GEORGE FRIDERIC HANDEL

http://www.youtube.com/watch?v=MS3vpAWW2Zc

COGNITIVE ALTERATIONS

Suzanne Sutherland, PhD, RN

Nursing 138/139
February 26, 2015

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

COGNITIVE IMPAIRMENT

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.

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?

DEVELOPMENTAL DELAY

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

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?

PHYSICAL DELAY
Diagnosis of cerebral palsy
Affected children may have
pronounced difficulty with motor skills
but unimpaired intelligence
http://www.youtube.com/watch?v=oNW
7avgkLBo&feature=related 1:30 2:00
Alex and Frederic Bilodeau

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

PREVENTING INTELLECTUAL
DISABILITY
Of the causes of intellectual disability,
which are most preventable?
A.
B.
C.
D.

Prenatal
Genetic
Perinatal
Postnatal

COGNITIVE IMPAIRMENT
Permanency: Unalterable
Treatment modalities at home:
stability, consistency, enrichment

Treatment modalities at school:

education, training

FACTS
Incidence about 2.5%
(under-identification in schools)
Etiology - genetic, prenatal, perinatal,
or postnatal
Pathophysiology syndromic versus
structural

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)

CLINICAL 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

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?)

MEASUREMENT TESTS

SIBLINGS
At first unaware
Subsequently protective
In teen years, often both embarrassed
and guilty, as well as protective

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

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?

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

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.

TREATMENT
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.

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/parentstrug
gle_DK.pdf

NURSING INTERVENTIONS

Respect for the person

Interpreter
Emotional support
First-line assessment pathology
versus style
Essential versus optional information
Modeler of behavior

PARENTAL GRIEVING PROCESS

Ken 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

GRIEVING PROCESS

EXAMPLE OF NESTED ITEMS

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.

Nested Dolls

DENIAL

ANXIETY

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!

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

DEPRESSION

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.

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.

GUILT
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.

SPECIAL OLYMPICS

OUT-OF-HOME PLACEMENT

At birth, used to be the norm

Now later-occurring
Selection of placements age-dependent
Inevitability

DOWN SYNDROME

Let me win. But if I cannot win, let me

be brave in the attempt.

DOWN 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

DEFINITIVE DIAGNOSIS

muscle weakness,
hyperflexibility, and
hypotonia
square hands with short
fifth finger
transverse palmar
crease (simian crease)
wide space between
first two toes
extreme placidity

THE PERSON

Down syndrome is definitively diagnosed

by which of the following?
a.
b.
c.
d.

The quadruple screen

Amniocentesis
Karyotyping
Presence of eight or more clinical features

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

NEONATAL 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

THE AUTISM SPECTRUM

DISORDERS

DOWN SYNDROME
RADIOGRAPHIC EXAMINATION
Why is periodic radiographic examination of
the neck essential for childrens health in
Down syndrome?
A.
B.
C.
D.

Webbing can be minimized through treatment

Adenoidal hypertrophy can obstruct the airway
Chronic sinusitis affects hearing and speech
Atlantoaxial stabilization can prevent quadriplegia

AUTISM

DSM V Two Related Subtypes

Social Communication Disorder

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

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

However Addendum

TEMPLE GRANDIN

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.

Herself
UC Davis series
http://www.youtube.com/watch?v=2wt1I
Y3ffoU
Claire Danes
http://www.youtube.com/watch?v=vwJc
6HkP8fc

INCIDENCE

DISTRIBUTION OF AUTISM

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

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

QUESTION no answer

NATURE VERSUS NURTURE

Nature (genetic)
Nurture (environmental)
Pre-natal environment (G + E)

If there is such a thing as G + E, in a singlegene scenario if the gene emanates from the
mother rather than the father, and a
substance is produced that is transplacental, what should be the effect?

All infants will be more severely affected.

The number of infants affected will be greater.
Both.
Neither.

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-screeningtool/autism-spectrum-quotient-test-for-adultsages-16-and-over/

GENDER DISTRIBUTION

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

I THINK.

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

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%

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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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

IDENTIFICATION
Usually by parents
Confirmed by pediatricians
M-CHAT (Modified Checklist for
Autism in Toddlers)
http://www.autismspeaks.org/whatautism/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

Late manifesters
Apparently normal
development until
about 12 months
Loss of language
Auditory disregard
No pretend play
Food preferences
No voluntary
interaction

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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AND

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.

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=uPg
YlG83vlY

AUTISTIC BEHAVIORS

PROTODECLARATIVE POINTING

On a developmental assessment, in
which areas would you expect to
see delays in an autistic child?
A.
B.
C.
D.

Personal-social
Fine motor
Gross motor
Language

AUTISTIC BEHAVIORS
Which behavior is NOT commonly found
in autistic children?
A.
B.
C.
D.

Savant or unusual aptitude

Communication impairment
Abnormal social skill development
Preference for sameness

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.

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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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

MORE
Routine healthcare
Caregiver respite
Support during the
grieving process
Sibling wellness

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

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

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 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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FRAGILE X SYNDROME
Fragile X syndrome often co-exists with
A.
B.
C.
D.

Autism
Fetal alcohol syndrome
Down syndrome
Childhood schizophrenia

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.

PREVALENCE
Which of the following has the highest
prevalence among children?
A.
B.
C.
D.

Down syndrome
Intellectual disability
Fragile X syndrome
The autism spectrum disorders

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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