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?

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)

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.

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

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.

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

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

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

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.

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.

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

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

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

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.