger puppets could be used to keep participants’ attention on the screen, but only when attention is waning.
• Action songs to which the children could participate can help to provide a break from talking and listening. We used them approximately each fifteen minutes.

References

Palikara, O., Ashworth, M., Castro-Kemp, S., & Van Herwegen, J. (in press). All views my own? Portraying the voices of children with complex neurodevelopmental disorders in statutory documents

### Williams syndrome is genetic: what does it (not) mean?

By Admin, on 22 June 2021

Williams syndrome is a rare genetic disorder that is caused by a microdeletion (1.5-1.8-Mb ) on the long arm of chromosome 7 (7q11.23 to be precise). The deletion affects approximately 28 genes, one of them being the gene ELN which codes for elastin in our body. However, there is no single gene in the pathogenic variants that causes WS.

I am no expert in genetics, nor do I have a medical degree, but I have worked with parents and children with WS for over 15 years and below are some questions parents often have when their child received a diagnosis for WS. So I will try to answer these questions to the best of my ability, using my experience of working with WS and the research out there.

How can WS be diagnosed?

WS is diagnosed genetically as well as clinically.

In the past WS was genetically diagnosed by a Fluorescence In Situ hybridization (FISH) test which involved taking a sample of blood from the child and inserting two probes, a control probe and a probe that reacts with ELN. The probe is a very small chemical that glows brightly when it detects a specific element on a chromosome. A scientist uses a special microscope to look at the chromosome and see how many bright spots are present. When a person has a WS deletion, only one bright spot for ELN can be seen  instead of two (one on each chromosome) but two spots appear for the control probe. These days a chromosomal microarray is used to genetically diagnose WS which scans either the entire genome or targeted regions of the genomes to detect copy number variants (losses or gains of chromosome material), which may be benign, pathogenic, or of uncertain clinical significance.

Clinically, WS is diagnosed when the following features are present: cardiovascular disease (especially SVAS or narrowing of arteries), distinctive facial features (broad forehead, short nose, wider nasal tip, wide mouth, thick lips, small chin), Connective tissue abnormalities (Hoarse voice, inguinal/umbilical hernia, bowel/bladder diverticulae, rectal prolapse, joint limitation or laxity, and soft, lax skin), cognitive delay, growth abnormalities (especially failure to thrive and poor weight gain in infancy) and endocrine abnormalities (including hypercalcemia and hypothyroidism).

What caused the deletion?

To put it simply: during the time chromosome 7 was duplicated, something went wrong and a section of the chromosome was not duplicated correctly. It is not caused by something that parents did or did not do. This also explains why the incidence of WS (about 1 in 18,000 life births) does not vary depending on country.

Why are parents tested when their child receives a diagnosis of WS?

In most cases parents are not affected at all. Yet, when the child receives a diagnosis of WS, parents are often asked to be tested as well. I always wondered why this is the case. Well, we all have variations in our genetic make-up, especially inversions where the genetic material is there but in a different order. About 6% of people have an inversion on the same region of chromosome 7 as WS but this inversion is considered typical. Yet, 25% of parents who have a child with WS have this typical inversion which is much higher than what is expected for the general population. As a parent with the inversion has 50% chance of passing on this inversion to their child, parents are asked to be checked in case they want to have future children. It is argued that parents who have the inversion might be more likely to have another child with WS. Yet, this is extremely rare (you are more likely to win the lottery) and has only been reported in 3 cases. However, if people with WS have children than these children have 50% chance of having WS as well. So, please rest assured, most parents who have a child with WS and have the inversion have other children who do not have WS.

My child has a smaller deletion does this mean my child will be affected less?

It has been argued, especially on the WSA website that: “The size of the deletion plays a role in the symptoms of WS. Individuals who have a deletion of 28 instead of 26 genes are less likely to have hypertension. Deletions longer than 28 genes are associated with more severe intellectual disability, and seizures (infantile spasms). Deletions shorter than 26 genes are associated with fewer symptoms which vary depending on what genes are deleted. “

Although I am no expert in this field, I would like to put these strong claims within the right context.

1. There is no direct relationship between genes and behavioural outcomes. We are all the product of our development, that includes our environment, the people we grow up with, the opportunities we have had, whether we have been hospitalised for a long time, etc. All of these factors will have an influence on who we become and what we can achieve.
1. These claims are based on studies with small sample sizes and mainly included adults with WS. However, these studies did not take development into account or how these individuals might have different from each other.
1. Genes interact with each other and thus, it is not only the size of the gene deletion that matters but also which genes are deleted. However, we do not know much about how genes interact with each other and thus, making conclusions about genes and behaviour from the size of the genetic deletion alone may be well incorrect.
1. Yes, from a scientific point of view analysing the relationship between the size of the deletion in WS and the outcome can be useful to see what each gene in our body does. However, from a practical and clinical point of view we need to be very careful with the conclusions that are being made about these studies (see my point about development and outcome).

Hope this has helped a little.

### Language abilities in Williams syndrome

By Admin, on 6 June 2021

Williams syndrome (WS) is commonly described as good language versus poor non-verbal abilities (such as block building). Although language abilities generally are a strength in older children and individuals with WS, almost all young children with WS show a significant delay at the start of language development. Our own research has shown that children with WS generally have a very limited vocabulary up until the mental age1 of 18 months and many children may not say their first word until they are older than two years. There are a number of reasons why language abilities may be delayed in WS. The literature, as well as our own research, suggests that factors such as delayed pointing abilities, gross motor abilities early on in life, delayed theory of mind abilities, poor joint attention, and oromotor-praxis difficulties contribute to the delay observed in many children with WS. In addition, there are a small number of children with WS who never develop language (most of them have very complex needs and often also comorbid disorders) and sometimes language development can be extremely slow: I have worked with children with WS who did not develop language until they were 9 years old!

Not only is language delayed in WS, it is also atypical in that language production abilities are much stronger than language comprehension ones. Many people find this difficult to understand as generally in development children are able to understand something before they are able to do it themselves. In our own research we have seen that individuals with WS across a large age range are very poor at understanding figurative expressions. Yet, when we looked at the language they produced when telling stories from a wordless picture book, they used the same amount of figurative expressions as typically developing children.

So how can they use expressions and words, but without necessarily understanding them? Individuals with WS have very good short-term memory abilities, especially phonological short term memory or memory for sounds, and have very good imitation abilities. This allows them to imitate words and even entire sentences but without necessarily putting any meaning to it. This would also explain why children with WS sometimes use words within a strange context: “I am going to evacuate this glass” (when drinking from a glass). So, even when children obtain a large vocabulary and use full sentences, it is important to check the child’s language comprehension when giving instructions as their language production might deceive you about their understanding!

So if your child is very young and not talking yet, what can you do to support your child’s language development?

• Keep talking to them, name objects and read books, children with WS often need a lot more repetition before the information “sticks”.
• As children with WS gesture and point less than typically developing children, the children themselves create fewer opportunities to receive language input from adults than their peers and thus, adults need to ensure they keep providing language input, even when their child does not explicitly ask for it.
• Most children with WS have an affinity towards music so songs can be a good way to teach children language. For example, you could prompt them to say the final word of the rhyme/song.
• When you talk about something make sure you show the object to them or point but wait until the child has moved his/her attention to it. Young children with WS find it difficult to move their attention from one place to another and need more time and prompting to do so.
• We know that motor development (being able to move around and manipulate the objects) is very important for language development but children with WS often have delayed motor development or fine motor difficulties: so show them the objects from different angles and allow them to explore things as much as possible (e.g. by moving them around or position them in various different positions: viewing an object from the top looks very different to viewing it from the side).
• Help them develop their oral motor skills by pulling funny faces, practice tongue muscles by sticking out tongue in various ways, blowing bubbles etc.
• Access specialist input such as speech and language therapy, music therapy, and physiotherapy

### Supporting reading in Williams syndrome

By Admin, on 22 May 2021

There are typically three ways to read words. One is a logographic approach in which children do not read any particular letters or the word itself but they recognise the shape of a word within a context. For example, very young children might recognise the word school because of the ‘ool’ shape. My own sister thought she could read when she was five years old because on the motorway she could recognise the word “exit”. This is the very first step in learning to read. The two other routes of reading are a phonics approach or sight word learning approach. In the phonics approach, children learn to recognise the letters and their matching sounds. Once the children know the letters and their sounds, the phonics approach allows children to read words that they have never seen before. For example, a child that has learned the letters C, A, T, B, and S can read the words: ‘cat’, ‘bat’, ‘sat’, ‘at’, ‘cab’ etc. Although the phonics approach is very good for learning to read new words you have never seen before, it requires you to break down the words in letters, sound each of these letters out, keep track of which letter you have sounded out and which one is next in line, and then blend of the different sounds back together at the very end. This approach therefore requires a lot of ‘working memory’ or the ability to hold information in mind and manipulate that information. In some languages, especially in English, not all words are written the way they are pronounced. For example, Leicester is not pronounced “lajtsesta”. So, for these words a phonics approach cannot be used and children need to learn to read some words by heart or use a ‘sight word reading approach’.

What approach works best for children with Williams syndrome?

Looking at their overall strengths and difficulties, children with Williams syndrome (WS) generally have good verbal short term memory (repeating information they have just heard) and have good phonological abilities. However, working memory abilities are lower than children of a similar chronological age. In addition, children with WS, also learn at a slower pace.
Reading abilities have been found to be delayed in children with WS. Yet, the abilities that relate to early reading proficiency, such as good phonological awareness and good vocabulary abilities, are similar to typically developing children. Still, there is a lot of variability in the reading abilities of children with WS with some children being quite fluent readers and others struggling to read. This is mainly because many children find it difficult to learn the letter-sound combinations. Finally, many individuals with WS who do read quite fluently still find it difficult to understand what they are reading.

There is only one study that I know of that has examined whether children with WS would benefit from a phonics approach or a sight word reading approach to reading. Beccera and colleagues (2009) included 44 children with WS aged 8 to 17 years old in their study and divided them according to their educational plan and parental reports in to those children who primary followed a phonics approach and those who followed mainly a whole word approach. They found that children who primarily followed a phonics approach had better reading outcomes compared to those in the whole-word approach. However, some caution is required when interpreting this study as children were not randomly allocated to a group in this study and the children in the phonics-approach had better working memory and higher overall cognitive abilities (they did find that reading levels correlated with overall IQ) than those children in the sight word reading approach.

So, what can we conclude thus far?
Young children with WS often struggle with early reading abilities because of 1) their overall language delay and limited vocabulary and we know that language and vocabulary is a precursor for reading in WS, 2) they have limited working memory abilities and thus they struggle to break down a word into their individual sounds, keep track of the letters they have sounded out, and then blend the sounds back together, 3) they have overall learning difficulties which makes both learning sound-letter combinations and learning sight word reading difficult.

In sum, using just one approach is unlikely to help children with WS to read. The Reading and Language Intervention (RLI) designed by Down Syndrome Education International and at The Centre for Reading and Language at the University of York, for children with Down Syndrome includes a reading strand and language strand. The reading strand focuses on both phonics and sight word reading whilst the language strand teaches vocabulary, utilising visual approaches to introduce and reinforce the meaning of new words in a variety of different contexts. Although we have no studies that have examined the impact of RLI training for children with WS, seeing the multi-faceted approach to reading and what we know about reading in WS, this approach might benefit children with WS as well as those with Down syndrome.

Reference:
Becerra AM, John AE, Peregrine E and Mervis CB (2009). Reading Abilities of 9 – 17-Year- Olds with Williams Syndrome: Impact of Reading Method. Front. Hum. Neurosci. Conference Abstract: 12th International Professional Conference on Williams Syndrome. doi: 10.3389/conf.neuro.09.2009.07.030

For more information about the Reading and Language Intervention (RLI) see: https://www.dseinternational.org/en-gb/resources/teaching/rli/design/

### Home Learning Environment of children with Williams syndrome

By Admin, on 21 May 2021

(Erica Ranzato, Andrew Tolmie, Jo Van Herwegen)

Photo source: Unsplash_Annie Spratt

Home Learning Environment (HLE) refers to all of the activities and opportunities provided by parents to support their child’s overall academic success. It includes the frequency of home learning experiences, the availability of resources that promote learning, children’s participation in the learning activities and parents’ attitudes towards learning. Cross-cultural research on typically developing populations suggests that the HLE during early years has a pivotal role for the development of children’s literacy skills (Senechal & LeFevre, 2014) and mathematical abilities (Mutaf-Yildiz et al., 2020).

We investigated for the first time the HLE of 24 primary school children with Williams syndrome (WS), using a parental web-based survey and our findings showed that:

• Literacy-based activities occurred more frequently than maths-based activities.
• Parents provided a varied HLE characterized by maths activities supporting different skills such as counting, digit recognition, arithmetic, and numeracy.
• Parents engaged with their child in both formal and informal[1] literacy and maths-based activities, but informal activities occurred more often when supporting counting and number recognition skills.
• Parents had high expectations for their child’s literacy and number knowledge skills at the end of primary school, but their expectations for their child’s arithmetic skills were significantly lower compared to the other categories.
• Parents that had higher expectations for their child were, in general, offering more frequent learning activities at home.
• When compared to a group of parents of primary school children with Down syndrome (DS), parents of children with WS provided maths-based activities that supported counting and number recognition less often than parents of children with DS. This might be explained by the fact that, although parents of children with WS recognised their child’s difficulties with mathematics, they may underestimate the difficulties that their child might have specifically with counting.

Although the HLE for children with WS was varied and parents had overall high expectations for their child’s academic abilities, it is recommended that parents provide short (5-10 minutes) but frequent activities that focus on maths as well as literacy development. If you would like further inspiration for informal mathematical activities, have a look at our Math@home work (https://www.ucl.ac.uk/ioe/departments-and-centres/departments/psychology-and-human-development/child-development-and-learning-difficulties-lab/educational-technologies-and-apps/mathshome).

We would like to thank all the parents who completed the web-based survey.

[1] Formal activities are the activities used by parents with the specific purpose of developing literacy or mathematical skills. Informal activities consist of real-world tasks during which parents’ teaching happens without an explicit purpose and the learning is likely to be incidental, such supporting maths learning through playing board games that involve numbers.

### School transition from primary to secondary school in children with Williams syndrome

By Admin, on 19 May 2021

Most children with Williams syndrome (WS) have difficulties with anxiety when they reach the end of primary school and they often prefer a clear structure. School transition is an important milestone in children’s school career that can be stressful. Although transition to secondary school can provide new opportunities in terms of more independence and new topics to study, it also brings a lot of fear of the unknown in terms of friends, new teachers, as well as new environment.

In our recent project around school transition, parents, educators as well as children reported their concerns before the child with WS moved to a secondary school as well as after. Before the transition, children with WS were worried about their new physical environment (where toilets would be and what to do when you get ill), parents had more social concerns and wanted to know more about who else would be in their child’s classroom as well as worried about bullying, whilst teachers were more concerned about how the child would cope with new freedom and food.

Teachers and parents expressed the need for better planning around transitions and based on our research we would like to make the following suggestions:

• Start the discussion around the child’s school transition early (start of year 5)
• Plan for additional support that might need to be in place for meeting the child’s needs in secondary schools
• Visit a number of schools (with the child with WS if possible) and try and meet the transition co-ordinators to discuss your child’s needs and the transition plan.
• Ask the child’s new school to send a map of the school and pictures of teachers with short description of nice facts about the teachers and school.
• Ask the child’s current school to provide an overview of what they found worked well and what didn’t
• Provide the child’s new school with an overview of what the child likes/ doesn’t like where the child’s strengths lies (maybe the child with WS can do a presentation or take photos themselves of their strengths, weaknesses, interests and needs.

We are currently writing up the results of this study and hope to disseminate more detailed findings soon.

This research was funded by a grant by the Baily Thomas Charitable Trust  to Jo Van Herwegen (collaborators Dr Olympia Palikara, Maria Ashwort hand Elizabeth Burchell)

### Education guidelines for children and young people with Williams syndrome

By Admin, on 17 May 2021

Recently, researchers from the Child Development and Learning Difficulties lab at UCL Institute of Education and Mary Immaculate College (Ireland) conducted focus group discussions with parents and educators (e.g. teachers, LSAs, SENCOs) regarding the reality of how schools and staff support learners with WS.

One of the main challenges highlighted by educators was the lack of information available with regards to how individuals with WS can be supported on a day-to-day basis with regards to their learning. Educators noted that, whilst parents were a good source of initial information, there was still a need for concise and easily accessible educational guidelines.

The focus groups also engendered a lot of discussion on what works in the classroom, and specifically what strategies have been used that have been successful in promoting learning in children with Williams syndrome. It was generally agreed that educators and parents alike should:

• Build upon the strengths and interests of the WS profile and keep the individual child in mind. For example, many children with WS gave an affinity towards music and this music affinity can help them to learn. In addition, many children with WS are eager to please and respond well to praise during teaching.
• Make the learning physical and concrete rather than abstract, especially when teaching mathematical concepts. Both teachers and parents felt that children were well adept at learning a wide variety of mathematical concepts when incorporating physical objects in the teaching (e.g. teaching addition with toys rather than figures on a worksheet).
• Always take the child’s sensory needs into account when teaching (is the classroom maybe too loud/ the environment too distractive?)
• Give the child multiple breaks (whether longer sensory breaks or 1 minute movement breaks) and provide learning in very small bursts of 5-10 minutes.
• Keep a diary of what works with the individual child and what doesn’t, and that this could be shared with colleagues
• Include clear communication between school and home: Young people with WS are not always able to clearly communicate what happened at school or home. Yet, events at home or school may impact on their anxiety and ability to learn/ concentrate. Also, parents knowing about what academic topics school is focusing on and how they support their child allows parents to support their child’s learning at home as well.

Overall, it was made clear that according to both parents and educators, education should not only include academic skills but should also include life skills as well as social skills, so that young people with WS may reach their full potential, become as independent as possible and a good quality of life in their adult years.

We would like to thank all of the parent and educators who took part in our focus groups.

Please keep an eye out on the new educational guidelines that we will be publishing by the end of this term (July 2021).

This research is being funded by the Williams Syndrome Foundation with a grant to Jo Van Herwegen

### Naughty behaviour and punishment in children with WSWilliams syndrome, Learning, school,

By Admin, on 22 April 2021

Children with WS have mild to moderate learning difficulties meaning that on average they function at the same level as typically developing (TD) peers about half their chronological age (so a 7-year-old with WS will have the reasoning abilities of a 3- to 4-year-old TD child). This is also the case for their language comprehension abilities, despite the fact that they may use a lot more ‘age-appropriate’ or ‘inappropriate language’. This has a number of consequences when it comes to ‘naughty behaviour’ and punishments in WS.

1)    First of all, children with WS often crave social contact with peers and others but often lack the communication and social skills to initiate or continue a conversation in an appropriate way. This often results in children either relying on stereotypical questions (e.g., asking about the weather) or repetitive questions. Similar to younger TD children, another way children with WS may try and build social relationships is through mimicking other people’s behavior and language (e.g., trying to be funny through the use of toilet humour). Children with WS have a very good memory for the phonology of a word and thus they can copy words really well.

2)    Although they are able to copy many words even within the correct context, children with WS have limited language understanding and thus often they do not understand the words they are copying. We have repeatedly found this in our own research (see Naylor & Van Herwegen, 2012; Van Herwegen et al., 2013).

3)    As children with WS are developmentally delayed, they often do not understand why a certain type of behavior or word is wrong and they often also struggle to understand the relationship between their actions and the consequence or punishment, especially when young (mental age younger than 6 years old).

Therefore, when children with WS show ‘naughty’ behavior, it is important to:

1)    Keep in mind the overall delay in language and reasoning abilities in children with WS

2)    Clearly explain using simple language, why a certain type of behavior or language is inappropriate.

3)    Ensure that the punishment is developmentally appropriate to the child and can be understood. In my view, punishment should be kept to a minimum as children with WS find it difficult to see the relationship between actions and consequences.

4)    Provide the child with alternative strategies to obtain the same desired outcome: providing them with language and social skills that will help them build relationships using appropriate behavior and language.

5) To help children deal with their frustration you can use sensory toys that they keep in their pocket which they can pite, squeeze, hit,…Bouncing on trampolines are also very effective to bounce off any anger or a scream cushion where they can vent their anger and frustration into.

6) Model appropriate behaviours.

References:

Van Herwegen, J., Dimitriou, D., & Rundblad, G. (2013). Development of novel metaphor and metonymy comprehension in typically developing children and Williams syndrome. Research in Developmental Disabilities, 34, 1300-1311.

Naylor, L. & Van Herwegen, J. (2012). The production of figurative language in typically developing children and Williams Syndrome. Research in Developmental Disabilities, 33(2), 711-716.