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.  

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 

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/
 

(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.

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)

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

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.

Hand-writing is still a core aspect of the national curriculum in the UK. Indeed, the new curriculum, implemented in 2014, focuses even more on hand-writing skills, linking a child’s handwriting to composition and spelling ability. However, other countries such as the USA have dropped handwriting skill from the curriculum as questions have been raised about the importance of hand-writing within a digital age. Think about how often do adults even write with a pen these days and is it not likely that the need to write with a pen will even decrease further when children will grow up?

Writing is a task that requires a number of cognitive processes to come together

One of the arguments put forward as to why handwriting is still important is that handwriting allows children to “feel” the letters and that this ‘learning by doing’ aids children’s spelling abilities.

However, handwriting does not only require complex motor abilities, it is also a complex cognitive skill. When writing with a pen a child engages at least in the following cognitive processes:

1) thinking of the sound they are going to write (verbal working memory)

2) retrieve appropriate visual representation of the sound from memory (visuo-spatial working memory)

3) find appropriate place on paper to start writing (visuo-spatial awareness) whilst remembering to hold the pen correctly (motor skills) and remembering the visual representation of the sound (verbal working memory and visuo-spatial working memory)

4) keep track of how to write the sound (visuo-spatial awareness)

repeat these steps for next sound.

Hence, writing with a pen requires a lot of 1) working memory abilities (processing more than 1 thing/information at the same time in the brain), 2) motor abilities, 3) visuo-spatial abilities. All of these are generally areas of difficulty for children with WS.

In comparison, writing with a keyboard on a computer involves the following:

1) think of sound you are going to write

2) retrieve appropriate visual representation of the sound from memory

3) find appropriate sound on computer (visuo-spatial ability but can memorised) and press button

repeat for next sound.

Therefore, writing with a keyboard limits the amount of cognitive processing (less working memory demands) and minimises motor demands and visuo-spatial demands. This may be beneficial for children with WS, especially when they are asked to write longer texts as even though some children with WS write, their writing can be slow and laborious. In addition, writing longer texts or a story requires children to keep in memory even more information as well as story structure etc, placing an even greater demand on children’s working memory abilities. However, it has been argued that as computers auto-correct and have spell checks, writing with a keyboard may limit children’s spelling abilities. In addition, we can question whether limitations or difficulties, such as handwriting, should be avoided by using a keyboard or receive more attention, practice and support instead.

Writing is important for children: children who read and write more get to develop their language abilities more and better language abilities lead to better writing abilities. However, children who have motor difficulties, as well as working memory and visuo-spatial difficulties, might benefit more from writing with a keyboard than writing with a pen, at least when writing longer texts.

By Dr Jo Van Herwegen

Children with WS are often delayed in their learning and most require specialist support (including speech and language therapy, physio therapy, occupational therapy, music therapy). I would say that all children with WS would benefit from 1-to-1 support to help their learning and to follow instructions. Besides that support, children with WS might benefit from the following strategies and approaches to support their learning and inclusion in the classroom:

Language development: Although language abilities generally are a strength in older children with WS, almost all young children with WS show a significant delay at the start of language development. In addition, children with WS often show better vocabulary compared to language comprehension. Therefore, the following adjustments should be made:

• Make instructions short or break them down into smaller chunks.
• Make eye contact and clearly say the child’s name before giving an instruction, as children with WS often miss that the instruction applies to them too when given in a group.
• Seeing their affinity with music, songs can help gain attention from children with WS and be used to teach children new facts and materials.
• Make sure objects and concepts are accessible/visible when talking about them and avoid abstract concepts.
• When explaining things always give the same example/explanation to start with and then expand with others, children with WS find it difficult to see similarities and analogies between things so explaining things in a different way might confuse them and hinder their learning.
• Grammatical understanding (especially prepositions such as “on”, “beside”, “between”) is often poor and people with WS might confuse sentences such as “the cat chases the dog” and “the dog chases the cat”. So always check the child with WS has understood what is expected from them.
• Figurative language or analogies are often used to explain new concepts. Yet, children with WS find figurative language, such as metaphors (i.e. the brain is like a computer), and analogies difficult to understand. So it is best to use more concrete language.

Peer relationships: Relationships with peers often stay superficial and people with WS tend to seek out social interactions with adults, instead of peers, even when older. In addition, individuals with WS may lack theory of mind abilities (understanding what others may be feeling or thinking) and may thus not able to respond appropriately to other people or in social situations. Therefore, the educational programme for children with WS should include some naturalistic play scenarios that include components of theory of mind as well as social situations and Social Stories that allow them to develop their social skills and establish friendships with peers.

Learning environment: Due to their distractibility and complex sensory needs, it is important that children with WS can receive some 1-to-1 teaching in a quiet room as this will allow them to concentrate better but also provide some vital time out from all the sensory input. This is important as too much sensory input can interfere with processing and the consolidation of the information being taught and lead to high levels of anxiety.

Managing behavioural and anxiety problems: Although most young children with WS are happy, people with WS are at an increased risk for high levels of anxiety and phobias. These tend to become worse with chronological age and at times of transition. These high anxiety levels can lead to outbursts of aggression or difficult behaviour. A fixed or predictable routine (e.g. a time schedule) is definitely recommended to reduce anxiety. In addition, using the same well-known support workers and preparing children with WS for any transitions might be needed.

Motor skills: Due to their low muscle tone, limited working memory abilities, and poor visuo-spatial awareness, writing with a pen is often a slow and laborious process in children with WS. Instead, the use of an iPad or keyboard would allow children with WS to develop their literacy (as well as language and learning).

The use of outdoor activities (such as climbing and balance activities) will help develop gross motor abilities. Furthermore, research has shown that people with WS have very poor visuo-spatial and navigation abilities but that exploration through self-locomotion can improve spatial abilities, spatial language as well as navigation abilities which in turn will aid independence long term.

General information processing and learning in WS: Research has shown that children with WS do not necessary integrate new information into their existing knowledge and theories. This means that it might be difficult for them to retrieve facts or they might take a long time to learn new things. A lot of repetition and explicitly showing children how new information relates to things they already know might be required. In addition, individuals with WS have a local processing information style which means that they will see/hear/focus on details at the expense of the wider context. So for example, when looking at a picture they may focus on certain details but not see how items go together. Similarly, when listening to a story they may remember details without understanding the wider context of the story or main storyline. Making children explicitly link information will therefore be essential. In addition, teaching children to group objects and semantic knowledge into categories will be required.

Reading development: Reading abilities have been found to be delayed in children with WS. And 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. In addition, many individuals with WS who do read quite fluently still find it difficult to understand what they are reading and show reading comprehension difficulties.

Reading difficulties are caused by visuo-spatial difficulties as well as poor working memory abilities.  Therefore, a phonological reading approach may not always work for children with WS and it is important that phonics lessons are combined with whole word reading and vocabulary lessons (for example such as in the Reading and Language Intervention or RLI for children with Down syndrome).

Mathematical development: Our research has also shown that, although children with WS know the counting names very well, their understanding of mathematical concepts is very delayed. Number abilities relate to domain specific abilities (such as approximate number abilities (ANS) or ability to see where there are more or less items as well as the number line) and some domain general abilities (including working memory (WM) and language abilities). Our research has shown that the ANS abilities in WS are impaired from infancy onwards and that individuals with WS have difficulties with WM. These difficulties can explain why, although they are very good at counting, people with WS fail to understand the meaning of counting or to complete formal mathematical tasks.

Due to the rarity of the disorder (about 1 in 20 000 life births), there are currently no intervention or training programmes that are aimed at WS specifically. However, children with WS often show language and attention difficulties that are similar to children with more common disorders such as Down syndrome (DS). In addition, although children with WS are very sociable, they also show social difficulties and anxiety issues that are common in children with Autism Spectrum Disorders (ASD). Therefore, children with WS might benefit from training programmes that have been found to be successful for children with DS and ASD.

The information provided here is very general and might not apply to all children or all ages. However, hopefully this summary provides some further information about what kind of support and strategies might be helpful for children with WS.

Some key references:

Brock J. Language abilities in Williams syndrome: a critical review. Dev Psychopathol. 2007;19: 97-127.

Martens MA, Wilson SJ, Reutens DC. Research Review: Williams syndrome: a critical review of the cognitive, behavioural, and neuroanatomical phenotype. J Child Psychol Psychiatry. 2008;49:576-608.

Thomas MSC, Van Herwegen J. Williams syndrome and language development. In:  Brooks P, Kempe V, Golson JG, editors. Encyclopedia of Language Development. Sage Publications;2014: 667-669.

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