• Central cyanosis, coagulation defects, clubbing of the fingers and toes, feeding difficulties, failure to thrive, and dyspnea
• decreased pulmonary blood flow

• Abnormally slow heart rate 
• Heart rate is reduced to less than 60 beats per minute (bpm), and extra beats and slow nodal rhythms also may be present.

• Heart rate of 200 to 300 bpm; can lead to congestive heart failure. The child is irritable, eats poorly, and is pale.

• Caused by a deficiency of surfactant. As the air sacs collapse, oxygen absorption and carbon dioxide elimination are hindered.

• A chronic inflammatory disorder characterized by bronchial smooth muscle hyperreactivity that causes airway constriction in the lower respiratory tract, difficulty breathing, and bouts of wheezing
• TX: Anti-inflammatory drugs, immunotherapy, environmental control. 
•  May limit activity level or avoid the environmental irritant 

• A multisystem disease that appears to be related to an impermeability of epithelial cells to chloride; as a result, the exocrine (mucus-producing) glands malfunction, producing secretions that are thick, viscous, and lacking in water. The thick secretions block the pancreatic ducts, bronchial tree, and digestive tract.

• Also called brittle bones, a disorder characterized by decreased bone deposition caused by an inability to form type 1 collagen. The bones are unusually fragile, and even minor trauma can cause a fracture.

•  Maternal education in handling and positioning to prevent fractures during activities.
•  Monitored movement activity.
• Monitored exercise and weight bearing.

• Excessive growth at the epiphyseal plates (i.e., tall stature); arachnodactyly (the fingers are long, slender, and curved, resembling a spider’s legs); skull asymmetry; and alterations in the joints, eyes, heart, and aorta

• Address any skeletal deformities (scoliosis) that interfere with function.

• Stunting of epiphyseal plate growth and cartilage formation. The limb bones continue to grow to appropriate widths but are abnormally short. Skull size is normal; face size may be small, with a prominent forehead and jaw and a small nose. Trunk growth is near normal. Skeletal abnormalities include lumbar lordosis, coxa vara, and cubitus varus.(most common cause of dwarfism)

• Surgeries
• Adaptive equipment + modified environments.

• Joint inflammation, joint contracture (stiff, bent joint), joint damage, and/or alteration or change in growth. Other symptoms include joint stiffness after rest or decreased activity (also referred to morning stiffness or gelling) and weakness in muscles and other soft tissues around involved joints. Children with JRA may have pain at times, may show signs of fatigue, and may have reduced range of motion in one or more joints; may need adaptive equipment 
• Pauciarticular: Less than 5 joints
• Polyarticular: Abrupt, painful onset; symmetric; more than 5 joints
• Systemic (still's disease): Polyarticular and involvement of other organs  

• Nonprogressive abnormalities in the developing brain that create a cascade of neurological, motor, and postural deficits in the developing child; also may show intellectual deficits, behavioral problems, seizures, psychosocial delay & feeding problems 
• Intervention: Antispasticity oral meds, injectable agents (BOTOX), othopedic surgery, neurosurgery

• ·      Active + passive ROM
• ·      Positioning + handling
• ·      Orthotics
• ·      CIMT
• ·      Intensive motor training
• Parent support and education

• Temporary, involuntary change of consciousness, behavior, motor activity, sensation, or automatic functioning; may occur frequently in children as a result of fever, acute illness, or central nervous system infection or after traumatic brain injury

• ·      Medication
• ·      Diet (force body to use fat not carbs)
• Surgery

• X linked disorder affecting boys; caused by a deficiency in the production of dystrophin 
• children show typical development at birth, symptoms usually begin to appear between 2nd and 6th year of life; shows Gower's sign 
• Goals: Maximize and prolong independent function and locomotion, inhibit physical deformity, provide access to full integration into society; stretching, aerobic, moderate resistance exercises & ROM activities 

This defect may be mild, with the laminae of only one or two vertebrae affected (occulta) and no malformation of the spinal cord, or it may involve an extensive spinal opening with an exposed pouch made up of cerebrospinal fluid (CSF) and the meninges (meningocele) or CSF, meninges, and nerve roots (myelomeningocele) 

Weakness or wasting of the small muscles of the hands and sensory diminution in the area of the hand and arm

• Entire cerebral cortex
• Tonic-Clonic: Awareness that the seizure is going to happen 
• Loss of consciousness, rigidity, rhythmic contractions of all extremities; loss of continence
• postictal period (drowsy or sleeps) may last up to 2 hours 
• Absence: Loss of awareness and motor activity (except blinking or rolling) for about 30 sec; glazed look, staring into space, unaware of environment. 
• Myoclonic: Contractions of a single muslce 
• Atonic: Loss of muscle tone 

• Begin at one locatoni, stay limited or spread to become more generalized
• Simple Partial: Clonic activity of face or extremities; visual and auditory hallucinations; nighttime awakenings; twitching of facial MMs. 
• Complex Partial: Usually similar to absence seizure with addition of disorientation, sensory experiences that are not real, automatic reactions (lip smacking, chewing, buttoning of clothes).
• Infantile Spasms: Usually result in intellectual impairments 

• initail muscles affected are the proximal muscles of the pelvis & shoulder girdles. 
• occurs anywhere from the 1st to 3rd decade of life. 

• autosomal dominant; onset in early adolescence
• affects face, upper arms, and scapular region
• decreased ability to raise arms above shoulder height, decreased mobility in facial MMs, masklike appearance 

Congenital MDs 

• Onset is in utero or during first year of life; brain involvement in addition to neuromuscular disorder 
• hypotonia, generalized muscle weakness, multiple contractures. 
• CMD I: without severe impairment of intellectual functioning
• CMD II: Involves muscle and brain abnormalities 
• CMD III & IV: involve muscle eye and brain abnormalities; (clubfoot, torticollis, diaphragmatic problems, congenital heart and spinal defects) 

  PR 118

• Loss consciousness prime indicator of ABI, though not necessary for serious trauma. most occur in less than one year or older than 15 years old, more boys than girls
•   Cause: most common is fall in or around home, also bikes, cars, etc
• Cognitive:  memory, attending & concentration, judgement, problem solving, process speed, distractibility, following directions, planning ahead, sequencing, reasoning, etc
• Tx:  pace Ax allow rest breaks, limit visual stimulation, use finger guiding to assist w tracking, color cue materials, instructions short & concise (attn), have child keep log of significant events, teach problem solving strategies
• Psychosocial:  withdrawn/depressed, low frustration tolerance, poor motivation, impulsive, disinhibited, aggressive
•  use calm & friendly approach,  reward appropriate behavior,  prepare child for changes & transitions, role play emotions    
• Problems: paralysis, coordination, body awareness, endurance, muscle strength, gross & fine motor tasks, tone, balance/equilibrium, fatigue, motor planning, visual field, hearing, tactile processing, oral motor
• Environment: use calendars & timers for day orientation, have child sit in front of classroom, prep child for tests in advance
• Prognosis: poor if in coma for longer than 1 month, under 7 yrs worse social & school outcome than older. In most cases prognosis is good

 CS 168

•  most common developmental disability, includes significantly impaired intellectual ability (2 SD below mean), onset before 18 yrs, & impairment of adaptive abilities necessary for independent living
• Assessment: IQ & tests of adaptive behavior, MAP, HELP
•  mild: can learn to 3rd-7th grade, social & vocational skills (most married & employed)
•  moderate: learn to 2nd grade, require support to function but can handle routine daily functions, & can do unskilled or semiskilled work in sheltered workshop
• severe: can learn to communicate & basic health habits, need extensive support
• profound: require caregiver assistance for basic survival needs, minimal self care
• 80% have comorbid problems (speech, ambulation, seizures, visual, chronic conditions)
•  early programs focus on facilitation of developmental milestones, enriching environment, developing self help, language & motor skills, educating & supporting parents
•   Treatment: inc Neurodevelopmental, behavior modification, sensory integration, human occupation - may include development self-care skills, independence in daily tasks, play skills, vocational interests, improve self-image

•  delay in 1 or more of developmental areas: cognitive, physical, communication, social/emotional, adaptive
• age range is 5 yrs or younger - covers early intervention & special education

• markedly restricted repertoire of Ax of interest. apparent bw birth & 3yrs
• unusual cpmbinations of sensory, communication & behavioral characteristics have negative effects on school, home & community Ax
• autism particularly severe limitations relating to others & disiplay ritualistic, stereotypical behaviors

Characteristics include disturbances in:

•  social interactions - impairs meaningful relationships.  poor eye contact, delayed/inappropriate facial expressions, aversion physical contact, lack social reciprocity, etc.
• communication - range mild to severe, can inc echolalia, syntax/atonal speech, lack emotion/inflection
•  behaviors - intolerance deviation from routine, resistance to any type change, attachments to bizarre objects.  motor patterns inc wiggling & flicking fingers, head rolling & banging, toe walking
• sensory & perceptual processing & associated impairments: overly sensitive to sound
•  comprehensive Tx inc: parent counseling, behavioral treatment, special ed in structured environments, SI therapy, social skills training, speech-language therapy, meds, family support.
• respond best to highly structured, specialized educational program that includes communication therapy, SI, social skills, training.

•  classified as a PDD - development normal until 6mo - neurodegenerative disorder characterized by loss of intellectual function (decelerated head growth), loss fine & gross motor skills, development stereotypical hand mmt, exclusive to girls
• can survive but usually nonambulatory, & nonverbal, no functional hand use (hand wringing & flapping common)
• prognosis generally poor & outcome is death.  Therapy focused on slowing decline, maintaining function as long as possible, enhancing quality of life

•  most common childhood neurobehavioral disorder
•  persistent & frequent pattern of developmentally inappropriate inattention & impulsivity with or hyperactivity
• exhibit inattention, hyperactivity, impulsivity which cause impairment to ADLs, easily distracted, inability to listen to instructions, difficulty organizing tasks, impulsivity
• Sx must present for 6mo to a degree that is maladaptive, interferes w occupational Ax inc self-care, academic, peers
• Therapy: cognitive-behavioral, behavior modification, educational interventions, social skills training, psychotherapy, modify environment, self-management techniques
• Assessments: Sensorimotor History, Sensory Integration & praxis

• group of problems that affect child’s ability to master school tasks, process information, & communicate effectively - often associated w other neurologic problems (ADHD). include variety of conditions: perceptual disabilities, dyslexia, developmental aphasia
• OT role: 
• young children: sensory integration, play & basic socialization & self-help
•  as age increases: additional  programs to promote social play, perceptual motor integration, & writing skills indicated

•  physical features: short, stocky stature & protruding abdomen, head small & flat on back, eyes upward slant. cardiovascular abnormalities common, obesity, high incidence respiratory infections
• motor planning, cognitive & language skills develop slowly

•  long, narrow skull, low-set, malformed ears, prominent occiput, small mouth & weak cry, webbed neck. low survival rate

•  eye ear & nasal anomalies, cleft lip & palate. 20% survive, most severely retarded & seizure disorders

• decrease in number of chromosomes. webbing of neck, congenital cardiac problems, small stature, obesity, underdeveloped ovaries & no secondary sexual characteristics, most do not have intellectual impairment, functional prognosis is good

• deletion of chromosome 5.  baby has weak, mewing cry, small head, wide spaced down slanting eyes, cardiac abnormalities, failure to thrive, microcephaly 

• XXY chomosomal pattern - mild disorder may not be noticed until adulthood.  LD & behavioral, emotional problems characteristic

• Most common inherited mental impairment. Meets early infant milestones. Speech delays common (especially boys), girls less effected speechà excessive shyness (may only be slightly delayed in speech), low endurance and easily distracted, hand mouthing (put a lot of objects including hands in mouth, Facial differences like large ears, long face, high foreheads, large heads.

• OT: fine and gross motor, motor planning, oral motor, SI problemsà tactile defensive is one of most common.

• Diagnosed by 10 yr., It can cause brain tumors, bone deformities, facial disfigurement, learning disabilities, and cancer (not predictable, spontaneous mutation). Ex. Loose depth perception, binocular and peripheral vision, hearing, winged scapula, general muscle weakness). No treatment..only try to slow tumor growth.

• (chromosome 15) Floppy babies (weak)that develop poorly. Obese (compelled to over eat, never feel full), reduced muscle tone and mental ability, sex glands that produce little or no hormones., emotionally immature, short.

• No cure, Strict diet, support and exercise, padlock refrigerator- can literally eat self to death, behavior management plan ( and structured environment) – anxiety can cause behavioral meltdown and may become aggressive, hormone replacement.

3 types: 

• A: deficiency in clotting factor (most common)
• B: Deficiency in clotting factor
• C: lack of functional clotting.

• Medical management: prophylaxis on regular schedule

Sensory motor neuropathy )3 types. Foot deformity, weakness of leg and foot muscles, sensory loss (maybe apraxia). Meds for pain.

• 4 types ( acute infant, chronic infant, chronic juvenile, adult onset). 
• Mutation, deletion, or rearrangement of chromosome. Progressive weakness of limb and respiratory muscles.

•  irritability, unusual sleepiness, pale, weakness, fast  HR, big spleen and pain on left side. Severe pain or fatigue. 
• Management: medicine and antibiotic, blood transfusion, bone marrow transplant.

• appetite for odd, non food, things-grass, crayons, dirt, pebbles, ect. Not sure what causes-may surface out of no where. Mineral deficiencies or mental illness?

• Hypersensitive, underactive, motorically disorganized/compulsive. Difficulty regulation sensory, motor, physiologic, attention, or affective processes. (auditory, visual, tactile, pain, vestibular).

• inability to synthesize, process, organize sensory info from body and environment.
•  Sensory Modulation (Sensory defensiveness, sensory registration problems), 
• Adaptive movement disorders (vestibular process disorder and developmental disorder), 
• Sensory Discrimination and perceptual disorders (tactile, proprioceptive, visual, other senses)

• inability of CNS ability to regulate own activity.
• Problems: sensorimotor, gravitational, auditory, movement level, olfactory, visual, attention, postrotary nagstygmus, sensory dormancy, sensory defensiveness, ect.

• Tactile Defensiveness: adverse reaction to touchà excessive emotion rxn, hyperactivity, behavior problems.
• OT:less defensive to touch, attention span, group activities and self care with out discomfort.

• SMD, brain unable to inhibit or modulation vestibular  impulses.  Abnormal anxiety/distress when gravity receptors  of vestibular system stimulated (head positioning, movement when feet not on ground)

• OT: increase tolerance to touch/stimuli, acceleration and deceleration, rotational movement tolerance, balance, stepping off/ climbing

• deficit in coordinating two sides of body, poor equilibrium, low muscle tone, communication difficulties due to underactive vestibular response and inadequate maturation of postural rxn.  Vestibular stimulation to influence balance, muscle, oculomotor responses, movements against gravity, ect. = 
• improved daily living skills, academic performace, play skills, attn. span and focus

• difficulty conceptualizing, planning, executing a nonhabitual motor act- originating in childhood. Unknown cause but begins early and continue to adulthood. Types: somatodyspraxia ( SI deficit), Postural, sequencing, oral and verbal, constructional, graphic.

• can’t distinguish between different sensory stimuli (of one sensory modality). Associated with autism, CP,  learning disabilities, premature, psychiatric disorders, traumatic head injury, stroke, tumor. SI, perceptual motor approaches, auditory integration training.

• #1 mental health for children (general anxiety), discomfort, high HR, poor coping, ect when parent not around. Strategies: help child understand anxiety, relaxation strategies, develop healthy ways of thinking, face fears.

• difficulty hearing normal speech or sounds. Caused by lesion in auditory canal  or middle ear (conductive), Lesion in inner ear or 8^th Cranial Nerve. Structural or genetic.

• uncontrollable tics, involuntary muscle movements, vocal issues, repetitive and distractive behaviors ( ex. Excessive blinking).
• OT: help eliminate tic- urge, feeling, signal from body makes tic occur. Find trigger and help fight “temptation” . Strategies to overcome or adapt

• if before most major developmental stages occurred then either blind (corrected vision less 20/200) or visually impaired. Components: visual reflexes (blink), visual acuity, accommodation, convergence, scanning. Most common cause it prematurity.
•  SI and developmental therapy.

• develop a Tx plan 
• screen to decide if further evaluation of the child is warranted
• eligibility or diagnositc testing to see of child is eligible for OT
• reevaluation to assess progress
• research or outcomes testing to evaluate intervention efficacy. 

i.Two levels of screening

1.Screening of developmental skills (language, motor, social, personal & adaptive skills)

2.Second level Screening: occurs when a healthcare professional or teacher identifies the child as being at risk for developmental delays.

ii.Screening tools (first level)

1.Ages and Stages Questionnaire

2.Bayley Scales of Infant and Toddler Developmental-screening test

3.Denver Developmental Screening test-II

4.First STEP

iii.Screening tools (second level)

1.Short Sensory Profile

2.Bruininks-Osertsky Test of Motor Proficiency (BOT-2)

• Ages and stages questionnaire
• Bayley Scales of infant and toddler development
• Denver Developmental screening test II
• First STEP 

• child's chronological age
• presenting problems
• parents' priorities
• available evaluation tools
• type of service delivery (direct, integrated, supervised, consultation) 
• amount of time and resources

• Hawaii early learning profile checklist 
• Erhardt Developmental Prehension Assessment 
• Assessment and Programing system for infances and young children 
• evaluation of childrens handwriting 

• Knox Preschool play scale
• HOME inventory 
• TPBA
• SFA 
• Sensory Processing measure 

• do not interfere with the natural course of events
• pay attention to the physical and social features of the environment
• record the childs behavior in observable and neutral terms

Used to gather data on a child's development, caregiver/child interactions and childs environments. 

• pediatric eval of disability inventory 
• caregiver/parent-child interaction feeding scale
• sensory profile 

A team of professionals evaluates the child together. This is beneficial to gain knowledge, understanding & perspective from other areas of expertise. 

created federal support for family centered services for children 0-3 and their families 

(OT participates in multidiciplinary evals to determine eligibility, asses. of family needs, resources and priorities in an IFSP 

• tells the degree or strength of the relationship between two scores or variables. 
• range from -1.00 to +1.00 

extent to which a test measures a particular theoretic construct; does the test discriminate groups? does the test capture progress in child's performance? 

the extent to which the items on a test accurately sample a particular behavior domain 

-

describes how well test scores reflect current performance 

-examined by comparing the relationship of a new test and existing test. 

Arthrogyrposis Multiplex Congenita

• Increased connective tissue around joints. Contracture of many or all of the childs joints at birth.  Muscles may be absent or not completely formed. With the most common type, amyoplasia, both LE and UE are involved.  Stiff, spindly, and deformed joints, may have clubfoot and hip dislocation, or paralysis.

•    Maintaining and increasing functional ROM + strength.
•    Splints, serial casts, daily stretching.
• Adapted equipment and modified environment

Occur more frequently in UE.

• ·      Amelia: Absence of limb or of distal limb segments.
• ·      Phocomelia: Missing 1 or more proximal segments of limb.
• ·      Paraxial deficiency: Proximal limb is correct, but the medial or lateral side of the rest of the limb is missing.
• ·      Transverse hemimelia: Amputation of limb segment across central area.
• ·    Polydactyly: Excess of fingers and toes
• Syndactyly: Webbing between fingers + toes

• Prostheses training
• Psychosocial, self-concept, + social play experiences.

Lateral curvature (of more than 10 degrees) + rotation of the spine.

• ·      Surgery
• ·      Back brace
• ·      Exercise to strengthen spinal and abdominal muscles.
• ADL adaptations, if nec.

Small, recessed jaw. Tongue can obstruct airway and asphyxiate baby.

• ·      Surgery
• Work on core posture muscles to facilitate breathing.

Contracted sternocleidomastiod muscle so that neck flexes to involved side , face turns to opposite side and is tilted upward

• Positioning, exercise, gentle stretching, soft collar brace.

Malformations of the CNS that occur early in uterine development.  Spinal column fails to close and is exposed to the environment. 3 major forms:

• Encephalocele: severe deficits, cognitive + motor impairments, hydrocephalus, + seizures.
• Anencephaly: Children do not survive infancy
• Spina Bifida: mild to severe, could have sensory + motor disturbances below lesion.

 Paralysis of LE, bowel and bladder incontinence common.

• Wheelchair training if nec,
• Adaptive equipment
• Bowel and bladder control

Buildup of CSF in the ventricles of the brain.  Can push cerebellum into foramen magnum.  Can cause visual + perceptual deficits, fine motor delays, sensory processing difficulties, cog. impairment, seizures, + death.

• IADL’s
• Functional mobility skills
• Self Care 

• Paralysis of arm due to injury (extreme shoulder flexion) to the brachial plexus. Muscle + sensory weakness in hand

• Prognosis can be good with early intervention (therapy, surgery, or it recovers on it’s own).

Standardized 

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