Paige Kiernan EDUC3629: Students with Special Needs

Case Study:

Harley - Year 1, Prader-Willi Syndrome

As stated in the case study, Harley is a year 1 student who has been diagnosed with

Prader-Willi syndrome, a lifelong condition without a known cure that affects

approximately 1 in 10,000 to 15,000 people (Dryden, 2019; Scott et al., 1999;

Prader-Willi Syndrome of Victoria, n.d.). Prader-Willi syndrome is a rare and complex

chromosomal disorder that results when genetic material on chromosome fifteen

derived from the father is either deleted or unexpressed (Dryden, 2019; Fidler et al.,

2003; Prader-Willi Syndrome Association of Victoria, n.d.). Whilst the severity and

characteristics differ in each person affected with the disorder (Scott et al, 1999),

some common characteristics include impaired speech and language skills, delay in

reaching motor milestones, rigid thinking, obsessive tendencies, poor muscle

strength, a diminished sense of arousal, and an increased appetite and reduced

satiety (Dryden, 2019; Atkin & Lorch, 2007; Prader-Willi Syndrome of Victoria, n.d.).

Given that a child with Prader-Willi syndrome’s language function and articulatory

skill are also severely affected, children may also begin to display some dysfluent

speech behaviours such as interjections, revisions, repetitive questioning, and

incomplete phrases (Atkin & Lorch, 2007). As well as being the driving force behind

many behavioural difficulties, Prader-Willi syndrome is also the most common

genetically identified cause of obesity (Atkin & Lorch, 2007; Dryden, 2019).

Impacts on Classroom Experience

Many children affected with Prader-Willi syndrome struggle to interpret subtle social

cues, learn social norms, and lack the ability to see things from another person’s

point of view (Dryden, 2019; Chedd et al., 2006). This can have a detrimental impact

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on a student’s classroom experience as any child who lacks appropriate social skills

is at risk of being rejected, victimized, or teased by their peers (Westwood, 2015;

Rosner et al., 2004). Temper tantrums are also common among children with

Prader-Willi syndrome, which has the potential to hinder the child’s relationship with

others and may ultimately lead to social isolation and cause the child to disengage

from their learning (Westwood, 2015; Rosner et al., 2004; Dryden, 2019).

Children diagnosed with Prader-Willi syndrome also often show high rates of

behavioural difficulties including underactivity, compulsions, stubbornness, and

aggressive behaviour (Rosner et al., 2004; Chedd et al., 2006; Dryden, 2019). The

majority of children who suffer from Prader-Willi syndrome are underactive, which

often manifests itself as a lack of enthusiasm or minimal interest in completing

assigned work (Chedd et al., 2006). These children often also exhibit compulsive

and repetitive behaviours that will likely impede their ability to work quickly and

effectively, such as repeatedly asking questions about the schedule and routine

within the classroom (Dryden, 2019). Children diagnosed with Harley’s condition are

also likely to have heightened emotional reactions to minimal changes in their

schedule (Dryden, 2019).

Another challenge children with Prader-Willi syndrome experience is a lack of short-

term auditory memory (Chedd et al., 2006). In a classroom setting this may mean

that a child will find it difficult to remember strings of verbally presented information,

as they struggle to transfer auditory information from their short to long term memory

(Chedd et al., 2006). This may be unintentionally misunderstood by the educator as

disobedience as the child is unable to process pieces of the directions (Chedd et al.,

2006).

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Another factor that would likely impact on Harley’s classroom experience is his low

muscle tone and strength. In Harley’s case, this lack of strength has negatively

impacted his fine motor skills and as such he may experience difficulties participating

in tasks such as drawing or writing (Chedd et al., 2006). Children who suffer from

Prader-Willi syndrome may also struggle to engage fully in tasks that require

substantial motor planning skills, so much so that simple tasks such as moving

around the classroom or changing rooms may prove challenging (Chedd et al.,

2006).

Another issue that is highlighted in the case study is Harley’s lack of expressive

language. This is a common characteristic among children with Prader-Willi

syndrome, as their expressive language usually lags behind their receptive language

or word comprehension (Chedd et al., 2006). This delay in language development

can negatively affect a child’s ability to communicate their wants and interests with

an educator, whilst also decreasing the opportunities for the child to interact

meaningfully with their peers (Lane et al., 2006).

Planning and Assessing Considerations

When planning for Harley, an educators programs should aim to address his specific

needs including use of expressive language, physical therapies, supervision around

food, and the development of a regular, healthy, exercise pattern ((Dryden, 2019;

Chedd et al., 2006). Given that visual processing is a strong cognitive skill for

students with Prader-Willi syndrome (Rosner et al., 2004), an educator should aim to

plan lessons that capitalize on these strengths whilst minimizing weaknesses in

other areas such as auditory short-term memory (Dryden, 2019). Another important

aspect that must be considered within planning is enhancing opportunities for Harley

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to engage in positive social interactions with his peers (Chedd et al., 2006;

Westwood, 2015).

In terms of assessment, Harley may experience difficulties demonstrating his

understanding of the content if the method of assessment requires him to use

language and motor skills that he does not possess (Westwood, 2015). This may

mean that Harley will require modifications to the format of an assessment, variation

in the mode of responding, or allocated additional time to complete a set task

(Westwood, 2015; Chedd et al., 2006).

Who is Responsible?

A variety of professionals play a key role in meeting Harley’s unique needs, namely a

physical therapist, occupational therapist and speech therapist (Scott et al., 1999;

Chedd et al, 2006). A physical and occupational therapist can work with Harley to

develop strategies to support his fine motor skills and motor development, whilst a

speech therapist can be helpful in working with Harley to improve his expressive

language skills, articulation, and voice quality (Chedd et al., 2006).

The educator as well as the child’s parents also play a major role in improving

Harley’s educational outcomes (Scott et al., 1999; Chedd et al, 2006). It is imperative

that teachers and families work closely together to share information regarding

routines and positive behaviour supports, so that these can be implemented both at

home and at school (Scott et al., 1999; Chedd et al, 2006). It is also important that

the classroom teacher incorporate goals from the child’s therapy into the regular

curriculum and classroom activities (Chedd et al., 2006). Lastly, the child themselves

also must be actively involved in monitoring their own behaviour and achievement

(Scott et al., 1999).

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Strategies to Adapt / Modify / Differentiate

In order to minimise Harley’s stress and anxiety surrounding his daily routine inside

the classroom, an educator must first create a predictable and consistent learning

environment (Dryden, 2019; Chedd et al., 2006). To do so an educator may provide

Harley with a visual schedule outlining the day’s events as well as the time and

duration of specific activities, which he may use to remind himself of his routine at

times when he is feeling particularly anxious or uncertain (Dryden 2019; Chedd et

al., 2006). To ease Harley’s stress surrounding transitions, an educator may provide

Harley with an anticipatory object, such as a ball when going to physical therapy or a

toy when going outside, which may aid in Harley’s adjustment to certain activities

(Chedd et al., 2006). It is also imperative that the educator prepare Harley for any

changes to his schedule, such as a field trip or school event, ahead of time in order

to reduce any distress and anger that he may feel (Dryden, 2019; Chedd et al.,

2006).

To offset Harley’s hyperphagia as a result of his condition, an educator must strictly

regulate what he eats throughout the day. Many children with Prader-Willi syndrome

are known to display food seeking behaviours such as stealing food, eating non-food

items and binge eating, which if not controlled may result in life-threatening obesity

(Dryden, 2019). It is therefore important that during school hours Harley is closely

supervised in all areas where food is readily available, and that access to food is

eliminated as far as possible (Dryden, 2019; Chedd et al., 2006; Scott et al, 1999).

To do this, an educator may store food outside the classroom in high or locked

cabinets, and serve food in appropriate portions for each individual child as opposed

to allowing children to serve themselves (Chedd et al., 2006; Dryden, 2019).

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Furthermore, food should never be offered as a reward for good behaviour (Dryden,

2019; Chedd et al., 2006; Scott et al., 1999).

As well as a focus on diet, educators must increase the amount of time devoted to

physical education and fitness in order to develop behavioural patterns of life-long

exercise and physical activity (Scott et al., 1999; Westwood, 2015). Within the

classroom, physical activity can be used to develop Harley’s strength, coordination,

balance and motor planning skills that have been negatively affected by his condition

(Chedd et al., 2006). In order to cater for Harley, an educator should incorporate

non-competitive activities into their program such as walking, low-impact aerobics,

and group exercise (Chedd et al., 2006). More so, it imperative that Harley is not

isolated from his peers during physical activity, but instead integrated with typical

developing children (Fidler et al., 2003; Westwood, 2015).

Given that Harley process information slower than his peers, an educator must put in

place strategies to aid in his attention and work completion (Dryden, 2019). Firstly,

when presenting Harley with verbal instructions for a task or project, they should be

broken down into smaller, more manageable steps with ample time given to process

each piece of information (Dryden, 2019; Chedd et al., 2006; Westwood, 2015).

Given that visual processing is a strong skill for children with Prader-Willi syndrome,

an educator should also make use of visual aids, such as photos, illustrations, and

videos, when presenting Harley with new information (Chedd et al., 2006; Rosner et

al., 2004). When learning new concepts and skills, Harley would also benefit from

high-quality explicit instruction whereby new knowledge and skills are taught through

direct teaching and modelling (Westwood, 2015).

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Given that Harley’s fine motor skills aren’t as developed as his peers, an educator

must also minimize his writing demands within the classroom by providing alternative

ways to complete a set task (Chedd et al., 2006). For example, rather than writing an

answer to a question, he may instead check an answer from a multiple-choice format

(Chedd et al., 2006). It is also important for Harley that unstructured, idle time within

the classroom setting is avoided as this can easily lead to maladaptive and

obsessive behaviours, such as repetitive questioning (Chedd et al., 2006; Dryden,

2019). If this behaviour becomes an issue for Harley, it may prove useful for the

educator to write down the answer to a preservative question on a piece of card that

Harley can refer back to if the behaviour was too reoccur (Chedd et al., 2006).

In terms of his social development, Harley must be explicitly taught the specific skills

and behaviours that will enhance his relationship with his peers (Chedd et al., 2006).

Given that Harley has issues understanding some of the more subtle aspects of

socialization, the educator must teach Harley appropriate social skills through a

combination of modelling, role play, rehearsing, and receiving feedback (Westwood,

2015; Chedd et al., 2006). To further enhance Harley’s social development, an

educator should also utilise group work within the classroom. By doing so Harley will

effectively be building his social skills as he interacts with his peers (Westwood,

2015).

Communicating with Parents

In terms of communicating with parents, Chedd et al. (2006) states sending a

notebook or diary containing information such as the child’s successes throughout

the day, any strategies that worked well, and any behavioural issues, back and forth

between home and school can help maximise consistency for the child. It would also

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be beneficial for Harley’s development if his caregivers met regularly with the

educator to discuss his progress within the classroom (Chedd et al., 2006).

Harley is a year 1 student who has been diagnosed with Prader-Willi syndrome, a

chromosomal disorder that results from missing genetic material on chromosome

fifteen (Fidler et al., 2003; Dryden, 2019; Prader-Willi Syndrome Association of

Victoria, n.d.). Whilst the characteristics of this condition differ from person to person,

for Harley some of the side effects he experiences include a lack of expressive

language, difficulty interpreting subtle social cues, obsessive behaviours, lack of

short-term auditory memory, and poor fine motor skills (Chedd et al., 2006; Dryden,

2019; Rosner et al., 2004). Given that all of these factors can have a significant

impact on Harley’s classroom experience, an educator must adapt and modify their

curriculum so that Harleys social, emotional, and academic needs are met. Some of

the ways an educator can do this is by explicitly teaching social skills, maintaining a

regular schedule within the classroom, controlling access to food, incorporating daily

physical activity, and breaking large tasks down into smaller parts (Chedd et al.,

2006; Westwood, 2015; Dryden, 2019; Scott et al., 1999). Lastly, an educator must

maintain regular communication with Harley’s primary caregivers in order to ensure

his success both inside and outside the classroom (Chedd et al., 2006).

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References
Atkin, K., & Lorch, M. P. (2007). Language development in a 3-year-old boy with

Prader-Willi syndrome. Clinical linguistics & phonetics, 21(4), 261-276.

https://doi.org/10.1080/02699200701243865

Chedd, N., Levine, K., Wharton, R.H. (2006.). Educational Considerations for

Children with Prader-Willi Syndrome. In: Butler M.G., Lee P.D.K., Whitman

B.Y. (Eds.) Management of Prader-Willi syndrome (p.302-316). Springer.

https://doi.org/10.1007/978-0-387-33536-0_11

Dryden, M. (2019). What school psychologists should know about Prader-Willi

syndrome. Communiqué (National Association of School Psychologists),

47(5), 4-6.

Fidler, D. J., Lawson, J. E., & Hodapp, R. M. (2003). What do parents wants? An

analysis of education-related comments made by parents of children with

different genetical syndromes. Journal of Intellectual & Developmental

Disability, 28(2), 196-204. https://doi.org/10.1080/1366825031000147120

Lane, J. D., Lieberman-Betz, R., & Gast, D. L. (2016). An analysis of naturalistic

interventions for increasing spontaneous expressive language in children with

autism spectrum disorder. The Journal of Special Education, 50(1), 49-61.

http://doi.org/10.1177/0022466915614837

Nationally Consistent Collection of Data of School Students with Disability. (n.d.).

Harley, Supplementary, Cognitive.

https://www.nccd.edu.au/case-studies/harley-supplementary-cognitive

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Prader-Willi Syndrome Association of Victoria. (n.d.). An introduction to Prader-Willi

Syndrome. http://www.pws.org.au/wp-content/uploads/2016/10/Introduction-

to-PWS-brochure.pdf

Rosner, B. A., Hodapp, R. M., Fidler, D. J., Sagun, J. N., & Dykens, E. M. (2004).

Social competence in persons with Prader-Willi, Williams and Down’s

Syndrome. Journal of Applied Research in Intellectual Disabilities, 17(3), 209-

217. http://doi.org/10.1111/j.1468-3148.2004.00200.x

Scott, E. M., Smith, T. E. C., Hendricks, M. D., & Polloway, E. A. (1999). Prader-Willi

syndrome: A review and implications for educational intervention. Education

and Training in Mental Retardation and Developmental Disabilities, 34(1),

110-116.

Westwood, P. (2015). Commonsense methods for children with special educational

needs (7th ed.). Routledge Falmer.

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