DEMOGRPHIC DETAILS
Joy
Jaazaniah, a girl starts out as a happy and healthy baby. She is the eldest of
3 siblings. She is 12 years old now. Normal development like any other child
grows. Then, around 6-18 months of life something goes terrible wrong. She
loses the ability to speak, she can’t use her hands to play with toys or even
feed herself and she may lose the availability to walk. Our local hospital
unable to diagnosed. Some doctor say could be Autistic, Cerebral palsy or any
rare diseases. We are unable to comprehend with speculations. Her parents did
final attempt, blood sample taken; send to Japan to find out what is wrong with
her. After several months we found out that she is diagnosed with Rett
Syndrome.
Have
you heard of “Rett Syndrome” I haven’t until my niece Joy Jaazaniah diagnosed
with it. What is Rett Syndrome? Through my assessment I will share how my niece
and rest of the girls diagnosed with Rett Syndrome nature, and appearance of
this syndrome had impacted physical, cognitive progress and socio emotional by collaborating
the two theories.
OBSERVATION
SETTINGS
My
observation carried out in various situations from the day she was born till
today. On a closer observation, a difference is seen from normal child growing
and special child like my niece. Rapidly observed during crucial time when I
took Child Physiology course. Rett Syndrome is a genetic neurological disorder
that affects girls between 6 – 18 months of age. The regression decease leads
to a lifelong handicap. My first observation was her floppy legs movements
indicate difficulty with balance and coordination. She started too walked on
her toes unlike other children. She is unable to express her feel of
excitement, anger, sadness or pain. She unable to articulated or says any
single words as she grows. Even thought when she was 12 months “Tertiary Circular Reaction” she is able
to say first recognizable words. Shaking different toys to hear sounds and
searches for object that have been concealed. After diagnosed gradually she had
severe communication impairments. She communicates with me through her smiles,
facial expression and eye contact. I spend most of my time talking to her,
reading spiritual book and children books.
DEVELOPMENT
MILESTONES
COGNITIVE
DEVELOPMENT
Rett
Syndrome is a disorder of the nervous systems, caused by mutation on the MECP2
gene location on the X Chromosome, that leads to development reversals. Joy is
unable to demonstrate her intellectual abilities. She cannot verbally
communicate; she is able to point to something, understand our conversation, questions
and she communicate to us through her beautiful eyes. Non verbal communicators like
me required to be attentive to gesture, facial expression, body language, even
the look in someone eyes. This will build her confident and feel secure. Her
intelligence is difficult to measure due to her motor difficulties and lack of
speech. To stimulate her cognitive development we begin with pre-literacy
skills exposure and repetition. I make a point spending time reading books,
play and watch cartoons with her. Learning
is a good for the brain, no matter what the neurological challenges may be. I
also use flash cards for her to know and see her surroundings. She expresses
herself when she is hungry and discomfort by crying or make noise. As she able
to point to something, I experiment Object
Permanence falls under Piaget Stages
of Cognitive Development “Sensorimotor”: 4 – 8 Secondary Circular Reactions. Same
method hid a toy under a blanket, while she was watching and observing whether
or not she search the hidden toy. After several attempt of me demonstrating, I
was happy to see she is trying her best to reach out her hand to move the blanket.
PHYSICAL
DEVELOPMENT
Children
diagnosed with Rett Syndrome tent to display both poor gross and fine motor
skills. A child’s motor ability with Rett Syndrome can be significantly
impacted by motor Apraxia and Ataxia. Motor apraxia is inability of
the body to perform a movement when prompted by the brain. Ataxia, means
without order or in coordination. The combination of motor apraxia and ataxia
clearly negatively affect the child’s ability to perform many gross skills used
in general physical education and other movement settings.
Gross
Motor Development
We
notice regression on Joy Gross Motor Skill when she was between 12 – 18 months.
Her physical Development Milestones regressed instead following the benchmark
of any normal child milestone development. She is unable to perform locomotors
skills such as hopping, jumping, throwing balls, kicking and catch things.
Diagnosed with Rett Syndrome is prone to have a Hypotonia. Lack of muscle tone and therefore may require support
from adult. She typically remains standing still with support while watching
others performs activities. It will take great amount of strength and co
ordination to perform activities. Base on Piaget’s Stage of Cognitive
Development, should be “Concrete
Operational” stage, however with
this diagnosed motor movements can be difficult. Muscles weaknesses, rigidity
or stiffness, dystonia is increased muscles tone with abnormal posturing of
extremity and scoliosis is curvatures of the spine are other prominent features
decline her to move. She needs wheelchair to move around with the support of
adult.
Fine
Motor Development
Loss
of purposeful hand movements; the grasp used to pick up small objects is
repetitive hand motions like wringing or constant placement of hands in mouth
also clasped behind the back or held at the sides. Even thought she was able to
feed herself a cracker or, passing an object from one hand to another and pick
up a tiny object unfortunately she can’t do any more as days goes by. She wants to move and perform the task, but
seems to be unable to tell the body to do. Her floppy arms and legs are the
result of neurological problem rather than lack of exercise. Lately I bought a
dog “Shih Tzu”. Upon my observation I notice her eye contact constantly looking
at the dog and smiling. I took my dog place on her lap. She slowing left her
hand and strokes the dog with a smile. It is really a great achievement for me.
COMMUNICATION
AND LANGUAGE DEVELOPMENTAL MILESTONE
Neurological
problems
like Rett Syndrome affect the muscles needed for speaking. Without ability to
communicate through speech or hand movement she unable to express her needs and
wants. Her non verbal communication is more on facial expression and body
language. She understands basic sound of native language but she unable to
response. She expresses herself by making sound and through her eye contact as
well crying when discomfort.
SOCIO-EMOTIONAL
DEVELOPMENAL MILESTONES
Many
children with Rett Syndrome go through a period of social withdrawal as part of
their regression period. While growing up she cries frequently, avoid eye
contact; she don’t like to go out in public like shopping mall and restaurants.
Noise often irritates her. She shows no interest in surrounding. She preferred
to be alone at home and play with her fingers. Social interactions are challenging for
children with Rett Syndrome at all stages. At the age for two she started to
lose grip associating with her peers and isolated herself by playing alone.
Music also has been found to be an affective motivation tool. I bought some
nice soft music to stimulate her mind; motivating tool that increase social
engagement and receive positive response from her. Sociocultural Theory : Lev Vygotsky “Zone of Proximal development and
Scaffolding” applies in my
family and I confronted as face challenges every day. However, we often find
ways to overcome challenges to create positive environment and learning tools
to help her. Meeting her unique behavioral by supporting creative positive
social skills. Creating opportunities for social interaction and exposing her
to the surrounding. Keep motivating her and give her hugs of love each time she
manage to adapt to the environment.
She
needs a lot of “Scaffolding” support
in Medical Concern. She is prominent to seizures. In additional to seizure,
child with Rett Syndrome often have Gastroesophageal reflux, constipation,
scoliosis and abnormal breathing and poor circulation. All these potential
medical issues can cause delays in the motor domain. She need constant
observation, care and support to go through daily life this in world.
THEORIES
Cognitive-Development
Theory JEAN PIAGET
Relating
his theory on Rett Syndrome child on Piaget stages of cognitive development
apply differently compare to normal child. Joy relates more on Sensorimotor Substages. Rett Syndrome
is a disorder of the nervous systems (Neurological Disorder) that leads to
developmental reversals. Hypotonia,
loss of transitional movements, excessive saliva and drooling, floppy arms,
legs and showing head growth beginning at approximately 5 – 6 months of age.
Believing nature and nurture interact to yield cognitive development for
special child like Joy. It is easy to relate Piaget theory for normal children,
how are we going to relate to special children diagnosed not only to Rett
Syndrome also Autistic, Dyslexia, ADHA and many more. Special need children are
significantly requires intensive accommodations and special learning approach
as well environment to suit their needs. How can we excel Piaget theories Sensorimotor, Preoperational, Concrete
Operational and Formal Operational to our special need children? It is something for me to brain storm,
designed for our potential special needs children.
Sociocultural
Theory: LEV VYGOTSKY
Vygotsky has developed a
socio-cultural approach to cognitive development.Vygotsky
(1978) sees the Zone of Proximal Development as the area where the most sensitive
instruction or guidance should be given - allowing the child to develop skills
they will then use on their own - developing higher mental functions. Vygotsky
also views interaction with peers as an effective way of developing skills and
strategies. He suggests that teachers use cooperative learning exercises where
less competent children develop with help from more skillful peers - within the
zone of proximal development. “Scaffolding”
support help child to enhance skills to leave independence. Special Children
need a lot of support to accomplish certain tasks. As a caregiver we need to be
aware of their capabilities and motor challenges of a child. She can easily
become frustrated or disinterested with an activity that challenging. Finding
out what motivates the child enables the child to stay more focuses on the
task. When we are assisting a child we also need to be sensitive on their movement,
any pain sensation any reaction for improvement. It is also good the keep tract
on child milestones. How long does a child need our support. We being with
simplest form of an activities or skill help the child process the task and
have a success before we moving on to a more complex skill.
