Lecture 6

Developmental Disabilities P020A

Course Objective #36

• Describe the cause and symptoms in the child suffering from the following types of malnutrition:– Kwashiorkor– Marasmus

Malnutrition

• Body does not get enough nutrients.

Causes of Malnutrition

• Inadequate diet• Problems with digestion

or absorption• Medical conditions

MalnutritionKwashiorkorMarasmusRicketsPellegra-these are the most severe forms of

malnutrition

Kwashiorkor

• Not enough protein in the diet.

KwashiorkorOccurrence

World -Wide

• Famine• Limited food

supply• Low levels of

education

U.S.A.

• Weaning

Kwashiorkor

• When kwashiorkor does occur in the United States, it is usually a sign of …

• child abuse and severe neglect

Kwashiorkor

Severe• infections • Irritability• Hepatomegaly Ascites• Lethargy• Dermatitis• Edema

Mild• △ skin pigment• h muscle mass• Diarrhea• i grow• Fatigue• Hair △

KwashiorkorDx test

• BUN• CBC• Creatinine clearance• Serum creatinine• Serum potassium• Total protein

KwashiorkorTreatment

h Calories & protein• 1st

– Calories– Slowly

• 2nd – Protein– Lactose free

KwashiorkorPrognosis

• “Children who have had this condition will never reach their full potential for height and growth”

• May be permanent– Mental & physical problems

KwashiorkorPrevention

• Diet– Carbohydrates– Fat

• 10% total cal.– Protein

• 12% total cal.

Marasmus• Overall calorie

deficiency

MarasmusS&S

• Tissue wasting• Stunted growth

MarasmusTreatment

• Slowly h calories

What is the difference?

Kwashiorkor• i protein

Maramus• i protein and

calories

Ricketts• Deficiency of

Vitamin D

RickettsS&S

• Bone deformities

RickettsTreatment

• Vitamin D • Sunlight

Pellagra• i Niacin

–(B complex vitamin)

PellagraS&S

• Scaly dermatitis• Inflamed

mucosa• Mental

disturbances

Pellagra• + Niacin into diet

Course Objective #37

• Differentiate between the following forms of cerebral palsy:– Spastic– Dyskinetic– Ataxic– Mixed

Cerebral Palsy

• Disorder of movement, balance and posture

• Results from a non-progressive lesion in the– Cerebellum

Cerebral Palsy (CP)

• Motor problems• May also have…

–cognitive impairments,–seizures–sensory loss

Cerebral Palsy (CP)

• h in neonatal care • h survival of very low birth

weight babies–> risk of CP

Cerebral Palsy (CP)

- ~ ½ of all CP is found in birth weights less than 5 ½ lbs.

Cerebral Palsy (CP)

- most often attributed to perinatal injury- Prenatally- Postnatally

Causes of CP:Prenatal:-infections -exposure to teratogens -maternal-fetal blood incompatibilities-faulty implantation of ovum

Causes of CP:

Perinatal:-asphyxia*-intra-cranial bleeding

Causes of CP:

Postnatal:-infection -brain abscess-head trauma-kernicterus

Types of CP

Classified by variations in motor symptoms Spastic* Dyskinetic Ataxic Atonic Mixed

Spastic CP• -#1• lesion located in pyramidal tract• “spasticity”• movement is slow, effortful,

restricted• occasionally jerky and explosive

Spastic CPFurther sub-typed by limbs affected:-all four limbs = quadriplegia-two like limbs, mostly legs = diplegia-three limbs = triplegia-legs only = paraplegia-limbs on one side only = hemiplegia-one limb = monoplegia

Spastic CPclassified by severity

• Mild: May lack only fine motor movements, have awkward gait, arms out for balance

• Moderate: problems with fine and gross motor and speech

• Severe: unable to walk, use hands, or speak

Extrapyramidal CP

-AKA Dyskinetic CP- Refers to one of the two nerve tracts in

the brain that control movement - Impairment in muscle tone and a variety

of abnormal motor patterns and postures

Extrapyramidal CP

--movements are uncontrolled and without purpose

-movements disappear with sleep and increase with stress

Extrapyramidal CP

Types of movement:• Athetosis*

– slow, wormlike, writhing. – If movements are jerky

• choreic & choreoathetoid

Extrapyramidal CP Subtypes

• Rigidity–Movement is impeded–Resistance

Extrapyramidal CP Subtypes

• Tremor–Rhythmic & pendulum-like

Ataxic CP

• 7% of all CP• disorder of balance / incoordination• lesion is in the cerebellum

Ataxic CP

-gait is unsteady-wide-based gait-

Ataxic CP

-hand movements awkward with tendency to under or over reach

-difficulty with repetitive finger movements or rapid turning

Atonic (Hypotonic) CP

-Usually hypotonic at birth and persists beyond 2-3 yrs.

-usually develops incoordination, ataxia, difficulty with rapid movements

-cognitive impairment can be severe-can also develop into athetoid form by age 3

Mixed Form CP

-combination of movement disorders-most common mix is spasticity and

athetosis-less common is ataxia and athetosis

CP Associated Disorders1) Speech Impairment may be the most disabling aspect of the disorder~ 2/3 of people with CP are speech impaired

CP Associated Disorders

2) Intellectual Impairment-assessment of intelligence is difficult due

to impairment in movement -ataxia & athetoid types less affected-spastic type more affected-mixed type greatest affected

CP Associated Disorders

3) Perceptual Disorders• visual, visual-motor, tactile, auditory• ADD / ADHD

CP Associated Disorders

4) Health Problems• seizures • orthopedic deformities• UTI• Respiratory difficulties• Eye abnormalities• hearing loss

Treatment of CP

1) Physical Therapy--starting early -prevents additional muscle tone problems

Treatment of CP

2) Surgery-lengthen or release muscles and

tendons-correct bone deformities-Botox injections with muscle building

exercises

Treatment of CP

3) Medications-not usually effective

Treatment of CP

4) Assistive Devices-for communication and movement

Treatment of CP

5) Supported Employment & Living-sheltered workshops, independent living

facilities-in adults: 1/3-1/2 will achieve

independence

Course Objective #38

• Explain the prevalence of seizure disorders in the DD population.

What do these people have in common?

Alexander the Great Danny GloverAristotle Agatha ChristieJulius Caesar Margaux HemingwayCharles Dickens Richard BurtonNapoleon Truman CapoteSt. Paul the Apostle Socrates Vincent Van Gogh

Epilepsy

- “epilepsy” from the Greek word meaning “condition of being overcome, seized or attacked”

- not a disease;

Epilepsy

-it IS a condition-characterized by sudden, brief seizures

whose nature and intensity vary from person to person

What is a seizure?

It may appear as:a brief starean unusual movement of the bodya change in awarenessa convulsion

How long do seizures last?

What is a seizure?

It may last:a few secondsa few minutescontinuously (status epilepticus)

What is a seizure?

-not all seizures indicate epilepsy!-a single seizure is NOT epilepsy!

What causes a seizure?

• high fever• TBI• i oxygen, • Hypoglycemia

What is a seizure?-the type depends on which part of the brain is

affected by the abnormal electrical impulse-divided into two major types: generalized

seizures and partial seizures

What is a seizure?-many different types of seizures-people may experience one type or more than

one type of seizure

Seizures

• Definition– Excessive discharge cerebral neurons

Seizures

–Partial–Seizure that begins in one part of the brain

Seizures– Partial

– Seizure that begins in one part of the brain• Simple

– Repetitive purposeless behavior– Dreamlike state– No loss of consciousness– Duration: < 1 minute– I.e. talking unintelligibly, dizziness, hand shake,

picking at clothes, chewing, smacking lips, spitting, fondling self

Seizures

• Classification– Partial

• Simple• Complex

– Repetitive purposeless behavior– Loss of consciousness– Lasts 2-15 minute– Not aware of behavior

Seizures

• Classification– Generalized

– Seizure that involves electrical discharges in the whole brain

• Absence Seizure– Occurs must often in children– Period of staring– Duration: several seconds

• Tonic-Clonic Seizure

Absence Seizure

• < 10 sec• ‘staring’• -usually begin between 4-14 yrs. age• -70% stop by age 18 yrs.• -may also have eye blinking, eyes roll-up• -no warning, no awareness of seizure, alert

immediately after

Atypical Absence Seizure

-usually last 5-30 seconds; usually more than 10 with gradual beginning and end

-stare, somewhat responsive, eye blinking, jerking of lips

-usually begin before 6 yrs.; continue into adulthood-most have < IQ and other types of seizures

Seizures

• Classification– Generalized

• Tonic-Clonic Seizure–(grand mal seizure)–Tonic – rigidity–Clonic – contraction and relaxation of all

muscles jerky, rhythmic

Seizures

• Possible Etiology– Underlying cause is electrical disturbance

emitting of abnormal, recurring, uncontrolled electric discharge

• Idiopathic origin• Head injury• Hypoglycemia• Brain tumor• Infection • Anoxia

Seizures

• Clinical manifestations– Prodromal phase

• Vague emotion• Min – hrs before seizure

Seizures

• Clinical manifestations– Prodromal phase– Aura

• Brief sensory experience• Precedes seizure by a few seconds• Usually the same aura each time

Seizures

• Clinical manifestations–Prodromal phase–Aura–Loss of consciousness

• May or may not occur–Seizure activity

Seizures

• Seizure activity– Tonic Phase

• Duration: 30-60 sec• Rigidity fall• Pupils fixed & dilated• Hands and jaws clenched• Stop breathing

– Clonic phase

Seizures

• Seizure activity– Clonic phase

• Contraction & relaxation of all muscles, jerky rhythmic fashion

• Incontinent• Frothing at the mouth• Dyspnea• Epileptic cry• Tongue chew• Lasts 1-2 minutes subsides

Seizures

• Characteristics – Post-seizure / postictal

• Recover period• Deep sleep• Confused

Seizures

• Medical management– Anticonculsants

• Caramazepine / Tegretol– S/E

» Blood dyscrasias» Hepatitis

• Clonazepam / Klonopin– S/E

» Hepatotoxicity» Behavioral changes

Seizures– Anticonculsants

• Phenytoin / Dilantin– S/E

» Gum hyperplasia» Nystagmus» Blood dyscrasias» PG cleft palate

Seizures

– Anticonculsants• Valproate / Depakene

– S/E» Hepatotoxicity» Blood dyscrasias

• Diazepam / Valium• Phenobarbital / Luminal

– S/E» Rash» h pain

Seizures

• Nrs Management w/ anticonvulsants– Do not stop abruptly seizures– Monitor levels– Take regularly– Inform of all meds– Alcohol only in moderation

Seizures

• Before Seizure– At risk for injury

• Padded side rails• Suction machine in room• Loose clothing• Know aura

Seizures

• Fear r/t possibility of a seizure– Take meds routinely– ID triggers

• Stress• New environment• Menstruation• Fever• Sleep deprivation• Alcohol

What photic stimulation can trigger a seizure?

• Give 3 examples!

Seizures

• Fear– Avoid photic stim

• Bight flickering lights• TV• Wear dark glasses

– Regular routine– Wear ID band

Seizures

• Ineffective coping– Embarrassed– Feel rejected, avoided, discriminated– Frequently lonely psych problems– Driving restrictions

Seizures

• Knowledge deficit– Take meds daily– records of meds & seizures– Drug levels routine– Avoid activities requiring alertness after meds– Showers or bath?

• Shower!

– Exercise– Sleep

Seizures

– Do not • pry open mouth• Insert anything in mouth• restrain

– Stay with pt

• Support patient– Privacy

During a seizure• Safety

– Ease to floor– Protect the head– Turn to side– Loosen clothing– In bed?

• Remove pillow• Side rails up

Seizures

– Duration– Unconsciousness– Weakness– Dysphasia– Post seizure behavior

• Observe & document– Circumstance (before) – Aura?– 1st – Movement– Area– Pupils– Automatisms– Incontinent

Seizures

• Recovery position• After a seizure

– Document– At risk for

• Hypoxia• Vomiting• Aspiration

Seizures

• Post Seizure Nursing care– Side lying– Bed low– Padded side rails– i stimulation

• Dim lights• Noise i

– VS– Check mouth– Clean pt– Allow to “sleep it off”

Status Epilepticus

• Defines– Prolonged seizure activity– Series of generalizes seizures

w/o full recovery btw attacks

– Medical emergency

Status Epilepticus

• Affects– Metabolic demand– Respiration– Anoxia– Brain damage

Status Epilepticus

• Medical Management– Goal

• Stop seizure activity• Airway

• Rx of choice– Diazepam

• Valium

Status Epilepticus

• Dx– Blood studies

• Electrolytes• Glucose• Phenytoin level

What is an AURA?

-some people experience an aura; a sensation or warning before a seizure starts

-varies from person to person-may occur enough in advance that person

can protect self from injury-seizure dogs help

What is an AURA?

-may manifest as a change in body temp, tension/anxiety, musical sound, strange taste, or odor

-precise description of aura helps diagnose the region of brain involved

-not always followed by full scale seizure -aura is a simple-partial seizure

Myoclonic Seizure

-brief, shock-like jerks of a muscle or muscle group-last 1-2 seconds; just one, or many in a row-usually both sides of body at once-seen in several epilepsy syndromes-many people have ‘normal’ myoclonus w/o having

epilepsy

Atonic Seizure

-AKA drop attacks or drop seizures-usually last < 15 seconds-sudden loss muscle strength-eyelids may droop, head may nod, drop items,

falls to ground-usually remains conscious-often begins in childhood; continues into

adulthood

Tonic-Clonic Seizure

-when massive bursts of electrical energy sweep through the whole brain at once, causing loss of consciousness, falls, convulsions, massive muscles spasms

Tonic-Clonic Seizure

-AKA Grand Mal-usually lasts 1-3 minutes-sudden onset, some preceded by aura-characteristics: immediate loss of consciousness two phases: Tonic and Clonic

Tonic-Clonic Seizure

Tonic Phase:-sudden contraction of all muscle groups-lasts 10-30 seconds, at times, loud cry-if standing, forcibly fall-pupils dilate, face is pale or flushed-eye roll up to one side or to the back-stops breathing!

Tonic-Clonic Seizure

Clonic Phase:-spasmodic jerking of all muscles-lasts 1-5 minutes or longer-breathing resumes, but labored-profuse sweating, salivation-tongue may be bitten-involuntary bowel/bladder-gradually becomes less violent and finally ceases

Tonic-Clonic Seizure

-may have quick recovery to consciousness with no effects, if episode is short

-often followed by stuperous state (postictal phase) and sleep

-upon waking; headache, fatigue, restlessness, agitation, sadness

Types of Seizures

Partial Seizures: -simple partial -complex partial -secondarily generalized

Partial SeizuresTwo forms: Simple Partial and Complex Partial -difference is whether or not there is a change in level

of consciousness

Causes: head trauma, serious infections, tumors of any kind, brain surgery, strokes, Alzheimer’s disease, arteriosclerosis

Simple Partial

-usually last < 2 minutes-do not lose consciousness-remain awake and aware-may talk normally-can remember what happened-can affect movement, emotions, sensation,

feelings in unusual and sometimes frightening ways

Complex Partial Seizures

-usually lasts 30 seconds to 2 minutes-affects larger area of the brain and affects

consciousness-cannot interact normally during-can’t remember what happened-will be in dreamlike, trancelike state-may talk, but unlikely to make sense-often takes place in temporal lobes-AKA “psychomotor epilepsy”

Secondarily Generalized Seizure

-starts as a partial seizure, then spreads and becomes a generalized seizure

Status Epilepticus

-condition in which there are continual seizures or a tonic/clonic seizure is barely finished before another begins and the person does not regain consciousness

-can produce exhaustion, severe anoxia leading to brain damage and cardiac arrest, brain hemorrhage and DEATH

Status Epilepticus

-can quickly become a LIFE-THREATENING situation!

-treated with IM or IV Diazepam and/or IV Phenobarbital

Treatment of Seizures

1) Medications:-single or combination of drugs-drugs control seizures – no cure!-partial seizures are more difficult to

control-meds must be taken consistently

Treatment of Seizures

2) Surgery:-when meds don’t work and seizures are

frequent and affect quality of life-remove small portion of the brain-or if coming from many sites on one side and

spreading, will cut connections between two sides (corpus callosotomy)

Treatment of Seizures

3) Vagus Nerve Stimulation (VNS)-electrical stimulation of the brain via the vagus nerve

in the neck-a small battery is implanted in the chest wall and

programmed to deliver short bursts of energy to the brain via the vagus nerve

-interrupts abnormal electrical impulses triggering seizures

Treatment of Seizures

4) Ketogenic Diet:-an option for children with hard to control

seizures-high in fat, low in carbohydrates, no sugar,

restricted calories-must be closely monitored by MD, RD, family-being used more often when other methods

fail

Nursing Actions During

-never leave them alone-if upright, lower to floor and side-lying safety

position to aid respirations-clear area of hazards, loosen tight clothing-do not restrain-padded tongue blade, if possible-padded bed rails, no pillows-accurate observations and documentation

Nursing Observations

-aura-presence or absence; if present, ability of patient to describe it

-cry-presence or absence-onset-initial sounds, movements, body parts

involved; chewing and salivation; posture; sensory changes

-tonic and clonic phases- movements, progression, duration of each

-skin color, respirations, pupillary changes, incontinence

Nursing Observations

-relaxation (sleep)-duration and behavior-postictal phase-duration, general behavior, ability to

remember, orientation, headache, injuries present-duration of seizure-from aura to relaxation-level of consciousness-changes and duration-presence of injury-to mouth, lips, tongue, soft tissue,

injury to extremities

Patient Education

-use of meds, including side effects, dose, timing, reporting side effects to Dr.

-importance of avoiding alcohol and illegal drugs

-safety measures top prevent injury during seizures

-good oral hygiene if taking Phenytoin-importance of adequate rest and diet

Patient Education

-importance of taking meds even if seizure free-community resources available-restrictions concerning driving-importance of follow-up care-need to avoid excessive stress-importance of wearing medical alert ID-importance of not over-protecting self