Parent-implemented Hanen program More than words in Angelman syndrome: A case study

Abstract

Children with Angelman syndrome (AS) exhibit significant social, communicative and cognitive difficulties. The aim of this case study was to describe the profile of communicative abilities of a child with AS, before and after the implementation of the Hanen program ‘More than words’ (MTW). Additionally, changes on the language directed at her in spontaneous interactions were analysed. Advances were observed regarding the quantity and quality of communicative acts displayed by the child, both in natural interactions and through the use of standardized instruments. Moreover, a different rate of development was noted, when comparing two time periods (with and without MTW intervention). Linguistic input provided by the mother also exhibited some variations after treatment. Our findings suggest that MTW could be a promising approach to use with the communicative challenges experienced by AS children. Further research is needed to add more evidence about the suitability of the MTW program for AS individuals.

Keywords

Angelman syndrome communication family-centered intervention Hanen program language

I Introduction

Angelman syndrome (AS) is a neurodevelopmental disorder caused by a deficient expression of the UBE3A gene, which is in turn due to several abnormalities of chromosome 15 (Dan, 2009). Four major molecular mechanisms are known to be involved in this syndrome. The most common one is the interstitial deletion of 15q11-q13 chromosome region (Peters et al., 2004b). Other variants include mutation in the UBE3A gene (Kishino et al., 1997), imprinting center defects (Yong-Hui et al., 1998) and paternal uniparental disomy. Several reports have suggested that these last genetic variants show a milder or atypical phenotype (Jolleff et al., 2006). In approximately 10% of the clinical cases, no genetic defect has been found, or at least, it is undetectable with existing technology.

According to prevalence data, AS affects between 1:10,000 and 1:40,000 live births (Clayton-Smith, 2001; Dan, 2009).The main clinical features of the neurobehavioral phenotype were summarized by Williams et al. (2006). All diagnosed cases present intellectual disability in the severe to profound range, lack of speech or minimal use of words, ataxic gait, unprovoked laughter or smiling, apparent happy demeanor, hyperactivity and short attention span. Although initially the laughing and smiling behaviors were considered pathological and context inappropriate (Williams and Frias, 1982), recent investigations have confirmed that these behaviors have a functional nature and occur more frequently in social interaction conditions (Mount et al., 2011; Oliver et al., 2002).

Other features that have been described include delay in the growth of head circumference and other traits such as tongue-thrusting, wide mouth, irregularly-spaced teeth, drooling, hypopigmentation, and eating and sleeping disturbances (Gentile et al., 2010). Seizures are very common and some stereotypic behaviors have been reported, such as hand-flapping and sensory-seeking behaviors like mouthing of hands or objects (Walz and Baranek, 2006) and attraction to water (Didden et al., 2006).

Regarding the communicative profile of individuals with AS, studies have shown a pronounced gap between expressive and receptive abilities (Andersen et al., 2001; Jolleff et al., 2006). Most children with AS do not develop oral speech and, in those cases in which it does develop, it typically consists of single words and word approximations (2 to 15) (Alvares and Downing, 1998). This severe impairment of expressive skills is more acute than would be expected on the basis of cognitive functioning level alone (Gentile et al., 2010). It is important to point out that greater impairments in communicative behavior are associated with lower levels of intellectual functioning, as well as with the presence of seizures and the use of anticonvulsive medication (Didden et al., 2004).

As few individuals with AS develop functional speech, most of them rely on other ways of communication. In this respect, Jolleff and Ryan (1993) found that the preferred form of communication was through gestures involving physical contact with the referent or the listener (e.g. push away unwanted objects). Furthermore, several forms of pre-linguistic behaviors have also been observed, such as pointing, reaching, looking at and giving items (Calculator, 2013; Penner et al., 1993). The use of aided approaches such as picture communication symbols and voice output devices has been reported (Andersen et al., 2001; Didden et al., 2009; Summers et al., 1995). However, hand gestures occur more rarely due to motor impairment (Alvares and Downing, 1998; Jolleff et al., 2006). In particular, Calculator (2014) conducted a study based on a survey that parents self-administered over the internet. Although electronic devices were rated very positively by approximately 50% of the parents, non-symbolic methods (e.g. natural gestures, non-speech vocalizations and physical manipulation) were deemed most important.

Different questionnaires, also completed by the parents, have been employed in order to achieve a better understanding of the main communicative functions used by AS individuals. According to this kind of assessment instruments, individuals with AS communicate primarily to request (to have their wants and needs met) and to reject (Didden et al., 2004). In contrast, many people with AS fail to (non)verbally label objects or activities, as well as to develop imitation abilities.

Regarding the strengths of AS individuals, two commonly-highlighted abilities have been noticed. One of them is non-verbal comprehension. A strong desire to interact socially with others has also been reported, compared to other kinds of intellectual disabilities (Clayton-Smith, 1993). However, difficulties have also been found in skills that are clearly linked to communication, such as joint attention initiation, which seems more impaired than the participant’s ability to respond to the joint attention bids of others (Summers and Impey, 2011). With regard to impairments in joint attention and imitation, there is increasing evidence suggesting comorbidity and/or overlap between AS and Autism Spectrum Disorders (ASD) (Bonati et al., 2007; Peters et al., 2004a). Nevertheless, there have been calls for caution in the over-diagnosis of autism in AS population, which may be due to the very low mental age of the research participants (Trillingsgaard and Ostergaard, 2004).

In relation to communication intervention studies in individuals with AS, there is a clear dearth of evidence. In one of the first studies about this topic, parents of nine children with AS were taught to use ‘enhanced natural gestures’, i.e. gestures consisting of motor components that were already in the child’s repertoire (Calculator, 2002). Although the children’s progress in acquiring these kinds of gestures was slow, the intervention program was considered by the majority of parents as acceptable, effective and easy to teach to others. However, some families argued that the excessive amount of time that they had to invest in order to implement the program interfered with the natural flow of everyday life.

Summers and Szatmari (2009) implemented discrete trial instruction with three children with AS to teach several functional skills, including following one-step directions, using picture symbols to request preferred items and asking for help to open a container. This training was rated positively by parents for helping to build functional skills in some children with AS. More recently, in an investigation conducted by Radstaake et al. (2012), participants were taught to use pictures and small objects as a means to request attention, tangibles and breaks from tasks. The results of the study showed a reduction in challenging behavior following intervention. Similar findings were observed when treatment was carried out by teachers in school settings (Radstaake et al., 2013).

This exploratory case study aimed to investigate the effects of using the Hanen program ‘More than words’ (MTW) (Sussman, 1999) for a mother and her child with a diagnosis of AS. Specifically, the purpose of this case report was to document changes produced in child communicative behavior and mother interactive style, following participation in the MTW program.

There is a paucity of research about communication intervention in AS. A naturalistic parent-focused intervention, such as MTW, might be a suitable match for the communicative profile that characterizes AS, and a positive response to such an intervention could be hypothesized. Due to areas of commonality between AS and autism, it seems meaningful to carry out educational and psychological interventions known to be effective in relation to autism (Trillingsgaard and Ostergaard, 2004). To date, only three studies have evaluated the effectiveness of the MTW program. McConachie et al. (2005) undertook an investigation in which 51 preschool children with ASD and their families participated. Although the results obtained did not determine advances in core deficits of ASD, they showed a significant increase in children’s vocabulary and improvement in parents responsive style, which seems to relate positively with language acquisition rate (Yoder and Warren, 1999). Likewise, Girolametto et al. (2007) studied the effects of the program on three children with ASD and their families. They employed microanalytic coding techniques to analyse the communicative interaction and found an increase in parents’ responsive communicative acts and in children’s lexical repertoire. Social interaction also improved significantly in relation with the amount of turn-taking episodes and the number of social initiations, besides other factors. Sixty-two children with risk indicators of ASD, average age of 20 months, took part in a randomized controlled trial (Carter et al., 2011). According to the data reported, the intervention had different effects on children’s communication depending upon their baseline. Progresses were more evident in children with typical autism and more limited play skills.

In MTW, parents are taught how to recognize, correctly interpret and respond appropriately to individual communicative acts, elements that are deemed to be essential in interventions with children with AS (Sigafoos et al., 2006). This underscores the construct of responsivity, referring to parental behavior that responds contingently to the child’s cues, follows the child’s lead and provides input and support that build on the child’s focus of attention and activity (Spiker et al., 2002). This is a basic tenet of MTW program.

On the other hand, while treating the aforementioned difficulties affecting joint attention and imitation, the MTW program emphasizes strong points such as eye contact, which children with AS find particularly reinforcing and highly resistant to satiation (Mount et al., 2011). Additionally, many of the strategies that are taught are also shared with other intervention programs that families of individuals with AS have been found to be highly useful (environmental sabotage or expectant delay) (Calculator, 2002). Finally, studies on communication in AS have mainly employed questionnaires completed by parents and caregivers, but have not used microanalytic coding techniques to assess parent–child interactions or standardized instruments of communication evaluation.

II Method

1 Case study participant

The participant was C, a female aged 4 years 4 months with a diagnosis of AS, confirmed through genetic testing for a deletion of 15q11-q13 chromosome region. She lived with her parents, both of whom held University degrees. Spanish was the primary language spoken at home and at school. Her weight at birth was of 2.3 kg. The pregnancy period was normal until the final phase. In the thirty-eight week of gestation, the mother suffered from pre-eclampsia, a stoppage of baby’s growth was detected, labor was induced and the baby was delivered by Caesarean section.

The parents noticed a developmental delay in motor and language areas, and at one year old C had still not produced any sound. Visual and auditory abilities were checked and found to be in the normal range. During her second year of life, C suffered from recurrent bouts of otitis, controlled with antibiotics.

Medical examinations were carried out including magnetic resonance and electroencephalography, and showed a generalized intercritical paroxysmal epileptiform activity. C was administered pharmacological treatment with valproic acid and vitamin supplements to stimulate the production of myelin in the central nervous system.

At one year of age, C began early intervention in hospital, and at 24 months she started to attend an Early Care Center. During this period she reached developmental milestones such as crawling, standing with support and walking holding a hand. She can now walk unaided but her movements are jerky.

C’s first seizure with convulsions occurred at 40 months of age. Concurrent with this crisis, she underwent genetic testing which produced a diagnosis of AS. She has since exhibited some moments of absence but has not suffered from seizures with convulsions. At four years old, C started school in an inclusive classroom with educational support (teacher assistance and speech language pathology).

2 Assessment

Assessments were carried out at three time points (see Table 1).

Table 1.

Instruments and procedures used at each assessment point.

Time 1: Six months prior to the start of Hanen program ‘More than words’ (MTW)	Time 2: Immediately before the start of MTW program (after six months without intervention)	Time 3: Three months after the intervention with MTW program
Anatomical and functional oral motor assessment	Development: Battelle Developmental Inventory
Communication: Communication Symbolic Behaviour Scales	Communication: Communication Symbolic Behaviour Scales	Communication: Communication Symbolic Behaviour Scales
	Pragmatics Profile	Pragmatics Profile
	Mother–child interaction	Mother–child interaction

a Anatomical and functional assessment

C exhibited a habitual open-mouth posture, protruding tongue, low muscle tone and abundant and frequent drooling. It was not possible to assess other aspects due to difficulties of collaboration. She chews food with her mouth open but has no eating or feeding problems.

b Developmental assessment

The Battelle Developmental Inventory (Newborg et al., 1996) is a standardized instrument that assesses the fundamental abilities involved in child development, from one month to eight years. Items are categorized in different areas: personal/social, adaptive, motor, communication and cognition. Assessment results are gathered through structured tasks, parent interviews and observation of the child in natural environments.

The outcomes of the initial assessment indicate generally delayed development, with an irregular profile of abilities across different areas (see Table 2). The adaptive area is the most advanced, while social abilities are more delayed. The results of the cognitive, communicative and motor areas oscillate between 7 and 9 months, in terms of equivalent age.

Table 2.

Results of the Battelle Developmental Inventory.

Areas	Raw score	Equivalent age
Personal-social	8	6 months
Adaptive	13	15 months
Motor	10	9 months
Communication	6	8 months
Cognitive	8	7 months
Global index of development	45	8 months

c The Communication and Symbolic Behavior Scales Developmental Profile (CSBS DP)

(Wetherby and Prizant, 2002), is a standardized instrument designed to evaluate social, communication and symbolic abilities of children whose functional communication age is between 6 months and 2 years. It is a face-to-face evaluation of the child interacting with a parent and a clinician during a series of communicative temptations designed to entice the child to communicate spontaneously. Materials used during the evaluation include action-based toys, books and play materials that evaluate how a child uses and plays with objects symbolically and constructively. It consists of the following sampling opportunities: (1) four communicative temptations, which are nonverbal situations designed to tempt the child to communicate; (2) sharing a book; (3) language comprehension probes of object names, person names, and body parts; and (4) a play sample with a stuffed animal and feeding toy set for symbolic play and blocks for constructive play. For each of the three CSBS DP measures, seven cluster raw scores (Emotion and Eye Gaze, Communication, Gestures, Sounds, Words, Understanding, and Objects Use), three composite raw scores (Social, Speech, and Symbolic), and a total raw score are derived. The Social Composite includes emotion and eye gaze, communication and gestures. Emotion and Eye Gaze includes a measure of the child’s use of gaze shifts for social referencing during interactions, a measure of sociability or sharing of positive affect states and a measure of gaze/point following. Communication includes the rate and function of communicating, that is, the number of communicative acts and the purposes they serve. Gestures encompass the variety of conventional gestures and the sophistication of hand gestures from contact to distal. The Speech Composite assesses sounds and words. Sounds consist of the vocal acts that are transcribable sounds and include a vowel sound. Syllables containing consonants and the inventory of different consonants are considered. Words measure the use of words and word combinations and the number of different words and different word combinations that the child uses in communicative acts during the communication sample. Finally, the Symbolic Composite measures the child’s ability to use deliberate behavior and to plan ahead through mental representation and the use of symbols. One subscale measures the understanding of words and the other subscale rates the use of objects.

In this case, the CSBS was used beyond the normative age range since the participant did not exceed a cognitive age equivalent of 24 months. The scale is not standardized in terms of the Spanish population; we therefore employed raw scores to give a measure of progress. The CSBS was administered six months prior to and immediately prior to the start of treatment, and, third, after the intervention. This allowed comparison of the rate of communication development during the six-month period in which the child had not received intervention and the growth rate before and after the intervention period.

d Pragmatics Profile

The mother was interviewed using The Pragmatics Profile of Everyday Communication in Children (Dewart and Summers, 1995), a qualitative assessment with functional questions answered by a caregiver across four main domains of pragmatics: communicative functions, response to communication, interaction and conversation, and contextual variation (see Table 3).

Table 3.

Initial assessment with the Pragmatics Profile.

(A) Communicative functions: range:	Communicative functions: Forms:
Attention directing	Touch, vocalization
Requesting
Requesting object	Pulls by the hand and waits; pulls by the hand, look at interlocutor, looks at referent; points with open hand
Requesting action	Raises arms to interlocutor; tries to climb up someone’s body
Requesting help	Gives an object and waits
Rejecting	Pushes away; shakes head
Greeting (on arrival; on departure)	Throws herself into the arms of the person
Self-expression and self-assertion
Expression of emotion: pleasure	Smiles, claps
Expression of emotion: upset	Cries
(B) Response to communication: Range:	Response to communication: Forms
Gaining child’s attention	As C does not always respond to her name, it is very often necessary to move close to her face, touch her and say her name
Interest in interaction	Looking interested and making eye contact; joining in the ‘conversation’ using sounds
Understanding of gesture	Looking toward the interlocutor is pointing
Acknowledgment of previous utterance	Smiling and looking; vocalizing
Understanding of speakers intentions	Gives an object and wait
a) Response to request for action	Looks at you but doesn’t seem to know what to do
b) Response to request for information	Points; gestures
Anticipation	Tickling; smiling; joining in and playing
Responding with amusement	Tickling; swinging in the air; funny rhymes
(C) Interaction and conversation:
Participating in interaction	Looking, touching, smiling and laughing
Initiating interaction	Touching; vocalizing; pointing; giving object
Maintaining an interaction	Body movement; vocalizing; catching eye; gestures
(D) Contextual variation:
Person	Preferences for certain individuals
Peer interaction	Plays alone, beside the other children; looks for children; gestures; touching

C communicated through touch and vocalization to direct attention. Her vocalizations consisted primarily of some expressions similar to ‘ma’ (with a schwa-like vowel) that were interpreted as mamá (‘mummy’) or más (‘more’) by her family. In order to request an object she pulled by the hand, pointed with palm and vocalized or gave an item to ask for help. She also rejected things by pushing them away or shaking her head. She was able to respond to her name, look where the interlocutor was pointing and initiate interactions through touching, pointing with palm and giving objects.

e Mother and child interaction

The mother–child dyad was videotaped in the university clinic during three activities, which included storybook reading (with a familiar book), a known social game and a daily routine (snack). The mother was encouraged to interact with her child as she would normally. The duration of videotaping was the same at pre-intervention and post-intervention (17 minutes). These elicitation procedures of spontaneous communication are proposed in MTW program and have also been employed in other investigations (Girolametto et al., 2007).

Quantity and quality of communicative acts displayed by the child in spontaneous interaction situations

Measures were obtained from the videotaped interactions between the mother and the child. They were transcribed and coded using the computer program Systematic Analysis of Language Transcripts (SALT; Miller et al., 2011). The number of communicative acts carried out by the child was computed using the Wetherby and Prizant (2002) criteria. For certain conduct to be considered communicative, it must fulfill the following conditions:

The act must be a gesture (giving an object, touching adult’s hand, arm, body or face, moving an object toward or away from adult, head shaking or nodding, throwing or dropping objects, raising arms, waving, making a depictive gesture, showing an object), a vocalization or a verbalization. As C can only point with palm we considered this as pointing, contrary to the requirements of Wetherby and Prizant who only consider pointing with finger as communicative act.

The act must be directed to the adult.

The act must be used with a communicative function (behavior regulation, social interaction, joint attention).

To assess the quality of the communicative acts, they were coded as either complex or simple communicative acts. The distinction was made on the basis of the criteria considered in other investigations that have evaluated the coordination of communicative means (Shumway and Wetherby, 2009):

In order to be considered complex communicative acts, three elements – vocalization, eye gaze and gesture – should be present.

When one of these elements was lacking, the act was classed as a simple communicative act.

Additionally, each communicative act was coded as an initiation or response, according to Wetherby et al. (1988) definitions:

Communicative acts self-initiated by the child: The child initiates a topic or communicates spontaneously without an adult speaking prior to the child’s act.

Communicative acts not initiated by the child: The child maintains the topic by responding to a previous statement, answering a question or imitating the previous communicative act.

Mother’s communicative style

In accordance with SALT directions, the language of the mother was transcribed and segmented into utterances (C-Units), defined as one main clause and any subordinate clauses attached to it (Loban, 1976). The parent interaction coding proposed by Girolametto et al. (2007) was followed in order to assess changes in mother communication style. This coding system divides utterances into ‘directive language input’ and ‘responsive language input’. The codes are mutually exclusive and each maternal utterance received only one code.

Directive language input consisted of four codes:

behavior control: mother calls the child’s name to get attention; uses a command to promote safety or reduce noncompliance (e.g. ‘look’, ‘wait, wait’, ‘don’t break this down’);

command: mother requests an action response (e.g. ‘sit down’, ‘let’s play with the house’, ‘shut the door’);

test question: mother asks a question to elicit a known answer (e.g. ‘where’s the duck?’, ‘where’s the milk?’);

promote communication: mother prompts child to communicate by using intonation cues or sentence completion (e.g. ‘one, two, …’). The mother does not direct the child what to say.

Responsive language input consisted of the following codes:

wh-question: mother asks an open-ended wh-question (e.g. ‘what do you want?);

yes–no question: mother asks a question requiring a yes–no response (e.g. ‘do you want to play?’);

choice question: mother asks a choice question (e.g. ‘do you want a cookie or a chip?’);

comment: mother uses comments that (a) acknowledge the child’s behavior (e.g. ‘very good’,); (b) imitates the child’s utterance (‘shaking head saying no’); (c) provides a label (e.g. ‘the house’, ‘the milk’); (d) expands the child’s utterance (e.g. ‘more cookies’, after the child utterance ‘ma’); or (e) makes a general comment or statement that is not a label, imitation, or expansion (e.g. ‘you’re taking two cookies’).

Uncodable utterances: adult’s utterance is incomplete (e.g. ‘it is …’) or the utterance is directed to the person who is videotaping the session.

3 Inter-rater reliability of mother–child interaction measures

Videotapes were transcribed verbatim using computer program SALT, and then rated independently by both of the study authors for child communication and for mother’s communication style. In all cases, inter-rater reliability was calculated by dividing the number of agreements by the number of agreements plus disagreements, and multiplying the resulting number by 100 to yield a percentage score (Sackett, 1978).

For the child’s communication acts, agreement was calculated for the boundaries of communicative acts (e.g. the onset and offset), and hence the number of acts, and their category: simplex/complex; self-initiated / not initiated.

In the interaction transcription before the intervention, inter-rater agreement was 92.6% for the number of communicative acts, 90.2% for the complexity of communicative acts, and 98.4% for self-initiated vs. not initiated communicative acts. Concerning the interaction transcription after intervention, inter-rater agreement was 94.6% for the number of communicative acts, 91.2% for the complexity of communicative acts, and 98.3% for self-initiated vs. not initiated communicative acts. Reliability for the total coding of the language sample obtained before intervention was 87.7%, and 93.3% after the intervention. A mean percentage of inter-rater agreement of 93.2% (range 87.7% to 98.3%) was obtained on the different variables used in the analysis, indicating a substantial level of agreement.

From transcripts of the mother’s communicative style, agreement was calculated for responsive and directive language. Before intervention, reliability for the individual responsive codes used was: 100% for wh-questions, 97.3% for yes–no questions, 100% for choice questions and 87.4%, for comments. For the individual directive codes, reliability was: 90.4% for behavior control, 94.2% for commands, 100% for test questions and 91.7% for promote communication. For the transcription made after intervention, reliability for individual responsive codes was 100% for the wh-questions, 100% for the yes–no questions, 100% for the choice questions and 90% for comments. For the individual directive codes, reliability was 95.1% for behavior control, 96.5% for commands, 100% for test questions and 98.3% for promote communication. The mean percentage of inter-rater agreement of 96.3% (range 87.4% to 100%) was on the different variables analysed, confirming a considerable degree of agreement.

4 Intervention program

The main goal of MTW program is to empower parents to build on everyday situations to foster communication development. During the program, parents identify their child’s stage of communication development, their learning style and their sensory preferences. They become skilled at applying responsive interaction strategies related to the communication goals that have been agreed previously in a collaborative way between the speech language pathologist and the family.

First, parents are taught how to attract the child’s attention, being face to face, and arranging the environment in such a way as to provide the child with a reason to communicate. Parents also learn to wait for a communicative attempt that promotes communicative initiative. They use strategies such as following the child’s lead, including their interests, interpreting their communicative signals, imitating their actions or vocalizations and intervening (‘intruding’) when the child is unengaged or involved in a repetitive behavior. To promote larger interactions parents learn to use structured and predictable routines such as contexts for increasing the length of their child’s engagement. They use simplified linguistic input, adjusted to the child’s level, and they employ visual cues in order to improve comprehension.

In this study, the MTW program was carried out for 11 weeks. The mother attended eight group sessions lasting two and a half hours, with four other families of children with a diagnosis of Autism Spectrum Disorder. The sessions were held at the University Clinic and consisted of a combination of interactive presentations, group discussions, videotape analysis and opportunities to practice. They were run by a speech language therapist, the first author, who had received specific training and certification by the Hanen Center. A detailed description of the MTW program can be found in the guidebook used by the parents (Sussman, 1999) and in the leaders’ guide, and an outline of the eight sessions is given in Appendix 1.

In addition to group sessions, three individual videotaping sessions of communicative interaction were conducted in order to monitor parents’ progress and child’s results. Within these situations, parents practiced program strategies with coaching and feedback from the speech language pathologist. The child’s goals and any concerns that may have arisen were discussed.

Treatment fidelity was recorded with the use of attendance data and by applying the strategies to the three individual videotaping sessions. They were also applied to different questionnaires used to monitor both the usefulness of these strategies and any issues in terms of their implementation.

An important aspect to consider is that children with AS are multimodal communicators (Calculator, 2013), and therefore a combination of signs, gestures, vocalizations and pictograms were chosen as an alternative/augmentative communication system. Determining child preferences was very important in order to apply the strategies taught in the program and to implement the multimodal system of communication. To this end, we used the Modified Choice Assessment Scale (Didden et al., 2006), a 92-item reinforcement scale designed to address the usual preferences of AS individuals and their commonly reported attraction to water, which includes 5 subscales (Edibles, Tangibles, Activities, Sensory, Escape, Avoidance and items related to water). Each item is rated on a 3-point scale. The child’s preferences were food and drinks (fruit, cookies and juice), activities (swinging, sliding), social games such as hide and seek, toys (cars, balls, balloons), music and unusual items like clothes pegs and toys to throw and stick.

III Results

The child’s performance was measured using the CSBS DP across two time periods of the same duration (Time 1: 6 months prior to treatment; Time 2: immediately before the start of MTW program; Time 3: three months after the end of MTW). Data from the Pragmatics Profile and the mother–child interaction were gathered at Time 2 and Time 3.

1 Communication and Symbolic Behavior Scales Developmental Profile (CSBS DP)

The CSBS DP scores demonstrated important advances in child’s communicative development. The results are shown in Table 4. The Social Composite showed a doubling of the total scores regarding the different aspects assessed. Unexpectedly, a decrease was noted in Emotion and gaze (–4) in the period between T1 and T2. This tendency was reversed after intervention (from 6 to 12 points). In Communication, an increase of only 3 points was detected during the pre-intervention period. This trend contrasts with a gain of 10 points post-intervention. In relation to Gestures, there is a 1-point gain between T1 and T2 and, again, an increased rate of change of 5 points post-intervention.

Table 4.

Scores on the Communication and Symbolic Behavior Scales: 6 months before the intervention started, one month pre-intervention and three months post-intervention.

		Time 1: 6 months before intervention Weighted raw score	Time 2: Pre-intervention Weighted raw score	Time 3: Post-intervention Weighted raw score
Cluster scores:
Social	Emotion and eye gaze	10	6	12
	Communication	8	11	21
	Gestures	1	2	7
Speech	Sounds	0	6	6
	Words	0	0	3
Symbolic	Language comprehension	0	0	3
	Object use	0	0	0
Composite scores:
Social		19	19	40
Speech		0	6	8
Symbolic		0	0	3
Total:		19	25	51

The total increase in Speech composite between T2 and T3 was 3 points. Three words emerged during the period of intervention: ame (dame ‘give me’), mamá (‘mummy’) and ma (más ‘more’). No new words had been produced during the six months prior to the start of the intervention. However, it is important to note the increase of 6 points in the number of sounds produced in the run-up to the intervention initiation, and there was no change in number of sounds between T2 and T3. However, during the intervention period C was able to combine those sounds and syllables to form three word approximations, an ability that had not been developed during the pre-intervention period.

An improvement of 3 points was observed in the Symbolic Composite in language comprehension between T2 and T3, whilst no change had been noted in the pre-intervention period. No changes were observed in relation with object use, action schemes and play at any time point.

2 Qualitative analysis of progress according to Pragmatics Profile

Regarding new forms of communication, parents reported that C began to use the word mamá (‘mummy’) to direct attention. She also started to use vocalizations to ask for help and learnt to wave in imitation. C was reported to have developed more ways to express emotions by asking for a hug. Language comprehension was strongly impaired at the onset of intervention. Although it continued to be greatly affected, some signs of responses to requests for actions and for information have been observed. She was able to fill in missing sounds or movements in social games. In addition, she frequently initiated interaction with visual contact and with the word mama. Table 5 shows the advances observed in some of the communication functions and forms of the Pragmatics Profile as compared to pre-intervention (T2).

Table 5.

Changes post-intervention with the Pragmatics Profile.

(E) Communicative functions: Range:	Communicative functions: Forms:
Attention directing	Besides touch, vocalization, the girl started to use mamá.
Requesting
Requesting Help	When giving objects and waiting, the girl vocalized.
Greeting (on arrival; on departure)	To say goodbye, she learnt to wave in imitation.
(F) Response to communication: Range:	Response to communication: Forms
Understanding of speakers intentions
Response to request for action	Apart from giving objects and waiting, the girl began to vocalize or initiate a movement in social games.

3 Mother–child interaction

a Quantity and quality of communicative acts displayed by the child

Twelve communication acts were coded in the pre-intervention assessment (T2), 10 of which were simple (83.34%) and two were complex (16.66%). Seven communicative acts (58.33%) were initiated by C while five (41.67%) were in response to an adult communication act. As it can be seen in Table 6, there was an increase in all of the variables considered. Post-intervention 27 communicative acts were identified (more than twice the pre-intervention total), 19 of which were initiated by C (70.37%) and 8 (29.63%) in response to the mother interventions. Regarding complexity, 16 were simple acts (59.25%) and 11 were complex (40.75%). Consequently, noticeable progress was observed in the frequency and the quality of child communication, as well as in terms of communication initiative.

Table 6.

Number and types of child communicative acts pre and post-intervention.

	T2 pre-intervention	T3 post-intervention
Total number of communicative acts	12	27
Simple	10 (83.34%)	16 (59.25%)
Complex	2 (16.66%)	11 (40.75%)
Self-initiated	7 (58.33%)	19 (70.37%)
In response	5 (41.67%)	8 (29.63%)

b Mother’s communication style

In the pre-intervention assessment (T2) 352 utterances were identified; 215 of them (61.07%) were responsive, while 128 (36.36%) were directive (see Table 7). The mother’s communicative style was mainly responsive before the intervention. This responsive style was accentuated after the intervention with MTW. First, a decrease of the number of utterances was noticed (352 utterances at T2 versus 255 utterances at T3, i.e. 97 utterances less). These outcomes are consistent with some of the strategies taught in relation to adjusting the input at children’s level (the 4S strategy, ‘Say less’ and ‘Stress’, ‘go Slow’ and ‘Show’). The number of responsive utterances fell by 20%, while directive utterances fell by 37.5%. Furthermore, an increase of 5.98% in responsive language was seen (from 61.07% to 67.05%), particularly in use of wh- and yes–no questions, considered by the program to be the kind of questions that keep an interaction going and, consequently, lead to longer communication exchanges. Meanwhile, the rate of directiveness decreased (from 36.36% at T2 to 31.35% at T3 ), particularly in the number of commands used.

Table 7.

Mother’s language: Responsive and directive input pre-intervention and post-intervention.

	T2 pre-intervention	T3 post-intervention
Total utterances:	352	255
Responsives:
Comments	199 (56.53%)	130 (50.98%)
Wh-questions	5 (1.42%)	13 (5.09%)
Yes–no questions	11 (3.12%)	28 (10.98%)
Choice questions	0 (0%)	0 (0%)
Total responsive:	215 (61.07%)	171 (67.05%)
Directives:
Behavior control	28 (7.95%)	18 (7.05%)
Commands	65 (18.46%)	43 (16.86%)
Test questions	10 (2.84%)	7 (2.74%)
Promote communication	25 (7.10%)	12 (4.7%)
Total directives:	128 (36.36%)	80 (31.35%)
Uncodable utterances:	9 (2.5%)	4 (1.56%)

IV Discussion

The main objective of this case study was to describe the profile of communicative abilities of a child with AS, before and after the implementation of Hanen program ‘More than words’ (MTW; Sussman, 1999). Specifically, we aimed to analyse the advances after intervention in the two following areas: child communicative development and mother’s communication style.

As regards child communicative development, improvement was observed across the different measures. There was a substantial increase in the number and complexity of the communicative acts displayed in spontaneous interactions. The child succeeded in communicating more frequently and effectively and through the combined application of different means. She also showed more communicative initiative. These results are consistent with those obtained through the standardized assessment, the Communication Symbolic Behaviour Scales. It was possible to compare C’s rate of communicative development between time periods of the same duration, one of them being without intervention with MTW. The outcomes clearly indicate a different trend in each phase as there were no changes in social and symbolic scores during the six months prior to intervention, although an advance in speech was noted. However, after the intervention, scores in Social, Speech and Symbolic domains were enhanced.

The mother’s communication style also exhibited some changes post-intervention. Even though her way of communicating was characterized by being responsive from the beginning, this feature was emphasized after the intervention. However, one aspect to consider is that in previous works dealing with this topic, there are not defined criteria about the increase in responsivity that is required to achieve significant changes in communication. Although responsivity is a dynamic construct of central importance to the development of children with intellectual disabilities, directiveness may be necessary when attempting to reengage an uninvolved child or elicit higher levels of participation (Girolametto, 1995). In fact, a more detailed analysis of the mother’s responsive utterances reveal that yes–no and wh-questions increased. Although these interaction forms may lead to extended exchanges, they seem to be more intrusive in comparison with comments, that showed a decrease. We think that the child characteristics, a very short attention span and motor restlessness, may lead the mother to use this kind of utterances to engage her in communication. The aim is to strike a balance between responsivity and directiveness so as to make the exact provisions depending upon the child behavior. Further research is needed in order to settle the optimal amount of responsivity that must be heightened in each particular case.

Finally, the mother was asked to comment on her satisfaction with the intervention methods and with the outcomes observed in her child’s communication development. She expressed highly positive opinions about the benefits of the program. She incorporated the newly-mastered skills into everyday contexts. For example, she placed reminder cues of the strategies and sample activities in every room of her house. This also enabled other people with regular contact with the child to follow them.

Despite these encouraging findings, the methodological limitations of the current study do not allow us to conclude that there is a causal relationship between child communication development and mother’s interactive style, nor does one exist between the intervention and the outcomes. Furthermore, this case study reports mostly qualitative and quantitative data not compared through statistical methods. Other strategies taught in the program have not been analysed, such as observing and waiting, using visual cues (pointing, showing, gestures) or arranging the environment, and these may be affecting the child communication.

In addition, there are several shortcomings that threaten the internal validity of this work, including lack of control for participant history and maturation, or factors such as support received at school, that may have influenced the results. Additionally the professionals that carried out the assessment were not blind to the purpose of the study. However, it is important to note that the different pace of progress observed in two time periods of the same duration, with and without intervention with MTW, could indicate the program may be worth investigating more systematically than it could be done here.

Additionally, in this particular case, we cannot overlook a range of factors that could lead to this good response: adherence to intervention, parents’ involvement, sociocultural level and the mother’s ability to be a sensitive observer of the strategies presented. That is to say, the mother was in what in the program is called ‘the awareness stage’ in the learning process. In this stage the learner is aware of her behavior and can apply her declarative knowledge to practice activities and this facilitated the speed of learning. The mother integrated the strategies as part of her communicative style, using them fluently and effortlessly, without continuous conscious application. In her own words, ‘Little changes in my communicative style have made a big difference in the communication with my child.’ The perception of the usefulness of the learned strategies is reinforcing and may lead the mother to keep on applying them as she sees the outcomes on her child in a relatively short period of time. Other works have stated the importance of qualitative aspects such as parent beliefs and experiences, as well as their existing pre-intervention interaction strategies (Baxendale and Hesketh, 2003; Glogowska and Campbell, 2000).

Although in recent years, various investigations have provided valuable information about the communicative abilities of children with AS, to our knowledge, this is one of the first case studies to closely examine their communication profile using coding techniques to evaluate parent–child interaction. These findings add to the body of knowledge about the communication profile of children with AS and their response to a family-centered intervention. Future studies can further research the feasibility of the socio-interactive approach of MTW within the AS population and their effects not only in the short-term but also in the long-term. That is a commitment that we have with these children and with their families.

Footnotes

Appendix

Appendix 1.

Description of the intervention program.

Week 1	Get to know more about your child’s communication.
	Strategy: Give your child a reason to communicate and then wait.
	Parents learn how to arrange their child’s environment in order to promote the child to attend and interact with them. They also analyse their child’s sensory preferences.
Week 2	Follow your child’s lead using the four ‘I’ way.
	Strategy: The four I’s: Include your child’s interests, Interpret, Imitate and Intrude.
	Parents learn to follow their child’s lead in terms of activities or interests with the purpose of establishing reciprocal social interaction on a shared topic. Parents learn to share the child’s interests by touching, pointing to, or commenting on the child’s focus. They imitate their child’ actions and sounds and interpret nonverbal communication by giving the child a language model (label or short phrase), adjusted to their level.
Week 3	Individual visit with video feedback.
Week 4	Make the connection with people games and music.
	Strategy: ROCK: Repeat what you say and do, Offer opportunities for the child to take a turn, Cue your child to take his turn, Keep it fun and keep it going.
	Parents learn how to create ‘people games’ based on their child’s sensory preferences. They are encouraged to set appropriate goals based on their child’s stage of communication. These goals are integrated in predictable routines and parents provide the necessary cues to help their child to take a turn.
Week 5	Help your child understand what you say: ROCK in your routines.
	Strategy: The 4 ‘S’s: Say Less, Stress, Slow, Show.
	Parents are instructed on how to adjust their input using short sentences, stressing the key words, speaking slowly and using contextual cues (gestures, objects).
Week 6	Use visual helpers.
	Strategy: How to use visual helpers to help children understand.
	Parents learn to improve their child’s comprehension through the use of visual helpers (photos, images), that support their understanding of what happens in different situations or show them how to perform a sequence of actions.
Week 7	Individual visit with video-feedback.
Week 8	Bring on the books.
	Strategy: ROCK and read (combining strategies).
	Parents learn to integrate all of the previously learned program strategies and apply them to reading books.
Week 9	Take out the toys.Strategy: ROCK and play (combining strategies).
	Parents learn to integrate all of the previously learned program strategies and apply them to playing with toys.
Week 10	Individual visit with video-feedback.
Week 11	The topic planned in the program deals with how to make friends, but in cases where the group is mainly composed of toddlers in the requester communication stage, it may be more pertinent to invite a guest speaker to talk about a selected theme. Parents were interested in learning how to manage behavior problems, so this was the issue covered in this session.

Declaration of Conflicting Interests

The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding

The author(s) received no financial support for the research, authorship, and/or publication of this article.

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