CHARACTERIZING PLAY SKILLS AND IDENTIFYING THEIR ASSOCIATION
WITH EXPRESSIVE AND RECEPTIVE LANGUAGE SKILLS IN YOUNG BOYS
WITH FRAGILE X SYNDROME
A Thesis
Presented to the faculty of the Department of Speech Pathology and Audiology
California State University, Sacramento
Submitted in partial satisfaction of
the requirements for the degree of
MASTER OF SCIENCE
in
Speech Pathology and Audiology
by
Alex Stewart
FALL
2013
CHARACTERIZING PLAY SKILLS AND IDENTIFYING THEIR ASSOCIATION
WITH EXPRESSIVE AND RECEPTIVE LANGUAGE SKILLS IN YOUNG BOYS
WITH FRAGILE X SYNDROME
A Thesis
by
Alex Stewart
Approved by:
__________________________________, Committee Chair
Robert A. Pieretti, Ph.D.
__________________________________, Second Reader
Ann Blanton, Ph.D.
____________________________
Date
ii
Student: Alex Stewart
I certify that this student has met the requirements for format contained in the University
format manual, and that this thesis is suitable for shelving in the Library and credit is to
be awarded for the thesis.
_______________________________
Ann Blanton, Ph.D.
___________________
Date
Department of Speech Pathology and Audiology
iii
Abstract
of
CHARACTERIZING PLAY SKILLS AND IDENTIFYING THEIR ASSOCIATION
WITH EXPRESSIVE AND RECEPTIVE LANGUAGE SKILLS IN YOUNG BOYS
WITH FRAGILE X SYNDROME
by
Alex Stewart
Little is known about the play skills of children with FXS and the relationship of
play and language acquisition in this population. There are no published studies
examining play skills in young males with FXS, even though play skills have been shown
to be directly related to the development of receptive and expressive language. Research
that attempts to identify more efficient and naturalistic language intervention approaches
for children with FXS is needed. This study aimed to provide a descriptive
characterization of play for 11 boys with FXS between the ages of 27 and 51 months.
This study was designed to answer the following research questions: (1) What are the
concurrent correlations between play skills, receptive and expressive language, and
cognitive abilities of young males affected by fragile X syndrome? (2) How can the play
of young males affected by fragile X syndrome be described and characterized?
A series of one-tailed Spearman rank-order correlation (rho) was performed to
provide quantitative data to answer the research questions. A significant correlation was
iv
found between the Mullen Visual Reception raw scores and frequency of Discriminative
Play Actions (r = .667, p = .025, one-tailed) and a marginally significant correlation was
found between the Mullen Visual Reception raw scores and frequency of Total Play
Actions (r= .588, p= .057). The correlations with the Mullen Receptive Language raw
scores were hovering for Object Interest (r= .529, p= .094), frequency of Discriminative
Play Actions (r= .557, p= .075), and frequency of Total Play Actions (r= .538, p= .088).
There was also a hovering negative correlation between the ADOS Autism Severity
Score and Diversity of Play (r= -.559, p= .093). It should also be noted that no
correlations were found between duration of the play sample and any of the play
variables.
Using item analysis of the PLS-5 and level of mastery results from the DPA, a
language and play development chart (see Table 4-1) was created to describe and
characterize the play of young boys with FXS ages 1-5. The information from this study
will provide clinicians with information specific to the developmental trajectory of young
boys with FXS, which may also provide insight into both assessment and therapy
standards. This should improve the overall service delivery to these clients.
____________________________, Committee Chair
Robert A. Pieretti, Ph.D.
____________________________
Date
v
ACKNOWLEDGEMENTS
The research included in this thesis could not have been performed if not for the
assistance, patience, and support of many individuals. I would like to extend my
gratitude first and foremost to my thesis advisor, Dr. Robert Pieretti, for mentoring me
over the course of my graduate studies. His insight led to a more refined and descriptive
research objectives. His perspective has helped me to thoroughly express my results and
observations to allow for better understanding for my readers. I sincerely thank him for
his confidence in me and his help over the course of the analysis and the writing of this
thesis.
I would additionally like to thank Dr. Ann Blanton for the support she has
provided me throughout my undergraduate and graduate studies. Her background in
research has only benefitted me, especially throughout the revision stages of this paper. I
cannot thank her enough for the example she has set for me as both a clinician and a
researcher.
This study would not have been possible without the help, guidance, and support
of Dr. Leonard Abbeduto, Dr. Andrea McDuffie, Dr. Angela John Thurman, and Ashley
Oakes. To Dr. Abbeduto and Dr. Andrea McDuffie, I cannot thank you enough for
providing me with the wonderful opportunity to participate in such an innovative research
study. Your wisdom and guidance helped me throughout the course of this study, as well
as during the writing process. Dr. Angela John Thurman, I want to thank you for your
constant support and insight into the research field, including your assistance during the
data analysis and interpretation process. To Ashley Oakes, you led by example and taught
vi
me to not be afraid of the research process, but rather enjoy the ride that it takes you on.
And to my all my coworkers at the UC Davis M.I.N.D. Institute, it was a pleasure
collaborating with you all throughout the course of this study. I am forever grateful for
this experience.
This research would not have been possible without the cooperation and
participation of the children and families involved in this study. I cannot express how
much I appreciate the knowledge you have given me and the help you have provided for
my research.
The larger study, in which this pilot study was associated with, was supported by
funding from the National Fragile X Foundation. I would like to thank this wonderful
association for the support they provide to the families of those affected by fragile X
syndrome and their involvement in innovative research that will further support these
families.
Finally I would like to extend my deepest gratitude to my parents, Teri and David
Hinz, and my husband, Scott Stewart, without whose love, support and understanding I
could never have completed this master’s degree. To my mom and dad, you have
constantly shown me that hard work and dedication pay off. To my loving husband, you
have given me confidence when I needed it, help when I asked for it, and stability though
I never requested it. I could not have made it through this process without you all.
vii
TABLE OF CONTENTS
Page
Acknowledgements ..................................................................................................... vi
List of Tables ................................................................................................................ x
Chapter
1. REVIEW OF THE LITERATURE ....................................................................... 1
Introduction ....................................................................................................... 1
Causes of Fragile X Syndrome ............................................................. 1
Prevalence of FXS ................................................................................ 2
Cognitive and Developmental Characteristics of Individuals
with FXS ................................................................................................3
Physical Characteristics of Individuals with FXS ................................ 4
Language Development in Individuals with FXS ................................. 5
The Relationship Between Play and Language Development .............. 8
Play and Typically-Developing Children ............................................. 9
Play and Children with Developmental Delays .................................. 15
Statement of the Problem ................................................................................ 16
2. METHODOLOGY ............................................................................................... 18
Design. ............................................................................................................ 18
Participants ...................................................................................................... 18
Evaluation Measures ....................................................................................... 20
Variables Analyzed ......................................................................................... 26
viii
3. RESULTS ............................................................................................................. 28
Individual Characteristics: Developmental Play Assessment ......................... 28
Individual Characteristics: Measures of Language, Cognition, and Autism
Symptom Severity........................................................................................... 31
Concurrent Correlational Analysis ................................................................. 37
4. DISCUSSION ....................................................................................................... 41
5. IMPLICATIONS, LIMITATIONS, AND FUTURE DIRECTIONS .................. 49
Implications..................................................................................................... 49
Limitations ...................................................................................................... 50
Future Directions ............................................................................................ 50
Appendix A. Letter of Consent ................................................................................... 52
Appendix B. Letter of Thesis Approval from UC Davis Advisor .............................. 54
References ................................................................................................................... 55
ix
LIST OF TABLES
Tables
2-1
Page
Sequences, Definitions and Examples of Developmental Play Categories (Lifter,
2001) …………….…………………………………………………………….25
3-1a
Descriptive DPA Data for Participants 1-6.….………………………………..29
3-1b
Descriptive DPA Data for Participants 7-12.….……………………………....29
3-2
Quantitative Statistics of Play Actions for the DPA.….……………………....31
3-3
Raw Language Scores by Subtest……………................................ .…………32
3-4
Standard Scores, Composite Scores, and Age Equivalents for PLS-5.………..32
3-5
Standard Scores, Composite Scores, and Age Equivalents for Mullen.……….33
3-6
Item Analysis of Participants’ Scores on the PLS-5 Auditory Comprehension
subtest………………………………… ................................... .………………33
3-7
Item Analysis of Participants’ Scores on the PLS-5 Expressive Communication
subtest………………………………… ................................... .………………35
3-8
Communication Development Inventory of Receptive and Expressive Vocabulary
and Autism Severity Score and Spectrum Classification …… .………………36
3-9
Psychometric Properties of the Play, Language, and other Variables used in
Correlational Analysis ………………………………… ......... .………………37
3-10a Concurrent Correlations between Play and Other Participant Characteristics...38
3-10b Concurrent Correlations between Play and Other Participant Characteristics...39
4-1
Language and Play Scale for Young Boys with FXS.….……………………...43
x
1
Chapter 1
Review of the Literature
Introduction
Fragile X syndrome (FXS), previously named Martin-Bell syndrome, was first
described in the 1940s by Julia Bell and James Martin. At that time, there was little
known about this syndrome, except that it was a form of intellectual disability that
seemed to be a familial disorder. The disorder was renamed in the 1980s after cytogenetic
testing revealed the location of this genetic mutation. Even more specific gene
information was discovered nearly 10 years later (Roberts, Hennon, & Anderson, 2003).
Though we have learned much about FXS in the last 70 years, this review of the literature
will reveal that research is still needed to further establish best practices in assessment
and treatment of individuals with this syndrome.
This chapter will focus on research findings in the following areas: (a) causes of
FXS, (b) prevalence of FXS, (c) physical characteristics of individuals with FXS, (d)
cognitive and developmental characteristics of individuals with FXS, (e) language
development of individuals with FXS, (f) the relationship between play and language
development, and (g) play and developmentally-delayed children.
Causes of Fragile X Syndrome
FXS is currently the most common inherited cause of intellectual disability and is
caused by the expansion of a trinucleotide (CGG) repeat on the fragile X mental
retardation 1 (FMR1) gene, which is found on the bottom portion of the X chromosome
at Xq27.3 (Hagerman, 2008). An unaffected individual has between 5 and 50 CGG
2
repeats in the FMR1 gene. An individual who carries the premutation of fragile X
syndrome will have anywhere between 55 and 200 CGG repeats in the FMR1 gene.
Those with the premutation can display differences in areas such as cognition,
psychological well-being, behavior, and neurology from those unaffected by FXS. These
issues include, but are not limited to, premature ovarian failure (POF), fragile Xassociated tremor/ataxia syndrome (FXTAS), and anxiety. FXS is inherited when a preor full-mutation female passes the affected gene to her offspring. Risk of passing on a full
mutation increases as the number of repeat sizes in premutation carriers increases. When
the repeat size in a permutation carrier increases when an allele with more than 100 CGG
repeats is passed on, there is a 100% risk that the offspring will inherit the full mutation
and will be born with FXS (Hagerman, 2008). An individual with the full mutation will
have 200 to 230 CGG repeats (Crawford, Acuna, & Sherman, 2001). The expansion of
the CGG repeat in FXS causes the gene to stop making its associated protein, which is
termed the Fragile X Mental Retardation Protein (FMRP). FMRP is expressed in the
brain and is responsible for experience-dependent learning and neural plasticity. Absence
of FMRP results in the manifestation of the behavioral characteristics of FXS (Hagerman,
2008).
Prevalence of FXS
Males with the FXS full mutation are more affected than females, because, unlike
females, they have only one X chromosome. If their sole X chromosome has the full
mutation, this means little or no FMRP is produced. When this occurs, the medical and
developmental problems of fragile X syndrome become most apparent. However, females
3
have two X chromosomes; thus, an unaffected X chromosome continues to produce
FMRP, resulting in the full mutation having less of an effect than it would on males
(Hagerman, 2008). The most current numbers suggest that the approximate prevalence of
FXS is 1 in 3,600 males. However, the prevalence of FXS has not been well documented
in females, because females infrequently demonstrate the associated intellectual disability
and their diagnosis may go unrecognized (Crawford, et al. 2001; Hagerman, 2008).
Cognitive and Developmental Characteristics of Individuals with FXS
FXS is accompanied by issues that disrupt typical development. A full mutation
often results in intellectual disability (ID) in males and learning disabilities in females,
but a broad range of involvement occurs in both groups (Hagerman, 2008; Roberts et al.
2003). As infants, hypersensitivity or hyperarousal to sensory stimuli may cause an
exaggerated startle response and irritability (Abbeduto, Brady, & Kover, 2007;
Hagerman, 2008). In the second year of life, some of these children will demonstrate
poor eye contact and tactile defensiveness (Hagerman, 2008). Social anxiety is also a
frequent symptom of children with fragile X syndrome, and can be evident in children as
young as two years of age (Abbeduto et al. 2007). Males with FXS may also develop
self-injurious behaviors around 30 months. It should also be noted that both hyperactivity
and attention deficit/hyperactivity disorder (ADHD) occurs in approximately 80% of
males with FXS with the full mutation (Hagerman, 2008). A co-morbid diagnosis of FXS
and autism occurs in approximately 25%-33% of males with FXS and approximately 5%15% of females with FXS (Bailey, Hatton, Skinner, & Mesibov, 2001; Hagerman, 2008).
However, some researchers suggest that while children with FXS may display symptoms
4
of autism, these symptoms may be attributable to the severity of cognitive delays in these
affected individuals (McDuffie, Kover, Abbeduto, Lewis, & Brown, 2012). Males with
FXS alone are noted to show more interest in social interaction than those with comorbid
FXS and autism or those with autism alone. It is important to recognize that most
children with comorbid diagnoses of FXS and autism have lower IQ scores (Bailey,
Hatton, Tassone, Skinner, & Taylor, 2001; Hagerman, 2008) and more impaired
receptive and expressive language than those with only FXS (Hagerman, 2008).
Physical Characteristics of Individuals with FXS
Common physical characteristics of individuals with FXS include an elongated
face, long and prominent ears, high palate, flat feet, hyperextensible finger joints, and soft
velvet-like skin, though a diagnosis cannot be made upon these features alone. Infants
with FXS also typically exhibit a large head and hypotonia (Hagerman, 2008). There are
also a variety of physical issues that accompany those with FXS. FMRP, the protein that
is deficient in FXS, is believed to be involved in formation of connective tissue.
Occasional joint dislocations, hernias, and recurrent otitis media are all common
problems in FXS as a result of loose connective tissue. Other medical problems of
individuals with FXS include: strabismus in 8%-30% of individuals with the diagnosis
(Hagerman, Hagerman; Hatton, Buckley, Lachiewicz, & Roberts, 1998), mitral valve
prolaspse and/or dilation at the base of the aorta in about 50% of adults, and
gatroesophageal reflux (GER) in the majority of infants with possible frequent vomiting
as a result (Hagerman, 2008).
5
Language Development in Individuals with FXS
Children with FXS display moderate to severe communication impairments from
an early age. These delays may be more severe than would be expected based upon their
cognitive delays alone (Abbeduto & Hagerman, 1997; Sterling & Warren, 2008). As in
many other syndromes, children with FXS demonstrate variability in strengths and
weakness across developmental areas. In fact, several studies have shown that despite
their cognitive issues, these children display some relative cognitive strengths, such as
storing and retrieving information from long-term memory (Abbeduto et al. 2007).
Cognitive delays are, however, likely to interfere with typical language processing. As
previously stated, FXS frequently occurs with some degree of intellectual disability (ID),
especially for males. According to the American Association on Intellectual and
Developmental Disabilities (2010), Intellectual Disability (ID) is characterized by
significant limitations in both intellectual functioning and adaptive behavior in
conceptual, social, and practical adaptive skills. For a diagnosis of ID, the disability must
originate prior to the age of 18 years of age. This likely interferes with typical language
processing and development due to interruptions in auditory short-term memory,
processing sequential information, and directing and sequencing attention.
Successful language development is dependent upon an individual’s ability to
process linguistic information. Owen’s (2010) Information Processing model has 4
distinct steps in its sequence: attention, discrimination, organization, and memory or
retrieval. In a typically-developing child, information processing happens effortlessly and
efficiently. As stimuli are presented (e.g., auditory or visual input), the brain chooses the
6
most salient or important information to attend to and discriminates this stimulus from
other extraneous stimuli. For example, this could be the ability to recognize that a person
is trying to initiate conversation, to listen to what the person is saying, and to attend to the
person’s facial expressions, all the while blocking out background noise or other visual
distractions. The overall meaning of what is being said or seen is then understood based
upon representations previously stored in long-term memory and organized in the brain
for later efficient retrieval. In a conversation, one must also hold some information in
working memory in order to keep the conversation going. While successful information
processing certainly requires a foundation of intact cognitive abilities, it also depends on
successful executive functioning. Executive functioning is the set of mental processes,
including attention, memory, organization, time and space management, planning, and
strategizing, that connects past and present experiences (National Center for Learning
Disabilities, 2013). Children who have a weak link in any part of the information
processing system (attention, discrimination, organization, retrieval) or its foundations
will likely have difficulty processing language (Owens, 2010).
In fact, most boys with FXS demonstrate moderate to severe communication
delays (Roberts et al., 2002), which begin in infancy. Prelinguistic skills, such as joint
attention, are an important part of early language development. Joint attention is the
ability to share an object of attention with a social partner, often including coordinating
eye gaze between the referent and the communicative partner (Sterling & Warren, 2008;
Tager-Flusberg & Sullivan, 1998). Joint attention allows a child to share an experience
with a parent or other social partner and produce one of the first possible communication
7
acts. When impaired or absent communication development is delayed and the
development of play skills can also be affected. In children with FXS, hyperarousal,
inattention, and hyperactivity are likely to contribute to impairments in joint attention
(Sterling & Warren, 2008). Imitation is another prelinguistic skill vital for speech and
language development. This gives the child a direct model for correct speech production
as well as a chance to explore new words and build vocabulary. Imitation also provides
the opportunity for episodes of turn-taking with a communication partner. (Rogers,
Hepburn, Stackhouse, & Wehner, 2003; Sterling & Warren). Interestingly, a study by
Sterling & Warren demonstrated that imitation in children with FXS was similar to that
of children with similar levels of cognitive development.
Around 12 months of age, typically-developing children are beginning to use their
first words. At this time, use of gestures and other nonverbal communication acts begin to
decrease. Despite intact imitation skills, children with FXS do not produce their first
words until they reach around 26 to 28 months old (Finestack, Richmond, & Abbeduto,
2009). Some research has shown that nonverbal communication may persist longer in
children with FXS, sometimes remaining into adolescence and adulthood, whether or not
verbal communication has fully developed (Murphy & Abbeduto, 2003).
Most studies agree that expressive language deficits, initially, are typically more
prominent than receptive language deficits within this population (Price, Roberts,
Vandergrift, & Martin, 2007; Roberts, Mirrett, & Burchinal, 2001) and that receptive
language develops at a faster rate. Nevertheless, most research regarding speech and
language in FXS has, unfortunately, examined only adolescents and adults (Roberts,
8
Hennon, & Anderson, 2003) and, therefore, little is known about the developmental
trajectory of communication in children with fragile X syndrome.
The Relationship Between Play and Language Development
Play, from a developmental perspective, serves many functions, such as
exploration of environment and objects, cognitive and social growth, and expression of
knowledge and feelings. Play allows children to explore their environment, incorporate
new information onto existing knowledge structures, and display their knowledge,
reflecting their level of development (Lifter, 2000; Rubin, Fein, & Vandenberg, 1983).
Research in the field of psychology consistently demonstrates that children’s play
activities follow a sequence of development and display the changes in children’s
knowledge as they develop (Lifter, Mason, & Barton, 2011). Play also teaches children
societal roles, rules, and values and the conventional uses of objects (Rubin et al. 1983),
as well as how to adapt to their environment (Lifter, 2000). The concept of
developmental play has been studied by many professionals across many disciplines and
research consistently shows that play as a vital part of development, cognitively, socially,
and linguistically (Bloom, Lifter, & Broughton, 1985; Lifter, 2000; Lifter & Bloom,
1989; Rubin et al. 1983; Ungerer & Sigman, 1981). Over the last forty years research has
consistently shown a relationship between receptive and expressive language skills and
functional and symbolic play skills in typically-developing children (Thiemann-Bourque,
Brady, & Fleming, 2012; Kelly & Dale, 1989; McCune-Nicolich, 1981) and children
with developmental disabilities (Mundy, Sigman, Ungerer, & Sherman, 1987; Terpstra,
Higgins, & Pierce, 2002; Thiemann-Bourque, et al. 2012).
9
Play and Typically-Developing Children
Play skills are an important part of language development for all children with
and without disabilities (Mundy et al. 1987). In typically-developing children, symbolic
and pretend play unfolds in a hierarchical manner. The Westby Play Scale was originally
developed in 1980 by Carol Westby by systematically observing typically-developing
infants, toddlers, and preschoolers in childcare centers, as well as preschool and
elementary school age children with developmental disabilities in special education
classrooms. In 2000, the Westby Play Scale was revised and has remained the gold
standard for speech-language pathologists and other professionals as an observational
assessment to evaluate a child’s language and play abilities. This scale provides
normative ages of the emergence of play and communication skills and the hierarchical
structure in which they typically develop (Westby, 2000). According to Westby, Phase
One of a child’s play development is the presymbolic stage, which includes Presymbolic
Level I and Presymbolic Level II. During these levels, typically-developing children
should be developing skills in areas such as object permanence, means-end or problem
solving, and object use while growth in communication skills is co-occurring. In
Presymbolic Level I, children 8- to 12- months are demonstrating emergence of object
permanence skills. They are aware of an object’s existence when it is out of sight and
begin to associate objects with their location. As children become more mobile, they are
problem-solving by crawling or reaching to gain access to toys and other desired objects.
Rather than mouthing all toys, they are beginning to discover parts of objects and toys,
using actions other than just mouthing. They may be patting, banging, throwing, or
10
turning objects to explore as well as experimenting with different toy actions. Children
may also involve others in their play, showing or giving items, engaging in joint attention
with a toy and a person, and may use nonverbal communication acts such as a request or
comment. In Presymbolic Level II, skills from the previous stage are continuing to
develop in 13- to 17-month old children. They also begin to recognize that objects exist
apart from their location and are capable of finding an object hidden in various locations.
Within this time frame, children are likely demonstrating their understanding of
containment, by putting objects inside a container, and conversely about separation, by
dumping them out. They are continuing to use nonverbal communication, such as giving
a toy to an adult for assistance or attention or requesting by pointing to a desired object.
These children are also starting to use common objects appropriately and their toy
knowledge is expanding through trial and error during use. They begin to recognize the
physical properties and actions of a toy, as well as how toys relate to each other such as a
car and its driver. Children’s communication acts should expand to use gestures and
vocalizations for the purposes of requesting, commenting, interacting, protesting,
indicating personal feelings, responding, greeting, and labeling. Single words are
produced, though they are limited to use in the context in which they were learned.
Throughout these Presymbolic levels, typically-developing children are both exploring
independently and beginning to function within a shared reality (Baron-Cohen, 1995),
allowing them to learn from others about object use, play, and communication.
Phase Two includes eight symbolic levels in which typically-developing children
begin to engage in pretend play, developing schemas and sequences, and expand their
11
play from basic object use. Their language also continues to develop functionally,
structurally, and semantically. At 17- to 19- months, children begin transitioning to more
symbolic acts as Symbolic Level I begins. They begin to demonstrate decontextualization
through mental representations, such as using objects as tools, finding invisibly-hidden
toys, using life-like toys for pretend play, and understanding the physical limitations of
some toys (e.g., solid ring cannot be stacked on ring stack). Short, single-action schemas
begin to develop using everyday activities in which the child has been actively
participating. These schemas are self-representational, meaning the child is the active
participant in the pretend play. Language functions continue to develop and labeling
abilities expand to include activity and more objects. Authentic verbal communication is
being established for functional relations, including recurrence, existence, nonexistence,
rejection, and denial, as well as semantic relations, including agent, object, action, state,
and indicating an object or person associated with object or person. At 19- to 22-months,
children continue to develop use of realistic props in pretend play throughout Symbolic
Level II. Schemas now include more familiar activities that they have observed, but have
not participated in (e.g., washing dishes). These short, schemas may now include pretend
play using multiple toys or demonstrating multiple actions. Children are also beginning to
perform play actions toward a doll (e.g., feeds doll) and directing the same single action
upon multiple recipients (e.g., feeds doll, then feeds self). More abstract language is also
beginning to develop as children refer to persons or objects which are absent and are
beginning to request information to expand their knowledge. Semantic relations are also
further expanded as they begin to use single words and word combinations such as agent-
12
action, action-object, attributive, dative, action-locative, possessive. Around 2 years of
age, Symbolic Level III begins and is characterized by more complex single schemas
which elaborate upon many steps need to perform activity (e.g., fill sink with water, put
pots in, wash dishes, and dry dishes). Children begin to expand their role in play by
reversing roles with play partners. At this time, they begin to recognize and reflect upon
the current actions of both themselves and the doll. Their utterances are lengthening to
phrases and short sentences while they begin to develop early morphological endings,
such as present progressive –ing, plurals, and possessives. Symbolic Level IV begins at
approximately 2 ½ years of age. Until this time, these children’s schema likely unfolded
with rigid sequence. At this point sequences are likely to reflect events that are
infrequently encountered by the child, but are salient for reasons either positive or
negative. They are continuing develop more advanced pretend play throughout this level,
using complementary roles and speech directed at a doll or figure. Further language
abilities are emerging concurrently including wh- question formulation and responses,
with the exception of “why” questions. During Symbolic Level V, approximately 3 yearold children continue to increase the flexibility of their pretend play. They continue to act
out frequently experienced schema, generating new outcomes and also begin to play out
unplanned episodic sequences. These children are also becoming more committed to the
roles they play, transforming into the character. It’s at this time that children begin to
engage in parallel or associative play with other children where their goals are similar,
but they are not directly playing together. Their language continues to expand for
purposes of reporting, predicting, or narrating a story at a basic level, while both past and
13
present tense forms are also beginning to appear. As Symbolic Level VI begins around 3
to 3 ½ years old, children are beginning to play with replica toys smaller scale than lifesize. Object substitution becomes increasingly frequent, including using blocks to build
small representational structures like houses for dolls or other figures. At this time their
pretend play is becoming more imaginative as they are acting out events they themselves
did not personally experience, including schemas such as “policemen” or “cowboys.”
Puppets and dolls may take a more active role in play, being given a voice by the child.
These children begin assigning roles to other children and may take on the role of
multiple characters in a single schema. Using more sophisticated language, they are now
able to project their feelings onto their doll or puppet, make indirect requests, change
their speech based on their conversational partner, use language to reason, and produce
metalinguistic and metacognitive language when speaking (e.g. “She said,” “I think,”
etc.). Their communicative inventory grows to add descriptive words such as shapes,
sizes, colors, textures, and spatial relations, though these words are not always used
accurately. Between 3 ½ and 4 years of age, children are beginning use language in a
larger variety of ways during Symbolic Level VII. They are beginning use props more
realistically, building 3-dimensional structures, verbally imagining props, setting the
scene for their schemes, and improving and expanding diversity on common play themes.
These schemes are more planned out, though these children begin to wonder “what if”
and may alter their outcomes as they explore. Dolls, puppets and other figures continue to
take on a more active role in these schemes as children are using them to act out their
scripts. For example, in one scheme, the child and the doll may take on multiple roles
14
demonstrating a higher level of pretend play. In this level, language may function to set
the scene or narrate the play schemes. These children’s language likely now contains
some modals (e.g., “can,” “would,” etc.) and conjunctions, and may include appropriate
responses to “why” and “how” questions. Language and play further develops around 5
years during Symbolic Level VIII. Language is being used even more as a structural part
of play, setting the scene for much more imaginative play. This level of play includes
elaborate schema that the child has definitely never been involved in. There may be
multiple scripts occurring simultaneously and a high level of organization is required to
incorporate props, figures, and other children to carry out these sequences of events.
Often, their play may contain a goal at the end, for example catching the aliens that
invaded the planet. At this time, their play becomes much more mature and so do their
language abilities, as they start to use relational term (e.g., “when,” “last,” etc.).
Throughout these levels, new elements of language are continuously developing, but it
should be noted that it is not until age 12 when most children have fully mastered these
linguistic elements (Westby, 2000).
In sum, the Westby Play Scale is used to determine how developed a child’s play
and language should be at any given age. It is, therefore, a helpful diagnostic tool for
clinicians and it is also used for interventionists to determine where they might be able to
begin therapy and how they might effectively incorporate play into this therapy. It is
important to note, however, that the Westby Play Scale was not designed primarily for
children with developmental disabilities, nor was it normed on them. It was based on the
abilities of typically-developing children and many of the levels of play which are
15
described by Westby are at a higher developmental level than may be reached by toddlers
and preschoolers with moderate to severe intellectual and developmental disabilities
Therefore, in the current thesis focusing on play development in young males with FXS,
a decision was made to administer Lifter’s Developmental Play Assessment (Lifter,
2000), which provides a more detailed picture of earlier emerging play levels.
Play and Children with Developmental Delays
Much research has indicated that children with developmental disabilities develop
play skills in the same sequence as typically-developing children (Field, Roseman,
DeStefano, & Koewler, 1982; Westby, 2000) but that they develop at a different rate and
in a different manner (Rogers, 1988; Williams, Reddy, & Costall, 2001; Lifter, Ellis,
Cannon, & Anderson, 2005, Malone & Langone, 1999). These children, unlike typically
developing children, may not be intrinsically motivated to move forward in play (Lifter,
2000). Some research has shown that their play is less frequent and demonstrates less
variety (Lifter, Sulzer-Azaroff, Anderson, & Cowdery, 1993). Cognitive delays can
impact play skills, just as delays in play skills can negatively affect cognitive
development (Hill & McCune-Nicholich, 1981; Lifter, Ellis, Cannon, & Anderson, 2005;
Pierce-Jordan & Lifter, 2005; Ungerer & Sigman). Physical, sensory, communication,
and social deficits have also been shown to compromise a child’s potential to engage in
and benefit from play (Malone & Lagone, 1999).
Much research has been conducted determining specific deficits in play across
developmental disorders. For example, several studies have found that children with
autism exhibit less variety and complexity in their play behaviors than children with other
16
disabilities and typically developing children (Lifter et al. 2011). Children with autism
also produce fewer symbolic play acts (Lifter et al. 2011; Sigman & Ruskin, 1999) with
less variety (Thiemann-Bourque et al., 2012), and they demonstrate poor engagement and
less frequent functional play acts, as well as fewer acts of pretend doll play (Sigman &
Ungerer, 1984; Thiemann-Bourque et al., 2012).These children also display less variety
and complexity in the production of play sequences (Lifter et al., 2011). Play sequences
that do occur are shorter in duration than those of other children (Sigman & Ungerer,
1984). Many researchers view play skill deficits in children with autism to be caused by
their difficulty generating new play behaviors (Lifter et al., 2011). Research has shown
that children with other developmental disabilities also exhibit delays in acquisition of
play skills and produce less sophisticated play skills overall when compared to typically
developing children. Though these children display improvements in their overall
development and functioning over time, they do not seem to match these
accomplishments in their play skills (Lifter et al. 2011). There are no published studies
specifically examining play skills in young males with FXS, the most affected group of
individuals diagnosed with FXS.
Statement of the Problem
Little is known about the play skills of children with FXS and the relationship of
play and language acquisition in this population. As previously stated, there are no
published studies examining play skills in young males with FXS, even though play skills
have been shown to be directly related to the development of receptive and expressive
language. Research that attempts to identify more efficient and naturalistic language
17
intervention approaches for children with FXS is needed. The current study will add to
the literature by providing a descriptive characterization of play for 11 boys with FXS
between the ages of 27 and 51 months. This data has the potential to inform clinicians
involved in the diagnosis and treatment of boys with FXS. This study was designed to
answer the following research questions: (1) What are the concurrent correlations
between play skills, receptive and expressive language abilities, and cognitive abilities of
young males affected by fragile X syndrome? (2) How can the play of young males
affected by fragile X syndrome be described and characterized?
18
Chapter 2
Methodology
Design
This pilot data was obtained as a part of larger study, funded by the National Fragile
X Foundation, investigating the effects of a parent-mediated language intervention for
young males with fragile X syndrome. The pilot was a single-center study completed at
the University of California Davis Medical Center’s Medical Investigation of
Neurodevelopmental Disorders (M.I.N.D.) Institute. The principal investigator was
affiliated with the M.I.N.D. Institute and was concurrently enrolled in the SpeechLanguage Pathology graduate program at California State University, Sacramento. The
larger study employed a single-subject, non-concurrent multiple baseline design. Parent–
child dyads participated in baseline and intervention sessions. The first parent-child dyad
participated in 3 baseline sessions and 16 intervention sessions (4 onsite, 12 distance).
Each successive dyad participated in additional baseline sessions prior to starting
intervention as required by the experimental design (e.g. 3, 6, 9, 12). The analyses
reported in this thesis, however, employed a concurrent correlational design in which all
data was collected at a single point in time. Data for the pilot study was collected during
the pre-treatment assessment for each participant prior to the initiation of the baseline
sessions for the larger intervention study.
Participants
Participants, along with their biological mothers, were recruited for the larger
study at the University of California, Davis Medical Center’s M.I.N.D. Institute. This
19
study included twelve male participants, between 2 and 5 years of age with a confirmed
diagnosis of full mutation fragile X syndrome. The participants were required to meet six
eligibility criteria. First, each participant had (1) a diagnosis of fragile X syndrome
documented through molecular genetic testing and (2) was between 2 and 6 years of age.
Each mother was required to document that, at the time of recruitment, their child (3)
used less than 10 different spoken words on a daily basis, (4) had been diagnosed with a
developmental delay or intellectual disability by a qualified professional, and (5) had no
uncorrected sensory or motor impairments that would preclude processing and
responding to verbal language input. In addition, once recruited, children needed to (6)
display at least one intentional communication act during a parent-child or examinerchild interaction with an initial baseline assessment or observation. Participating mothers
were required to (1) be the biological mothers of the target children and (2) report that
they were the child’s primary care provider. Each mother also was required to report that
(3) she had earned a high school degree prior to age 19 and (4) that she had not been
hospitalized within the past six months for a psychiatric condition. Finally, participating
mothers were required to be (5) at least 21 years of age.
The child participants enrolled in the study were functioning developmentally at a
level lower than a typically developing 5 year old child and were also minimally verbal.
Thus, the biological mothers were asked to provide informed consent for the child’s
participation. Though twelve participants were enrolled in this study, data was used for
only eleven of the children due to one child’s inability to complete all necessary
assessment tasks.
20
There was no monetary compensation for this study. Each child received a picture
book at the conclusion of the play assessment. Parent inducements for participating in the
larger study included knowledge learned about child language development and parent
strategies for providing language stimulation to the child, strategies for supporting
parent/child interaction through play, and strategies for decreasing challenging behaviors
that may have been interfering with ongoing parent/child interactions. Gains for all
researchers and research assistants were purely educational, not financial. This study
poses no more than minimal risk because the anticipated harm is no greater than what
occurs in daily life activities for the participants.
Evaluation Measures
At the first visit of the intervention study, baseline assessment information was
collected to evaluate the client’s receptive and expressive language, cognition, and autism
symptom severity. These measures were later used as a part of the correlational analysis
along with the Lifter’s Developmental Play Assessment (DPA) (Lifter, 2000). The
Autism Diagnostic Observation Schedule (ADOS) (Lord, et al. 2001) Module 1, as well
as the Toddler Module, was used as the standardized measure of autism symptom
severity. Eleven participants were given the ADOS Module 1, which is designed for
children 31 months of age or older, who do not yet primarily use phrase speech. One of
the twelve participants was given the Toddler Module because his chronological age at
the onset of the study was below 30 months. The ADOS consists of a series of examiner
prompts designed to elicit specific behaviors or symptoms of autism (i.e., calling the
child’s name to engage social responsiveness). The severity score was calculated using
21
the Gotham Algorithm (Gotham, Risi, Pickles, & Lord, 2007). The Gotham algorithm is a
formula used in conjunction with scores from the ADOS to demonstrate better diagnostic
validity than using the ADOS diagnostic algorithm. When using the ADOS algorithm,
items related to repetitive behaviors and restricted interests are not included. These items
are considered using the Gotham algorithm and this allows for better predictive validity
of an autism diagnosis. The Mullen Scales of Early Learning (Mullen, 1995) was the
standardized measure used to evaluate overall cognitive development and language
abilities. The subtests given included: (1) visual reception, (2) fine motor, (3) receptive
language, and (4) expressive language. Language comprehension and production were
also measured using the Preschool Language Scales-5 (PLS-5) (Zimmerman, Steiner, &
Pond, 2011). This standardized assessment uses toys and books to evaluate auditory
comprehension and elicit verbal expression in a more naturalistic environment for the
children. The MacArthur Bates Communicative Development Inventory (CDI) (Fenson,
et al. 2007), a parent report measure, was also used to further examine each child’s
receptive and expressive communication inventory.
The language-based assessments were performed by a speech-language pathology
clinical fellow and the assessment of autism symptoms and severity was performed by a
licensed speech-language pathologist and special education professional. The DPA,
which was the focus of this study, was the only assessment administered by the principal
investigator of this pilot study. All measures were scored and double-scored by two
trained researchers on this project. Data was then entered into the computer and doublechecked once entered by another researcher before being used for analysis.
22
The Developmental Play Assessment (DPA) Instrument (Lifter, 2000) was used to
evaluate play abilities. This play assessment was created using the taxonomy of play
abilities adapted from a study conducted by Lifter & Bloom (1989), in which the play of
14 children was observed for 1-hour sessions on a monthly basis from 8 months until 2
years of age. The knowledge gained from this study provided the foundation for creation
of the DPA. Though the foundational study (Lifter & Bloom, 1989) was performed using
typically-developing children, the true intent of this instrument is to evaluate the play of
children with developmental disabilities to determine what they know, what they are
trying to learn, and what they do not know in terms of play skills. The DPA is beneficial
for both therapists and researchers, as it provides not only quantitative data appropriate
for detailed analyses of play skills, but also determines the play categories that the child
has mastered, those which are emerging, and those which are not yet present.
Ideally the DPA play sample takes a total of 30 minutes, allowing the child 7-8
minutes with each toy set. However, due to noted variations in attention and object
interest, the examiner is allowed to shorten or lengthen the sample duration based on each
individual child. Using DPA methods, four sets of toys were sequentially presented to the
child and the child was encouraged to play with the toys. Lifter provides a suggested
group of toy sets to best elicit a variety of play actions throughout all play categories.
Similar toys from the sample sets were used in our assessment. The following toy sets
were used in this study:
Group 1: Five-piece circle puzzle, with pieces in
Beads, in bowl with string beside
23
Small dump truck, with driver inside cab
6 foam blocks (3 cubes, 3 cylinders), grouped but not stacked
Stuffed Lamb
Group 2: Peg board, next to pegs
Pegs, in plastic box with lid atop, ajar
Larger nested cups, nested
Baby doll with clothes on
Blanket
Spoon
Comb
Mirror
Group 3: 4 Farm Animals (colt, horse, cow, pig), in plastic box with lid atop, ajar
Small boy family figure, laying flat on back
Three-Part Train, coupled
Cup, Saucer, and Pitcher, separated
Nuts and bolts, separated and inside bowl
Group 4: Ring Stack, rings on
Balls, in bowl
Stuffed bear
Foam blocks, in box without lid
Car
Throughout the assessment, the examiner observed the child’s play with each set of
24
toys and responded to the child if the child initiated conversation with the examiner. It is
acceptable for the examiner to reflect play actions that have already been performed by
the child, but the examiner is cautioned not to demonstrate any play actions that the child
has not already produced. This allows for the most reliable observation of what play
skills are truly within that child’s repertoire. Within the assessment, the examiner may
direct the child’s attention to other toys within the set to provide maximum opportunities
for play actions. If the child becomes fixated on one specific toy or plays with a toy
perseveratively, the examiner may remove this toy and redirect the child to other toys
within that set (Lifter, 2000). Each play sample is videotaped by a second researcher and
used for later analysis.
A chart of targeted behaviors is used to analyze play in children with developmental
disabilities. This chart was created to mirror the developmental progression studied by
Lifter and Bloom (1989). The eight play stages described in Table 2-1 by Lifter are
further divided into 14 different categories of play actions. Stage One of the DPA
includes indiscriminative actions, also known as undifferentiated play actions, which is
the most basic level of play. At this level the child is treating all toys and objects alike,
performing actions such as mouthing, banging, shaking, or inspecting an object. This
fundamental level of play allows the child to explore objects, learning their physical and
conventional properties using trial and error. All further stages of play are considered
discriminative or differentiated actions. Differentiated play actions, which include stages
two through eight of the DPA, require some knowledge of the item or object and this
knowledge is demonstrated when a child performs a purposeful action upon an object.
25
Development progresses throughout the play stages: Children begin by exploring, they
move to proper object use, and then play develops into functional, basic and complex
pretend play.
All assessment sessions were videotaped and the data was coded on a PC computer
using the DPA scoring strategy (Lifter, 2000) and subsequently coded using Playcoder
software, which was created to aid in reliably and quickly score the DPA (Bruckner &
Yoder, 2007; Tapp & Yoder, 2003). While playing the video clip in one window,
Playcoder can be opened in an adjacent window. This allows the researcher who is
scoring the assessment to pause the video clip when a play action is observed and then
record that play action in the Playcoder program. An action is coded by first selecting the
toy set, followed by the specific toy used, and then selecting the action performed on that
toy (e.g. Toy Set 1—Dump truck and driver—Pushes truck on floor or table). Once all
actions are coded, Playcoder uses a software algorithm to generate a report that includes
frequency statistics such as number of times each toy is touched, number of times each
action was performed on a toy, and total number of toys touched.
Table 2-1
Sequences, Definitions and Examples of Developmental Play Categories (Lifter, 2001)
Play Category
Level
I
Indiscriminate Actions
II
Discriminative actions
on single objects
Take apart
combinations
Definition
Example
All objects are treated
alike
Differentiates among
object, preserving their
physical or conventional
characteristics
Separates configurations
of object
Mouthing and banging
Rolls round beads, squeezes
stuffed animal
Takes pieces out of puzzle
26
III
Presentation
combinations
General combinations
Pretend self
IV
Specific combinations
based on physical
attributes
Child as agent
V
Specific combinations
based on conventional
attributes
Single scheme
sequences
Object substitutions
VI
VII
Doll as agent
VIII
Multi-scheme
sequences
Socio dramatic play
Thematic fantasy play
Recreates combination of
objects according to
original configuration
Creates combinations of
objects that result in
nonspecific combinations
Relates object to self with
pretend quality
Preserves unique physical
characteristics of objects
in configuration
Extends familiar actions to
doll figure
Recreates conventional
actions on objects in
configuration
Extends same action to
two or more figures
Uses one object to stand
for another
Moves doll figure as if it is
capable of action
Recreates a sequence of
actions with figure
Adopts familiar role in
play theme
Adopts role of TV, book
or movie character
Puts pieces into puzzle, nests
nesting cups
Puts beads in cup, puts boy in
box
Brings empty cup to mouth
Stacks nesting cups, stacks
blocks, strings beads
Feeds doll, combs doll’s hair
Places cup on saucer, wears
string of beads
Extends cup to baby doll and
then to examiner
Uses block for phone, use
bowl for hat
Puts brush into doll’s hand
Wipes doll’s face and puts to
bed
Plays house or doctor
Plays Superman or
Wonderwoman
Variables Analyzed
Several variables were used for the concurrent correlational analysis in this study.
DPA data variables included: Indiscriminative Action Frequency which totals all
indiscriminative play actions, Discriminative Action Frequency which totals all
differentiated play actions, Diversity of Play which totals the number of different types of
differentiated play actions, and Object Interest which totals the number of different toys
used for differentiated play actions. Time is also used as a variable in these analyses.
Because this assessment allows for variation in duration of exposure to the toy sets
included in the DPA, the times were adjusted to allow for comparability across
27
participants. Times were adjusted so that each child’s play was coded from the first three
minutes during which each toy set was presented to the child. Two children had sample
times just under three minutes for one of the four toy sets. This is noted within the data,
but missed play actions were considered to be minimal and thus to have negligible impact
on the data set. Other variables analyzed were chronological age, Mullen Visual
Reception subtest raw scores, Mullen Fine Motor subtest raw scores, Mullen Receptive
Language subtest raw scores, Mullen Expressive Language subtest raw scores,
MacArthur-Bates CDI Receptive Vocabulary score, MacArthur-Bates CDI Expressive
Vocabulary score, and ADOS Autism Severity Score. Mullen raw scores were chosen
rather than standard scores due to the limited abilities of the participants. Because of the
age and low scores obtained from these participants, raw scores display more variability
and are likely to be more useful in the correlational analyses as most children would have
scored at floor using standard scores. The PLS-5 results were used to evaluate each
child’s specific language skills and to determine which skills were acquired throughout
each year of development in this sample of children.
28
Chapter 3
Results
Individual Characteristics: Developmental Play Assessment
Several tables summarize each participant’s performance on the DPA. Tables 31a and 3-1b display the descriptive scores used to identify client’s level of mastery for
each play category. Within a play category, Lifter’s DPA uses the frequency of
discriminative play actions and the frequency of different action types to determine
whether this play category is mastered, emerging, or absent. Mastery of a play category
occurs when the child’s frequency of play actions reaches at least ten and the child also
demonstrates at least four different types of play actions. A play category is considered
Emerging when a child displays four or more play actions and two or more different
types of actions. A category is considered Absent from the child’s repertoire if less than
four play actions or only one type of action is observed during the play sample. The
tables indicate the frequency of play actions, followed by the number of different types of
actions (e.g. 10/4), with the exception of the Indiscriminative Actions category. This
category can include many different actions on an object, such as mouthing and banging.
These were not differentiated in coding because the child is treating all objects alike
during this exploratory phase of play.
Table 3-1a
Descriptive DPA Data for Participants 1-6
Participants:
#1
#2
#3
#4
#5
#6
Duration of Session:
11:45
12:00
12:00
12:00
12:00
12:00
29
I
II
III
Indiscriminative Actions:
Treats all objects alike
32
21
25
24
16
39
Discriminative Actions:
Actions on single objects
6/5
9/4
2/2
3/1
20/4
2/2
17/7
11/4
19/7
5/5
17/6
17/9
17/4
35/3
22/6
5/4
5/2
5/4
0
0
7/2
1/1
9/3
2/2
4/2
1/1
0
0
1/1
1/1
4/1
0
0
0
0
0
8/3
2/2
0
0
0
2/1
0
0
0
0
0
0
6/5
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0
0
2/2
0
0
0
0
Take Apart Combinations:
Separates configurations of
objects
Presentation Combinations:
Re-assembles presentation
General Combinations:
Assembles undifferentiated
configurations
Pretend Self:
Relates objects to self in pretend
IV
V
VI
VII
Specific Physical Combinations
Preserves physical characteristics
in configuration
Child-as-Agent:
Extends familiar actions to dolls
or figures
Specific Conventional
Combinations:
Preserves conventional features in
configuration
Single-Scheme Sequences:
Extends same action to multiple
figures
Substitutions:
Uses one object as substitute for
another
Doll-as-Agent:
Attributes actions to dolls or
figures
Multi-scheme Sequences:
Extends different actions to dolls
or figures
Table 3-1b
Descriptive DPA Data for Participants 7-12
Play Categories
#7
#8
#9
# 10*
# 11
# 12
Duration of Session:
12:00
11:17
12:00
--
12:00
12:00
30
I
II
III
IV
V
VI
VII
Indiscriminative Actions:
Treats all objects alike
32
7
18
--
39
10
Discriminative Actions:
Actions on single objects
9/3
0
10/6
--
1/1
16/4
15/4
6/4
21/8
--
19/7
9/4
11/3
0
27/5
--
11/3
7/2
0
0
1/1
--
0
2/1
2/1
0
1/1
--
1/1
0
2/1
0
0
--
0
0
0
0
5/2
--
0
0
0
0
0
--
0
0
0
0
2/3
--
0
0
0
0
0
--
0
0
3/2
0
0
--
0
0
0
0
0
--
0
0
Take Apart Combinations:
Separates configurations of
objects
Presentation Combinations:
Re-assembles presentation
General Combinations:
Assembles undifferentiated
configurations
Pretend Self:
Relates objects to self in
pretend
Specific Physical Combinations
Preserves physical
characteristics in configuration
Child-as-Agent:
Extends familiar actions to
dolls or figures
Specific Conventional
Combinations:
Preserves conventional features
in configuration
Single-Scheme Sequences:
Extends same action to
multiple figures
Substitutions:
Uses one object as substitute
for another
Doll-as-Agent:
Attributes actions to dolls or
figures
Multi-scheme Sequences:
Extends different actions to
dolls or figures
*Participant 10 was unable to perform the required tasks of the Developmental Play
Assessment (DPA) due to social anxiety. Therefore, he was subsequently dismissed from
this pilot study.
Table 3-2 displays quantitative play variables derived from the DPA data
collected. Object Interest represents the number toys that the participant touched using
31
differentiated play actions (Carter et al. 2011; McDuffie, Lieberman, & Yoder, 2010).
Diversity of Play, represents the number of unique actions observed. This is calculated by
counting the number of different action types, excluding indiscriminate actions (Yoder,
2006) and is similar to the notion of number of different words in a language sample
Discriminative Action Frequency totals all differentiated play actions (similar to Total
Number of Words in a language sample), while Total Action Frequency includes the total
frequency of all play actions including indiscriminative actions.
Table 3-2
Quantitative Statistics of Play Actions for the DPA
Participant
Age
#1
#2
#3
#4
#5
#6
#7
#8
#9
#11
#12
3.25
2.92
3.33
2.25
3.33
3.58
4.08
2.75
3.83
2.58
4.25
Object
Interest
14
7
9
6
8
11
9
3
10
9
7
Diversity of
Play
25
15
20
12
18
22
17
5
23
13
13
Discriminative Play
Action Frequency
56
58
50
14
52
29
42
6
65
32
34
Repetitive
Play Score
2.24
3.87
2.50
1.17
2.89
1.32
2.47
1.20
2.83
2.46
2.62
Total Action
Frequency
88
79
75
38
68
68
74
13
85
71
44
Individual Characteristics: Measures of Language, Cognition, and Autism
Symptom Severity
Several tables summarize each participant’s performance on the standardized measures of
language, cognition, and autism symptom severity.
Table 3-3 displays the raw scores for the expressive and receptive language subtests
for both the Mullen and the PLS-5. Because these tests are normed using typically-
32
developing children, raw scores are reported, instead of standardized scores, to allow for
the most sensitivity of the measures. The receptive and expressive language raw scores
from the Mullen were also used in the correlational analysis, discussed later in this
chapter. The Mullen visual reception and fine motor raw scores are also displayed here.
Table 3-3
Raw Language Scores by Subtest
Participant
Visual
Reception
#1
#2
#3
#4
#5
#6
#7
#8
#9
#11
#12
28
33
20
25
26
25
40
14
31
20
23
Mullen Raw Scores
Fine
Receptive
Motor
Language
19
19
16
19
24
23
29
13
25
18
19
Expressive
Language
21
26
13
12
24
26
30
12
27
23
15
PLS Raw Scores
Auditory
Expressive
Comprehension Communication
11
21
9
10
11
16
24
6
21
15
6
24
34
19
21
26
34
37
12
33
26
19
19
25
15
22
22
25
28
15
27
24
14
Tables 3-4 and 3-5 show the standard scores, or T-scores, composite scores, and age
equivalents for PLS-5 and the Mullen, respectively.
Table 3-4
Standard Scores, Composite Scores, and Age Equivalents for PLS-5
Age:
Auditory
Comprehension
Standard Score
Expressive
Communication
Standard Score
Total Language
Standard Score
1
2
3
4
5
6
7
8
9
11
12
3.25
2.92
3.33
2.25
3.33
3.58
4.08
2.75
3.84
2.58
4.25
62
98
50
73
67
78
77
50
76
76
50
58
77
50
77
64
64
64
54
68
75
50
57
86
50
74
63
69
69
50
70
75
50
33
Total Language
Age Equivalent
1.42
2.17
1.0
1.42
1.67
2.17
2.5
0.75
2.25
1.75
0.92
Table 3-5
Standard Scores, Composite Scores, and Age Equivalents for Mullen
1
2
3
4
5
6
7
8
9
11
12
Age:
Receptive
Language TScore
Expressive
Language TScore
3.25
2.92
3.33
2.25
3.33
3.58
4.08
2.75
3.84
2.58
4.25
20
35
20
20
20
20
26
20
20
20
20
20
28
20
20
20
20
20
20
20
20
20
Fine Motor
T-Score
20
20
20
20
20
20
20
20
20
33
20
25
45
20
39
20
20
39
20
22
21
20
50
67
49
55
49
49
58
49
49
54
49
1.67
2.25
0.92
0.83
2.0
2.25
2.75
0.83
2.33
0.92
1.17
0.83
1.83
0.67
0.75
0.83
1.33
2.17
0.42
1.83
1.25
0.42
Visual
Reception
T-Score
Early Learning
Composite
Standard Score
Receptive
Language Age
Equivalent
Expressive
Language Age
Equivalent
Tables 3-6 and 3-7 represent the item analysis performed using each participant’s
responses on the PLS-5. The participants are arranged in ascending order, according to
chronological age. This was used for determining language skill acquisition throughout
each year of development in an attempt to better understand the developmental
progression of language skills in young boys with FXS.
Table 3-6
Item Analysis of Participants’ Scores on the PLS-5 Auditory Comprehension subtest
Age:
2.25
2.58
2.75
2.92
3.25
3.33
3.33
3.58
3.83
4.08
4.25
34
Participant:
Item:
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
18
19
20
21
22
23
24
25
26
27
28
29
30
31
32
33
34
35
36
37
38
39
40
41
42
43
44
45
46
47
48
4
1
1
1
1
0
1
0
0
0
0
1
0
0
0
0
0
0
11
1
1
1
1
0
1
0
0
1
1
1
0
0
1
1
0
1
0
0
0
0
0
0
8
1
1
1
1
0
0
1
1
1
0
1
0
1
0
0
1
1
0
1
0
0
0
0
0
0
2
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
0
1
0
0
1
1
0
0
0
0
0
0
1
1
1
1
1
1
1
0
0
1
1
1
0
0
0
0
0
0
3
1
1
1
1
0
0
0
0
0
0
5
1
1
1
1
1
1
1
1
1
1
1
0
0
1
1
0
1
1
0
0
0
0
0
0
6
1
1
1
1
1
1
1
1
1
1
1
1
1
0
1
1
1
0
1
1
0
1
0
0
1
0
1
0
0
0
0
0
0
9
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
0
0
0
0
0
0
7
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
1
0
0
0
1
1
0
1
0
0
1
1
0
0
0
0
12
1
1
1
1
0
0
0
0
0
0
35
49
50
51*
0
0
*Items #52-65 were omitted because they were not administered to any of the
participants because they all previously achieved a ceiling.
Table 3-7
Item Analysis of Participants’ Scores on the PLS-5 Expressive Communication subtest
Age:
Participant:
Item:
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
18
19
20
21
22
23
24
25
26
27
28
29
30
31
32
33
34
2.25
4
1
1
1
0
1
1
1
1
1
0
1
0
0
1
1
1
1
0
0
0
0
0
0
2.58
11
1
1
1
1
1
1
1
0
1
1
1
1
0
0
0
0
0
0
2.75
8
1
1
1
0
1
1
0
0
0
0
1
0
0
0
0
0
1
0
0
0
0
0
0
2.92
2
1
1
1
0
1
1
1
1
1
0
0
0
0
0
0
3.25
1
1
1
1
0
0
1
0
0
0
0
1
1
0
0
0
0
0
0
3.33
3
1
1
1
0
1
1
1
0
0
0
1
0
0
0
0
0
0
3.33
5
1
1
1
1
1
1
1
1
1
1
1
0
0
1
0
1
1
0
0
0
0
0
0
3.58
6
1
1
1
1
1
1
1
0
1
1
1
1
1
0
0
0
0
0
0
3.83
9
1
1
1
1
1
1
1
0
1
0
0
0
0
0
0
4.08
7
1
1
1
1
1
1
1
1
1
0
1
0
1
0
0
0
0
4.25
12
1
1
1
1
1
1
0
0
0
1
0
0
1
0
0
1
0
0
1
0
0
1
0
0
0
0
0
0
36
35
36
37*
0
0
*Items #38-67 were omitted because none of the participants were administered these
items because they previously achieved a ceiling.
Table 3-8 displays results from a parent report measure, the MacArthur-Bates
Communication Development Inventory: words and gestures form, which totals noted
words in each child’s receptive and expressive vocabulary. This table also includes the
ADOS results, including the Gotham severity score and classification.
Table 3-8
Communicative Development Inventory of Receptive and Expressive Vocabulary and
Autism Severity Score and Spectrum Classification
Participant:
#1
#2
#3
#4
#5
#6
#7
#8
#9
#11
#12
MacArthur Bates CDI
# of Words
# of Words
Understands &
Understands
Says
128
1
254
71
254
0
303
33
177
2
193
40
65
206
0
0
257
32
71
38
61
0
ADOS
Severity Score
Gotham
Classification
4
4
6
n/a*
4
4
6
6
1
4
10
ASD
ASD
Autism
n/a*
ASD
ASD
Autism
Autism
Non-Spectrum
ASD
Autism
*Participant #4 was given the Toddler module of the ADOS and, therefore, a severity
score and autism spectrum classification was unable to be calculated using the Gotham
algorithm.
37
Concurrent Correlational Analysis
Tables 3-9, 3-10a, and 3-10b display the results of concurrent correlational
analysis used to determine any possible associations between the variables of play,
language, and autism symptom severity. Table 3-9 describes the order statistics, mean,
standard deviations for the variables used in the correlational analysis. Sample size was
consistently eleven for all variables, with the exception of correlations involving the
ADOS severity score because one participant was administered the Toddler module (due
to his age) for which severity scores are not available. .
Table 3-9
Psychometric Properties of the Play, Language, and other Variables used in
Correlational Analysis
Age
Mullen Visual Reception Raw
Score
Mullen Fine Motor Raw Score
Mullen Receptive Language
Raw Score
Mullen Expressive Language
Raw Score
MacArthur-Bates CDI Words
Understands
MacArthur-Bates CDI Words
Understands & Says
ADOS Severity Score
Time (in seconds)
Object Interest
Diversity of Play
Discriminative Play
Action Frequency
Total Play Action Frequency
N
Minimum
Maximum
Mean
11
11
2.25
14.0
4.25
40.0
3.2864
25.909
Standard
Deviation
0.62771
7.0775
11
11
13.0
12.0
29.0
30.0
20.364
20.818
4.5005
6.6456
11
6.0
24.0
13.636
6.2333
11
0.0
336.0
198.727
111.4245
11
0.0
206.0
38.455
60.3098
10
1.0
10.0
4.900
2.3310
11
677.0
720.0
714.7273
13.29730
11
3.0
14.0
8.455
2.8413
11
5.0
25.0
16.636
5.8185
11
6.0
65.0
39.818
18.7233
11
7.0
39.0
23.9091
10.83009
38
Tables 3-10a and 3-10b display results from the one-tailed Spearman rank-order
correlation (rho) correlations. A significant correlation was found between the Mullen
Visual Reception raw score and frequency of Discrimative Play Actions (r= .667, p=
.025, one-tailed) and a marginally significant correlation was found between the Mullen
Visual Reception raw score and frequency of Total Play Actions (r= .588, p= .057). The
correlations with the Mullen Receptive Language raw scores were hovering for Object
Interest (r= .529, p= .094), frequency of Discriminative Play Action (r= .557, p= .075),
and frequency of Total Play Actions (r= .538, p= .088). There is also a hovering negative
correlation between the ADOS Severity Score and Diversity of Play (r= -.559, p=
.093). It should also be noted that no correlations were found between Time and any of
the play variables.
Table 3-10a
Concurrent Correlations between Play and Other Participant Characteristics
Object Interest
Diversity of
Play
Discriminative
Action
Frequency
Total Action
Frequency
Time
Age
Visual
Reception
Fine Motor
Object
Interest
1.000
-.896**
.000
.446
Diversity
Play
.896**
.000
1.000
-.679*
Discrimin.
Action
Frequency
.446
.085
.679*
.011
1.000
Total Action
Frequency
.647*
.011
.382
.123
-.046
Time
Age
ADOS
.068
.421
.068
.422
.202
.364
.136
.447
.084
.342
-.526
.059
-.559*
.047
-.531
.085
0.11
--
.447
.275
.152
.057
.674*
.011
.068
.421
.364
.136
.333
.159
.395
.382
.123
.068
.422
.447
.084
.469
.073
.473
-.046
.447
.202
.275
.342
.152
.667*
.013
.428
1.000
-.237
.241
-.105
.379
.147
.333
.124
.237
.241
1.000
-.311
.176
.271
.210
.449
-.105
.379
.311
.176
1.000
-.350
.146
.597*
-.323
.181
-.131
.359
.256
.237
-.375
.143
-.365
39
Receptive
Language
Expressive
Language
CDI
Understands
CDI
Understands &
Says
ADOS
.114
.529*
.047
.486
.065
.046
.447
.204
.071
.517
.052
.429
.094
.196
.282
.064
.095
.557*
.038
.485
.065
.355
.142
.101
.358
.323
.166
.490
.063
.196
.281
.562*
.083
.433
.091
.401
.111
.539*
.043
.463
.026
.531*
.046
.213
.264
-.064
.426
-.032
.150
-.520
.062
-.543
.053
-.439
.102
-.431
.274
.425
.384
.036
.076
.463
.107
-.526
.059
-.559*
.047
-.531
.057
-.323
.181
-.131
.359
.256
.237
1.000
--
*Correlation is significant at the 0.05 level (1-tailed).
**Correlation is significant at the 0.01 level (1-tailed).
Table 3-10b
Concurrent Correlations between Play and Other Participant Characteristics
Object Interest
Diversity of
Play
Discriminative
Action
Frequency
Total Action
Frequency
Time
Age
Visual
Reception
Fine Motor
Receptive
Language
Expressive
Language
CDI
Understands
CDI
Understands &
Visual
Reception
Fine
Motor
Receptive
Language
Expressive
Language
CDI
Understands
.333
.159
.469
.073
.667*
.395
.114
.473
.071
.428
.529*
.047
.517
.052
.557*
.486
.065
.429
.094
.485
.046
.447
.196
.282
.355
CDI
Understands &
Says
.204
.274
.064
.425
.101
.013
.095
.038
.065
.142
.384
.147
.333
.271
.210
.350
.146
1.000
-.811*
.001
.771*
.003
.795**
.002
.616*
.022
.636*
.124
.358
.449
.083
.597*
.026
.811**
.001
1.000
-.804**
.001
.700**
.008
.442
.087
.549*
.323
.116
.433
.091
.531*
.046
.771**
.003
.804**
.001
1.000
-.920**
.000
.347
.148
.710**
.490
.063
.401
.111
.213
.264
.795**
.002
.700**
.008
.920**
.000
1.000
-.554*
.039
.873**
.196
.281
.539*
.043
-.064
.426
.616*
.022
.442
.087
.347
.148
.554*
.039
1.000
-.569*
.562*
.036
.463
.076
-.032
.463
.636*
.018
.549*
.040
.710**
.007
.873**
.000
.569*
.034
1.000
.018
.040
.007
.000
.034
--
40
Says
ADOS
-.375
.143
-.365
.150
-.520
.062
-.543
.053
*Correlation is significant at the 0.05 level (1-tailed).
**Correlation is significant at the 0.01 level (1-tailed).
-.439
.102
-.431
.107
41
Chapter 4
Discussion
This pilot study was conducted to answer the following research questions:
(1) What are the concurrent correlations between the play skills, receptive and
expressive language abilities and cognitive abilities of young males affected by fragile X
syndrome?
(2) How can the play of young males affected by fragile X syndrome be described
and characterized?
This pilot study began with 12 participants and ended with 11 participants, due to
one participant’s social anxiety which precluded his ability to complete all necessary
assessment tasks. Standardized measures of language, cognition, and autism symptom
severity were conducted as a part of a larger intervention study at the UC Davis M.I.N.D.
Institute. The Developmental Play Assessment (DPA) was also administered and was the
main focus of this study, providing both quantitative and qualitative data. As a part of the
larger study, a correlational analysis was performed using each participant’s age, raw
scores from the subtests of the Mullen Scales of Early Learning, raw scores from the
MacArthur-Bates CDI, autism severity scores derived from the ADOS, length of play
sample, and scores from the DPA. The correlational analysis performed was a Spearman
rank-order correlation (rho) using one-tailed significance levels. Results from this
analysis revealed that, as a child’s visual reception skills increase, typically their play
actions increase in number and the number of their discriminative play acts increases.
Visual reception, a portion of this study’s measure of cognition, is a skill which measures
42
the child's ability to process information using patterns, memory and sequencing (Mullen,
1995). The correlational analysis also revealed that as a child’s receptive language skills
develop, their number of discriminative play acts, number of discriminative play acts on
different toys, and number of their total play actions may increase. Statistical evidence
also suggests that for some children, as their autism symptom severity decreases, these
children begin to demonstrate more diverse play skills. These findings, when combined
with the data gathered from the PLS-5, are helpful in answering the stated research
questions in this study.
The DPA was found to be an appropriate assessment instrument for children with
FXS and it is recommended for both researchers and clinicians by the principal
investigator of this pilot study. Use of a criterion-referenced assessment, such as the
DPA, proves better suited than a norm-referenced assessment for children with
developmental disabilities. The DPA instrument, in particular, provides the examiner
with knowledge of which play level is emerging and what activities are developmentally
relevant to that child (Lifter, 2000). These activities are those beyond what the child has
mastered, but are those that the child has begun to experiment with.
Table 4-1 was formulated to describe how play correlates with receptive and
expressive language in young boys with FXS. In this table, a descriptive developmental
trajectory of play and language is described for children with FXS from 1 to 4 years of
age. This scale was constructed by performing an analysis of language items from the
PLS-5 and using mastery criteria calculations for the DPA. In this scale, mastered
language skills were determined by selecting the items in which at least 50% of sample
43
participants in that age group had correctly answered. Mastered play levels for each age
range were determined by selecting the level in which at least 50% of participants had
met mastery criteria for that play category.
Table 4-1
Language and Play Scale for Young Boys with FXS
Age:
1 years*
2 years
Auditory Comprehension
Attention to People:
Glances momentarily at a
person who talks to
him/her
Enjoys caregiver’s attention
Actively searches to find a
person who is talking
Anticipated what will
happen next
Interrupts activity when you
call his/her name
Looks at objects or people
the caregiver points to and
names
Attention to Environment:
Reacts to sounds other than
voices in the environment
Turns head to locate the
source of sound
Expressive Communication
Vocal Development:
Appropriate suck/swallow
reflex
Vocalizes soft, throaty sounds
Varies pitch, length, or
volume of cries
Vocalizes pleasure and
displeasure sounds
Social Communication:
Responds to speaker by
smiling
Vocalizes when talked to,
moving arms and legs
during vocalizations
Protests by gesturing or
vocalizing
Attempts to imitate facial
expressions and
movements
Vocal Development:
Vocalizes two different vowel
sounds
Combines sounds
Vocalizes two different
consonant sounds
Babbles two syllables
together
Produces syllable strings (two
or three syllables) with
inflection similar to adult
speech
Gesture:
Uses a representational
(symbolic) gesture
Uses gestures and
vocalizations to request
objects
Demonstrates joint attention
Play
1A—
Indiscrimiative
Actions
2B—TakeApart
Combinations
44
Responds to a new sound
Looks for object that has
fallen out of sight
Gesture:
Understands what you want
when you extend your
hands and say “Come
here”
Follows routine, familiar
directions with gestural
cues
Play:
Mouths objects
Shakes and bangs objects in
play
Demonstrates functional
play
Demonstrates relational play
Demonstrates self-directed
play
Engages in pretend play
Engages in symbolic play
3 years
4 years
Vocabulary/Connected
Speech:
Responds to an inhibitory
word (e.g. “No”)
Understands a specific word
or phrase without the use
of gestural cues
Identifies photographs of
familiar objects
Identifies things you wear
Understands the verbs “eat”,
“drink”, and “sleep” in
context
Recognizes action in
pictures
Vocabulary/Connected
Speech:
Identifies familiar objects
from a group of objects
without gestural cues
Follows commands with
gestural cues
Follows commands without
gestural cues
Vocabulary/Connected
Speech:
Identifies basic body parts
Understands pronouns (me,
Social Communication:
Seeks attention from others
Plays simple games with
another while using
appropriate eye contact
Participates in a play routine
with another person for at
least 1 minute while using
appropriate eye contact
Initiates a turn-taking game
or social routine
Vocabulary/Connected
Speech:
Uses at least one word
Imitates a word
Uses at least five-words
Social Communication:
Takes multiple turns
vocalizing
3A—
Presentation
Combinations
Vocabulary/Connected
Speech:
Names objects in photographs
Vocal Development:
Produces different types of
consonant-vowel (C-V)
combinations
2A—
Discriminative
Actions
45
my, your)
Understands use of objects
Identifies advanced body
parts
Basic Concepts:
Understands spatial concepts
(in, on, out, of ,off)
without gestural cues
Understands quantitative
concepts (one, some, rest,
all)
Identifies colors
Social Communication:
Uses words for a variety of
pragmatic functions
Vocabulary/Connected
Speech:
Names a variety of pictured
objects
Morphology/Syntax:
Understands sentences with
post-noun elaboration
Understands pronouns (his,
her, he, she, they)
Emergent Literacy Skills:
Points to letters
*The actions described at the 1 year age-level represent language items and a play level
that was previously mastered by all participants. The language items noted were those
below the basal of all participants.
As described earlier, play and language, of typically-developing and children with
developmental disabilities, form an important developmental relationship. However,
children with developmental disabilities present with challenges unique to them or their
disability which often slows skill acquisition and may prevent this development from
mirroring the hierarchical development of children with developmental disabilities.
The results from this study were used to describe the developmental progression
of language and play in children with FXS, ages one to five. This developmental
trajectory is unlike that of typically-developing children and groups of children with other
developmental disabilities. The following is a discussion of this unique progression:
46
From 1 to 2 years of age, these children have strengthened their oral-facial
muscles, are beginning to imitate facial expressions, and they are beginning to
experiment with their voice pitch and volume. They use vocalizations or gestures to
express positive and negative feelings or to acknowledge persons speaking to them. As
they are exploring with their voice and expression, they are exploring toys in the earliest
stages of play. At this time, they are treating all objects alike during play, mouthing,
banging, shaking, or inspecting, as they begin to learn what they are and what they do.
This period of development is one of exploration, across all areas.
From 2 to 3 years of age, attention continues to develop as they are able to locate
a person speaking, respond to their own name, and they demonstrate joint attention. They
are also beginning to direct their attention to other things in their environment, including
reacting to sounds other than voices, object permanence, and a basic understanding of
gestural cues. Receptive vocabulary expands to include words like “eat”, “drink”, and
“sleep”, other actions, clothing, familiar objects, and inhibitory words like “no”. Their
expressive language includes at least 5 words, as well as more gestures and vocalizations
to request objects. Socially, they seek attention, imitate turn-taking during a
game/routine, and engages in a play routine with a partner for at least one minute. As
their play exploration continues, these children are engaging in take-apart combinations
(e.g., knocking down stacked blocks, taking off a dolls clothes) to continue developing an
understanding of what things are and what they do. This period of development can be
described as the attention stage, as the child is learning what is important to direct their
47
attention to, is developing joint attention, and is seeking attention from a familiar
communication partner for social engages, as well as wants and needs.
From 3 to 4 years of age, vocabulary is a main point of development. These
children are beginning to identify a familiar object within a group of other objects and are
beginning to name photographed objects. They are also mastering following commands
without gestures and are beginning to take turns vocalizing. Play development continues
with mastery of presentation combinations, which describes the ability to assemble
objects or toys into their intended configuration (e.g., putting animals into barn, putting
clothes onto a doll). At this time, play is aiding in the child’s ability to categorize or
classify their vocabulary words.
From 4 to 5 years of age, receptive vocabulary continues to expand to include:
basic and advanced body parts, colors, letters, pronouns (e.g., me, my, she, they), spatial
and quantitative basic concepts. They continue to expand their expressive vocabulary for
naming objects and begin to use words for the purpose of social communication. Play
now includes discriminative actions, which are those that demonstrate knowledge of the
object’s physical or conventional characteristics (i.e. rolls round bead, squeezes a stuffed
animal). As new descriptive vocabulary is emerging, the child’s attention is focused on
these details and continually expanding their knowledge.
Each stage of play and language development is not only unique to children with
FXS but also demonstrates distinct areas of development which correspond with one
another. The results from this study reveal that although the play and language
development in children with FXS is more delayed and inconsistent than that of TD
48
children and those with other developmental disabilities, areas of play and language
development appear to correspond with one another. In this sample study, it was shown
that play and language continue to form a developmental bond.
49
Chapter 5
Implications, Limitations, and Future Directions
Implications
As previously stated, the research done regarding play and language in children
with FXS, as performed in this pilot study, is unprecedented though the information is
vital. Studies have continually shown that the areas of play and language, in typicallydeveloping children and children with various developmental disabilities, are each
influential in the development of the other. However this had not been studied in children
with FXS until now.
From a clinical perspective, play is often used as a tool for a clinician to connect
with children and a tool for young children to explore their environment and learn new
things. In therapy, before beginning to work with a child, it is important to understand
what skills the child has mastered, what he or she is beginning to learn, and what the
child does not know how to do. This provides a baseline and gives the clinician ideas as
to what goals the child should begin working toward. The DPA was created for this
purpose and gives professionals the ability to reliably compare the abilities of children
with developmental disabilities in relation to typically developing children.
The results of this study may influence the direction of speech therapy for
children with FXS. Children with FXS have many unique physical and developmental
characteristics that differentiate them from children with other developmental disabilities.
The information from this study will provide clinicians information specific to the
developmental trajectory of young boys with FXS, which may also provide insight into
50
both assessment and therapy standards. This should improve the overall service delivery
to these clients.
Limitations
There are several limitations to this study that should be considered. First, the
number of participants limits the generalizability of this study. The children chosen
needed to meet the inclusion and exclusion criteria, which limited the overall size of the
study. Second, only standardized language measures were used to evaluate the child’s
abilities. Past studies have shown that many standardized assessments do not represent
the true language abilities of children with developmental disabilities. Within the
confines of a research study, it may be difficult to truly represent all participants’
abilities. Third, due to the anxiety that typically occurs within children with FXS, it
initially proved difficult for children to willingly engage in the play assessment. This may
have impacted the scoring, as the time allotted for each toy set within the DPA was
adjusted to 3 minutes each. Some children performed more play actions and possibly
more types of play after the coding was discontinued.
Last, in order to truly draw conclusions on typical developmental trajectory for
this population of children, future research must be performed using a larger sample of
children.
Future Directions
As previously stated, there are no current published studies regarding play in
children with FXS or the correlations between play and language in FXS. Time and
again, play and language have been consistently shown to develop in a parallel manner.
51
Play and language both support each other during development, regardless of whether the
child has a developmental disability or not. Children with developmental disabilities are
not all the same. As highlighted in this thesis, there are many unique characteristics of
children with FXS, such as their possible autism symptoms, difficulty with executive
functioning, and issues with attention and memory, that are likely affecting their
language, play, and cognitive development. This makes the need for studies such as these
important and even more imperative in such understudied groups.
Future studies should attempt to isolate and evaluate other developmental
correlates. For example, a study examining the correlation between autism symptom
severity and play and language development may highlight skill acquisition for specific
subsets of the FXS population.
52
Appendix A
Letter of Consent
Dear Parent:
My name is Alex Stewart and I work as a Project Assistant at the UC Davis
M.I.N.D. Institute’s Laboratory on Language Development in Neurodevelopmental
Disorders. I am also a graduate student at California State University Sacramento in the
Speech Pathology Program. I am conducting a thesis research study under the supervision
of Dr. Robert Pieretti about the play skills and language abilities specifically of young
children with fragile X syndrome. This is important because of the small amount of
research done on this subject.
If you agree to participate in this study, a project staff member will conduct a
developmental play assessment with your child during your first visit to the M.I.N.D.
Institute for the Intervention Project. During this assessment, a variety of toys will be
presented to your child and he will be encouraged to play with the toys in an unstructured
manner. The play assessment will last approximately 30 minutes during which the project
staff member will observe your child’s play skills. You will also be able to observe the
assessment from the observation room. These interactions will be videotaped to be
analyzed later.
It is possible that your child may not benefit directly from participating in this
study. The potential benefit is that you may learn how to support your child’s language
development. In addition, the results of this study may help us to develop more effective
interventions for enhancing the development of other young children with fragile X
syndrome.
All information collected will be kept confidential. Your child will be assigned a
number that will be placed on all documents related to their performance and these
documents will be stored in a locked file cabinet, only accessible by approved staff.
Information linking child’s identity to data will be stored separately. Videotapes of the
play and language evaluations will be stored on the M.I.N.D. Institute’s firewall and
password-protected server, labeled only by a number. These tapes will be stored indefinitely
and be may be used for training purposes. There is no compensation for participating in this
project. Your child will receive motivational stickers for completing each assessment and
a picture book at the conclusion of the assessments.
If you do not want your child to participate in this study, it will in no way affect
your participation in the Intervention Project. By agreeing to participate in this study, you
will also be allowing me to access your child’s assessment scores from the Intervention
Project. If you give permission to participate, you may change your mind at any time
during the study and your child can be removed from the study.
If you would like your child to participate in this study, in addition to the
Intervention Study, please sign the attached sheet and return this letter to me or another
project staff member, along with your informed consent before beginning the study.
If you have any questions about any part of the project, please feel free to contact Andrea
McDuffie, Project Manager, my Thesis Supervisor, Dr. Robert Pieretti, or you may
contact me directly.
53
Sincerely, Alex Stewart
Project Director
Speech Pathology Department
California State University, Sacramento
I give permission for my child
_________________________________________________to participate in this study.
Signature:_______________________________________________________
Date:___________________________________________________________
54
Appendix B
Letter of Thesis Approval from UC Davis Advisor
December 2, 2013
Robert Pieretti, Ph.D., CCC-SLP
Ann Blanton, Ph.D., CCC-SLP
Department of Speech Pathology and Audiology
California State University, Sacramento
6000 J Street
Sacramento, California 95819
RE: Alex Stewart Thesis: Characterizing Play Skills and Identifying Their Association
with Expressive and Receptive Language Skills in Young Boys with Fragile X Syndrome
Dear Drs. Pieretti & Blanton:
I have reviewed the final draft of the thesis that Alex Stewart is completing for her
culminating experience in the Master’s Degree Program in Speech Pathology and
Audiology at Sacramento State.
The thesis satisfactorily represents Alex’s work and the original research conducted by
our research team at the U.C. Davis MIND Institute.
Best,
Andrea McDuffie, Ph.D., CCC-SLP
Laboratory on Language Development in Neurodevelopmental Disorders
UC Davis M.I.N.D. Institute
55
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