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Reducing challenging behaviors and fostering efficient learning of children with CHARGE syndrome

2005; Wiley; Volume: 133A; Issue: 3 Linguagem: Inglês

10.1002/ajmg.a.30548

1552-4833

J.P.M. van Dijk, Arno de Kort,

Tracheal and airway disorders

The literature on CHARGE syndrome has begun to address the behavioral problems. Hartshorne and Cypher [2004] in a study of 100 persons with CHARGE, found a relationship between deafblindness and behavioral problems. Positive correlations were also found between age and the number of behavioral problems and between the number of medical impairments and autistic behavior traits. Another survey study (N = 71) [Souriau, this issue] found lack of impulse control, difficulties with social relationships and communication in this population. Two Dutch studies support previous results. Van den Boom [2003] used stringent criteria to select subjects with a confirmed diagnosis of definite CHARGE syndrome (N = 27), finding significantly more behavior problems in the CHARGE population when compared to the norm group of the Child Behavior Checklist (CBCL). Fifty-four percent of the children in this sample demonstrated self-abusive behavior and 74% needed extra time to process information. Important is the finding that neurological disorders had a relationship with problem behavior. Van Tartwijk [2004] studied 121 persons enrolled in a center for persons with multiple sensory impairments, finding that one third of the persons with deafblindness could be rated (CBCL) as having "severe" or "very severe" behavior problems (N = 65), and this was very prominent in persons with CHARGE. Over a period of 2 years, the authors subscribed to the CHARGE Listserv on the internet (CHARGE@yahoogroups.com), where parents exchange thoughts about their children and raise questions concerning medical and behavioral problems. More than 4,000 messages were read and categorized. The most prominent topics of conversation had to do with medical issues, especially feeding problems, motor problems (learning to walk), visual problems (detachment of retina, impact of colobomas on behavior), and cochlear implants. The second most common cluster of topics had to do with behavior. Self-injurious behavior and aggression towards other persons and materials were discussed frequently, as were the lack of adaptive behavior skills, withdrawal, and the question of a possible relationship between autism and CHARGE. These survey studies are of great value for beginning to understand CHARGE syndrome and its possible repercussion for the person's development; however, survey studies are inadequate in helping understand the problems of a particular individual. We begin by considering the role of sensory systems and communication. There is ample evidence that "challenging behaviors" are common in persons with visual impairment [Van Dijk, 1982, 1991; Mar and Cohen, 1998]. However, the assessment of these behaviors must take into account the cause of the visual impairment. For example, there is a strong relationship between congenital cataracts and stereotyped behavior such as light gazing and pushing fingers in the eyes [Van Dijk, 1982, 1991]. Such unproductive behaviors will often diminish without direct intervention with the child simply by reducing strong lighting. Some children with CHARGE refuse to go outside in bright sunshine. The reason for this is likely that the pupils of some children with CHARGE (with iris coloboma) do not contract in bright light conditions [Hefner and Davenport, 2001]. This problem can often be ameliorated by the use of tinted glasses. Some parents have commented on the listserv that the visual impairment due to the presence of retinal colobomas seems to affect their child's behavior. Similar clinical examples can be given about the effects of hearing loss on behavior. Hearing impairment is the most common feature of CHARGE, with the loss ranging from mild to profound. Some children will cover their ears and even scream when they are exposed to a certain noise. This behavior might be well explained by the phenomenon of "loudness recruitment." The person with this problem has an inner-ear hearing loss, which exhibits an abnormally rapid rise in the sensation of loudness with only a small increase in sound stimulus intensity. Vestibular difficulties due to the absence or abnormality of the semi-circular canals in the inner ear are increasingly recognized as a significant problem for children with CHARGE [Admiraal and Huygen, 1997]. A child with this impairment will experience motor delays and problems orienting to the environment [Brown, this issue]. There is increasing agreement concerning the vital role of problems with expressive communication in behavior difficulties in CHARGE [Van den Boom, 2003; Hartshorne and Cypher, 2004; Souriau, this issue; Thelin and Fussner, this issue]. It can be assumed that when important persons in the child's environment respond to the child's initiatives, he or she feels understood, which, in turn, has positive consequences for his or her behavior. Recent published research studies have shown convincingly that lack of adult reaction can be a serious impediment to the interaction and communication of children with multiple sensory impairment who are in the early stages of communicative development. Daelman [2003] analyzed communicative behavior of four subjects in the pre-symbolic level of communication. She defines this behavior as: "behavior, which serves as a vehicle for sending a message to an adult." One of the subjects was a deafblind child with CHARGE syndrome. Almost 1,000 communicative behaviors were transcribed and categorized. One of the important findings was that in 50% of the child's intent to "send a message," either the adult did not pick up the message, or did not communicate to the child that his message was received. In her study on "effective programming of behavior problems" in children with multiple sensory impairment, Janssen et al. [2003] found similar results. Her research discloses clearly that to a large extent the "behavior problems" in this population are actually idiosyncratic behavior of the subjects. Very often, the expression of needs and wants of the child are so vague and hard to discern that the adult misses them entirely. As a consequence of this, the child withdraws or initiates (self) aggressive outbursts [Janssen et al., 2003]. One important outcome of this study was to recognize that teachers and care workers can be trained in a rather limited number of sessions (10× 1½ hr) to receive and to understand idiosyncratic patterns of communication and learn how to respond to it. Changes in adult (teacher, caregiver, etc.) behavior can lead to a significant decrease in the "problem" behaviors of the children. We have observed that teachers and parents typically initiate many more communicative interactions with a child than they respond to. This can be a particular problem in educational settings for persons with multiple sensory impairments. It is often the hearing and sighted person who takes the initiative in the communication process and controls the total process. This is not only detrimental for the development of language [Chen and Haney, 1995], but also for the enhancement of the child's self-esteem. Challenging behavior can be described as any repeated pattern of behavior, or perception of behavior, that interferes with, or is at risk of interfering with, optimal learning or engagement in social interactions with peers and adults. Examples of such behaviors include prolonged tantrums, physical aggression, disruptive vocal and motor responding (e.g., screaming, stereotypy), property destruction, self-injury, noncompliance, and withdrawal. Before establishing a protocol for management, it is important to get a clear picture of these behaviors: how they developed, when they occur, how often, and in what circumstances. Video analysis is a very useful tool for data collection that allows the target behavior to be observed and recorded as it occurs [Janssen et al., 2002; Daelman, 2003]. The context in which the behavior occurs can be recorded, making it possible to reach agreement between different observers. Viewing the video in slow motion makes it possible to assess and quantify (challenging) behaviors that otherwise may have escaped observation. Although time consuming, this process produces extremely valuable information [Nind and Hewitt, 1994]. We have used video extensively in analyzing the challenging behaviors of persons with CHARGE [Van Dijk and De Kort, 2004]. Problem behaviors are quite often related to a preceding event. In one instance, the self-abusive behavior of a child with CHARGE was noted to start a short time after the care worker announced that lunch was ready. In another case, a clear relationship could be observed between playing a ball game and self-aggression [Van Dijk and De Kort, 2004]. In these cases, it is instructive to observe the reaction of the care worker to the behavior. Did he or she postpone lunch or was the ball game stopped? In the theory of behavior-analysis this approach is called ABC analysis: events are observed which precede (Antecedent) the episode of problem Behavior (B) and what follows the behavior (Consequence) [Alberto and Troutman, 2003]. The Motivation Assessment Scale (MAS) [Durand and Crimmins, 1992] is a useful questionnaire for determining the function of challenging behaviors. This 16-item scale relates the behavior to four potential reinforcing factors: attention, tangible (wanting to get something) avoidance, and sensory (sensory stimulation seeking). The information from this scale is a good starting point for intervention. medical history; parent/teacher interview. Analysis of logs from service providers; observation of target behavior during prolonged periods of time; Motivation Assessment Scale; ABC/video analysis; formulation of hypothesis of intervention. Reaching consensus; period of intervention; follow-up. To illustrate, we present the case of an 11-year-old boy, Roberto (not the boy's real name) with CHARGE syndrome. Colobomas in both eyes, including the macula. His vision is severely impaired in the left and mildly impaired in the right eye. Unaided hearing loss is 60 dB on both sides. There are significant feeding problems for which an intensive program has been designed. He attacks people by grabbing their clothes or hair; he often refuses to go from one location to another, he then throws himself on the floor and hits his face with his fists; and he also hits himself on the nose. The mother and grandmother have both kept logs of Roberto's behavior on weekends and holidays. Teaching and care staff have completed daily reports of Roberto's activities, his mood and behavior. The MAS was completed by both care staff and teaching staff. In the analysis of the notes in the logs, it has become clear that there are two main causes for Roberto's challenging behavior. One is probably epileptic type of activity in the brain or migraine. Behaviors such as grabbing persons are followed by crying and requests for consolation. The "tangible" score on the MAS revealed the second reason for Roberto's "attacking" behavior: it occurs when Roberto wants to obtain an object he may not have or when he is requested to stop playing with the object. One hundred eighty minutes of videotape was digitalized for analysis. The results of the analysis were unambiguous. When Roberto was asked to go to another room, he would not make any attempt to leave the location. When the request was repeated with urgency, the boy would throw himself on the floor and turn his back to his service provider. When this person tried to put him on his feet, Roberto would refuse and try to pull her hair or clothing. However, when the service provider would stand beside him and with outstretched arms invite Roberto to stand up, he would watch her by peeping through his fingers. After a few minutes, he would pull his hand away and look her straight in the face. When he was again invited by the caregiver quietly to move on, Roberto would take her hand and stand up and continue his way without any fuss. Identical observations have been recorded several times. In one instance, it took Roberto 15 min to start with a new assignment. Before he was ready to take up a new task, he would look at a picture postcard for 5 min, vibrate with a plastic bottle against his teeth for the same length of time, and then finally look at the object of reference for the assignment for a few minutes and then stand up and leave the room to go to the workshop. It appears that making changes or transitions (e.g., leaving a situation or passing over an object to some one else or responding to a request) is an enormous effort for Roberto. It gives him feelings of discomfort, perhaps because his brain is not organized to respond quickly to new demands from the environment. Roberto wants almost complete control over his world and the people around him in order to prevent unexpected changes. He almost literally "chases them out." This avoidance behavior is his way of keeping himself from succumbing to stress. It is widely reported that children with multiple sensory impairment often feel at the mercy of their environment [Van Dijk, 1999]. Due to their idiosyncratic behavior, the signals they use to communicate their distress are often overlooked or misinterpreted. For many of these individuals, the "only way out" is to exhibit behaviors such as those used by Roberto. Transition time: Prevent stressful situations by giving Roberto time to prepare himself for another activity. Use reference books and calendars to announce the next activity (communication). Social reinforcement: The educators will lift their thumb and say "Good boy" when Roberto manages to transfer from one situation to another without problems. Tangible reinforcements: Give Roberto a sticker when he has completed an assignment. Introduce mutual games with care-giver/teacher and peers. Provide massage for exercise. Prescribe Depakine to help Roberto control his outbursts. When Roberto starts hitting his face or nose, the staff will hold a small pillow in front of his face. It was decided to utilize the above procedures for 3 months. Video recording, observations, and behavior logs were continued during this time. The case study of Roberto illustrates a case of a boy under great stress. He is unable to cope with the demands of his environment. Normally hearing and sighted persons are often unaware what it means for a child with impaired senses to experience life in a scattered world of moving persons and objects, with unidentifiable sights and sounds. Adult persons with multiple sensory impairment report that they experience the world around them as chaotic and lacking structure. These people complain about the stress of having limited control over their own activities and decisions. A number of studies have been published about the negative impact of stress on human development [Boyce et al., 1992; Burgess et al., 1995; Rothenberger and Huther, 1997; Black, 1998; Nelson and Carver, 1998; Perry and Pollard, 1998]. These studies reveal a relationship between brain formation and the ability of the child to cope with stress. Imposing activities on children that they are unable to escape undermines the development of the neurological system and even destroys existing neuronal pathways. Brain systems regulate the capacity to cope with the changing social environment [Kraemer, 1992]. These systems, which are the same as the ones that mediate attachment, are organized hierarchically. It is a challenge to discuss this neuro-biological organization relative to (multi-)sensory impairments, yet it demonstrates how important assessment is in determining the most favorable conditions for enabling a child to build such systems. First of all, the child must give attention to the (social) stimuli and determine their significance. For example: a child must be able to perceive an approaching person and then be able to remember that this is a person who provides comfort. The process of determining whether or not a stimulus is significant is called "gating." The turning of the head in the direction of that person, tracking the movements visually, and hearing the footsteps becoming louder and louder increases cortical synaptic density. Clearly children with CHARGE syndrome have a disadvantage in such situations. The information is simply not available to them. A second mechanism fundamental to the regulation of a child's relationship with the world is his or her ability to anticipate the effect of a stimulus; whether it is rewarding, punishing, or neutral. If a child responds and there is a positive consequence, it is likely that both the stimulus and the consequence will be connected and stored in the brain. This connection occurs nearly instantaneously. In children who see and hear normally, the connections happen continuously, often without any intervention on the part of the educator. This incidental learning is hampered in children with sensory deficits, especially when in combination with mental or physical disabilities. A third mechanism to help cope with the demands of the physical and social world is the child's pattern of arousal and relaxation, including sleep. In periods of high vigilance, gating and conditional learning are built; during sleep, these behaviors are integrated at the gating level so that in the next occurrence of a situation, the problem will be treated as routine. This rhythm seems to be essential in providing the human being with sufficient adaptive capacity. In people who succumb to stress, this mechanism is malfunctioning. One can only speculate as to whether the poor sleeping patterns which are often reported to exist in children with CHARGE are related to the failure of this arousal-sleep mechanism. From a neuro-biological point of view, it appears that the daily life of an individual with sensory impairment(s) or with multiple disabilities should be carefully organized in such a way that periods of intensive learning are followed by periods of relaxation in which routines have a prominent place and then by periods of sleep. A fourth mechanism is organizational plasticity. In order to adapt to the changing demands and challenges from the environment, coping mechanisms should be fine-tuned and readily available. One can observe that severe problems at this level occur in virtually all people with congenital sensory impairment or with multiple disabilities. When the social environment demands quick adaptation, it appears that the neuro-biological systems are inadequate. Clear stress phenomena can be observed that may lead to refusal, self-abuse, and inappropriate (social) behavior. Often this behavior can be explained because the previous hierarchical stages, as discussed above, did not receive a distinct place in the educational approach. However, sometimes the demands placed upon the person by the "hearing and seeing" world are simply too overwhelming. In the environment of a person with multi-sensory impairment people come, go, touch, smell, talk, shout, request, demand, and push and pull without warning and all in a matter of seconds. It is not uncommon for a deafblind person to be approached inappropriately by individuals who are unable to communicate with them or read their body language [Daelman, 2003]. In summary, if the challenging behaviors exhibited by individuals with CHARGE syndrome are to be effectively reduced, it is important that intervention take into account and address the underlying causes and the consequences of the behaviors. The following are possible therapeutic interventions based on the above discussion. A child who is (multiple) sensory impaired has difficulty registering the initiatives or the responses of the caregiver. As a result, only weak (if any) connections will be established in the child's brain. In the case of a proficient perceptual system, solid connections will be established. The young child needs only a minor clue to remember the previously learned event. This leaves him/her with a great deal of energy remaining for learning new contingencies. A comprehensive network of events is established by which the child is able to judge stimuli according to the pleasure they provide or the distress the cause (appetite-aversion). When the child has learned that both mother and grandmother are providing pleasurable experiences, he/she will have no problems transferring from mom to grandma. If this early learning has been impeded, every change from one person to another will take additional time. Initially, the child may fuss and refuse to sit on the lap of the other person. In comparison with the child without sensory impairment, a child with combined vision and hearing loss will require a much longer time to adapt to a new situation. If this is not taken into consideration, behavior problems are likely to arise. Intervention strategies should include the allowance of extra time for the child to process information and adapt to new situations. In the case study of Roberto, we described how, when under stress, Roberto would exhibit all types of challenging behaviors. Situations, which may cause undue stress should be prevented to the greatest extent possible. In Roberto's case, this meant giving him time to adjust himself. This can be enhanced by using a calendar system to graphically show when an upcoming event will occur and to provide several verbal reminders of what is to come: "Roberto, we will be going soon," "Roberto, are you ready to go?" This requires a great deal of adjustment and patience on the part of the caregivers. No hurry, no unexpected events, no force or commands, but simply offering help at the moment he is ready to move [Van Dijk and De Kort, 2004]. When there is a recurrence of events, the brain starts to make the connection that after situation A it is likely that situation B will occur. This is called anticipation. Anticipation is a very important process in stimulating the working of the brain, in particular the establishment of neural pathways. Once a routine has been established, it takes far less energy to switch from situation A to B. In the case of Roberto, when he comes home from school, he goes to his personal room where he plays with a favorite object (plastic bottle) and then he goes to the dinner table. Every day, the same routine is repeated. This enhances smooth transitions and helps prevents conflict. During recent decades, it has become possible to assess hearing and visual impairment in very young children. There is strong evidence that diagnosis and intervention before 6 months of age significantly improves outcomes of communication, behavior and learning. This applies to children with multiple sensory impairments as well. The essence of this intervention is to enhance the development of a healthy attachment bond between the child and the principal caregiver. Strategies have been developed that can foster such attachment [Janssen et al., 2002; Riksen-Walraven, 2002]. It goes without saying that the senses of hearing and sight play an important role in this relationship. In case of impairment, the fitting of low vision aids, hearing aids and cochlear implants at an early age can be very important. In conclusion, behavior in children with CHARGE syndrome presents major challenges to caregivers and families. Careful, detailed observation and assessment can elucidate many causes of these behaviors. Carefully coordinated, consistent management plans to control the environment and alter reactions to behaviors can be a major step towards reducing the frequency and intensity of these disruptive behaviors. The result can create an environment more conducive to social interaction and learning for these children.