Chapter 5 Intellectual and Developmental Disabilities

Chapter 5: Intellectual and Developmental Disabilities

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Topics • Definition• Prevalence• Causes• Identification• Psychological and behavioral characteristics• Educational considerations• Assessment of progress• Early intervention• Transition to adulthood

What’s in a name?

• Idiot, moron, imbecile

• Feeble-minded

• Mental retardation– Still the classification used in Pennsylvania

today

• Intellectual and developmental disabilities

Definition

Reasons for caution Concern about misdiagnosis of ethnic

minorities Possible stigma associated with the

diagnosis Belief that intellectual disability is a socially

constructed condition

2002 AAIDD definition

• Mental retardation is a disability characterized by significant limitations both in intellectual functioning and in adaptive behavior as expressed in conceptual, social, and practical adaptive skills. This disability originates before age 18.– IQ less than ____/75– Need to have both low IQ and limited

adaptive behavior

5 Assumptions of the AAIDD Definition

• Must consider context, age, and community• Assessment must be valid and consider

culture; language; sensory, motor, and behavioral issues; and differences in communication

• Individuals have both limitations and strengths

• Should develop profile of needed supports• Life functioning can improve over time with

use of appropriate supports

Supports• Supports

– Resources and strategies that promote development, education, interests, and well being

• Levels of support (1992 AAMR)

– Intermittent—as _________– Limited—daily or weekly for _____ period of time– Extensive—daily or weekly for _____ period of time– Pervasive—hourly or __________

• Types of support– Natural– Service-based– Technological

Classification of Mental Retardation(American Psychological Association)

IQ Score

Mild 50-70

Moderate 35-50

Severe 20-35

Profound 00-20

2002 Pennsylvania Definition

• Mental retardation--"significantly subaverage intellectual functioning that is accompanied by significant limitations in adaptive functioning in at least two of the following areas: Communcation, self-care, home living, social/interpersonal skills, use of community resources, self-direction, functional academic skills, work, leisure, health, and safety. The onset must occur before the individual’s 22nd birthday.”

(§4210.101a)

IQ below 70

Types of Support in PA• Learning

– “Services for students with a disability who require services primarily in the areas of ___________, ___________, __________, or speaking or listening skills related to academic performance”

• Life skills– "Services for students with a disability who

require service primarily in the areas of academic, functional or vocational skills necessary for __________ living.”

Prevalence of Intellectual Impairments

Theoretical prevalence of intellectual impairmentbased on normal curve = 2.27%

Prevalence of Intellectual Impairments

• Actual prevalence =

• Discrepancy between theoretical and actual prevalence may be due to:– consideration of adaptive behavior– classification as learning disabled for

those with higher IQs– classification as autistic

1%1%

Prevalence of People with Mental Retardation by Classification

Profound 2%

Moderate10%

Severe3%

Mild85%

Causes• Prenatal causes (before ______)

Chromosomal disorders• Down syndrome• Williams syndrome• Fragile X syndrome• Prader-Willi syndrome• Inborn errors of metabolism

• PKU

• Developmental disorders of brain formation• Microcephalus• Hydrocephalus

• Environmental influences• Fetal alcohol spectrum disorders• Rubella

Down Syndrome

Three typesTrisomy 21MosaicismTranslocation

Trisomy 21Down Syndrome

Trisomy 21 47 chromosomes in all

cells due to trisomy of 21st chromosome

92% of all cases of Down Syndrome

Related to increased parental age

Maternal age and Trisomy 21 Down Syndrome

Characteristics ofDown Syndrome

Physical Characteristics Short stature Obesity Hyperflexibility of joints Hypotonicity (low _____ _______)


Distinctive facial features Folds of skin around eyes Small ears with very small lobes Flat appearance to face; small nose

with small bridge Protruding tongue due to:

small mouth short roof of mouth low muscle tone in tongue

Sparse, fine hair


Physical Characteristics• Distinct hands and feet

– simian crease across palms– small hands with short stubby

fingers – little finger curves in instead of

being straight– short stubby toes with wide

space between big and 2nd toe


Health Characteristics Myopia ( ____ sightedness) and/or strabismus ( _____ eyes) in 60% Heart defects in 50% Respiratory problems Higher rate of leukemia Higher rate of Alzheimer's Conductive hearing loss in 66-89% Hypothyroidism in 50-90% Eczema and/or dry skin in 50% Generally sterile Shorter life span

50 years average life expectancy


Cognitive characteristics Better on visual-spatial tasks than verbal-auditory tasks Girls are about 5-10 IQ points higher than boys Declining IQ as person ages

highest IQs in infancy and early childhood development plateaus in middle childhood most adults have moderate MR deterioration of adaptive skills beginning at 40

Language characteristics Grammar is akin to that of 3 year old Receptive language is better than expressive language 90% have articulation problems May see more gesturing than actual language use


Sociobehavioral characteristics Generally are very sociable and friendly

outgoing likely to use sociability to get out

of difficult tasks may do better in inclusive settings may prefer playing with younger

children 75% described as stubborn

increased depression with age

Williams Syndrome• Caused by microdeletion

of the 7th chromosome (7q11.2)

• Characteristics:– mild mental retardation in

75%– attention deficit disorder

and hyperactivity– overly friendly with adults

• incessant chatter• inappropriate and

repetitive speech– excessive anxiety & worry

7q11.2

Williams Syndrome

• Characteristics:– strengths in auditory rote

memory and language– extreme weakness in

perceptual and motor – auditory and textile

hypersensitivity– high incidence of absolute

pitch– cardiovascular disease

• narrowing of the main arteries leading from the heart

Middle two rows are copied spatial designs (top row) by two 11-year-old children with Williams Syndrome. Their copies distort spatial properties while preserving other design aspects, such as color. Bottom row drawings are by a six-year-old child without Williams syndrome.

Williams Syndrome

• Characteristics:– connective tissue abnormalities

• joint limitation or laxity, and soft, lax skin

• hoarse voice

• bowel/bladder diverticulae and rectal prolapse

– delayed rate of growth, including low stature and a slight build

– distinctive facial characteristics

Characteristics of Williams Syndrome

• “________- like facial features

– broad brow– short nose, full nasal

tip,– long philtrum– full lips – wide mouth– small jaw– prominent earlobes

Fragile X

Trinucleotides on the X chromosome expand from the usual 6-50 repetitions to morethan 200 repetitions

Fragile X symptoms become apparent @ 200 repeats;

The more repetitions, the more symptoms we see

•aka Martin-Bell Syndrome or Marker X syndrome•caused by a trinucleotide repeat expansion of the FMR1 gene on the _____ chromosome

Characteristics ofFragile X

• distinct face long narrow face prominent jaw large protruding ears high arched palate

Characteristics ofFragile X

long hands and palms large testes after puberty seizures in 20% autism-like features in 7-25% (Hatton, et al., 2003)

normal life expectancy ADHD mild—moderate intellectual impairment

in boys; borderline IQ in girls decline in IQs over time

perseverative speech generally better verbal skills than one

would expect given IQ good adaptive behavior

Prader Willi Syndrome

• Caused by deletion of part of 15th chromosome (15q11-13) that derives from father

• most common cause

Characteristics ofPrader Willi Syndrome

Distinct faces down turned mouth almond shaped eyes

Hypopigmentation in 50% Hypotonia (_____ muscle tone)

delayed motor development failure to thrive and feeding problems in infancy morbid obesity after infancy obesity related health problems

More Characteristics of Prader Willi Syndrome

short stature relative to family adult males—avg. 5 ft. 1 inch adult females—avg. 4 ft. 10 ins. small hands and feet

delayed secondary sex characteristics; infertility

day time sleepiness and high threshold of pain

abnormal temperature regulation

Cognitive Functioning in Prader Willi Syndrome

Average IQ is 70 Strengths in visual processing Social behavioral issues (_______ _________)

Hoarding or stealing food

Picking at skin, especially front of hands and legs

Temper tantrums

Stubbornness

Compulsive Upset by changes in order Anxiety

Prader Willi Syndrome Treatment

• Growth hormone to increase height• Weight management

low calorie diet less than 600 kcals per day in children less than 1000 kcals per day in adults

limited food intake regular exercise

Question

• Jose has intellectual disabilities. As an infant, he was lethargic and had difficulty eating; however, at about one year of age he became obsessed with food. This is characteristic of

a)Down syndrome

b)Williams syndrome

c)Fragile X syndrome

d)Prader-Willi syndrome

Phenylketonuria (PKU)

recessive gene on 12th chromosome

______ error of ________ Causes body to not be able to

metabolize phenylalanine (found in ________ and nutrasweet)

Builds up in brain and causes brain damage

Treatment involves diet with no protein or nutrasweet Diet must be followed until ______

Phenylketonuria (PKU)

the longer we wait to begin diet, the lower the IQ will be

Age of treament and IQ in PKU (Hanley et al.)

93.5

71.654.5 55.5

40.8

0

20

40

60

80

100

0-2

months

2-6

months

6-12

months

12-24

months

over 24

months

age

IQ

Screening Tests

• Maternal serum screening

• Nuchal translucency sonogram

• Amniocentesis

• Chorionic villus sampling

• Newborn screening

Amniocentesis

• Take sample of amniotic fluid and analyze it

• Done at 13-16 weeks• 1/250 risk of

miscarriage

Chorionic Villus Sampling (CVS)

• Take sample of chorion, placental tissue

• Done at 7-11 weeks • 1% chance of

miscarriage

Hydrocephalus

Can result in intellectual impairment

Untreated hydrocephalus Treated hydrocephalus

Hydocephalustreatment

Treat with shunt Amount of intellectual

impairment depends on shunt’s ability to drain fluid

Can do fetal surgery and implant shunt in fetus

Monitor child for signs that shunt is malfunctioning, blocked or infected

– increased lethargy, fever, enlarged head size

MicrocephalusMicro= ___________

Cephalus or cephalo = _____________

Newborn with microcephaly

Microcephaly

Causes of microcephaly

• Maternal protein deficiency

• Various genetic disorders

School age children with microcephaly

Although text says “life expectancy is short,” life expectancy is usually

related to size of head

Fetal Alcohol Spectrum Disorder

Fetal alcohol syndrome•Have all 4 characteristics

Fetal alcohol effects syndrome•Have 3 of 4 characteristics

•Usually lack the distinctive facial appearance

Fetal Alcohol Spectrum Disorder

Characteristics of Fetal Alcohol Spectrum Disorder

• History of prenatal alcohol exposure

• Growth deficiency for height and/or weight

• Distinctive facial appearance including: short eye slits flat mid face thin upper lip short, upturned nose smooth or long

philtrum misaligned or

misshapen secondary

teeth


Flat midface

Smooth philtrum

Epicanthal folds


• History of prenatal alcohol exposure • Growth deficiency for height and/or weight • Distinctive facial appearance• Brain or CNS damage as evidenced by:

Microcephaly Reduced size of caudate nucleus in basal ganglia– (responsible for memory, attention, and cognition) Intellectual impairment tremors and or seizures hyperactivity ocular problems attention deficits poor coordination poor impulse and anger control difficulties with judgment & abstract reasoning memory impairments

Fetal Alcohol Effects Syndrome (FAES)

• Aka alcohol-related neurodevelopmental disorder (ARND)

• Fewer physical signs• Occurs 3 times more

frequently than FAS (Streissguth, 1997)

Fetal Alcohol Spectrum DisorderSecondary characteristics

90% develop mental health problems 60% quit school 60% trouble with law 50% incarcerated at some time 49% inappropriate sexual behavior 35% alcohol or drug problems

Congenital Rubella

aka German measles Problematic if mom gets rubella

during early part of pregnancy or right before pregnancy

Rubella: Pregnancy outcomes in mothers who had congenital

rubella (Sever, Hardy et al.,)

Age of infection

Percentage of Affected infants

Preconception 0-28 days before

conception

43%

First trimester 0-12 weeks after

conception

51%

Second trimester

13-26 weeks after

conception

23%

Third trimester 27-40 weeks after

conception

0%

Most severe

outcomes

Effects of Congenital Rubella

congenital cataracts blind intellectual impairment deaf deaf-blind hydrocephaly microcephaly heart defects

Congenital cataracts

Causes (cont’d)

• _______ causes (at the time of birth) Anoxia Low birth weight Infections passed from mother

Syphilis Herpes

Anoxia (lack of or too little ______)

• Don’t know exactly how much anoxia is needed to produce brain damage

• Causes of anoxia: Breech birth Prolonged labor Precipitous birth Placenta previa Cephalo-pelvic

insufficiency Umbilical cord prolapse or

cord wrapped around baby’s neck

Drugs use to speed labor or sedate mother

Low Birth Weight

• Full term babies – born after ______ weeks

• weigh less than 2500 grams (_______ lbs) Aka “small for gestational age”

Low birth weight vs. Prematurity

Time of gestation

Weight

Premature Less than 36 weeks

Less than 5.5 lbs.

Low birth weight

More than 36 weeks

Less than 5.5 lbs.

Low Birth Weight

Causes of Low Birth Weight

Maternal smoking Maternal malnutrition Maternal cocaine use Multiple and close

pregnancies Teen or young mother

Effects of Low Birth Weight

Death 5 times increased risk

Low IQ Microcephaly Failure to thrive

syndrome

Congenital Syphilis Syndrome• Sexually transmitted disease in mother• Caused by a spirochete bacteria • Bacteria crosses the placenta and damages the fetus

– Crossover cannot occur until 16th-18th week of pregnancy

• Can prevent by: Treating infected pregnant women with penicillin prior to the 16th

week of pregnancy Give untreated babies penicillin after birth

Effects of Congenital Syphilis Syndrome

• Syphilis in baby

• Fetal death 30% are stillborn (Mavrov et al., 2001)

• Distension of brain ventricle system• Intellectual impairment in 6% (Mavrov et al., 2001)

Congenital Herpes

Viral infection spread through exposure to Herpes lesions

Prevent exposure to lesions through Caesarian delivery

Effects of Congenital Herpes Seizures Death

50% die within 6 or 7 days Intellectual Impairment and/or Cerebral

Palsy Herpes lesions on baby

Causes (cont’d)

• Postnatal causes (_____ birth) Biological postnatal causes

Traumatic brain injury** Encephalitis Meningitis Lead poisoning

Psychosocial postnatal causes

**Discussed in depth in Chapter 13

Meningitis & Encephalitis

Encephalitis--inflammation of head or brain

Meningitis--inflammation of meninges

•Can occur at any time; •Can result in death, intellectual impairment, hearing loss, deaf-blindness, and/or finger/toe loss

Lead Poisoning

Lead accumulates in brain and bodily organs

Can cause: Learning disabilities Lower IQ Borderline or mild intellectual impairment Behavior problems Neurological problems Kidney disease

Sources of Lead

Paint chips

Lead pipes &solder

In old plumbingToys, jewelry, blinds,

and ceramic

items from China

Dirt from areas

near freeways

Psychosocial

Socioeconomic status (SES)

High Middle Low

0-20 IQ 1 1 1

20-50 IQ 4 4 4

50-75/80 IQ 10 25 50

75/80-90 IQ 50 170 300

Retardation prevalence per 1,000 School-age children by SES and IQ level

Psychosocial

Children who live in poverty for first 5 years of life average 9 IQ points lower than those who have never lived in poverty (Duncan et al., 1994)

Common correlates of low income

• Poor language and verbal skills of parents• Low maternal education• Few books and toys• Limited educational opportunities and expectations• Single and/or teenage mothers

– reduced supervision– reduced time spent with parent– emotional support may be lacking– lack of knowledge of child care

• Inadequate prenatal care• Multiple pregnancies in short time period• Limited routines, rituals and consistency

– sometimes, constant, unpredictable changes (Bernheimer, 2003)

PsychosocialTalk addressed to Child by Parent

0

2000

4000

6000

12 18 24 30 36

age in months

vocabula

ry

UpperSESMiddleSESLow SES

From Greenwood, Hart, Walker, Risley (1994)

Talk addressed by Child to Parent

0

500

1000

1500

2000

2500

3000

12 18 24 30 36

age in months

vocabula

ry

Upper SES

Middle SES

Lower SES

Low maternal education

Low vocabulary development in child

Low Mental Age in child

Low achievement in school

Early school drop out

Parenthood

Vocabulary size of the child is the single most important predictor of reading success (Anderson & Nagy, 2002)

IdentificationIntelligence tests

IQ tests for children (WISC-IV)

Cautions in using and interpreting IQ tests IQs can _______ from one

administration to next All tests are culturally

biased Younger the child, the less

valid the test IQ does not guarantee

outcomes

Adaptive behavior skills Parent or teacher answer

questions concerning the ability of student to perform adaptive skills

Vineland

Need both

Psychological and Behavioral Characteristics

• Genetic syndromes and behavioral phenotypes

• Specific learning problems Poor working memory Poor language comprehension and production Poor self-regulation (metacognition) Need external motivation

learned helplessness

Poor social development Increased gullibility

Educational Considerations

Functional academics– NCLB access to the general curriculum in:

_________, __________, and _____

Instruction in real-life settings with real materials

Grocery shopping in grocery store

Educational Considerations

Systematic instruction– Involves systematic

________, consequences for performance, transfer of stimulus control, and data based decisions

Constant time delay Progressive time delay

Prompt types• Ambiguous verbal• Specific verbal• Modeling• Gestures• Priming

– Enough physical help to start performing

• Physical assistance– Faded through

graduated guidance

Question• Karen is trying to transfer control away from

prompts to a more naturally occurring stimulus. She tells her daughter “ Pick up your toys. Put them in that box (points to box). “Over the next few days, she will tell her daughter to pick up her toys, but will wait 10 seconds before telling her to put them in the box. This is an example of

a) Progressive time delay

b) Constant time delay

c) Intermittent time delay

Functional behavioral assessment (FBA)

• Finding out the consequences, antecedents, and setting events that maintain the behavior– Used to identify the purpose or function of the behavior

• 5 most common functions of behavior1.______ attention2.escape from a ________3.access to ______ ________4.self stimulation / sensory stimulation

5.lack of knowledge or skill

Positive behavioral intervention and support (PBIS)• Support ______ behavior rather than ______ undesirable behavior

Service delivery models• Regular class with support (inclusion)

– Class wide peer tutoring– Co-teaching

• Resource room– Pennsylvania law limits 20 students to a

resource room

• Special class– in PA, usually 12-15 life skills or learning support

students– in PA, usually 8 students with multiple disabilities

• Special school– day school– residential school

• Hospital or homebound

More restrictive

Less restrictive

Assessment

Progress monitoring • Academic skills

Curriculum-based measurement (CBM)

• Adaptive behavior skills

Outcome measures Academic skills

PSSA-testing accommodations

PASA—alternative assessment

Adaptive behavior skills

Quality of lifeNeed

both types

Early InterventionPrograms designed for:

Prevention• _______ Preschool

Project• At age 27,

– Higher graduation rates

– Fewer arrests– Less teen pregnancies– Less welfare– Higher income

Further development• Language• Conceptual skills• Behavior• Motor skills

or

Transition to Adulthood• Self-determination

– Ability to make personal choices, plan and regulate one’s life, and be a self _______

– Cultural differences– Can it be carried too

far?

Transition to Adulthood• Person-centered planning

– Focus on the person– Supports a team approach– Vision, purposes, and

goals– Understand focus person’s

past, present and future life– Develop actions for

change, mutual support, personal and team development, and learning

– Usually creates the best contextual fit

Goals of Person Centered Planning (Iovannone, 2004)

1. Being present and participating in community life

2. Gaining and maintaining satisfying relationships

3. Expressing preferences and making choices in everyday life

4. Having opportunities to fulfill respected roles and live in dignity

5. Continuing to develop personal competencies

Transition to Adulthood: Community Adjustment

Community residential facilities

• aka ______ ______• 3-10 folks• Supervision

Supported living• Own home or

apartment • Support

Transition to Adulthood: Employment

Sheltered Workshop• no integration• less than minimum

wage• "non-meaningful"

work• no opportunities for

advancement• support, often

continuous

Transition to Adulthood: Employment

Supported Competitive• integrated• prevailing wage and

benefits• real, meaningful work• opportunities for

advancement• ongoing, unlimited support

– support in form of job coach, natural supports or technological supports

Documents

Chapter 5 Intellectual and Developmental Disabilities