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 Malnutrition Kwashiorkor Marasmus Rickets Pellegra -these are the most severe forms of malnutrition Kwashiorkor • Not enough protein in the diet. Kwashiorkor Occurrence World -Wide U.S.A. • Famine • Limited food supply • Low levels of education • 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 △ Kwashiorkor Dx test • • • • • • BUN CBC Creatinine clearance Serum creatinine Serum potassium Total protein Kwashiorkor Treatment h Calories & protein • 1st – Calories – Slowly • 2nd – Protein – Lactose free Kwashiorkor Prognosis • “Children who have had this condition will never reach their full potential for height and growth” • May be permanent – Mental & physical problems Kwashiorkor Prevention • Diet – Carbohydrates – Fat • 10% total cal. – Protein • 12% total cal. Marasmus • Overall calorie deficiency Marasmus S&S • Tissue wasting • Stunted growth Marasmus Treatment • Slowly h calories What is the difference? Kwashiorkor Maramus •i • i protein and calories protein Ricketts • Deficiency of Vitamin D Ricketts S&S • Bone deformities Ricketts Treatment • Vitamin D • Sunlight Pellagra • i Niacin –(B complex vitamin) Pellagra S&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 CP Further 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 CP classified 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 Disorders 1) 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 Glover Aristotle Agatha Christie Julius Caesar Margaux Hemingway Charles Dickens Richard Burton Napoleon Truman Capote St. 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 stare an unusual movement of the body a change in awareness a convulsion How long do seizures last? What is a seizure? It may last: a few seconds a few minutes continuously (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 During a seizure • Safety – – – – – Ease to floor Protect the head Turn to side Loosen clothing In bed? • Remove pillow • Side rails up – Do not • pry open mouth • Insert anything in mouth • restrain – Stay with pt • Support patient – Privacy Seizures • Observe & document – – – – – – – – Circumstance (before) Aura? 1st Movement Area Pupils Automatisms Incontinent – – – – – Duration Unconsciousness Weakness Dysphasia Post seizure behavior 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 Seizures Two 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