REPETITIVE BEHAVIORS IN CHARGE SYNDROME: DIFFERENTIAL DIAGNOSIS AND TREATMENT OPTIONS

Transcription

1 REPETITIVE BEHAVIORS IN CHARGE SYNDROME: DIFFERENTIAL DIAGNOSIS AND TREATMENT OPTIONS By Veronika Bernstein, PhD Laurie S. Denno, MA, LMHC August, 2003

2 Introduction Over the past 15 years in our roles as neuropsychologist and behavior specialist at the Perkins School for the Blind, Deafblind Program, we have worked with, evaluated and consulted on numerous students with deafblindness. All students are unique. Each has their own set of strengths and abilities. Many students with whom we have worked also display behavior challenges. As a group, the many students we have known with CHARGE Syndrome often display a specific set of challenging behavioral characteristics. The range of behavioral challenges is the same as in all educational settings. However, the CHARGE students appear to display a much higher frequency of repetitive behaviors when compared to other students of similar age, sensory impairment and functional ability. The repetitive behaviors often do not always appear to serve a function and it can be extremely difficult to redirect the CHARGE students from repetitive behaviors. We have documented this finding in our previous work (Denno and Bernstein, 1997). This difference is not just a communication issue. Many of the students with whom we work have good communication abilities. This difference is not just a sensory issue (Moss, 1999, Murdoch, 2000). Many of the students with whom we work have functional hearing and/or functional vision. We believe that, for many individuals with CHARGE, repetitive behaviors are a characteristic of their diagnosis and not a function of their sensory or communication impairments. But in either event, when these students are in school or participating in family activities, their repetitive behaviors interfere with their ability to fully access the environment. Therefore, treatment becomes critical. We presently have 14 students in the Deafblind Program with CHARGE. We have had a lot of experience designing treatment to meet students individual needs. We would like to share this process with you in hope that you will be able to successfully use some of these strategies with the individuals with CHARGE that you know. Student Evaluations 1

3 We evaluated 29 students using the Compulsive Behavior Checklist (A. Gedye, 1992). Fourteen students are presently enrolled in the Deafblind Program at the Perkins School for the Blind. The other 15 students were seen during evaluations and/or individual consultations. (See Table 1 for summary of participant characteristics.) We have previously used this checklist and other standardized assessment tools to evaluate the unique behavioral characteristics of CHARGE students who attend our school. We found that the Compulsive Behavior Checklist was the most helpful tool in differentiating CHARGE students from other deafblind students in terms of repetitive behaviors. The Compulsive Behavior Checklist measures the number, intensity and redirectability of observed repetitive behaviors in five areas including ordering, completeness, cleaning, checking and grooming. In addition to a large number of common repetitive behaviors listed on the checklist, the checklist allows for idiosyncratic behaviors under the other heading for each of the five categories included. The checklists were filled out by the authors with assistance from family members and/or classroom staff. Results As previously noted, CHARGE students obtain a high score for repetitive behaviors. This group of students averaged 11.5 repetitive behaviors in an average of 4.2 categories. The break out by categories is as follows: ordering, 2.8 behaviors per student, completeness/incompleteness, 2.8 behaviors per student, cleaning/tidiness, 3.2 behaviors per student, checking/touching, 1.6 behaviors per student and grooming, 1.1 behaviors per student. Some of the unusual behaviors which were noted include must do things in a certain order, must eat specific foods at specific meals, shirt must be tucked in, objects must be in their place, a schedule must be followed in order, things must be empty (to be finished) and things must be perfect e.g., all mistakes must be undone. In addition several students desire pressing or tightness of clothing, look for 90 degree angles and express unusual interest in numbers or dates. A number of students display frequent perseverative 2

4 questions about people (where are they, when will they be back, when will they work), schedules, changes and vacations. (Please see Graph 1 for a summary of repetitive behaviors broken down by age group.) Twenty one of twenty nine students, or 72%, spend one or more hours per day involved in repetitive behaviors. For 83% of students, the behaviors significantly interfere with the student s daily routine. For 72% of students, the behaviors significantly interfere with the student s social activities and relationships. When parents or teachers attempt to redirect the students from the repetitive behaviors, 48% of the time, the student goes back to doing the behavior as soon as the adult withdraws. In 34% of the students, attempts to redirect the students from the repetitive behaviors result in hitting or kicking directed toward the adult or self injurious behavior including bites to self, hitting self or head banging. The responses to redirection are relatively consistent. Forty six percent of students who respond poorly to redirection respond occasionally or often by engaging in aggression toward others. Thirty one percent of students who respond poorly to redirection respond occasionally or often by engaging in self injurious behavior. Diagnosing and Treating Challenging Repetitive Behaviors It seems the case can be made that many students with CHARGE Syndrome engage in high frequency repetitive behaviors and that they respond poorly to redirection. In response to this finding, what is to be done? We have had many years of clinical experience in assisting parents and teachers of CHARGE students to define and treat various types of repetitive behaviors. We would like to share with you the process and techniques we have found helpful. Please see the attached flow chart for a brief summary. 1. Observation As trained clinicians, we have years of experience in observing and defining behavior. For our observations, we do not use a formal assessment tool. However, we generally observe students for two to four hours in a variety of settings. It is important to observe students in both structured and unstructured settings and in group and 3

5 individual activities. It also is helpful to observe students who are not interacting with adults. A free play activity is good for this purpose. We take many notes. Sometimes we count behaviors which appear problematic. A time sample can be helpful for this. (Example: count the number of times the student attempts to straighten chairs during a 30 minute interval). 2. Defining Behavior for Consideration After an observation, we usually discuss what we saw with teachers and/or parents. They have a wealth of information and often just need someone to organize it for them so repetitive behaviors can be categorized. We usually make an extensive list of repetitive behaviors and write out very specific definitions so that everyone agrees on what we are talking about. 3. Functional Analysis of Behavior We next look at the repetitive behaviors in relationship to the environment. We discuss what happens before the behavior, what happens after the behavior and what the student gets from or for doing the behavior. This can be a difficult task for non clinicians. People often tend to be overly subjective and interpret more than they should. We know as adults who work with youngsters with CHARGE, we are often called upon to speak for them. However, in this case, we must let the behaviors speak for themselves. One easy tool to use to take the subjectivity out of the process is the Motivational Assessment Scale (MAS) (Durand and Crimmins, 1988). This assessment can be used by anyone with a good definition of the behaviors under review. It takes only a few minutes to fill out and when you re finished, if the behaviors serve a function, you can tell what it is. Functions include getting sensory input, getting attention, getting material things and avoiding situations. 4. Treatment Options Treatment Options are based on functions/cause. You do not treat all students who repetitively tap their cheek the same way. We divide repetitive behaviors into four main categories and then work through the treatment options to find the most effective treatment for each individual student. Sometimes you can not be sure about the function/cause of a repetitive behavior. Sometimes you can only 4

6 diagnose OCD after repetitive behaviors or maladaptive routines fail to respond to a behavioral approach. Finding the correct treatment usually is a trial and error process. It takes a lot of time. Everyone must be patient and remain positive. You also will need excellent data collection. Hard, written down data is critical to this process. Because so many adults work with each student, individual observations can be confusing. People tend to remember what happened yesterday and forget to look at the big picture. In addition, behavior change takes time and happens in increments. You may not be able to tell the difference between doing a behavior 5 times a minute and 3 times a minute unless you count. Get everyone on board the data collection train. The four main categories of repetitive behavior and treatment options are listed below: Self Stimulatory Behaviors: Many CHARGE students have significant sensory impairments. We all know that everyone needs a certain amount of stimulation for neurological satisfaction. This area is often the first place to investigate when looking at repetitive behaviors. Treatment options include: a. SMI (sensory motor integration); a routine of sensory input activities prescribed by an Occupational Therapist designed to increase input in specific areas. b. teach new sensory behaviors to replace self stimulatory behaviors; sometimes these new behaviors are more socially appropriate or are less physically hurtful to the student. Focus on the topography of the existing behavior and try to replace it with something which will give the same sensory feedback. Use reinforcers to increase the new behavior. Make a decision when it is all right for the student to engage in self stimulatory behavior and when it is not. Redirect to other activities when the time is not right. Set aside self stimulation time. c. teach other new behaviors; increase the student s overall repertoire of functional behaviors. Sometimes learning to do more behavior with naturally decrease the need for self stimulation. Use reinforcers to increase other behavior. 5

7 d. self stimulatory behaviors as a reinforcer; use these highly preferred behaviors contingent on other less preferred activities. Alternate between the two sets of behaviors. e. consult a behavior therapist; you often can be more effective using the above strategies with a little help from someone who is an expert in teaching new or replacement behavior. f. consult with a psychiatrist; usually medication does not decrease self stimulatory behaviors except by decreasing overall activity/alertness level. Maladaptive Routines/Behaviors: CHARGE students display the same types of behavioral challenges that all students display. Many times they have learned to do things the wrong way because they did not have visual or auditory models due to their sensory impairments. Also, sometimes parents and teachers do not have the training, time or energy to teach deafblind students the correct routine or behavior which other youngsters or students just get. a. replace maladaptive routines/behaviors with adaptive ones; use task analysis. Teach the routine step by step. Redirect or interrupt maladaptive responses. Reinforce new routines/behaviors even if they are heavily prompted. Get the student doing the right thing anyway you can and then reinforce. Fade prompts as the adaptive routine gains strength. Use of behavioral consultation for this type of teaching can be helpful. b. change the environment to disrupt the routine; a major change in a schedule/procedure can cause the student to temporarily stop a maladaptive routine. At this point teach a new routine. Reinforce the new routine. c. reinforce flexibility; CHARGE students are nothing if not routine oriented. From a young age communicate about and practice that things change, things are canceled, people are absent and sometimes the car does not go. Teach responses to these changes such as oh well, he ll be back tomorrow, shucks, we ll have to do something else, we ll have fun doing a new thing, it s a surprise day. d. consult a behavior therapist; you often can be more effective using the above strategies with a little help from someone who is an expert in teaching new or replacement behavior. 6

8 e. consult with a psychiatrist; medication does not usually help with maladaptive routines. Hard work involving teaching is the key. Call a teacher or behavior consultant instead. Tics: Many CHARGE students appear to have tic disorders (Tourette s Disorder is included in this category.) Tics are repetitive behaviors which have a neurologic basis. Tics are defined as a sudden, rapid, recurrent, nonrhythmic, stereotyped motor movement or vocalizations. It is experienced as irresistible but can be suppressed for varying lengths of time (DSM IV). Tics can get worse with stress and fatigue and better when students are absorbed in an activity or asleep. a. give the movement a name; so the student can identify it. The first step in changing a behavior is bringing it to the student s attention. b. allow time to tic or breaks for tics; label the behavior, schedule time for the behavior, tell the student this is the correct place to do the behavior. c. social consequences; for some students awareness is not enough. They need to know that tics may draw undesirable attention to them and may frighten or intimidate others. While you don t want to undermine your CHARGE student s self esteem or draw attention to their differences, it is important to be realistic. Giving them coping strategies in public through problems solving and practice can be very helpful. d. consult a behavior therapist; you may be able to get help with some of these strategies. Focusing on the CHARGE student s self concept and setting up coping practice sessions can be very helpful. e. consult with a psychiatrist; medication can be very helpful in reducing or controlling tics. You should definitely consider this option. Obsessive Compulsive Behavior: Many CHARGE students appear to have some degree of obsessive compulsive disorder (OCD). This idea is not well documented but we are certain that most (but not all) of the 29 students we included in the above data can be diagnosed with OCD. OCD is recurrent obsessions or compulsions that are severe enough to be time consuming (more than 1 hour per day) and cause marked distress or significant impairment (DSM IV). Obsessions are persistent ideas, thoughts, impulses or images that are intrusive and inappropriate to the situation. Compulsions are repetitive behaviors which are reported to reduce anxiety but not 7

9 produce pleasure. Many individuals with OCD feel driven to perform the compulsive behavior. a. redirect compulsive behaviors; this sounds easy but can be extremely difficult. It is easier to redirect to a functional, hands on activity than it is to stop the behavior. However, keeping busy and active can be helpful. b. allow first repetitive behavior and then redirect; same as above but allows student to have some relief from the driven feeling of the obsessions c. teach internal controls; give the behavior a name, make a rule about the behavior, (the rule is when, where and how the behavior can be performed) review the rule about the behavior on a regular basis, use reminders about the rule. Offer positive strategies about what to do instead of the compulsive behavior. d. allow OCD time ; schedule time for the behaviors to occur without any redirection. Let the student know that this is the time to do OCD behavior. Use this treatment in conjunction with redirection and rules as above. e. approach and resist therapy; where the student places himself in a situation where the obsession is likely to occur and then resists doing the compulsive behavior. This is the treatment of choice (paired with medication) and should only be considered if working with a trained therapist with experience in treating OCD. Ask specifically about this approach and the therapist s training. You may find such treatment in a clinic that specializes in anxiety disorders and desensitization procedures. f. consult with a psychiatrist; medication can be extremely helpful in reducing or controlling OCD. Medication can make other treatments and self control techniques much more helpful. You should definitely consider this option. Behavior Treatment Trends We Have Seen with CHARGE Students We have gone through the above diagnostic and treatment procedure with approximately 20 students. Nothing works for everyone. We acknowledge the unique characteristics of all our students. However we have discovered some strong trends based on our previous 8

10 research and more recent observations. First, let us rule out what usually does not work. In the loser category we have; reprimands negative feedback loss of privileges or preferred activities physically stopping the behavior and restraints (except in an emergency). In the sometimes works category we have; using increased positive attention for appropriate, useful behavior at the student s functional level, tangible reinforcers (candy, tokens, pennies, stickers, prizes, special activities) given contingent on appropriate, useful behavior at the student s functional level and/or given contingent on the absence of undesirable repetitive behaviors contingent effort, having the student engage in a repetitive behavior involving some effort e.g., writing 10 sentences contingent on turning off the lights (the effort does not have to relate directly to the behavior observed). In the works most of the time category we have: changing an established routine to break a pattern of repetitive behaviors having a daily predictable schedule of activities (probably decrease anxiety) having a busy schedule of activities (little free time) decreasing environmental stimulation o time to sit and relax when repetitive behaviors are high o using a relax time ; sitting apart from others (sometimes a quiet and dimly lit place is helpful) o contingent Time Out (TO); if behaviors are disruptive to the environment waiting strategies, e.g., Rubic s cube, silly putty, paper clips, rubber bands, other manipulatives specific relaxation procedures; relaxation therapy, 10 deep breaths, yoga, deep pressure 9

11 Summary Treating students with CHARGE Syndrome and repetitive behaviors can be a challenge for the most dedicated parents and experienced teachers. These behaviors can interfere with all aspects of learning, be stigmatizing to the child and prevent the development of functional skills such as communication and social interaction. Since many CHARGE students appear to engage in repetitive behaviors, some at an extremely high rate and for many years, it is important to be on the look out for and recognize them when they are emerging. Don t just give them over to deafblindness and accept them. This is a huge disservice to our students. Of course, some repetitive behaviors are developmental. They appear and then drop out of the repertoire of the student over time. However, when the repetitive behaviors remain in the student s repertoire and interfere with learning, it is time for further evaluation. Since we have known and worked with so many students with CHARGE, we have developed a strategy for observing, defining, evaluating and treating all four types of repetitive behaviors which are most often seen. We have had good success with using this process and many of the students with whom we have worked have shown good reductions in interfering repetitive behaviors. Of course results vary. Some students have fluctuating levels of repetitive behaviors. Some students have dramatically increased repetitive behaviors in times of stress or illness. Others maintain a relatively high frequency of repetitive behaviors, but the behaviors do not interfere with on going learning or daily activities. The students, their families and staff must learn to live with and work around them. The need for thorough functional analysis of repetitive behaviors can not be over emphasized. This process can take a lot of time, months in fact. Sometimes one can not be sure of the analysis and must test out different hypotheses. You should seek out someone with a specific background in behavior analysis and therapy, behavior management in an educational setting or a behavior therapist. Also, in the case of tics and OCD, close work with a child psychiatrist who is familiar with special education and these types of diagnoses is 10

12 essential. You may have to search for the correct one. You may have to education the psychiatrist on your child s sensory impairments. Of course, you are used to that. You do it all the time. You ll know it s the correct one when you meet him/her. He will listen to what you say and ask many, many questions. He will tell you what diagnosis he is considering and give you treatment options. Read information about the diagnosis. Extrapolate for your own child s sensory and intellectual functioning. No one knows your child better than you do. We hope this outline will be helpful to you. We have found it helpful to our students and their families and teachers. We rely on each other a good deal of the time to brainstorm through these issues. Students with CHARGE Syndrome have many challenging issues. Repetitive behaviors appear to be part of who they are. This is all right. We all have unique characteristics. However, uniqueness should not prevent full access to the educational environment, social interactions, the community or the world. If your child with CHARGE does display high frequency repetitive behaviors which interfere with access to one or all of these areas, consider making a determined effort to change the behaviors. You have nothing to lose and your child has everything to gain. 11

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14 Table 1. SUMMARY OF PARTICIPANT CHARACTERISTICS (all students with CHARGE) Age Group Number of Students Vision Impairment Hearing Impairment Intel.Functional Impairment Blind Mod. Mild. Deaf Mod. Mild. Sev. Mod. Mild Average 3 6 y.o y.o y.o y.o y.o TOTAL:

15 y.o y.o y.o y.o y.o ORDERING COMPLETENESS CLEANING CHECKING/TOUCH GROOMING OTHER 14

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17 Bibliography Denno, L. S., and Bernstein, V. (1997). Behavioral Characteristics of CHARGE Association. Proceedings of the National Conference on Deafblindness, Washington D.C. Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition Durant, V. Mark, and Crimmins, Daniel, B. (1992). Motivational Assessment Scale (MAS), Monaco and Associates, Inc. Topeka, KS. Gedye, A. (1992). Compulsive Behavior Checklist. Habilitative Mental Healthcare Newsletter, Vol. 11. Moss, Kate. (Summer 1999). Looking at self stimulation in the pursuit of leisure or I m okay, you have a mannerism. SEE/HEAR Newsletter, Texas School for the Blind and Visually Impaired Outreach and Texas Commission for the Blind. Murcoch, H. (2000). Repetitive behaviors in children with sensory impairments and multiple disabilities: Summary of a dissertation. DBI Review: The Magazine of Deafblind International,