pervasive developmental disorders a case study. andy age 6 degenerative hearing loss diagnosed at...

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Pervasive Developmental Disorders A case study

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Page 1: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Pervasive Developmental Disorders

A case study

Page 2: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Andy

Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification. Cochlear implant surgery June of 1999 at age 4. Use is consistent. Student is committed to use. Speech and listening skills not improving. Relies on sign language for meaningful communication. Entered Special Needs Preschool at age 3 and has been

receiving services since.

Page 3: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Concerns

Andy has always engaged in self stimulating behaviors. When he entered preschool, he was orally and tactically

defensive. Not able to adjust to unexpected deviations in routine. Tends to obsess over objects being in line, rituals. Repeats himself. Learns rote material with little repetition, but cannot

generalize. Easily distracted; not able to divide attention.

These behaviors are obstacles to Andy’s instruction. I wanted to gain an understanding to be better

able to serve him and to address issues as needed.

Page 4: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Andy is currently receiving services for his hearing loss. Because an official diagnosis would not provide additional services, it is not being pursued. However, to provide effective services , an understanding of PPD is essential.

Of particular concern was his self-stimulating behavior. He is currently mainstreamed in a first grade class. This behavior is noticeable to the other students and becomes the target of their teasing.

Page 5: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Behavior: Crossing arms for self-stimulation when excited.

Intervention: Began with touching arms and telling him to put his arms down. The cue was lessoned to a meaningful look at his arms.

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BASELINE

INTERVENTION

Page 6: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Pervasive developmental disorder (PPD) is a broad term used to

describe a set of symptoms that occur along a continuum of

severity. This continuum is often referred to as Autism Spectrum

Disorders (ASD).

Page 7: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

The DSM-IV criteria consists of a set number of characteristics from three categories:

A. (1) qualitative impairment in social interaction, (2) qualitative impairments in communication, (3) restricted, repetitive, and stereotyped patterns of

behavior, interests, and activities, and,

B. Delays or abnormal functioning in at least one of the following areas, with onset prior to age 3 years: (1) social interaction, (2) language as used in social communication, or (3) symbolic or imaginative play.

C. The disturbance is not better accounted for by Rett's disorder or childhood disintegrative disorder.

Page 8: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Characteristics associated with PPD

are:

1. Withdrawn behaviors

2. Unsocial behaviors

3. Problems using language to

communicate

4. Repetitive patterns of behavior

5. Problems with imaginative play

6. Unable to deviate from schedule

7. Self-stimulating behavior

Page 9: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Some common disorders on this continuum are:

1. Asperger’s Syndrome2. Pervasive Developmental Disorder3. Rett’s Syndrome4. Childhood Disintegrative Disorder5. Autism

NICHD reports that 1in 500 people have some form of autism.

Page 10: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Asperger’s Syndrome

Unsocial behaviors Repetitive patterns of behavior Problems with imaginative play Good language skills Normal or near normal IQ

Page 11: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Pervasive Developmental Disorder

Signs of developmental problems, but without the significant autism symptoms.

Characteristics vary widely, but tend to be of a lesser degree than autism.

Sometimes referred to as Pervasive Developmental Disorder-not otherwise specified, or PDD-NOS.

Page 12: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Rett’s Syndrome

Has only been seen in girls. Development is normal until

6-18 months, then language and motor abilities are lost.

The development of the nervous system is not controlled leading to the loss of acquired skills.

Normal milestones are stunted.

Rate of progression is specific.

Page 13: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Current Research

NIH’s collaborative Program of Excellence in Autism has also identified a genetic origin for

Rett’s Syndrome.

Girls have two X chromosomes, therefore some genes are repeated.

Randomly one gene will shut down and the remaining one will control the process.

The disorder is the result of the mutation on the gene that makes methyl cytosine binging protein 2 (MECP2).

The abnormality interferes with the biochemical regulator (MECP2) of the gene’s expression.

Page 14: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

The defect prohibits a gene system from being shut down allowing too much of a “good” protein to be produced.

During infancy a balance system is in place to decrease the interference of that protein.

At the age of 2, the balance system no longer functions allowing the effects of the overproduction to surface.

It is believed that the syndrome is limited to girls because males have only one MECP2 producing gene. Therefore, if it is mutant, they have no “back up” copy and die before birth.

Page 15: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Autism

1. Withdrawn behaviors2. Unsocial behaviors3. Problems using language to communicate4. Repetitive patterns of behavior5. Problems with imaginative play6. Seizures (30%)7. Most often seen in boys8. Hypersensitivity to stimulation (visual, auditory, tactile), especially to being touched and loud noises9. Little or no eye contact

10. Digestive disorders are common

Page 16: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

11) Lack of eye contact 12)Inappropriate affect 13)Echolalia 14)Non-responsive to verbal cues 15)Inappropriate attachment to objects 16)Severe emotional outbursts without apparent

cause

Page 17: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

150 sibling pairs found strong evidence to indicate chromosomes 2 and 7 are involved with autism.

Another possible location, although the correlation is not as strong, is chromosomes 16 and 17.

Chromosome 7 is known to be linked to language disorders.

Not exactly sure what is wrong on the gene to cause the disorder.

In Fall of 2001, NIH’s collaborative Program of Excellence in Autism identified a suspected chromosome for autism.

Page 18: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

In the Fall of 2002, NIH’s Collaborative

Program of Excellence in Autism (10

multidisciplinary programs)

identified a gene, HOXA1, that may

predispose a person to autism.

Page 19: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Childhood Disintegrative Disorder

Normal development to age 2 then rapidly lose acquired skills (language, self-help).

Usually presents between 36-48 months, but can happen up to age 10.

Child loses interest in the environment. Very rare.

Page 20: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Interventions

PDD is usually treated with either: behavioral therapy,

pharmacological therapy, or a combination of

both.

Page 21: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Behavioral Therapy

One-on-one speech Communication therapy

– Picture Exchange Communication System (PECS)

Modeling to teach social, self-help skills, communication and learning

This approach is generally effective, depending heavily on the intensity of the

therapy and the severity of the child.

Page 22: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Pharmacological Therapy

Usually used for seizures, self-injurious behaviors, digestive difficulties

Secretin Therapy– A hormone involved in intestinal processes– A double blind study where each participant

received both treatment and saline, using a form of secretin derived from pigs, found no difference between it and placebo. (Journal of the American Academy of Child and Adolescent Psychiatry, Nov 2001)

A list of scientifically unreplicated therapies can be found at http://www.autism-biomed.org/unproven.htm

Page 23: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

PDD and deafness

Hearing loss (HL) is difficult to detect in children with PDD. One of the hallmarks of PDD is non-responsiveness to auditory cues. This makes it difficult to know if the child is not hearing or just not responding.

Oral communication is problematic without HL. Visual communication requires attending and eye

contact. Typically people with PDD avoid both of these.

Tactile defensiveness and over sensitivity to stimuli may prevent hearing aid fitting and use. Risk of damage and injury are increased in the presence of self-stimulating or injurious behaviors.

Page 24: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Resources

National Institute of Child Health and Human Developmentwww.hichd.nih.gov

General Informationwww.geocities.com/Heartland/Fields/6979

Autism Biomedical Information Network(Includes complete DSM-IV criterion)www.autism-biomed.org

Autism society of ALwww.autism-alabama.org

Asperger’s Disorder DSM-IVhttp://web.syr.edu/~rjkopp/data/as_diag_list.html

Personal Accounts and linkshttp://web.syr.edu/~rjkopp/autism.html

Page 25: Pervasive Developmental Disorders A case study. Andy Age 6 Degenerative hearing loss diagnosed at age 2. Bilateral hearing aid use began at time of identification

Resources, cont.

Autism/PDD Resources Networkhttp://www.autism-pdd.net/

Governer’s Council for Developmental Disabilities for GAhttp://www.ga-ddcouncil.org/pubs.htm

Autism Society of America’s Greater GA Chapterwww.asaga.com

GA Tools for Lifewww.gatfl.org

Parent to Parent of GAparenttoparentofga.org

GA State Resources at NICHCYwww.nichcy.org/stateshe/ga.htm

Hands on Atlanta-Autism Society of America http://www.handsonatlanta.org/calendar/where.asp?orgid=266