Chapter 7 Autism Spectrum Disorders
(Pervasive Developmental Disorders)
PATHOPHYSIOLOGY
Autism spectrum disorders (ASDs), also known as pervasive developmental disorders, comprise a group of neuropsychiatric disabilities involving varying degrees of restricted, repetitive, and stereotyped patterns of behavior as well as impairment in communication skills and social interaction. ASD diagnoses range from the milder Asperger’s disorder to the more severe autistic disorder. The Diagnostic and Statistical Manual of Mental Disorders, fourth edition, text revision (DSM-IV-TR) outlines specific observable behavioral components related to social interactions, communication, and repetitive and stereotypical behaviors and other indicators, presented for Asperger’s disorder in Box 7-1 and autistic disorder in Box 7-2. Intact language skills distinguish Asperger’s disorder from autism. Rare forms of ASD include Rett syndrome (Box 7-3) and childhood disintegrative disorder (CDD) (Box 7-4). In both disorders there is normal development followed by a profound regression of cognitive abilities. Rett syndrome occurs only in girls and is likely genetically based. The DSM criteria for pervasive developmental disorder, not otherwise specified, are presented in Box 7-5.
Box 7-1 Diagnostic Criteria for Asperger’s Disorder
A. Qualitative impairment in social interaction, as manifested by at least two of the following:
B. Restricted repetitive and stereotyped patterns of behavior, interests, and activities, as manifested by at least one of the following:
C. The disturbance causes clinically significant impairments in social, occupational, or other important areas of functioning
D. There is no clinically significant general delay in language (e.g., single words used by age 2 years, communicative phrases used by age 3 years)
E. There is no clinically significant delay in cognitive development or in the development of age-appropriate self-help skills, adaptive behavior (other than social interaction), and curiosity about the environment in childhood
F. Criteria are not met for another specific pervasive developmental disorder of schizophrenia
From American Psychiatric Association: Diagnostic and statistical manual of mental disorders, ed 4, text revision (DSM-IV-TR), Washington, DC, 2000, The Association.
Box 7-2 Diagnostic Criteria for Autistic Disorder
A. A total of six (or more) items from (1), (2), and (3), with at least two from (1), and one each from (2) and (3)
B. Delays or abnormal functioning in at least one of the following areas, with onset before age 3 years:
C. The disturbance is not better accounted for by Rett syndrome or Childhood Disintegrative Disorder.
From American Psychiatric Association: Diagnostic and statistical manual of mental disorders, ed 4, text revision (DSM-IV-TR), Washington, DC, 2000, The Association.
Box 7-3 Diagnostic Criteria for Rett Syndrome (Females Only)
B. Onset of all of the following after a period of normal development:
From American Psychiatric Association: Diagnostic and statistical manual of mental disorders, ed 4, text revision (DSM-IV-TR), Washington, DC, 2000, The Association.
Box 7-4 Diagnostic Criteria for Childhood Disintegrative Disorder*
A. Apparently normal development for at least the first 2 years after birth as manifested by the presence of age-appropriate verbal and nonverbal communication, social relationships, play, and adaptive behavior
B. Clinically significant loss of previously acquired skills (before age 10 years in at least two of the following areas):
C. Abnormalities of functioning in at least two of the following areas:
D. The disturbance is not better accounted for by another specific pervasive developmental disorder or by schizophrenia.
*Childhood disintegrative disorder is much rarer than autism.
From American Psychiatric Association: Diagnostic and statistical manual of mental disorders, ed 4, text revision (DSM-IV-TR), Washington, DC, 2000, The Association.
This category should be used when there is a severe and pervasive impairment in the development of reciprocal social interaction or in verbal and nonverbal communication skills, or when the stereotyped behavior, interests, and activities are present, but the criteria are not met for a specific pervasive developmental disorder, schizophrenia, schizotypal personality disorder, or avoidant personality disorder. For example, this category includes “atypical autism”—presentations that do not meet the criteria for autistic disorder because of late age at onset, atypical symptomatology, or subthreshold symptomology (i.e., fewer than six items), or because of all three reasons.
From American Psychiatric Association: Diagnostic and statistical manual of mental disorders, ed 4, text revision (DSM-IV-TR), Washington, DC, 2000, The Association.
The exact cause of autism is unknown. Some studies show abnormal brain development beginning in the first months of life. In spite of popular belief, there is no scientific evidence to date that immunizations (e.g., measles, mumps, and rubella vaccine) cause autism. The co-occurrence of seizure disorders and some degree of mental retardation is common.
INCIDENCE
1. One in every 500 children has some form of pervasive developmental disorder (PDD).
2. Prevalence rate of autistic disorder is approximately 2 to 5 in 10,000 births.
3. Asperger’s disorder is more common in males.
4. Prevalence rate of Asperger’s disorder is not established.
4. Prevalence rate of Rett syndrome is 1 in 10,000 to 15, 000 births.
CLINICAL MANIFESTATIONS
A diagnosis of an ASD is made based on behavioral symptoms. Possible indicators of ASDs are as follows:
Communication
1. Does not babble, point, or make meaningful gestures by 1 year of age
2. Does not speak one word by 16 months
3. Does not combine 2 words by 2 years
5. Loses language or social skills
6. May remain mute throughout life
7. May use language in unusual ways
8. Repeats certain phrases over and over
10. May have large vocabulary but difficulty sustaining conversation
11. Has difficulty understanding body language and tone of voice
12. Tone of voice and body language may not reflect what person is saying
Social Symptoms
1. Appears indifferent to others and prefers being alone
2. May resist or passively accept hugs and cuddling
3. Seldom seeks comfort (at older age)
4. Slow to learn to interpret what others are thinking and feeling
5. Misses subtle social cues (e.g., grimace or wink)
6. Difficulty regulating emotions (i.e., may be disruptive and aggressive, cries easily or inappropriately, has verbal outbursts)
Repetitive, Stereotypical Behaviors
1. Exhibits odd repetitive motions (e.g., flapping arms, walking on toes)
2. Suddenly freezes in position
3. Spends hours lining up toys
5. Becomes distressed with change in routine (e.g., times for dressing, taking a bath, going to school)
6. May focus intently on one thing (e.g., learning about vacuum cleaners, train schedules, numbers, symbols, or science topics)
COMPLICATIONS
Autism is not associated with complications; however, the child with autism may have secondary diagnoses such as depression, obsessive-compulsive disorders, and anxiety.
LABORATORY AND DIAGNOSTIC TESTS
1. Developmental assessments—Ages and Stages Questionnaire (ASQ), Greenspan Social-Emotional Growth Chart, Temperament and Atypical Behavior Scale (TABS).
2. Screening tools specific to autism.
3. Parent or caregiver interviews may include semistructured format or structured formats such as ADI-R.
4. Child observations in various settings and times are conducted in the home, the school, and other community settings familiar to the child (ADOS).
6. Assessment of adaptive functioning.
7. Assessment of cognitive functioning.
8. Assessment of verbal and nonverbal language—SCQ, Peabody Picture Vocabulary Test-Revised (PPVT-R).
MEDICAL MANAGEMENT
Many behavioral programs exist to treat ASDs, applied behavioral analysis (ABA) being the most widely accepted. The objective of any good program is to reduce inappropriate behavior and increase communication and appropriate social behaviors. Behavioral plans should be structured and individualized. Parental involvement is essential. In some cases, medication is used. Antipsychotic medications are prescribed for severe behavioral problems. Medications are also used to treat co-occurring symptoms of anxiety, depression, and obsessive-compulsive disorders. Many medications are currently used “off label” (not approved by the United States Food and Drug Administration [FDA] for use in children), but psychopharmacologic studies are underway.
NURSING DIAGNOSES
NURSING INTERVENTIONS
Interventions for Inpatient Treatments, Hospitalizations, or Outpatient Visits
1. Provide quiet, structured environment.
2. Take vital signs with sense of calmness, with as little distraction as possible.
3. Give one instruction at a time.
4. Tell child or youth what to expect, and use simple explanations.
6. Have child or youth visit same clinician on each outpatient medical appointment.
7. Use same routine each visit.
8. Remain composed even when child or youth is screaming.
9. Encourage parents to be present.
Infants, Toddlers, and Preschoolers
1. Refer to early intervention program for development of individualized family service plan (IFSP) and interdisciplinary treatment plan; or, if child, refer to preschool program for individualized education plan (IEP) (see Appendix G) that provides opportunities for developmental learning (Appendix B):
2. Refer parents and caregivers to family resource centers and/or parent information centers that provide early intervention services to parents of infants and toddlers with disabilities for parental support and assistance with informational needs and respite services.
3. Collaborate with other interdisciplinary professionals to formulate IFSP and/or IEP that is based upon individual needs, is family-centered, has measurable objectives, and includes periodic evaluations.
4. Serve as health resource consultant to community service coordinator.
5. Assist family in navigating service systems to obtain needed services for child and family.
6. Refer to the Discharge Planning and Home Care section in this chapter.
School-Age Children
1. Collaborate with other interdisciplinary professionals to formulate IEP that is based upon individual needs, is individual- and family-centered, has measurable objectives, and includes periodic evaluations.
2. Collaborate with IEP team on identification of health-related needs and development of IEP objectives.
3. Assist family in navigating service systems to obtain needed services for child and family.
4. Refer to the Discharge Planning and Home Care section in this chapter.
Adolescents
1. Collaborate with other interdisciplinary professionals to formulate transition IEP that is based upon individual needs, is youth-centered, has measurable objectives, and includes periodic evaluations.
2. Collaborate with IEP team on identification of health-related transition needs and development of IEP objectives.
3. Provide input on transition plan related to health-related needs.
4. Assist family in navigating service systems to obtain needed services for child and family.
5. Refer to the Discharge Planning and Home Care section in this chapter.
Discharge Planning and Home Care
1. Instruct parents, family members, and child or youth and reinforce information about the behavioral and speech and language manifestations (social difficulties, speech and language limitations, characteristic repetitive and stereotypic behaviors), and long-term outcomes and prognosis of the autism diagnosis.
2. Educate parents, family members, and child or youth about long-term management strategies and community resources needed to access services (refer to Appendix G).
3. Refer families to early intervention programs to address child’s needs for treatment services.
4. Participate as a member of an interdisciplinary team to develop plan of services to address family-centered goals and objectives based on child’s individual needs.
CLIENT OUTCOMES
1. Child will be diagnosed early, enabling participation in early treatment and intervention.
2. Child or youth will achieve highest potential of biopsychosocial functioning.
3. Child or youth will achieve highest possible level of self-sufficiency.
4. Child or youth will demonstrate highest achievable level of autonomy, self-determination, and self-advocacy.
5. Family will demonstrate ability to cope with child’s or youth’s behaviors and needs and to access needed services.
6. Parents will demonstrate attachment and responsive parenting behaviors.
7. Parents will demonstrate ability to accept child’s limitations and recognize child’s strengths.
American Psychiatric Association. Diagnostic and statistical manual of mental disorders, ed 4. Washington, DC: The Association, 2000. text revision (DSM-IV-TR),
Chez MG, Chin K, Hung PC. Immunizations, immunology and autism. Semin Pediatr Neurol. 2004;11(3):214.
Masi G. Pharmacotherapy of pervasive developmental disorders in children and adolescents. CNS Drugs. 2004;18(14):1031.
National Institute of Mental Health. Autism spectrum disorders (pervasive developmental disorders), 2006. www.nimh.nih.gov/publicat/autism.cfm Accessed December 15, (website):
Souders MC, et al. Caring for children and adolescents with autism who require challenging procedures. Pediatr Nurs. 2002;28(6):555.
Sparks SM, Taylor CM. Nursing diagnosis reference manual, ed 6. Philadelphia: Lippincott, Williams & Wilkins, 2005.
Spence SJ, Sharifi P, Wiznitzer M. Autism spectrum disorder: Screening, diagnosis, and medical evaluation. Semin Pediatr Neurol. 2004;11(3):186.
Vastag B. Autism interventions come of age. JAMA. 2004;291(23):2807.
