- Selected Topics
- Accessing the General Curriculum
- Auditory Training
- Calendar Systems
- Concept Development
- Daily Living Skills
- Environmental Considerations
- Harmonious Interactions
- Lilli Nielsen and Active Learning
- Orientation & Mobility
- Play & Recreation
- Social Interactions
- Tactile Strategies
- Universal Design for Learning
- van Dijk Approach
Behavioral Issues in Children with CHARGE Syndrome - Bibliography
1st CHARGE Syndrome Conference for Professionals: Conference Program and Handouts --CHARGE Syndrome Foundation, Inc. CHARGE Syndrome Foundation, Inc. (2009)This is a loose-leaf notebook containing printed copies of handouts and slides provided for presentations at the 1st CHARGE Syndrome Conference for Professionals held July 23, 2009 in Bloomingdale, IL.
9th International CHARGE Syndrome Conference: Conference Program and Handouts --CHARGE Syndrome Foundation, Inc. CHARGE Syndrome Foundation, Inc. (2009) This is a loose-leaf notebook containing printed copies of handouts and slides provided for presentations at the 9th International CHARGE Syndrome Conference held July 24-26, 2009 in Bloomingdale, IL.
Adaptive Behavior in Children with CHARGE Syndrome --Salem-Hartshorne N.; Jacob, S. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005. (2005) The "R" in the mnemonic "CHARGE" has historically stood for "Retardation of Growth and Development." Early medical reports describing mental retardation in CHARGE syndrome have not used convincing means to assess this attribute. This article investigated the range of developmental ability in individuals with CHARGE as measured through an adaptive behavior scale, the Adaptive Behavior Evaluation Scale (ABES) [Carney (1995): The Adaptive Behavior Evaluation Scale home version technical manual-revised. 126p.], over time. Parents of individuals with CHARGE syndrome were surveyed twice over a 4-year time span (N=100, 85) to obtain information about adaptive behavior and specific CHARGE characteristics. There was a significant decline in ABES scores over the 4-year period. However, at Time two, one-half of the children achieved a standard score above 70. Correlation and regression analyses at Time one and Time two revealed negative relationships between (a) age at walking, (b) degree of hearing impairment, (c) degree of visual impairment, and (d) medical involvement variables and the dependent variable, adaptive behavior. In both studies, age at walking was the best predictor of scores. Analysis of variance confirmed that medical involvement and degree of vision impairment were related to adaptive behavior scores because they were also related to age at walking. Age at walking and medical involvement at Time one were found to be significantly different between those who improved and declined in adaptive behavior scores over time. Adaptive behavior scores from both studies revealed a much broader and higher-reaching range of ability for this population than has been previously reported in the literature. Available on the web: http://onlinelibrary.wiley.com/doi/10.1002/ajmg.a.30546/full
Autistic-like Behavior in CHARGE Syndrome --Hartshorne TS.; Grialou TL.; Parker KR. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005, 257-261. (2005) Children with CHARGE syndrome frequently exhibit moderate to severe behavior difficulties, and are often diagnosed with obsessive-compulsive disorder, attention deficit disorder, Tourette syndrome, and autism. Hartshorne and Cypher (2004) surveyed parents of 100 children with CHARGE worldwide and confirmed the prevalence of behaviors that are associated with these disorders. They also found behaviors that could be described as typical of persons who are deafblind. The present study examined whether the autistic-like behaviors of children with CHARGE are more similar to those of children who are deafblind, to those of children who are autistic or are unique to CHARGE. Surveys including the Autism Behavior Checklist (ABC) were mailed to families of 204 children with CHARGE, and 160 usable surveys were returned (78%). Total scores on the ABC for children with CHARGE were significantly different from the norms for those with autism, and those who were deafblind. However, the variance for CHARGE was larger than for the normative groups, and 27.5% of those with CHARGE could be classified as autistic. The pattern of subscale scores for those with CHARGE differed from the other normative groups. Available on the web: http://onlinelibrary.wiley.com/doi/10.1002/ajmg.a.30545/full
"B" in CHARGE: The Behavioral Phenotype --Nicholas, Jude; Hartshorne, Timothy S. 14th DbI World Conference on Deafblindness Conference Proceedings, September 25-30, 2007, Perth, Australia. (2007)This is the text of a workshop presentation given at the 14th DbI World Conference on Deaf-Blindness. The presentation describes that after 10 years of seeking to understand the challenging behavior in CHARGE syndrome, a tentative behavioral phenotype is starting to emerge.
"B" in CHARGE: The Hunt for the Source of Behavioral Challenges --Nicholas, Jude; Hartshorne, Timothy S. 14th DbI World Conference on Deafblindness Conference Proceedings, September 25-30, 2007, Perth, Australia. (2007)This is the text of a workshop presentation given at the 14th DbI World Conference on Deaf-Blindness. The main purpose of the presentation is to feature the work that is taking place to better understand challenging behavior in CHARGE syndrome.
Behavior in CHARGE Syndrome: Introduction to the special topic --Hartshorne, Timothy S.; Hefner, Margaret A.; Davenport, Sandra L.H. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005, 228-231. (2005) CHARGE association (CA) consists of a non-random association of ocular coloboma (C), heart anomaly (H), atresia of choanae (A), retarded growth and/or development (R), genital hypoplasia (G), and ear anomalies and/or hearing impairment (E). A prospective multidisciplinary study of 31 Swedish patients with CA was undertaken in order to describe the associated malformations and functional deficits, find possible etiological factors and identify critical time periods for the maldevelopment. The clinical files were analyzed, the mothers answered a questionnaire on history of prenatal events, and a clinical evaluation of systemic findings, vision, hearing, balance, speech, oral and swallowing function, and neuro-psychiatric function, especially autism, was performed. The most frequent physical abnormalities affected ears (90%), eyes (90%), brain (61%), heart (52%), retarded growth (48%), genitals (38%), choanae (35%), and facial nerve (32%). Sixty-one percent of the patients were visually impaired or blind, and 74% had hearing loss or deafness. Problems in balance, speech, and eating were common. Forty percent of the patients had autism/atypical autism, and 82% had developmental delay. Three children were born following assisted fertilization and two mothers had diabetes. The mothers reported infections, bleedings, and drug use during pregnancy. Analysis of possible critical time periods suggested that most malformations were produced early in pregnancy, mainly during post conceptual weeks 4, 5, and 6. A multidisciplinary approach is essential in the assessment and management of CA. Available on the web: http://onlinelibrary.wiley.com/doi/10.1002/ajmg.a.30541/full
Behavior Symposium: Panel A --Brown, David, M.A.; van Dijk, Jan, Ph.D. Cleveland, OH: Proceedings of the 6th International CHARGE Syndrome Conference, July 25-27th, 2003, Cleveland, OH. (2003)This presentation presents several case studies to describe the relationship between sensory difficulties and resulting behavior. Describes several scenarios and the resulting behavior and provides examples of how to modify that behavior into something more positive. Also available is a CD-ROM containing an audio version of this presentation.
Behavior Symposium: Panel B --Denno, Laurie S., M.A.; Souriau, Jacques, D.E.A.; Hartshorne, Timothy, Ph.D. Cleveland, OH: Proceedings of the 6th International CHARGE Syndrome Conference, July 25-27th, 2003, Cleveland, OH. (2003)These presenters describe various types of behavior associated with children with CHARGE Syndrome. The first presenter describes common repetitive behaviors in children with CHARGE. These include self-stimulatory behavior, maladaptive routines, tics, and obsessive-compulsive behavior (OCD). The second presenter describes a questionnaire completed regarding behavior in the domains of social skills, communication, and cognitive skills in children with CHARGE Syndrome. The final presenter describes the prevalence of autistic like behavior in children with CHARGE Syndrome. Also available is a CD-ROM containing an audio version of this presentation.
Behavior Symposium: Panel C --Nicholas, Jude, Ph.D.; Williams, George, M.D. Cleveland, OH: Proceedings of the 6th International CHARGE Syndrome Conference, July 25-27th, 2003, Cleveland, OH. (2003)This presentation describes a case study using the BRIEF, which assesses executive function in children and adolescents. The BRIEF consists of two rating forms, a parent questionnaire and a teacher questionnaire. Describes the usefulness of using this tool in evaluating children with CHARGE Syndrome. A second speaker describes the brain of a child with CHARGE Syndrome from a neuroscience point of view. Describes emotional experience, sensory deprivation and dysfunction, pain and stress, ineffective communication, neurotransmitters, and drug use and abuse. Also available is a CD-ROM containing an audio version of this presentation.
Behavior Symposium: Panel D --Blake, Kim, M.D.; Graham, John M., M.D., Sc.D. Cleveland, OH: Proceedings of the 6th International CHARGE Syndrome Conference, July 25-27th, 2003, Cleveland, OH. (2003)This presentation describes behavioral phenotype of CHARGE Syndrome. Discusses a study by the Canadian Pediatric Surveillance Program (CPSP), preliminary results and future work in this area. A second speaker describes behavioral phenotype in CHARGE Syndrome compared with other common syndromes. Also available is a CD-ROM containing an audio version of this presentation.
Behavioral Features of CHARGE Syndrome (Hall-Hittner syndrome) Comparison With Down Syndrome, Prader-Willi Syndrome, and Williams Syndrome --Graham JM Jr.; Rosner B.; Dykens E.; Visootsak J. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005, 240-247. (2005)CHARGE syndrome, or Hall-Hitner syndrome (HHS), has been delineated as a common syndrome that includes coloboma, choanal atresia, cranial nerve dysfunction (particularly asymmetric facial palsy and neurogenic swallowing problems), characteristic ear abnormalities, deafness with hypoplasia of the cochlea and semicircular canals, genital hypoplasia, and variable heart defects, orofacial clefting, tracheo-esophageal fistula, renal anomalies, thymic/parathyroid hypoplasia, spine anomalies, short broad neck with sloping shoulders, and characteristic facial features. We conducted behavioral and personality assessments in 14 boys with HHS syndrome aged 6-21 years, and compared their characteristics with similar data from 20 age-matched boys with Down syndrome (DS), 17 boys with Prader-Willi syndrome (PWS), and 16 boys with Williams syndrome (WS). We used the Reiss Profile of Fundamental Goals and Motivation Sensitivities, the Achenbach Child Behavior Checklist (CBCL), and the Aberrant Behavior Checklist (ABC). All 14 boys with HHS were legally deaf, and 10 of the 14 were also legally blind. In comparison these other syndromes, boys with HHS had behavior that resembled autistic spectrum disorder. They were socially withdrawn, lacked interest in social contact, and manifested reduced seeking of attention from others, with hyperactivity and a need to maintain order. Though the boys with HHS showed decreased social interaction, they were not as socially impaired as in classic autism. Their language was delayed due to dual sensory impairment, cranial nerve deficits, and chronic medical problems, but their language style was not abnormal (no echolalia or jargon, no scripted phrases, and no pronoun reversal). Boys with HSS appeared frustrated, but they were not aggressive, or at risk for delinquency, manifesting few stereotypic behaviors or unusual preoccupations. They did not have a restricted repertoire of activities and interests. Their behavioral features appeared to be due to dual sensory impairment affecting hearing and vision, rather than to primary autistic spectrum disorder, but successful remediation requires similar educational interventions, which are discussed herein.
Behavioral Features of CHARGE Syndrome: Parents' Perspectives of Three Children With CHARGE Syndrome --Lauger K.; Keedy W.; Cornelius N. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005, 391-399. (2005) As the population first diagnosed with CHARGE Syndrome has aged, information has emerged about specific behavioral features of this syndrome. Once the medical features are managed, it is the behavioral features that interfere with our children's ability to form reciprocal relationships, to gain formal communication skills, to make the educational gains they are cognitively capable of, and to ultimately care for themselves. In telling the informal case studies of our children, we hope their behavioral commonalities, in spite of their medical and educational differences, will be apparent as well as the complexity of the multiple types of behavior observed. We remain hopeful that answers can be found that will help our children and our families cope with this very debilitating feature of CHARGE. Available on the web: http://onlinelibrary.wiley.com/doi/10.1002/ajmg.a.30557/full
Behavioral Phenotype --Hartshorne, Timothy S. The ASHA Leader, October 17, 2006, p. 41. (2006)Very brief article highlighting some of the research aimed at describing a behavioral phenotype for CHARGE Syndrome. Recent research supports the presence of executive dysfunction, or problems with shifting, initiating, inhibiting, or sustaining actions based on prefrontal cortex activity. Other areas being explored include the presence of a regulatory disorder making it difficult for the child to regulate complex processes such as their sleep-wake cycles, hunger-satiety cycle, manage their emotions, etc. Research has also found indications of significant sleep disorders in well over half of these children which can have a significant impact on behavior.
The Behavioral Phenotype in CHARGE: The "B" in CHARGE --Hartshorne, Tim. Costa Mesa, CA: 8th International CHARGE Syndrome Conference, July 26-29, 2007, Costa Mesa, CA. (2007) PowerPoint Presentation. Offers a brief history of development of information about behavior and CHARGE, personal history and steps in developing a working model of a behavioral phenotype. Steps include: understanding the syndrome, observing children, review research, understand the impact of sensory deficits, parenting styles, impact of early medical issues, cognitive ability, etc. Offers a first draft of a behavioral phenotype, benefits of having one, possible intervention plans and considerations for the IEP.
Behavioral Profiles and Symptoms of Autism in CHARGE syndrome: Preliminary Canadian Epidemiological Data --Smith IM; Nichols SL; Issekutz K; Blake K. --Canadian Paediatric Surveillance Program. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005, 248-256. (2005)Individuals with CHARGE syndrome were identified through the Canadian Pediatric Surveillance Program (CPSP). From this population-based cohort (n=78), we present data on developmental and behavioral characteristics for the first 13 individuals (eight males, five females) for whom assessments are complete. Standardized parent questionnaires on development and behavior were followed by a structured telephone interview, with a specific emphasis on symptoms of autistic spectrum disorder (ASD). Preliminary results confirm that individuals with CHARGE syndrome have relatively low adaptive behavior skills, motor impairments being particularly significant. Most individuals did not present with significant behavior problems; however, evidence of ASD symptoms was judged to be moderate to strong in six of the ten children who were above the age of 4-5 years. Results are discussed with reference to the challenges inherent in the diagnosis of autism in individuals with sensory impairments, and to the implications for understanding the etiology of CHARGE syndrome and of ASD.
Behavioural Phenotypes in CHARGE Syndrome: A Study of Behavioural Aspects in People With CHARGE Syndrome --Hoevenaars-van den Boom, Marcella, A.A.; Vervload, Mathijs P.J.; Admiraal, Ronadl J. C. 14th DbI World Conference on Deafblindness Conference Proceedings, September 25-30, 2007, Perth, Australia. (2007)This is text of a workshop presentation given at the 14th DbI World Conference on Deaf-Blindness. This presentation describes the aim of a study of the existence of a pattern of behavioural characteristics in people with CHARGE syndrome.
Can Specific Deficits in Executive Functioning Explain the Behavioral Characteristics of CHARGE Syndrome: a case study --Nicholas J. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005, 300-305. (2005) I present the case of a 12-year-old girl diagnosed with CHARGE syndrome. As an infant she had shown typical characteristics of CHARGE. Neuroimaging revealed asymmetrical ventriculomegaly. Both her teacher and parents reported academic and psychosocial problems. Neuropsychological assessment revealed significant executive dysfunction, including attention difficulties. We discuss the possible association between executive deficits and school and psychosocial problems. This case also highlights the importance of evaluating executive function in children with CHARGE, which is particularly useful in efficiently identifying needs for direct intervention. Available on the web: http://onlinelibrary.wiley.com/doi/10.1002/ajmg.a.30558/full
Challenging Behaviors in CHARGE Syndrome: Psychiatric and Behavioral Approaches to Assessment and Treatment --Wachtell, Lee Elizabeth; Kahng, Sung Woo. Miami Beach, FL: 7th International CHARGE Syndrome Conference, July 22nd - July 24th, 2005, Miami Beach, Florida. (2005)Copy of powerpoint presentation. Authors review research about sensory deprivation and attachment and compare the resulting behaviors to the behavioral phenotype in CHARGE syndrome. Treatment options discussed emphasize touch and communication.
CHARGE Syndrome: Relations Between Behavioral Characteristics and Medical Conditions --Vervloed, Mathijs; Hoevenaars-van den Boom, Marella; Knoors, Harry; van Ravenswaaij, Conny; Admiraal, Ronald. American Journal of Medical Genetics, 2006, 140A:851-862. (2006) The behaviors and medical problems in 27 persons with CHARGE syndrome were studied, because it was hypothesized that their behavior might be partly dependent on the heterogeneous medical status. With the exception of more tics, cardiac surgery was associated with positive behaviors: less withdrawn behavior, better mood, and a more easy temperament. Tube feeding was also related to positive behavior, since participants with a history of tube feeding showed less intense behavior. Cerebral deficits were associated with three problem behaviors: more intense and withdrawn behavior and a worse mood. Deafblindness was associated with developmental delays in expressive and overall communication level, and recurrent middle ear infections correlated with delays in written language. Of all medical conditions, only the presence or absence of heart defects and cardiac surgery could differentiate between the participants with regard to the number of behavioral problems. Participants with heart surgery especially, had less behavior problems. The number of operations and hospitalizations was not associated with behavior, but the total length of the hospitalizations was. Long hospital stays were associated with less problem behavior, especially internalizing behaviors. Cerebral and heart problems did not result in longer hospital stays, whereas esophageal reflux did. Age effects were reflected in older participants, who showed more internalizing problems. Heart surgery and hospitalization may be protective factors, but the protection might not be the actual surgery or hospital stay, as there may be other variables that are the actual cause, such as reduced vitality or altered parent child interactions after heart surgery. The study could not confirm a significant association between medical conditions and autism found in previous studies. Available on the web: http://onlinelibrary.wiley.com/doi/10.1002/ajmg.a.31193/full
CHARGE Syndrome 101: Characteristics and Diagnostic Criteria --Hefner, Meg. Miami Beach, FL: 7th International CHARGE Syndrome Conference, July 22nd - July 24th, 2005, Miami Beach, Florida. (2005)An article that reviews the 1998 revised diagnostic criteria for CHARGE, illustrated with pictures of ears, hands, facial asymmetry. Also, briefly reviews medical management issues, behavior, assessment and development.
CHARGE syndrome: 2005. --Carey JC. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005, 227. (2005)Editorial.The discovery in 2004 of mutations in the gene, CDH7 in 10 of 17 individuals with CHARGE establishes it as a syndrome. This makes the timing of this special issue on CHARGE even more important. These articles are particularly unique for this journal in their detailed coverage of the behavior phenotype but this is an important theme for medical geneticists involved in the management of individuals with this syndrome.
CHARGE Syndrome "behaviors": Challenges or Adaptations? --Brown D. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005, 268-272. (2005) Sometimes behaviors reported as challenging are more often adaptive responses to severe levels of multi-sensory impairment. A therapy/educational response may be more productive. Multi sensory is more than deaf-blind. Issues related to balance, hearing, vision, and cranial nerve anomalies are discussed. These are followed by comments about sensory integration (SI) and communication. Finally there are examples of behaviors that were moved into “non-challenging” category by taking a multi-sensory perspective toward them. Available on the web: http://onlinelibrary.wiley.com/doi/10.1002/ajmg.a.30547/full
CHARGE Syndrome: Developmental and Behavioral Data --Souriau J; Gimenes M; Blouin C; Benbrik I; Benbrik E; Churakowskyi A; Churakowskyi B. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005, 278-281. (2005)Report on research conducted in France. Focus on the behavioral and developmental issues related to CHARGE. A questionnaire to be completed by families was developed and completed by 71 families. Results are discussed. Although this is only preliminary research, it does offer a description of behaviors that can be observed in a significant number of children with CHARGE. Further research is needed.
Charge Syndrome: Dual Sensory Impairment, Cognitive Development and Anxiety --Reau, Charlotte; Blouin, Corinne; Tap, Christine. DBI REVIEW, #39, January-June 2007, pp. 34-35. (2007)The three authors are psychologists working in France. Christine is also Headteacher of CESSA, a school for deafblind children. This is the third article of a series of three which discuss methodology, cognitive development and the first clinical observations about anxiety. In this article they discuss their clinical observations which are centered around the expression of language, the nature of anguish in their expression, the loss of objects and tactile sensitivity in place of spoken language.
Charge Syndrome Revisited (2007): A 5 Year Follow-Up of 6 Children with CHARGE Syndrome --van Dijk, Jan; Nelson, Cathy; de Kort, Arno. AapNootMuis Educainment. (2007) This is an updated version of the following CD-ROMs: Living With CHARGE: Assessment, Prevention and Intervention of Challenging Behavior (2002) and Living With CHARGE: How to Deal with Challenging Behaviors and Enhance Communication (2005). In this newest edition, four of the original children have been recorded again and two new children have been added. Topics include intervention strategies specific to challenging behaviors, feeding problems, depression, cochlear implantation, communication, and literacy. Cost: $35.00. Available from: Vision Associates. Web: https://visionkits.com/
Conference Account: CHARGE Syndrome Behavior Symposium --Hartshorne, Tim. CHARGE ACCOUNTS, vol. 13, #3, Fall 2003, pp8-9. (2003)This article provides a synopsis of the CHARGE Syndrome Behavior Symposium. It includes information on the four goals of the conference, information on each presentation, and a summary of the conference itself.
2011-0377 Deaf-Blindness, Self-Regulation, and Availability for Learning: Some Thoughts on Educating Children with CHARGE syndrome --Brown, David. RESOURCES, vol. 16, #3, Fall 2011, pp. 1-7. (2011) Available on the web: http://www.cadbs.org/newsletter/resources-fall-2011/
An Ecological Approach of Development --Souriau, Jacques. Miami Beach, FL: 7th International CHARGE Syndrome Conference, July 22nd - July 24th, 2005, Miami Beach, Florida. (2005)The purpose of this presentation is to identify the parameters in the environment that facilitate active exploration of the world, information processing, active learning, sustained interaction and communication. It is assumed that looking at behaviors from an ecological point of view can help designing better contexts for children's development. Unusual behaviors, instead of being seen as deviations from the normal, could also be addresses as indications of how the world is perceived and as opportunities for socially functional development.
Executive Function in Charge Syndrome --Hartshorne, Timothy S.; Nicholas, Jude; Grialou, Tina L.; Russ, Joanna M. Child Neuropsychology; Aug 2007, Vol. 13 Issue 4, p333-344. (2007) This study addressed the presence of executive dysfunction in children with CHARGE syndrome, a genetic disorder with multiple physical anomalies and severe challenging behaviors. Ninety-eight children were included in the study. More than half received clinically significant scores on the Behavior Rating Inventory of Executive Function (BRIEF; Gioia et al., 2000) scales of Shift, Monitor, and the Behavioral Regulation Index, with additional high scores on Inhibit and the Global Executive Composite. Associations were found with the age the child first walked, scores on the Autism Behavior Checklist (ABC; Krug et al., 1993), and being classified as deafblind. Difficulties with making transitions and flexible problem solving, monitoring their work and their effect on others, and acting on impulse, may be related to the behavioral difficulties exhibited by children with CHARGE. Interventions targeting improved self-regulation may help to manage this challenging behavior.
How to Recognize, Acknowledge and Support the Ability of a Child with CHARGE Syndrome in Finding Solutions to Communicate and to Meet Challenges in their Daily Life --Ang, Ulla Holmqvist; Hedstrom, Kristina. 14th DbI World Conference on Deafblindness Conference Proceedings, September 25-30, 2007, Perth, Australia. (2007)This is text of a workshop presentation given at the 14th DbI World Conference on Deaf-Blindness. This presentation describes three children with CHARGE syndrome and how to recognize challenging behaviors as attempts at communication.
Life, Liberty, and the Pursuit of Happiness: Can emotional competence ever be an educational goal? --Brown, David. Costa Mesa, CA: 8th International CHARGE Syndrome Conference, July 26-29, 2007, Costa Mesa, CA. (2007)PowerPoint Presentation. Explores the areas of emotional intelligence, social and emotional competence, and development; the critical importance of the communication partner's ability to note and respond to the child's psychological and emotional needs; and the risk factors that CHARGE Syndrome presents, e.g., problems with self-regulation, neurological and sensory difficulties and communication competence.
Medical & Development Issues in CHARGE Syndrome --Davenport, Sandra L.H. Austin, TX: Texas Deafblind Project. 2005 Texas Symposium on Deafblindness. (2005)This article contains brief descriptions of medical management issues including: swallowing problems, gastroesophageal reflux, compromised airway, recurrent otitis media, middle ear bone malformations, sinusitis, scolliosis, detached retinas, short stature, disturbed sleep cycles and chronic constipation. Development issues are also briefly described and include input impairment, output impairment, intelligence and psychological assessment, and behavior issues.
A Neurodevelopmental Perspective of CHARGE Syndrome: The Impact of Altered Development on Learning, Behavior and Being --Lauger, Kim. Costa Mesa, CA: 8th International CHARGE Syndrome Conference, July 26-29, 2007, Costa Mesa, CA. (2007)With CHARGE, the most basic and essential tasks of life, breathing, eating, and moving are often impacted from the beginning. These stressors then alter the functioning of the autonomic nervous system. Due to the interconnectedness of systems, irregularities in one system impact the development of other systems. This paper discuses this impact and how this manifests itself in behavior. Recommended reading and definitions are included. Reprints of two articles by Judith Bluestone of the HANDLE Institute are also included.
Observing, Defining, and Treating Repetitive Behaviors in CHARGE Syndrome --Denno, Laurie. Miami Beach, FL: 7th International CHARGE Syndrome Conference, July 22nd - July 24th, 2005, Miami Beach, Florida. (2005)This is a set of tables related to diagnosing and treating challenging repetitive behaviors. Begins with a flow chart showing how observation leads to defining behaviors to functional analysis to identification and differentiated treatments. This is followed by a list of descriptive definitions of behaviors and a discussion of the success of various treatment strategies.
2012-0256 Parent and Teacher Perceptions of Challenging Behavior in their Children and Students with CHARGE Syndrome --Sheriff, Lorna Kathleen. Texas Tech University. (2012) A dissertation in special education submitted to the graduate faculty of Texas Tech University in partial fulfillment of the requirements for the degree of doctor of education. Available on the web: http://repositories.tdl.org/ttu-ir/bitstream/handle/2346/46966/SHERIFF-DISSERTATION.pdf?sequence=1.
Reading Behavior as Communication --Hartshorne, Tim; Grassick, Sharon Barrey. Miami Beach, FL: 7th International CHARGE Syndrome Conference, July 22nd - July 24th, 2005, Miami Beach, Florida. (2005) Copy of a PowerPoint presentation. Focus on communication for infants/ toddlers in medical situations as well as youngsters post-hosital, defines broad range of communicative behaviors, offers tips, and a grid that describes the effect of sensory losses.
Reducing Challenging Behaviors and Fostering Efficient Learning of Children With CHARGE Syndrome --van Dijk JP; de Kort A. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005, 273-277. (2005) Letter. Describes a single-subject case study. Author states that if the challenging behaviors exhibited by individuals with CHARGE are to be effectively reduced, intervention must take into account and address the underlying causes and the consequences of the behaviors. Concludes that 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.
Repetitive Behaviors in CHARGE Syndrome: Differential Diagnosis and Treatment Options --Bernstein V; Denno LS. American Journal of Medical Genetics, Vol 133A, Issue 3, March 15, 2005, 232-239. (2005) Twenty-nine students with CHARGE syndrome were evaluated using the Compulsive Behavior Checklist (CBC). Most of the students obtained a high score for repetitive behaviors, averaging 11.5 repetitive behaviors per student. Most students' repetitive behaviors significantly interfered with their daily routine. Thirty-four percent of the students responded to redirection with aggression toward themselves or others. Many of these behaviors do not respond well to traditional behavioral techniques. Through observation, definitions, and using functional analysis of behavior, repetitive behaviors were assigned to one of four categories. Each category has different treatment options. A case study of an adolescent girl with CHARGE syndrome who has profound deafness, colobomas, and moderate cognitive delay is presented. She displayed severe behavior challenges and significant medical issues. The course of treatment is outlined over 5 years. Behavior management, medical management, and psychiatric management were used in regulating her behavior, medical issues, and anxiety disorder. Close coordination between disciplines and excellent family involvement led to a very positive outcome. The student, now 19 years old, has made progress in her educational program and has been able to experience more community integration. Available on the web: http://onlinelibrary.wiley.com/doi/10.1002/ajmg.a.31193/full
Self-destructive Behaviour in a Nine-year-old Boy with CHARGE Syndrome --van Delden, Geert W. 14th DbI World Conference on Deafblindness Conference Proceedings, September 25-30, 2007, Perth, Australia. (2007) This is a brief summary of a workshop presentation given at the 14th DbI World Conference on Deaf-Blindness. This presentation is a case study of self-destructive behaviour in a young boy with CHARGE syndrome.
Sleep Disturbances in CHARGE Syndrome: Types and Relationships with Behavior and Caregiver Well-Being --Hartshorne, Timothy S.; Heussler, Helen S.; Dailor, A. Nichole; Williams, George L.; Papadopoulos, Dimitrios; Brandt, Kimberly K. DEVELOPMENTAL MEDICINE and CHILD NEUROLOGY, vol. 51, #2, pp. 143-150. (2009) Children with CHARGE syndrome frequently develop moderate to severe behavior difficulties and are often diagnosed with obsessive-compulsive disorder, attention deficit disorder, Tourette syndrome, and autism. Anecdotal reports have indicated that sleep is also affected. However, the prevalence and types of sleep disturbance have not been identified. This study investigated sleep disturbances in 87 children with CHARGE syndrome, aged 6 to 18 years. Eighteen of the children were deaf-blind. There were 52 males and 35 females. Instruments included measures of sleep (Sleep Disturbances Scale for Children [SDSC]), behavior (Developmental Behaviour Checklist [DBC]), and carer well-being (Malaise Inventory). On the SDSC, 57.5% received scores considered significant for sleep disturbances, with disorders of initiating and maintaining sleep, sleep breathing disorders, and sleep-wake transition being the most common. The SDSC was significantly correlated with the DBC (p=0.010) and the Malaise Inventory ( p=0.003). Regression analysis found that both problem behavior and sleep disturbances contributed to the prediction of scores on the Malaise Inventory. Being both deaf and blind (p=0.001), experiencing frequent middle-ear infections ( p=0.015), and starting to walk at an older age ( p=0.007) were associated with more sleep disturbance. Craniofacial anomalies were not. The study highlights the importance of addressing the sleep difficulties associated with CHARGE syndrome relating both to airway management and to disorders of initiating sleep.
Some Common Educational and Behavioral Implications of CHARGE Syndrome --Brown, David. Brantford, Ontario: Canadian Deafblind and Rubella Association. 13th DbI World Conference on Deafblindness Conference Proceedings, August 5-10, 2003, Mississauga, Ontario, Canada. (2003) This is the text of a workshop presentation given at the 13th DbI World Conference on Deaf-Blindness. The paper describes the broad medical aspects of CHARGE and the implications of these things for the children's functioning, development, behavior and educational needs.
Your Voices Heard: Results of the Survey --Whitman, Barbara Y.; Hefner, Meg; Davenport, Sandra. CHARGE ACCOUNTS, vol. 13, #2, Summer 2003, pp. 9-11. (2003) This article provides results of CHARGE Accounts Fall 2003 survey. It includes demographic information, statistical analysis, and three tables of results. Topics included the importance of various behaviors, various treatments, and topics of interest.