Running head:
BEST PRACTICES 1
Best
Practices in Education for Students with Angelman Syndrome
Rita
Molino-Sell
Walden
University
BEST PRACTICES 2
Abstract
Children with Angelman
Syndrome are globally developmentally disabled creating a variety of needs to
consider for cognitive assessment, placement, and instruction. This paper offers a vision of three stages to
increase the opportunities for children with Angelman Syndrome to participate
in inclusion. Best Practices in
education need to be considered when creating an IEP for an individual: this
includes classroom placement.
BEST PRACTICES 3
Best
Practices in Education for Students with Angelman Syndrome
Angelman Syndrome (AS) is a neuro-genetic disorder that
creates individuals to be globally delayed.
A partial deletion or mutation in maternal chromosome 15 is the cause of
AS. AS was first described in 1965 by
Harold Angelman, who noticed a group of children in his pediatric practice had
flat heads, made jerky movements, had protruding tongues, and had a curious
high rate of smiling, happy demeanor, and bouts of laughter. People diagnosed with AS are often referred
to as Angels due to the name of the condition and also because of their happy
and smiley demeanor.
Approximately 1/20,000 births are diagnosed with AS. Because it is a rare and unknown condition by
many medical and educational professionals, this population is often
underserved in both fields. Often AS
children are misdiagnosed with autism and/or cerebral palsy. This misdiagnosis often leads to
inappropriate educational placement and instruction. AS individuals have unique abilities and
learning styles that need to be considered when assessing and teaching them.
There are listed consensus criteria to describe and
diagnose Angelman Syndrome. It must be
noted that not all Angels will have all of these traits. It is important to remember that each angel
is unique and has his/her own personality traits and learning abilities and
style.
The consensus criteria as per Williams, Beaudet,
Clatyton-Smith, et al. (2005):
·
Developmental delay, functionally severe
BEST PRACTICES 4
- Movement or balance disorder, usually ataxia of gait
and/or tremulous movement of limbs. Movement disorder can be mild. May not
appear as frank ataxia but can be forward lurching, unsteadiness,
clumsiness, or quick, jerky motions
- Behavioral uniqueness: any combination of frequent
laughter/smiling; apparent happy demeanor; easily excitable personality,
often with uplifted hand-flapping or waving movements; hypermotoric
behavior
- Speech impairment, none or minimal use of words;
receptive and non-verbal communication skills higher than verbal ones
Frequent (more than 80%)
- Delayed, disproportionate growth in head circumference,
usually resulting in microcephaly (≤2 S.D. of normal OFC) by age 2 years.
Microcephaly is more pronounced in those with 15q11.2-q13 deletions.
- Seizures, onset usually < 3 yrs. of age. Seizure
severity usually decreases with age but the seizure disorder lasts
throughout adulthood.
- Abnormal EEG, with a characteristic pattern, as
mentioned in the text. The EEG abnormalities can occur in the first 2
years of life and can precede clinical features, and are often not
correlated to clinical seizure events.
Associated (20 - 80%)
- Flat occiput
- Occipital groove
- Protruding tongue
- Tongue thrusting; suck/swallowing disorders
- Feeding problems and/or truncal hypotonia during
infancy
- Prognathia
- Wide mouth, wide-spaced teeth
- Frequent drooling
- Excessive chewing/mouthing behaviors
- Strabismus
- Hypopigmented skin, light hair and eye color (compared
to family), seen only in deletion cases
- Hyperactive lower extremity deep tendon reflexes
- Uplifted, flexed arm position especially during
ambulation
- Wide-based gait with pronated or valgus-positioned
ankles
- Increased sensitivity to heat
- Abnormal sleep wake cycles
- Attraction to/fascination with water; fascination with
crinkly items such as certain papers and plastics
- Abnormal food related behaviors
- Obesity (in the older child)
BEST PRACTICES 5
- Scoliosis
- Chronic
Constipation
Upon reading this long list of traits, one might believe this
population is severely mentally retarded.
Earlier reports and definitions of AS listed severe mental retardation
as the first descriptor of this population (Williams, Fria, Optiz, 2005),
however with an increase in awareness and in depth studies, it has been found
and believed that Angels are able to learn and have a higher level of cognition
and receptive skills than originally thought by professionals (Summers &
Szmarti, 2009).
This population is often overlooked for inclusion opportunities
due to various reasons: inappropriate or
ineffective assessments, untrained teachers, misguided belief systems regarding
inclusion, limited knowledge and awareness of AS, limited funds in school
budgets, lowered expectations for this population, and their various needs.
Inclusion opportunities for any
special needs population is an age old discussion. For decades arguments for both sides of
inclusion or self-contained classrooms has been going on within districts and
parents. Some districts are better
equipped to have successful inclusion programs. The No Child Left Behind Act
(NCLB) 2001 primary purpose was to make schools and teachers accountable for
how well their students are learning. The Individuals with Disabilities
Education Improvement Act of 2004 (IDEA) partnered with NCLB have revised
standards for academic achievement for special needs children, along with the
measurement of their functional performance.
BEST PRACTICES 6
Parents and educators have questions about
inclusion and the term Least Restrictive Environment (LRE).
Many believe that the IDEA requires schools
to practice inclusion. In fact, the term "inclusion" is relatively
new and is not included in the IDEA statute or regulations. The term used in
IDEA is “mainstreaming”.
According to
www.wrightslaw.com:
The IDEA's LRE
or mainstreaming policy, school
districts are required to educate students with disabilities in regular
classrooms with their nondisabled peers, in the school they would attend if not
disabled, to the maximum extent appropriate.
It
is often a misunderstanding that IDEA states that it is the law and a “right”
to have full inclusion for a developmentally disabled child. There are times when a self-contained
classroom would be most appropriate for a given student. However, it is imperative to acknowledge that
all factors need to be considered when making the decision for a student’s
Individualized Education Program (IEP) for full inclusion, partial inclusion,
or a self-contained classroom. Parent
involvement is a critical aspect of the decision making as well.
The
important question to ask is; realistically, can we expect children with
Angelman Syndrome to thrive in inclusion programs or are they destined for
self-contained classrooms? What are the
best practices for this population in regards to assessment, instruction, and
placement?
Best Practices in Education
What
does the term “Best Practices” mean? It
can be defined as “what works” in a particular environment or situation. Grover
J. Whitehurst, as assistant secretary for Educational Research and Improvement
at the U.S. Department of Education in 2001, defined evidence-
BEST PRACTICES 7
based education as “the integration of professional
wisdom with the best available empirical evidence in making decisions about how
to deliver instruction.” The application
of educational theory and research to make decisions for a child’s education
involves more than how to deliver instruction, it also involves decision making
for assessments and placement not just instruction.
According
to the State Education Resource Center (
www.ctserc.org)
there are nine standards to assess Best Practices:
1.
A Clear and
Common Focus; Administrators, teachers,
students, and parents share and commit to clearly articulated and understood
common goals based on the fundamental belief that all students can learn and
improve their performance. There is
clear evidence of school practices to support this belief
2. High
Standards and Expectations: Each teacher
believes “all students can learn and I can teach them.”
Staff members are dedicated to helping
every student achieve challenging state and local standards. All students are engaged in an appropriately
ambitious and rigorous course of study in which the high standards of
performance are clear and consistent and the conditions for learning are
modified and differentiated. This
results in all students being prepared.
3. Strong Leadership: School leadership is focused on
enhancing the skills, knowledge, and motivation of the people in the
organization and creating a common culture of high expectations based on the
use of skills and knowledge to improve the performance of all students.
Leadership fosters a collaborative atmosphere between the school and the
community while establishing positive systems to improve leadership, teaching, and
student performance.
4. Supportive, Personalized Relevant Learning: Supportive learning environments provide
positive personalized relationships for all students while engaging them in
rigorous and relevant learning.
5. Parent/Community Involvement: Parents and community members help develop,
understand, and support a clear and common focus on core academic, social, and
personal goals contributing to improved student performance and have a
meaningful and authentic role in achieving these goals. The school community
works together to actively solve problems and create win-win solutions.
Mentoring and outreach programs provide for two-way learning between students
and community/business members.
6. Monitoring, Accountability, and Assessment: Teaching and learning are continually
adjusted on the basis of data collected through a variety of valid and reliable
methods that indicate student progress and needs. The assessment results
BEST PRACTICES 8
7. are interpreted and applied appropriately to improve
individual student performance and the instructional program
8. Curriculum and Instruction: Align curriculum with core learning
expectations to improve the performance of all students. Students achieve high
standards through rigorous, challenging learning. Staff delivers an aligned
curriculum and implements research-based teaching and learning strategies.
Students are actively involved in their learning through inquiry, in-depth
learning, and performance assessments.
9. Professional Development: Ongoing professional development aligned with
the school’s common focus and high expectations to improve the performance of
all students is critical in high-performing schools. These professional
development offerings are focused and informed by research and school/classroom-based
assessments. Appropriate instructional support and resources are provided to
implement approaches and techniques learned through professional development.
10. Time and Structure:
Flexibly structured to maximize the use of time and accommodate the
varied lives of their students, staff, and community in order to improve the
performance of all students. The structure of programs extends beyond the
traditional school day and year as well as beyond the school building. The
program draws on the entire community’s resources to foster student
achievement.
Assessment, Placement, and
Instruction
Having
every aspect of development being affected from AS, it may prove to be
difficult to accurately assess cognitive abilities. AS individuals are nonverbal, visually
impaired, fine motor and gross motor skills are affected and delayed, many have
severe sensory processing disorders, hyperactivity, limited attention span, and
many have seizure disorders along with other medical and physical issues. Any one of these preceding traits can create
difficulty and limitations in how accurately a child’s cognitive ability can be
assessed. Cooper (2010) found that in
many cases when children who are nonverbal and have significant motor
disabilities (such
BEST PRACTICES 9
as Angelman Syndrome) “may need adaptations of
testing materials or processes, specifically of input (presentation) and output
(response) modes.”
Mukhopadhyay
& Nwaogu (2009) stated that
”Augmentative Alternative Communication has proven to be beneficial in the
instruction of children who are nonverbal
Augmentative and alternative communication (AAC) means any method of
communication that helps an individual with severe communication disorders to
compensate for activity limitation and enhances participation in various
communicative interactions.” Perhaps
further research in the use of AAC for assessment purposes would be
advantageous in finding and implementing best assessment practices for AS
students. There is limited literature on appropriate assessment
practices for this specific population.
It would be beneficial to parents, educators, and AS individuals alike
if further research was done to review assessment tools and practices for this
population.
Best practices
in placement for children with AS and subsequently instruction and curriculum
is a topic that needs further investigation as well. There are many opinions in regards to
inclusion vs. self-contained classrooms.
Implementing best practices in education for this population: assessment,
placement, and instruction will fail to occur
if an increase in awareness and knowledge of Angelman Syndrome does not
happen. Misdiagnosis, misunderstanding,
and underestimating the abilities and capabilities of a child with AS are also
great contributors to the lack of inclusion opportunities for AS children. I have a vision to improve the opportunities
of inclusion. It will take time, effort,
fortitude, and several stages to attain this vision.
BEST PRACTICES 10
Stage 1: Increase Awareness and Knowledge
As stated earlier, misdiagnosis is a
primary contributor to the lack of inclusion opportunities for children with
Angelman Syndrome. Many medical
professionals do not know of this syndrome or lack extensive knowledge of this
disorder to correctly diagnose children.
Often children are misdiagnosed with autism and/or cerebral palsy
(Williams, Lossie, & Driscoll, 2001).
There may be some similarities amongst these conditions; however, there
are many specific identifying behaviors and traits attributed to Angelman
Syndrome. Another contributor to
misdiagnosis could be that parents may be overwhelmed with all of the aspects
in caring for a special needs child and not know that their child is
misdiagnosed.
The first step in achieving an increase in AS
students in inclusion classrooms is to bring awareness, educate, and inform
parents, medical, and educational professionals. In order to accomplish this, I plan on
providing pediatric offices with educational brochures and information regarding
the criteria in diagnosing Angelman Syndrome.
The earlier a child is correctly diagnosed the quicker appropriate
educational and therapeutic plans can be set into place.
Stage
2: Establish the Existing Belief System
Towards Inclusion vs. Self-Contained Classrooms
I will evaluate and analyze various
educational trends. I will look at surrounding districts and assess what is
working and not working within their inclusion classrooms. Next, I will survey
and interview teachers, students, and parents to receive feedback about current
teaching practices. I will also look at current belief systems from educators
and parents on inclusion including any obstacles or apprehensions to pursue
increasing the amount of inclusive
BEST PRACTICES 11
classrooms. In order to establish an existing belief
system in regards to inclusion vs. self-contained classrooms, a
self-administered survey can be implemented for parents and educators. A data
analysis of local districts and the percentage of developmentally disabled
students placed in their home schools inclusively or segregated or placed in a
special school with no opportunity for inclusion. Also a contrast and comparison amongst
districts can be completed to determine satisfaction level of parents and educators
with inclusion policies and practices.
Face to face interviews would also be beneficial to acquire a data base
to establish current belief systems.
These surveys, interviews, and data analysis, hopefully will contribute
to establishing a needs assessment of an increase in the opportunities for
children with Angelman Syndrome to be included in educational settings with
their non disabled peers.
Stage
3: Educate and Inform
Upon completion of gathering and
interpreting data, sharing an opportunity with leaders, professionals, and
parents to educate and inform them on the benefits inclusion and ways to
implement inclusion for students with Angelman Syndrome. Invitations to open forum discussions and/or
lecture style seminars can be beneficial.
Finally, I will develop effective instruction that models and coaches
teachers by implementing strategies that promotes profound learning for the
diverse population in their classrooms. Support staff will be available as
needed for the students with IEPs. Students who require additional services
will be able to access those services within their school day and as push in
therapy (when possible) and not be entirely segregated from their peers. McCarty (2006) stated several benefits to
inclusion. “The special
BEST PRACTICES 12
needs student is
able to develop relationships with peers that are non-disabled. The general education student also benefits
from understanding people with disabilities”.
Academic
benefits are not only for the student with special needs but also for general
education students. “Academic benefits
for general education students include having additional special education
staff in the classroom, providing small-group, individualized instruction, and
assisting in the development of academic adaptations for all students who need
them” (Hines, 2001, P. 3). Research has
also shown that special-needs students in inclusion do better academically and
socially than comparable students in non-inclusive classes (Irmisher, 1995).
There
is research that also depicts disadvantages to inclusion. Increase in support staff equates to
increase in cost to districts. Ongoing teacher training adds to the cost as
well. Some have argued that the
non-disabled students will lose learning opportunities due to an increase in
the special needs students’ disruptive behavior (McCarty, 2006). Educators and parents of children in general
education worry that full inclusion will lower the standard of learning for the
class and make it less of a priority than socializing (Irmsher, 1995).
Conclusion
In
order to follow Best Practices throughout the implementation of the stages to
increase the opportunities of AS students for inclusion, I will follow and
abide by the nine standards listed above.
There is research on inclusion for students with various developmental
delays and disabilities. There is limited research on inclusion for students
with Angelman Syndrome. Leyser &
Kirk (2011) recently published an article on parent’s perspectives on inclusion
and their AS child’s schooling. This
study showed that parents who children who were involved in some form of
inclusion had a more favorable attitude toward inclusion compared to parents
who
BEST PRACTICES 13
were mainly
educated in segregated settings. This
finding reinforces the need to inform parents of children with Angelman
Syndrome all of their options in regard to inclusion. Many parents shared satisfaction with the
positive relationship with teachers they perceived as caring and committed. Yet 40% of the parents in this study were
critical in regards to their relationships with the school and district
administration. Perhaps this is a red
flag for leaders to be more open to improve communication and relationship
status with parents of special needs children.
I
am in complete agreement with Leyser & Kirk (2011) when they stated “future
research should continue to examine the perspectives of parents of children
with different diagnostic characteristics and educational needs regarding
inclusion.” A thorough and in depth
study would benefit this population greatly.
Future research to investigate which forms of alternative assessment and
instruction solicit the best responses from AS children is needed to determine
appropriate classroom placement.
Inclusion
can be cost-effective if approached and implemented correctly. Assessing the individual child’s needs and
what support systems will need to be in place for the teacher and student in
order for inclusion to be considered successful and beneficial to all involved.
A child with Angelman Syndrome presents unique
considerations for inclusion. Perhaps an
additional question that needs to be asked is if total inclusion, partial
inclusion, or self-containment is the most appropriate for a child with
AS? More research needs to be done on
the benefits of inclusion, and partial inclusion (for example part of the day
in self-contained classroom, and part of the day spent in inclusion with non
disabled peers) for students with Angelman Syndrome.
BEST PRACTICES 14
AS
children are capable of learning. They
just have additional special needs that if properly assessed, can thrive in all
areas of academia. In order to step
into their full potential, they need the same opportunity to access all the
benefits their non disabled counterparts have in education.
Finally,
it must be recognized that each individual has his/her own unique needs,
capabilities, learning styles, and disabilities that require individual
attention. Each parent with a special
needs child, specifically Angelman Syndrome, needs to be informed of what their
options are for their child’s education.
The best decision is a well informed decision.
References
Angelman, H.
(1965). Puppet Children: A report of three cases. Developmental Medicine
& Child Neurology, 7(6), 681-688.
Cooper, M. A.
(2010). Cognitive Assessment of Nonverbal young Children Who Have Significant
Motor disability: challenges and
Solutions. Physical Disabilities:
Education and Related Services, XVIII, 19-33.
Hines, R. A.
(2001). Inclusion in middle schools. Urbana, IL: Eric Clearinghouse On
Elementary And Early Childhood Education, University Of Illinois.
Irmsher, K.
(n.d.). Inclusive Education in Practice: The Lessons of Pioneering School Districts.
www.eric.ed.gov. Retrieved October 14, 2011, from
www.eric.ed.gov/PDFS/ED380913.pdf
Leyser, Y.,
& Kirk, R. (2011). Parents' Perspectives on Inclusion and Schooling of
Students with Angelman Syndrome: Suggestions
for Educators. International Journal of Special Education, 26(2),
79-91.
Mukhopadhyay,
S., & Nwaogu, P. (2009). Barriers to Teaching Non-Speaking Learners with
Intellectual Disabilities and Their Impact on the Provision of Augmentative and
Alternative Communication. International Journal of Disability,
Development and Education, 56(4),
349-362. Retrieved from EBSCOhost
Petersen, M. B.,
M.D., K. B., Hansen, L. K., & Wulff, K. (1995). Clinical, cytogenetic, and
molecular diagnosis of Angelman syndrome: Estimated prevalence rate in a Danish
county. American Journal of Medical Genetics, 60(3), 261-262.
Runswick-Cole,
K. (2008). Between a Rock and a Hard Place:
Parents’ Attitudes to the Inclusion of Children with Special Educational
Needs in Mainstream and Special schools.
British Journal of Special Education, 35(3), 17-180. Retrieved from EBSCOhost.
Summers, J.,
& Szatmari, P. (2009). Using Discrete Trial Instruction to Teach Children
with Angelman Syndrome. Focus on Autism and Other, 24(4),
216-226.
Williams, C.,
Beaudet, A., Wagstaff, J., Clayton-Smith, J., Knoll, J., Kyllerman, M., et al.
(2006). Angelman syndrome 2005: updated consensus for diagnostic criteria. American
Journal of Medical Genetics, 140(5), 413-418.
Williams, C. A.,
Lossie, A., & Driscoll, D. (2001). Angelman Syndrome: Mimicking Conditions
and. American Journal of Medical Genetics, 101, 59-64.
