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1COGNITIVE ALTERATIONS
Suzanne Sutherland, PhD, RN
Nursing 138/139
February 26, 2015
GEORGE FRIDERIC HANDELhttp://www.youtube.com/watch?v=MS3vpAWW2Zc
For unto us a child is born
Unto us a son is given
Unto us a son is given
For unto us a child is born
And the government shall be upon his shoulders
Isaiah 9:6
OBJECTIVES
After completion of this segment, the student will be able to
Explain the distinction between developmental delay and intellectual disability (cognitive impairment).
Describe the characteristics of a child with intellectual disability.
Explain why identification of cognitive impairment is important for optimal development.
Discuss the familys reaction to having a child with a cognitive alteration and the anticipated grieving process.
Describe the appearance and behaviors of a child with Down syndrome, a child with fragile X syndrome, and a child with autism.
Discuss the rise in diagnosed autism spectrum disorders and formulate an opinion of why this rise has occurred.
Describe the various roles of the nurse in caring for the family of a child with cognitive impairment.
COGNITIVE IMPAIRMENT
Permanent condition
Also called intellectual disability
Involves impairments of general mental abilities that affect adaptive functioning in three domains, or areas: conceptual, social, and practical.
Schools identify 1% to 2% of children
Symptoms beginning during the developmental period
Diagnosis based on severity of deficits in adaptive functioning
WHY DIAGNOSE, MEASURE,
AND TREAT?
The nature and severity of a specific
impairment represent a given set of facts,
embodying the raw material with which the
family must work in order to craft an adult
who can be, at least to a degree,
communicative and self-sustaining.
Constants: Syndromes, IQ, physical limitations
Variables: Environments of home and school
DEVELOPMENTAL DELAY
Term used when intellectual impairment may or may not be present
Not meeting a developmental norm on time
Manifested before age 18
Need for a combination of services
Attributable to mental impairment, physical impairment, or both
Incidence increasing why?
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2PHYSICAL DELAY
Diagnosis of cerebral palsy
Affected children may have pronounced difficulty with motor skills
but unimpaired intelligence
http://www.youtube.com/watch?v=oNW7avgkLBo&feature=related 1:30 2:00 Alex and Frederic Bilodeau
COGNITIVE IMPAIRMENT
Permanency: Unalterable
Treatment modalities at home:
stability, consistency, enrichment
Treatment modalities at school:
education, training
THE VALUE PLACED ON
THE CHILD WITH
COGNITIVE IMPAIRMENT
The family
The educational system
Society
The person with cognitive impairment
The nurse we are socially appropriate and we respond to children positively
FACTS
Incidence about 2.5%
(under-identification in schools)
Etiology - genetic, prenatal, perinatal, or postnatal
Pathophysiology syndromic versus structural
PREVENTING INTELLECTUAL
DISABILITY
Of the causes of intellectual disability,
which are most preventable?A. Prenatal
B. Genetic
C. Perinatal
D. Postnatal
NEUROPLASTICITY
Cells in one area assume functions of a damaged or absent area (age-limited)
Cells reroute signals around nonfunctioning cells (all ages)
The sooner the rerouting occurs, the better the outcome should be (the
argument for prompt intervention)
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3CLINICAL MANIFESTATIONS
Mild: cognitive delays
Moderate: developmental delays; delays in motor development, speech
Severe: little speech; pronounced delays in motor development, speech
Profound: delays across all areas; often identifiable syndromes
SIBLINGS
At first unaware
Subsequently protective
In teen years, often both embarrassed and guilty, as well as protective
DIAGNOSIS
Thorough physical examination and history (Is this child physically within normal range?)
Routine developmental assessment (Is a delay present?)
Standardized tests of intellectual and adaptive functioning (How far from average are the childs cognitive functioning
and behavior?)
DEVELOPMENTAL
ASSESSMENT TESTS
Denver Developmental Screening Test II (Denver II)
Vineland Adaptive Behavior Scale
AAMRs Adaptive Behavior Scale
These are NOT IQ tests
- rule out, not in
- results vary if child is ill, tired
- must be repeated if abnormal
MEASUREMENT TESTS
Determination whether intellectual disability exists - Bayley, Wechsler Preschool and Primary, Peabody
Quantification Stanford-Binet (100 is normal)
Bayley, Wechsler, Peabody, Leiter also yield normed 100-based scores
Tests of Adaptive Behavior
Scores on many tests of IQ and adaptive
behavior produce scores that are
normed to 100. What two things does this imply if the curve is normal?
A. More people score 100 on the test than any other
number.
B. If a persons score isnt 100, (s)he isnt normal.
C. Any large randomly obtained group (50 or more)
should have an average score of about 100.
D. Having a test score less than 100 is abnormal.
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4TREATMENT
Health maintenance
Early intervention for better adaptation
Education keyed to childs abilities and needs (strength-needs assessment)
Extra effort/work for everyone
Infant stimulation, preschool programs, high teacher-to-student ratios, work
training in high school
THE FAMILY ENVIRONMENT
The family as therapist
The family as comfort
School subjects versus enrichment
PARENTAL COUNSELING
What is the general purpose of counseling for parents of children with intellectual disability?
A. It teaches parents things they need to know to maximize childrens outcomes.
B. It educates parents about ways that they can prevent intellectual disability in subsequent children.
C. It allows parents to recognize their feelings and assures them that those feelings are normal.
D. It teaches parents how to grieve effectively, so as to achieve resolution.
NURSING INTERVENTIONS
Respect for the person
Interpreter
Emotional support
First-line assessment pathology versus style
Essential versus optional information
Modeler of behavior
The Impact of Childhood Disability:
The Parent's Struggle
by Ken Moses, Ph.D.
Ken Moses is a psychologist originally
from the East coast, who has practiced
in Illinois for many years.
He has lectured throughout the United
States on the parents experience of having a child with a major disability.
http://www.pent.ca.gov/beh/dis/parentstruggle_DK.pdf
PARENTAL GRIEVING PROCESSKen Moses
Denial denial of reality or of one of its aspects; denial of the importance of the reality in ones childs life or in ones own
Anxiety a state of chronic uneasiness, often accompanied by physiological symptoms such as hyperventilation, nervousness, or hyperactivity
Fear a state of dread, apprehension, or trepidation related to the future
Depression overwhelming, heavy, deep sadness or sorrow; the inability to see a clear and positive future
Guilt the taking upon oneself of responsibility for a past event
Anger rage, indignation, or hostility, as a result of pain and injury
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5GRIEVING PROCESS
Parents may be able to self-identify all of their stages of grieving, except
______
Different items, same family varying stages regarding each
A feeling process, not a cognitive one
Life may be a series of concentric eggs, or nested items.
EXAMPLE OF NESTED ITEMS
Nested Dolls
DENIAL
Denial of reality or of one of its aspects; denial of the importance of the
reality in ones childs life or in ones own
This is too overwhelming for me.
Function: ??
Interventions: ??
What not to say: Youre in denial!
ANXIETY
A state of chronic uneasiness, often accompanied by physiological
symptoms such as hyperventilation,
nervousness, or hyperactivity
This is too large for me.
Function: ??
Interventions: ??
What not to say: Calm down!
FEAR
A state of dread, apprehension, or trepidation related to the future
Things can happen to those about whom I care.
Function: focuses on reality, prioritization
Manifestations: overuse of healthcare system, hypervigilance, overprotection
Interventions: ??
What not to say: Dont be afraid.
DEPRESSION
Overwhelming, heavy, deep sadness or sorrow; the inability to see a clear and positive future
Functions: a silent request for someone to listen, to be with
Being with is the essence of good psychiatry.
Interventions: ??
What not to say: Cheer up. Its not as bad as it could be.
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6GUILT
Taking upon oneself the responsibility for a past event
Manifestations: searching for causes, inability to get on with it
This has a cause.
Functions: ?? (antidote to what?)
Interventions: ??
What not to say: Its not your fault.
ANGER
Angerrage, indignation, or hostility, born of pain and injury
Manifestations: emotion out of proportion to the circumstances
This isnt fair. Why me?
Function: ?? (antidote to what?)
Interventions: ??
What not to say: Youre being unreasonable. Dont be so angry.
SUPPORT FOR FAMILIES
Acceptance of the child Mrs. Anderson
Truth in small bites
Feeling-focused interactions
Denial and teaching
Good decisions begin with good facts.
OUT-OF-HOME PLACEMENT
At birth, used to be the norm
Now later-occurring
Selection of placements age-dependent
Inevitability
SPECIAL OLYMPICS
Let me win. But if I cannot win, let me be brave in the attempt.
DOWN SYNDROME
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7DOWN SYNDROME
Incidence: 1 in 660 to 800 live births
Relative risk: 1 in 1500 when mother is 30; 1 in 100 when she is 40
BUT more common in mothers 35 or younger
Detection/screening: DNA blood test, then amniocentesis or chorionic villus sampling
Diagnosis: kayotyping Variant: mosaicism
CLINICAL MANIFESTATIONS
upward-slanting eyes
epicanthic folds
small nose
medium to large tongue with small to medium mouth
high arched palate
muscle weakness, hyperflexibility, and hypotonia
square hands with short fifth finger
transverse palmar crease (simian crease)
wide space between first two toes
extreme placidity
DEFINITIVE DIAGNOSIS
Down syndrome is definitively diagnosed
by which of the following?
a. The quadruple screen
b. Amniocentesis
c. Karyotyping
d. Presence of eight or more clinical features
THE PERSON
HEALTH CONCERNS
Heart defects (especially septal) 4 in 10
Otitis media
Increased incidence of upper respiratory illnesses
Gastrointestinal abnormalities
Hypothyroidism
Ocular cataract
Atlantoaxial instability
Down-associated Alzheimers
WELLNESS MAINTENANCE
Down syndrome growth charts
Immunizations
Infant stimulation programs
Socialization
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8NEONATAL MANAGEMENT
Before 1960
The do not feed order
Not restricted to Down Syndrome
Sign on babys crib
Implications
Today
Neonatal screening for hearing
Echocardiogram
Family assessment
Tentative diagnosis
Order for karyotyping
TRENDS
1950s
Life expectancy about 9 years
IQ profoundly intellectually disabled
Not raised at home
School not available for institutionalized or
group home children
21st Century
Life expectancy 50 to 58 years
IQ moderately intellectually disabled
Raised at home
Schooling available for all
DOWN SYNDROME
RADIOGRAPHIC EXAMINATION
Why is periodic radiographic examination of
the neck essential for childrens health in Down syndrome?
A. Webbing can be minimized through treatment
B. Adenoidal hypertrophy can obstruct the airway
C. Chronic sinusitis affects hearing and speech
D. Atlantoaxial stabilization can prevent quadriplegia
THE AUTISM SPECTRUM
DISORDERS
AUTISM
Primarily a disorder of both social
interaction and social communication
Manifested by altered behavior patterns
and social interactions, and often by
impaired communication with humans
Probably a combination of genetics and
environment
DSM V Two Related Subtypes Social Communication Disorder Persistent difficulties in the social use of verbal and nonverbal
communication.
The deficits result in functional limitations in effective communication, social participation, social relationships, academic achievement, or occupational performance, individually or in combination.
Onset of the symptoms is in the early developmental period.
The symptoms are not attributable to another medical or neurological condition or to low abilities in the domains or word structure and grammar, and are not better explained by autism spectrum disorder, intellectual disability, global developmental delay, or another mental disorder.
Autism Spectrum Disorder Persistent deficits in social communication and social interaction
Restricted, repetitive patterns of behavior, interests, or activities
Symptoms must be present in the early developmental period
Symptoms cause clinically significant impairment in social, occupational, or other important areas of current functioning
These disturbances are not better explained by intellectual disability (intellectual developmental disorder) or global developmental delay
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9However Addendum
Individuals with a well-established DSM-IV
diagnosis of autistic disorder, Aspergers disorder,
or pervasive developmental disorder not otherwise
specified should be given the diagnosis of autism
spectrum disorder. Individuals who have marked
deficits in social communication, but whose
symptoms do not otherwise meet criteria for
autism spectrum disorder, should be evaluated for
social (pragmatic) communication disorder.
TEMPLE GRANDIN
Herself
UC Davis series
http://www.youtube.com/watch?v=2wt1IY3ffoU
Claire Danes
http://www.youtube.com/watch?v=vwJc6HkP8fc
INCIDENCE
Incidences of all forms of autism increasing rapidly all forms combined now as high as 10 per 1000 (1%)
True autism about 1.3 to 2 per 1000
Aspergers syndrome 2 in 10,000
DISTRIBUTION OF AUTISM
Like schizophrenia, 4 boys to 1 girl Assuming 1% (1 in 100) are affected, why
is the occurrence rate for males 1.6%?
Females are NOT necessarily more severely affected (textbook error).
Incidence varies by area
Artifactual versus real increase
Monochorionic versus dichorionic twins
NATURE VERSUS NURTURE
Nature (genetic)
Nurture (environmental)
Pre-natal environment (G + E)
QUESTION no answer
If there is such a thing as G + E, in a single-gene scenario if the gene emanates from the
mother rather than the father, and a
substance is produced that is trans-
placental, what should be the effect?
All infants will be more severely affected.
The number of infants affected will be greater.
Both.
Neither.
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10
SECOND QUESTION no answer
Assume that the incidence of autism is the same in males and females, that the root causation is purely genetic, but that something causes girls not to manifest the severe symptomatology as frequently. What are the most likely candidates for this something? (Consider hormones, socialization, birth trauma, and whatever else seems plausible.)
http://adultautism.us/free-autism-screening-tool/autism-spectrum-quotient-test-for-adults-ages-16-and-over/
GENDER DISTRIBUTION
Whiteley et al. (2010), England and Ireland
Increasing over-representation of males
Overall male:female ratio 7.38:1
ASD/NOS 6.84:1
Autism 6.54:1
Aspergers 12.07:1
So, based on 7.38:1, if incidence is 1 in 100, this means the chance for a girl is 0.24% but
for a boy is 1.76%
ASSOCIATED SYNDROMES
Fragile X about 1/3 of children with Fragile X are also autistic
Rett Syndrome gene mutation, behaviors similar to those of autism
Tourettes some overlap but controversial
CAUSES
Not childhood schizophrenia
Not maltreatment
Not refrigerator mothers (late 60s)
Not a single-gene defect
Seizures - 1/3 to 3/4 of autistic children
Complex genetics with environmental trigger
Trigger likely to be neuroactive
PROPOSED TRIGGERS
MMR (thiomersol) (refuted)
Immune stress
Prenatal virus, ?prion?
Pesticides, pollutants
Animal hormones
Something hormonal (4:1 or greater ratio)
Stress response during delivery
Congenital rubella or influenza
Maternal smoking
I THINK.
Pre-existent tendency
An incident like hypoxia
Or combined insults
Or surgery/pain
Mothers are more stressed as the family size increases (because they
know more). This may account for the
not first child finding.
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11
PROPOSED GENETICS
Single-gene (refuted)
Three to ten genes working in concert
Up to 100 genes, co-relating
On the X chromosome or not
Fetal testosterone levels (both genders) (Baron-Cohen)
Different for different subtypes (UCD)
Look at the sisters and mothers!
USUAL EARLY FINDINGS
Early manifesters
Sleep disorder
Auditory hypersensitivity
Food aversion
Swallowing disorder
Hyperactivity
Impaired eye contact
Late manifesters
Apparently normal development until about 12 months
Loss of language
Auditory disregard
No pretend play
Food preferences
No voluntary interaction
IDENTIFICATION
Usually by parents
Confirmed by pediatricians
M-CHAT (Modified Checklist for Autism in Toddlers)
http://www.autismspeaks.org/what-
autism/diagnosis/mchat
AUTISM CAUSE
What is the probable cause of autism?
A. Aloof, refrigerator mothers who cannot relate to their children
B. A single recessive gene
C. An interaction between genetics and
environment
D. Reaction to the MMR vaccination
LATER BEHAVIORS
Poor communication
Unusual forms of play
Bizarre body movements
Repetitive actions
Withdrawal
Absent eye contact
Gastric reflux, food intolerances
AND
Unusual hand movements
Sensitivity to tactile stimulation
Altered responses to pain
A resistance to usual teaching methods
Solitary activities (repeated, apparently meaningless activities that the child finds essentially important and seems compelled to repeat in sequence)
Key objects or toys
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12
AND
Obsessive behaviors concerning eating, dressing, moving, and the physical
environment, including the people in it
Stiff gestures
Awkward gait
Waving or flipping the hands back and forth. If repeated, these body movements are often
accompanied by repetitious sounds.
AND
Rocking, moaning, rubbing head
Seeking contact with the hands or head by slapping an object or banging the
head into it, or as extreme delight in
touching or holding certain objects
Frustration: striking self on head
https://www.youtube.com/watch?v=uPgYlG83vlY
AUTISTIC BEHAVIORS
On a developmental assessment, in
which areas would you expect to
see delays in an autistic child?
A. Personal-social
B. Fine motor
C. Gross motor
D. Language
PROTODECLARATIVE POINTING
In protodeclarative pointing, children point to objects they want, or wish to
see more closely. It is a request.
Instead, an autistic child will often put one hand on an adults wrist or forearm and advance it toward a desired object
to signal wanting that object, in a kind
of proxy reach.
AUTISTIC BEHAVIORS
Which behavior is NOT commonly found
in autistic children?
A. Savant or unusual aptitude
B. Communication impairment
C. Abnormal social skill development
D. Preference for sameness
THE GOLDEN TICKET
Lovaas small sample Early Intensive Behavioral Intervention
High teacher-to-student ratio
20 to 25 hours per week
Parental inclusion
Two year duration
Randomly-assigned experiments support the treatment
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13
EFFECTIVENESS?
All children showed some improvement
Most dramatic improvement in younger children
Some studies recommend more hours, which seem to be associated with
better outcomes
NURSE INTERVENTIONS
Routines are essential
Parents are essential
How do you give him meds?
No fear of danger
Support parental treatments if there appears to be no harm diet modifications, probiotics, secretin
hormone
MORE
Routine healthcare
Caregiver respite
Support during the
grieving process
Sibling wellness
AUTISM EARLY DIAGNOSIS
Why is early identification of autism
important?
a. Parents of autistic children need support
b. Early intervention fosters better outcomes
c. Prevention of subsequent pregnancies is
essential
d. Cure is possible only if diagnosed by 18
months
FRAGILE X SYNDROME
Almost exclusively boys affected severely
Incidence varies: .25 to .8 per 1000
Information on Internet and in text misleading (male carriers, females affected)
Female carriers
Nucleic acid repeat on X chromosome
Repeat more than 200 times is the syndrome, less than 200 times is the carrier state.
Randi Hagerman @ MIND Institute
Fragile X Manifestations
Moderate to severe intellectual disability
Large ears
Cupping of the ears
Velvet-like skin
Slim build
Broad and somewhat squinting eyes
Hyperextensible joints
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14
FRAGILE X SYNDROME
Fragile X syndrome often co-exists with
A. Autism
B. Fetal alcohol syndrome
C. Down syndrome
D. Childhood schizophrenia
PREVALENCE
Which of the following has the highest
prevalence among children?
A. Down syndrome
B. Intellectual disability
C. Fragile X syndrome
D. The autism spectrum disorders
LET ME SUM UP. Two to three percent of the human population has a degree of
intellectual disability.
Siblings of children with intellectually disability may have to expend extra patience and assume added responsibility. This could affect their lives positively, adversely, or both.
Caregivers and siblings of children with intellectual disability undergo a grieving process related to their separation from a significant dream.
Down syndrome is the most frequent and most widely recognized chromosome disorder of humans.
Autism is a developmental disorder, characterized by extreme difficulty communicating with and relating to the environment, and manifested by bizarre behavior, delayed language acquisition, poor social relations, impairment of self-care skills, and altered sensory responses.
Because of behavior disturbances and disordered sleep patterns, caregivers of autistic children experience substantial stress.
LAST THOUGHTS
The nurse is extremely powerful for acceptance and better treatment of persons
with cognitive delay, both within AND
OUTSIDE the workplace. Watch verbiage.
It is a privilege to care for someone who needs you. This is often the point of view of the parent of a child with cognitive
alterations. Pity is inappropriate. Share the
parents joy in the child.
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