EDUC2000 Assessment 1Individual Case Profile

Ricky Lee

Student ID 18237508

Background

Liam Johnson, an age 16 male, is a senior high school student in year 11 at a private

school, and has suffered from Becker muscular dystrophy (BMD) – a variant of

Duchenne muscular dystrophy (DMD) – since age 10. Liam was asymptomatic in the

first year of his life and reached several gross motor skill milestones, such as sitting,

standing, and rolling, however he learnt to walk later than normal at the age of 16

months. In the years before the onset of his disability, he experienced cramps/pains

in the calves, had some difficulty in walking and running and experienced slowly

progressing weakening of the muscles. In his early adolescence, after clinical

examinations and blood tests he was diagnosed with a learning disability but has

progressed with tutorial assistance. He does not take any steroid medication and

remains ambulatory, but compensates his weakening muscles with a waddling gait.

He also experiences shortness of breath and fatigue. Liam mainly uses physical

therapy and bracing to combat contractures and the risk of heart failure due to

cardiomyopathy. No other immediate or distant family member if Liam’s family has

any musculoskeletal difficulties

The Learning Disability

Belonging to a group of disorders known as dystrophinopathies, severe Duchenne

and milder Becker muscular dystrophy are neuromuscular disorders – caused by the

deficiency or impaired function of the protein dystrophin associated with mutations in

the dystrophin gene (Dastur, Gaitonde, Khadilkar, & Nadkarni, 2008; Socialstyrelsen,

2013). Muscular dystrophy is genetic regardless of type, but not all family members

may have this condition, sons of a female carrier have 50% chance of inheriting the

disease and a man with the disease cannot pass it on to his sons but his daughters

will become healthy carriers (Parenting and Child Health, 2016; Socialstyrelsen,

2013).

This genetic disorder is characterised by progressive muscle wasting and weakness,

causing a decline in muscle strength as the muscle degenerates (Muscular

Dystrophy Foundation Australia, 2012). While DMD can show signs of muscle

weakness as early as age 3 but as a milder version of DMD, BMD usually onsets in

the teens or early adulthood, and is slower and less predictable than DMD, with

affected individuals remaining ambulatory beyond 16 years and some leading near

normal lives. Muscle degeneration with BMD usually begins with the hips, pelvic

area, thighs and shoulders; degeneration rate may greatly vary from person to

person. (Muscular Dystrophy Foundation Australia, 2012; Wang, et al., 2011;

WebMD, 2016)

BMD can cause discomfort, pain, restricted mobility and impediment of daily

activities through conditions such as contractures which cause extreme discomfort

during a range of motion therapy, and scoliosis which can result in back pain and

interferes with sitting and sleeping (Zebracki & Drotar, 2008). BMD is also associated

with cardiomyopathy, a heart condition which weakens the cardiac muscle,

preventing the efficient pumping of blood and can cause shortness of breath, fatigue,

a feeling of lethargy, palpitations, fainting attacks and sometimes chest pains

(Genetics Home Reference, 2012; Cardiomyopathy Australia, 2016).

There is no current cure for muscular dystrophy but conditions can be improved and

muscle deterioration can be slowed by working with a team of specialists such as

neurologists, orthopaedists, pulmonologists, physical therapists and cardiologists

(Muscular Dystrophy Foundation Australia, 2012). Physical therapy and bracing can

help maintain muscle tone and raise flexibility, reducing joint contracture severity;

exercises to keep the back straight and surgery (spinal fusion) can reduce the pain

and severity of scoliosis; medication belonging to a group called corticosteroids are

effective at slowing the course of muscular dystrophy but data for or against its use

with BMD is lacking and there are side effects such as weight gain and lower bone

density; splints, standing frames and wheelchairs (manual and power) can help with

mobility, voice activated software can assist social interaction and learning for

individuals with affected mobility and hand control (Muscular Dystrophy Foundation

Australia, 2012).

Challenges in the secondary school context

Physical

As Liam does not use any movement aids and is ambulatory, he has much greater

mobility than an individual that relies on them, but due to due to muscle weakness

and being prone to fatigue, he is not able to fully participate in physical education

classes, however this does not mean that they should be excluded. There are many

benefits for individuals with muscular dystrophy if they stretch their muscles and be

as active as their condition permits (Muscular Dystrophy Association of New

Zealand, 2013).

To effectively adapt lessons to allow as much participation as possible in this

scenario, the teacher should set realistic goals for the Liam, such as walking and

light jogging a lap around the oval while the others run 2 in the same time, adjusting

activities such as by changing the size of the court for a game of soccer and giving

him a different role such as referee or score keeper during events like sports days so

that he is still included in the event.

Social/behavioural/emotional

The limitations that are placed on Liam by his disability can make it difficult for him to

keep up with others during activities, sports and games. Activity restrictions have

been shown to increase social isolation which can consequently impede the

development of independence and self-identity (Zebracki & Drotar, 2008).

Some children who are ill feel ‘different’ than their peers may be hesitant to approach

them in social relationships, blame their condition for their problems and be absence

from school for medical appointments which can lead to fewer opportunities to

develop interpersonal skills (McDevitt, Ormrod, Cupit, Chandler, & Aloa, 2013).

Therefore educators should work to facilitate peer relationships as much as possible

by adapting activities, working to foster positive understanding towards disability

within peers and train their social skills to improve quality of life and provide

emotional support.

Cognitive development

Individuals with BMD do not usually have cognitive problems and are not as severe

as those in DMD if present (Kerr & Butterfield, 2013). Doctors believe that there may

be subtle cognitive and behavioural deficits due to dystrophin abnormalities in the

brain and that learning problems in people with BMD occur in 3 general areas –

attention focusing, verbal learning and memory (Muscular Dystropy Association,

2016).

Home Environment

Liam comes from a supportive family that is relatively well off and is able to provide

for any needs that he has, his family is very much supportive but his mother is

somewhat overprotective of him due to his disability. Liam’s parents have

researched the topic of his disability and understand what is needed for his treatment

and have also communicated with the teachers so that they understand Liam’s

needs.

By being able to provide for his needs, Liam’s parents make sure that he has the

ability to maintain his health, seeking the right information and help when needed. As

Liam’s mother is overprotective of him, there is a risk that Liam will find it hard to

become independent and nurture self-esteem and to feel in control of his life.

Parents, carers and families are the most important influence in a child’s life, helping

shape the attitudes and values that support young people in their education,

therefore it is essential for educators to understand and build partnerships with them

to maximise student engagement and achievement (Ministerial Council on

Education, Employment, Training and Youth Affairs, 2008). By connecting with the

teachers in their son’s school Liam’s parents have ensured that they understand

what Liam is going through and what is outside his control so that they are able to

accommodate him, creating individual educational plans to help him, making their

lessons more inclusive.

Overall School Experiences

1. As his condition made it so that he physically weak and easily tired, Liam found it

hard to do any exercise that was too intense such as physical education lessons and

was unable to participate in many activities or made to sit out which made him feel

socially isolated from his peers. This can be remedied by practicing inclusion,

adjusting the lessons to allow him equal participation.

2. One of the problems that Liam faced after developing his condition was social

issues, as he was teased and at times bullied, for simply being different to the other

children and not being able to keep up with them in physical activities. This caused

him to feel socially isolated and feel that approaching others was difficult, resulting in

few friends through the years and a low self-esteem. In this case it is necessary for

the educator to explain to the other students what Liam was going through and to

practice inclusion as much as possible, in or outside the classroom and to stop

bullying immediately when seen.

3. The private school that Liam goes is spacious and has many two-story buildings.

One of the daily challenges that Liam faced was in navigating in this environment as

like most secondary schools, different subjects are held in different classrooms,

requiring the students to move constantly between lessons and breaks, sometimes

from one end of the campus to the other. With his condition making it harder to

traverse long distances and climbing stairs regularly difficult due to gradual muscle

weakness, Liam was often late to his classes. As this is an issue that is outside of

the student’s control, it requires the teachers to think of ways to accommodate him,

such as giving more time to move between classes and consideration of the routes

he needs to take.

Consent Form

References

Cardiomyopathy Australia. (2016). CARDIOMYOPATHY EXPLAINED. Retrieved

from http://www.cmaa.org.au/cardiomyopathy-explained.html

Dastur, R., Gaitonde, P., Khadilkar, S., & Nadkarni, J. (2008). Becker muscular

dystrophy in Indian patients: Analysis of dystrophin gene deletion patterns.

Neurology India 56(3), 374-8. Retrieved from http://search.proquest.com.db

gw.lis.curtin.edu.au/docview/236939916/7710AEAD3F5F42E1PQ/2?

accountid=10382

Genetics Home Reference. (2012). Duchenne and Becker muscular dystrophy.

Retrieved from https://ghr.nlm.nih.gov/condition/duchenne-and-becker-

muscular-dystrophy

Kerr, L. M., & Butterfield, R. (2013). Duchenne and Becker Muscular Dystrophy.

Retrieved from https://www.medicalhomeportal.org/diagnoses-and-

conditions/duchenne-and-becker-muscular-dystrophy

McDevitt, T. M., Ormrod, J. E., Cupit, G., Chandler, M., & Aloa, V. (2013). Child

Development and Education. Frenchs Forsest: Pearson Australia.

Ministerial Council on Education, Employment, Training and Youth Affairs. (2008).

Melbourne Declaration on Educational Goals for Young Australians.

Muscular Dystrophy Association of New Zealand. (2013). A teacher’s guide to

Duchenne muscular dystrophy in primary schools. Retrieved from

http://www.mda.org.nz/media/23708/2013%20Duchenne%20MD%20primary

%20manual.pdf

Muscular Dystrophy Foundation Australia. (2012). Facts about Duchenne and

Becker Muscular Dystrophies. Retrieved from Muscular Dystrophy WA:

http://mdaustralia.org.au/wp-content/uploads/2012/07/002_ducene_becker-

july-2012.pdf

Muscular Dystropy Association. (2016). Becker Muscular Dystrophy (BMD).

Retrieved from https://www.mda.org/disease/becker-muscular-dystroph

y/signs-and-symptoms

Parenting and Child Health. (2016). Physical Disability. Retrieved from http://www.c

yh.com/HealthTopics/HealthTopicDetails.aspx?p=114&np=306&id=1874

Socialstyrelsen. (2013). Becker muscular dystrophy . Retrieved from https://www.s

ocialstyrelsen.se/rarediseases/beckermusculardystrophy

Wang, Q., Yang, X., Yan, Y., Song, N., Lin, C., & Jin, C. (2011). Duchenne or Becker

muscular dystrophy: A clinical, genetic and immunohistochemical study in

China. Neurology India 59(6), 797-802. Retrieved from http://search.pr

oquest.com.dbgw.lis.curtin.edu.au/docview/916768592/7710AEAD3F5F42E1

PQ/7?accountid=10382

WebMD. (2016). Understanding Muscular Dystrophy - the Basics. Retrieved from

http://www.webmd.com/children/understanding-muscular-dystrophy-basics

Zebracki, K., & Drotar, D. (2008). Pain and activity limitations in children with

Duchenne or Becker muscular dystrophy. Developmental Medicine and Child

Neurology 50(7), 546-52. Retrieved from http://search.proquest.com.db

gw.lis.curtin.edu.au/docview/195603707/7710AEAD3F5F42E1PQ/19?

accountid=10382