EDAC225 Assessment 1 Melissa Kelly

EDAC225: Family Studies & Disability

Discussion Paper (Case Study)

This article examines the context and condition of Evie, a 5 year old girl who was
diagnosed with type 1 Usher syndrome at the age of 3 years old (Better Health
Channel, 2011). She is living with her mother, father and brother (7 years old) within the
outer suburbs of Melbourne and suffers from little language delay, few balance
problems and presently, moderate vision impairment (Dammeyer, 2012). Following a
newborn hearing screening, Evie was given a diagnosis of profound congenital
sensorineural hearing loss by an otorhinolaryngologistwith a hearing range of 90-110
decibels (Dammeyer, 2012; United States National Library of Medicine, 2012),. This
meant that Evie was unable to hear almost all sounds (Australian Hearing, 2010).

Upon recommendations from their otorhinolaryngologist, followed by extensive

research by her mother, the decision was made to move forward with simultaneous
bilateral cochlear implantation at the age of (Abbas, Brown, Gantz, Hughes, Lowder,
Preece, Rubinstein, Tyler, & Wolaver, 2002; Buchman & Das, 2005; National Institute
for Health and Care Excellence, 2013). Cochlear implants can aid in an increase in
auditory skills, speech understanding and oral linguistic development by directly
stimulating the cochlea and are extensively recommended as early in life as possible
(Cochlear, 2013; Henricson, Lidestam, Lyxell, Mller, & Wass, 2012; May-Mederake,
2012). Additionally, bilateral implantation can assist in determining the orientation of
sounds, speech understanding and intelligibility of speech (Abbas, Brown, Gantz,
Hughes, Lowder, Preece, Rubinstein, Tyler, & Wolaver, 2002; Balkany & Brown, 2012).
This procedure has been shown to make the age-appropriate language development
achievable for the profoundly deaf and greatly aided Evie to be able to develop speech
at the typical age (Archbold & ODonoghue, 2009).

At 28 months, Evies parents first noticed signs that she might suffer from

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balance issues and night blindness; when she would stumble and fail to see others in
dimly light environments (Boystown National Research Hospital, 2012). Consultations
with the optometrist diagnosed retinitis pigmentosa, a disease that causes damage to
the retina and causes sight to diminish, starting with night vision, then peripherals until
complete blindness (Better Health Channel, 2011; Brown, Fishman, & Grover, 1998).
From there Evie was tested for the autosomal recessive disorder and was found to have
mutations in the myosin VIIA gene, evidence of Usher type 1b (Moller, 2003).

Throughout Evies life so far, she has required various levels of support from a
speech-language pathologist, an audiologist, an ophthalmologist (to regularly monitor
the regression in Evies eyes), and an art therapist (Boystown National Research
Hospital, 2012). An art therapist was recommended by Evies early childhood educator.
This person supports her in dealing with understanding the condition, any concerns she
has over differences between herself and other children (the cochlear implant), and
helping her prepare for what changes she will face in the future; both the physical
developments of her condition and changes in social dynamics when she will begin
school. Evie may in the future also need a orientation and mobility (O&M) instructor, as
this will allow Evie to develop skills she may need as her sight declines (cane travel) as
well as safe skills for situations when her sight is not efficient, such as power outages
(Boystown National Research Hospital, 2012).

Obtaining a diagnosis early in life allowed Evie and her family to foster
partnerships with such practitioners in order to create a family-centred atmosphere and
promote optimal development and teaching behaviours that take into account
deterioration over time (Boystown National Research Hospital, 2012; Porter, 2012).
Furthermore, this early intervention and support has allowed Evie to maintain a fairly
typical lifestyle for a 5 year old; developing friendships, playing games. Her condition
may also be attributed to an increase in curiosity and empathy towards the deaf blind
community and people with other physiological disabilities.

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Partnership between families, teachers and the wider

community

Evies condition has had a profound impact on the dynamics and lifestyle of her
family, both positive and challenging effects. The initial diagnosis of profound hearing
loss came as a shock to both parents, who had already raised a healthy boy, and had
just received a newborn girl with the same hopes and dreams attached (Needham &
Needham, 2012). Intense concerns over their daughters ongoing quality of life became
the driving force for research into rehabilitation possibilities and locationing of support
agencies, and created an environment of inquiry and research within the household.
This focus on appointments and therapy for Evie developed into tension and jealousy
from her brother, who was not only struggling with a needy new sibling to compete for
attention with.

Both parents were forced to face misconceptions they held about deafness and
blindness; that Evie would not be able to talk, hear or understand speech, that hearing
aids are the only possible implants, that she could have very poor quality of life being
deaf blind and that she would not be able to achieve a level near to typical development
(Action on Hearing Loss, n,d; Gundersen, Kongsrud, Lund, 2005; Mouvet, Matthijs,
Loots, Taverniers, & Van Herreweghe, 2013). There was additionally feelings of guilt as
her parents came to terms with the fact that Usher syndrome is an autosomal recessive
inheritance, thereby, a condition resulting from a condition both parents carriers of
(Centre for Genetics Education, 2013).

Evies father had no previous knowledge of cochlear implants and had only
encountered a moderately deaf child during his own childhood who did take to their
hearing aid and preferred to just rely on sign. Consequently, he took the news that Evie
suffered from hearing loss as her being reliant on others understanding sign language
for the rest of her life. Alternatively, Evies mother became purely focused on finding
every possible resource for helping her development on an age-appropriate level. This
determination led to high stress levels and less quality time within the nucleus family.

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Evies grandparents held many misconceptions regarding these sensory

impairments as a sign of diminished intelligence and inability to living an average life,
coming from an age when such children were most often institutionalised (Darling &
Seligman, 2007). The diagnosis of Usher syndrome similarly raised concerns from her
parents about her capacity for cognitive skills, however, research showed that apart
from possible issues with semantic decision making, children with type 1 Usher
syndrome and bilateral cochlear implants(CI) generally show increased development
and working memory than other deaf children with CI (Henricson, et. al., 2012).

Individual Support
With a condition such as Evie's, there is a need for highly trained professionals to
provide intensive differentiated instruction (Boystown, 2012). Initially this would have
focused on developing listening and spoken language which is best addressed early on
as the younger a child is exposed to auditory stimuli the more likely they appear to be
able to understand spoken language and use spoken language themselves (May-
Mederake, 2012). With the early cochlear implantation, it will have a positive influence
of language as well as general development however, particular note should be taken of
the deaf community; possessing its own language (sign language), pattern of beliefs,
values, customs, arts, institutions, social forms and knowledges it provides a rich
context of support for such conditions (Ashman & Elkins, 2002; Deaf Australia, 2013;
Spencer & Marschark, 2003,as cited in Mouvet, et. al., 2013). Specialists should
consider the benefits and complications of bilingual approach to communication,
integrating sign into the languages spoken at home and school, but attempt to create
consistent communication methods wherever possible throughout development
(Mouvet, et. al., 2013).

Nonetheless, support from such professionals is meaningless unless continued

into the wider social systems of the child's life (Bronfenbrenner, 1979). It is imperative
that parents and family members have been educated on the types of strategies
professionals are employing in order to make these consistent with the home

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environment as all factors and changes are interconnected (Bowes & Grace, 2009;
May-Mederake, 2012).

This can similarly be applied to the school environment. Families should search
out educational institutions with a extensive whole school approach to the principles of
inclusion, access and equity; that includes positive attitudes to professional
development and community engagement (Meldrum & Peters, 2011). Professionals can
use this opportunity to reflect on the beliefs, perceptions and attitudes within a context
and how this can affects a childs disability; particularly encompassing a focus on
activity limitations and participation restrictions, which should be differentiated from the
impairment (Bowes & Grace, 2009; World Health Organisation, 2013). Educators should
avoid interindividual assessment and aim on creating the least restrictive environment
for deaf/blind children. This may include accommodations such as having lesson plans
available for the child and their learning support (if applicable) prior to the class, to allow
the student to review the material prior to class and thereby be less likely to be left
behind information is not delivered correctly during class (Ashman & Elkins, 2002). The
child could also benefit from writing backup of content when oral communication is
taking place. Other strategies for creating the most ideal environment for a deaf/blind
child to access learning within the classroom are;
always facing the class/student when speaking to allow for lip reading
placing the students chair where power points are accessible to accommodate
for assistive information communication technology (ICT) devices
debrief with the student to monitor their engagement
create a reflective diary to assess the relationships between student involvement,
lesson plan and content.
(Ashman & Elkins, 2002)
Above all, however, it is stressed that professionals and family members hold high
(though realistic) expectations of a child. That they may develop positive self-esteem
and and perceived quality of life and outlook for the future.

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EDAC225 Assessment 1 Melissa Kelly

Application to professional values

Family systems theory provides a framework for understanding the diverse
organisation and relationships within a family (Constantine, 1993). Understanding of this
theory has helped me to consider the differences not only in the types of families;
nuclear families, same-sex couples, number, sex and age of children, and abilities (the
structure), but also the intricacies of relationships within this paradigm (Morgaine, 2001).

In the case of Evie, her condition inadvertently creates stress and demands that
leave less attention available for maintaining the relationships between husband and
wife, parent and son. This in turn creates a negative emotional atmosphere within the
sibling subsystem at a very early stage in their relationship.

Furthermore, I must recognise that within a family system, each element has its
own characteristics (Morgaine, 2001). As such, one must not assume that there is also
agreement on certain things. One parent may be intent on cochlear implants, while the
other may want the child to have the opportunity to embrace the deaf community, whilst
another family member may doubt a deaf/blind childs cognitive ability to advance at all.
I have seen through learning AUSLAN that some deaf customs can seem odd or rude;
such as my teachers sign name, which means fat. To the hearing students, this was a
horrid thought that that could be a persons nickname within this culture, but to her it
was a name given to her early in life by deaf friends and is an affectionate label.
Additionally, there could be varying levels of preferred involvement in the rehabilitation
process as far as learning sign language or maintain professional strategies into the
home environment. Many parents may take the birth of a deaf child as a challenge or
endearing trait, while some are left with a feeling of psychological or functional
inadequacy (p. due to their inability to use intuitive communication (Meadow-Orlans
and Spencer, 1996, as cited in Mouvet, et. al., 2013). However, in most cases, a
variation of these are felt by individuals at different stages.

In a sense, the inclusion we maintain for all differences in children must be maintained
for parents; particularly when we could provide valuable support for parents struggling in

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a novel situation such as the birth of a disabled child. As such, as an educator, I must
have and represent myself as being open to all thoughts and opinions from those
around me, and understand that each situation is an opportunity for professional
development.

*Evie was created for the purposes of this case

study and does not represent any real individual

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EDAC225 Assessment 1 Melissa Kelly

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