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 Page

2-1 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 X- associated tremor/ataxia syndrome (FXTAS), and anxiety. FXS is inherited when a pre- or 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 year- old 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 life- size. 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?

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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 Speech-

Language 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 examiner- child 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.

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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 double- checked once entered by another researcher before being used for analysis.

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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

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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 Definition Example Level I Indiscriminate Actions All objects are treated Mouthing and banging alike II Discriminative actions Differentiates among Rolls round beads, squeezes on single objects object, preserving their stuffed animal physical or conventional characteristics Take apart Separates configurations Takes pieces out of puzzle combinations of object

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III Presentation Recreates combination of Puts pieces into puzzle, nests combinations objects according to nesting cups original configuration General combinations Creates combinations of Puts beads in cup, puts boy in objects that result in box nonspecific combinations Pretend self Relates object to self with Brings empty cup to mouth pretend quality IV Specific combinations Preserves unique physical Stacks nesting cups, stacks based on physical characteristics of objects blocks, strings beads attributes in configuration V Child as agent Extends familiar actions to Feeds doll, combs doll’s hair doll figure Specific combinations Recreates conventional Places cup on saucer, wears based on conventional actions on objects in string of beads attributes configuration VI Single scheme Extends same action to Extends cup to baby doll and sequences two or more figures then to examiner Object substitutions Uses one object to stand Uses block for phone, use for another bowl for hat VII Doll as agent Moves doll figure as if it is Puts brush into doll’s hand capable of action Multi-scheme Recreates a sequence of Wipes doll’s face and puts to sequences actions with figure bed VIII Socio dramatic play Adopts familiar role in Plays house or doctor play theme Thematic fantasy play Adopts role of TV, book Plays Superman or or movie character 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.

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Chapter 3

Results

Individual Characteristics: Developmental Play Assessment

Several tables summarize each participant’s performance on the DPA. Tables 3-

1a 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

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I Indiscriminative Actions: Treats all objects alike 32 21 25 24 16 39

II Discriminative Actions: Actions on single objects 6/5 9/4 2/2 3/1 20/4 2/2

Take Apart Combinations: Separates configurations of 17/7 11/4 19/7 5/5 17/6 17/9 objects III Presentation Combinations: Re-assembles presentation 17/4 35/3 22/6 5/4 5/2 5/4

General Combinations: Assembles undifferentiated 0 0 7/2 1/1 9/3 2/2 configurations Pretend Self: Relates objects to self in pretend 4/2 1/1 0 0 1/1 1/1

IV Specific Physical Combinations Preserves physical characteristics 4/1 0 0 0 0 0 in configuration V Child-as-Agent: Extends familiar actions to dolls 8/3 2/2 0 0 0 2/1 or figures Specific Conventional Combinations: 0 0 0 0 0 0 Preserves conventional features in configuration VI Single-Scheme Sequences: Extends same action to multiple 6/5 0 0 0 0 0 figures Substitutions: Uses one object as substitute for 0 0 0 0 0 0 another VII Doll-as-Agent: Attributes actions to dolls or 0 0 0 0 0 0 figures Multi-scheme Sequences: Extends different actions to dolls 0 2/2 0 0 0 0 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

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I Indiscriminative Actions: Treats all objects alike 32 7 18 -- 39 10

II Discriminative Actions: Actions on single objects 9/3 0 10/6 -- 1/1 16/4

Take Apart Combinations: Separates configurations of 15/4 6/4 21/8 -- 19/7 9/4 objects III Presentation Combinations: Re-assembles presentation 11/3 0 27/5 -- 11/3 7/2

General Combinations: Assembles undifferentiated 0 0 1/1 -- 0 2/1 configurations Pretend Self: Relates objects to self in 2/1 0 1/1 -- 1/1 0 pretend IV Specific Physical Combinations Preserves physical 2/1 0 0 -- 0 0 characteristics in configuration V Child-as-Agent: Extends familiar actions to 0 0 5/2 -- 0 0 dolls or figures Specific Conventional Combinations: 0 0 0 -- 0 0 Preserves conventional features in configuration VI Single-Scheme Sequences: Extends same action to 0 0 2/3 -- 0 0 multiple figures Substitutions: Uses one object as substitute 0 0 0 -- 0 0 for another VII Doll-as-Agent: Attributes actions to dolls or 3/2 0 0 -- 0 0 figures Multi-scheme Sequences: Extends different actions to 0 0 0 -- 0 0 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 Object Diversity of Discriminative Play Repetitive Total Action Interest Play Action Frequency Play Score Frequency #1 3.25 14 25 56 2.24 88 #2 2.92 7 15 58 3.87 79 #3 3.33 9 20 50 2.50 75 #4 2.25 6 12 14 1.17 38 #5 3.33 8 18 52 2.89 68 #6 3.58 11 22 29 1.32 68 #7 4.08 9 17 42 2.47 74 #8 2.75 3 5 6 1.20 13 #9 3.83 10 23 65 2.83 85 #11 2.58 9 13 32 2.46 71 #12 4.25 7 13 34 2.62 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

Mullen Raw Scores PLS Raw Scores Visual Fine Receptive Expressive Auditory Expressive Participant Reception Motor Language Language Comprehension Communication #1 28 19 21 11 24 19 #2 33 19 26 21 34 25 #3 20 16 13 9 19 15 #4 25 19 12 10 21 22 #5 26 24 24 11 26 22 #6 25 23 26 16 34 25 #7 40 29 30 24 37 28 #8 14 13 12 6 12 15 #9 31 25 27 21 33 27 #11 20 18 23 15 26 24 #12 23 19 15 6 19 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

1 2 3 4 5 6 7 8 9 11 12 Age: 3.25 2.92 3.33 2.25 3.33 3.58 4.08 2.75 3.84 2.58 4.25 Auditory Comprehension 62 98 50 73 67 78 77 50 76 76 50 Standard Score Expressive Communication 58 77 50 77 64 64 64 54 68 75 50 Standard Score

Total Language 57 86 50 74 63 69 69 50 70 75 50 Standard Score

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Total Language 1.42 2.17 1.0 1.42 1.67 2.17 2.5 0.75 2.25 1.75 0.92 Age Equivalent

Table 3-5

Standard Scores, Composite Scores, and Age Equivalents for Mullen

1 2 3 4 5 6 7 8 9 11 12 Age: 3.25 2.92 3.33 2.25 3.33 3.58 4.08 2.75 3.84 2.58 4.25 Receptive Language T- 20 35 20 20 20 20 26 20 20 20 20 Score Expressive Language T- 20 28 20 20 20 20 20 20 20 20 20 Score

Fine Motor 20 20 20 20 20 20 20 20 20 33 20 T-Score Visual Reception 25 45 20 39 20 20 39 20 22 21 20 T-Score Early Learning Composite 50 67 49 55 49 49 58 49 49 54 49 Standard Score Receptive Language Age 1.67 2.25 0.92 0.83 2.0 2.25 2.75 0.83 2.33 0.92 1.17 Equivalent Expressive Language Age 0.83 1.83 0.67 0.75 0.83 1.33 2.17 0.42 1.83 1.25 0.42 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

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Participant: 4 11 8 2 1 3 5 6 9 7 12 Item: 1 1 2 1 3 1 4 1 5 0 6 0 7 1 8 1 9 1 10 0 11 1 12 0 1 13 1 1 14 0 1 1 15 0 1 1 16 1 1 1 1 1 1 1 1 1 1 17 1 1 1 1 1 1 1 1 1 1 18 1 1 0 1 1 1 1 1 1 1 1 19 1 1 1 1 1 1 1 1 1 1 1 20 0 0 0 1 1 0 1 1 1 1 0 21 1 1 0 1 1 0 1 1 1 1 0 22 0 0 0 1 0 0 1 1 1 1 0 23 0 0 0 1 0 0 0 1 1 1 0 24 0 1 0 1 1 0 0 1 1 1 0 25 0 1 0 1 1 0 1 1 1 1 0 26 1 1 1 1 1 1 1 1 27 0 0 1 0 0 0 1 1 28 0 0 1 0 1 1 1 1 29 0 1 1 0 1 1 1 1 30 0 1 1 0 0 1 1 1 31 0 0 1 0 0 0 1 1 32 0 1 0 0 0 1 1 1 33 0 1 0 1 1 1 34 0 0 0 0 0 0 35 0 0 0 1 0 0 36 0 1 0 0 0 37 0 1 0 0 1 38 0 0 1 0 1 39 0 0 0 0 40 0 1 1 41 0 0 0 42 0 0 0 43 0 0 1 44 0 1 45 0 0 46 0 0 47 0 48 0

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49 0 50 0 51*

*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: 2.25 2.58 2.75 2.92 3.25 3.33 3.33 3.58 3.83 4.08 4.25 Participant: 4 11 8 2 1 3 5 6 9 7 12 Item: 1 2 3 4 1 5 1 6 1 7 1 8 1 9 1 1 1 1 1 10 1 1 1 1 0 11 1 1 1 1 0 12 0 0 0 1 0 13 1 1 1 1 1 14 1 1 1 1 1 1 1 0 15 1 1 0 1 1 1 1 0 16 1 1 0 1 0 1 1 1 17 1 1 0 0 0 1 1 0 18 0 1 0 1 0 0 1 1 1 0 19 1 1 1 1 1 1 1 1 1 1 20 0 1 0 1 0 0 0 1 1 1 0 21 0 0 0 0 0 0 0 0 1 1 0 22 1 1 0 1 0 0 1 1 1 1 1 23 1 1 0 1 0 0 0 1 1 1 0 24 1 1 0 1 1 0 1 1 1 1 0 25 1 1 1 1 1 0 1 1 1 1 1 26 0 0 0 1 0 0 1 1 1 0 27 0 0 0 0 0 0 0 0 0 0 28 0 0 0 0 0 0 0 1 1 0 29 0 0 0 0 0 0 0 0 0 0 30 0 0 0 0 0 0 0 0 1 0 31 0 0 0 0 0 0 0 0 0 0 32 0 0 0 0 33 0 0 34 0 0

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35 0 36 0 37*

*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

MacArthur Bates CDI ADOS # of Words Participant: # of Words Understands & Severity Score Gotham Understands Says Classification #1 128 1 4 ASD #2 254 71 4 ASD #3 254 0 6 Autism #4 303 33 n/a* n/a* #5 177 2 4 ASD #6 193 40 4 ASD #7 65 206 6 Autism #8 0 0 6 Autism #9 257 32 1 Non-Spectrum #11 71 38 4 ASD #12 61 0 10 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.

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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

Standard N Minimum Maximum Mean Deviation Age 11 2.25 4.25 3.2864 0.62771 Mullen Visual Reception Raw 11 14.0 40.0 25.909 7.0775 Score Mullen Fine Motor Raw Score 11 13.0 29.0 20.364 4.5005 Mullen Receptive Language 11 12.0 30.0 20.818 6.6456 Raw Score Mullen Expressive Language 11 6.0 24.0 13.636 6.2333 Raw Score MacArthur-Bates CDI Words 11 0.0 336.0 198.727 111.4245 Understands MacArthur-Bates CDI Words 11 0.0 206.0 38.455 60.3098 Understands & Says 10 1.0 10.0 4.900 2.3310 ADOS Severity Score

11 677.0 720.0 714.7273 13.29730 Time (in seconds)

11 3.0 14.0 8.455 2.8413 Object Interest

11 5.0 25.0 16.636 5.8185 Diversity of Play

Discriminative Play 11 6.0 65.0 39.818 18.7233 Action Frequency 11 7.0 39.0 23.9091 10.83009 Total Play Action Frequency

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

Discrimin. Object Diversity Action Total Action Time Age ADOS Interest Play Frequency Frequency Object Interest 1.000 .896** .446 .647* .068 .364 -.526 -- .000 .085 .011 .421 .136 .059 Diversity of .896** 1.000 .679* .382 .068 .447 -.559* Play .000 -- .011 .123 .422 .084 .047 Discriminative .446 .679* 1.000 -.046 .202 .342 -.531 Action .085 0.11 -- .447 .275 .152 .057 Frequency Total Action .674* .382 -.046 1.000 .237 -.105 -.323 Frequency .011 .123 .447 -- .241 .379 .181 Time .068 .068 .202 .237 1.000 .311 -.131 .421 .422 .275 .241 -- .176 .359 Age .364 .447 .342 -.105 .311 1.000 .256 .136 .084 .152 .379 .176 -- .237 Visual .333 .469 .667* .147 .271 .350 -.375 Reception .159 .073 .013 .333 .210 .146 .143 Fine Motor .395 .473 .428 .124 .449 .597* -.365

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.114 .071 .095 .358 .083 .026 .150 Receptive .529* .517 .557* .323 .433 .531* -.520 Language .047 .052 .038 .166 .091 .046 .062 Expressive .486 .429 .485 .490 .401 .213 -.543 Language .065 .094 .065 .063 .111 .264 .053 CDI .046 .196 .355 .196 .539* -.064 -.439 Understands .447 .282 .142 .281 .043 .426 .102 CDI .204 .064 .101 .562* .463 -.032 -.431 Understands & .274 .425 .384 .036 .076 .463 .107 Says ADOS -.526 -.559* -.531 -.323 -.131 .256 1.000 .059 .047 .057 .181 .359 .237 --

*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

Visual Fine Receptive Expressive CDI CDI Reception Motor Language Language Understands Understands & Says Object Interest .333 .395 .529* .486 .046 .204 .159 .114 .047 .065 .447 .274 Diversity of .469 .473 .517 .429 .196 .064 Play .073 .071 .052 .094 .282 .425 Discriminative .667* .428 .557* .485 .355 .101 Action Frequency .013 .095 .038 .065 .142 .384 Total Action .147 .124 .323 .490 .196 .562* Frequency .333 .358 .116 .063 .281 .036 Time .271 .449 .433 .401 .539* .463 .210 .083 .091 .111 .043 .076 Age .350 .597* .531* .213 -.064 -.032 .146 .026 .046 .264 .426 .463 Visual 1.000 .811** .771** .795** .616* .636* Reception -- .001 .003 .002 .022 .018 Fine Motor .811* 1.000 .804** .700** .442 .549* .001 -- .001 .008 .087 .040 Receptive .771* .804** 1.000 .920** .347 .710** Language .003 .001 -- .000 .148 .007 Expressive .795** .700** .920** 1.000 .554* .873** Language .002 .008 .000 -- .039 .000 CDI .616* .442 .347 .554* 1.000 .569* Understands .022 .087 .148 .039 -- .034 CDI .636* .549* .710** .873** .569* 1.000 Understands & .018 .040 .007 .000 .034 --

40

Says ADOS -.375 -.365 -.520 -.543 -.439 -.431 .143 .150 .062 .053 .102 .107

*Correlation is significant at the 0.05 level (1-tailed).

**Correlation is significant at the 0.01 level (1-tailed).

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: Auditory Comprehension Expressive Communication Play 1 years* Vocal Development: 1A— Appropriate suck/swallow Indiscrimiative reflex Actions 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 2 years Attention to People: Vocal Development: 2B—Take- Glances momentarily at a Vocalizes two different vowel Apart person who talks to sounds Combinations him/her Combines sounds Enjoys caregiver’s attention Vocalizes two different Actively searches to find a consonant sounds person who is talking Babbles two syllables Anticipated what will together happen next Produces syllable strings (two Interrupts activity when you or three syllables) with call his/her name inflection similar to adult Looks at objects or people speech the caregiver points to and names Gesture: Uses a representational Attention to Environment: (symbolic) gesture Reacts to sounds other than Uses gestures and voices in the environment vocalizations to request Turns head to locate the objects source of sound Demonstrates joint attention

44

Responds to a new sound Looks for object that has Social Communication: fallen out of sight Seeks attention from others Plays simple games with Gesture: another while using Understands what you want appropriate eye contact when you extend your Participates in a play routine hands and say “Come with another person for at here” least 1 minute while using Follows routine, familiar appropriate eye contact directions with gestural Initiates a turn-taking game cues or social routine

Play: Vocabulary/Connected Mouths objects Speech: Shakes and bangs objects in Uses at least one word play Imitates a word Demonstrates functional Uses at least five-words play Demonstrates relational play Demonstrates self-directed play Engages in pretend play Engages in symbolic play

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 3 years Vocabulary/Connected Social Communication: 3A— Speech: Takes multiple turns Presentation Identifies familiar objects vocalizing Combinations from a group of objects without gestural cues Vocabulary/Connected Follows commands with Speech: gestural cues Names objects in photographs Follows commands without gestural cues 4 years Vocabulary/Connected Vocal Development: 2A— Speech: Produces different types of Discriminative Identifies basic body parts consonant-vowel (C-V) Actions Understands pronouns (me, combinations

45

my, your) Understands use of objects Social Communication: Identifies advanced body Uses words for a variety of parts pragmatic functions

Basic Concepts: Vocabulary/Connected Understands spatial concepts Speech: (in, on, out, of ,off) Names a variety of pictured without gestural cues objects Understands quantitative concepts (one, some, rest, all) Identifies colors

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 typically- developing 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.

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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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