Peds_ Exam 2 Blueprint.docx

PEDIATRICS EXAM 2 BLUEPRINT MODULE 5: INTRACRANIAL REGULATION (10) Examine the concept of intracranial regulation related to the care of the pediatric patient. Discuss normal physiological processes of the brain. Describe three categories of dysfunction and risk factors. Discuss the levels of preven...

PEDIATRICS EXAM 2 BLUEPRINT MODULE 5: INTRACRANIAL REGULATION (10) Examine the concept of intracranial regulation related to the care of the pediatric patient. Discuss normal physiological processes of the brain. Describe three categories of dysfunction and risk factors. Discuss the levels of prevention for the care of the client with alteration in intracranial regulation. Determine the appropriate nursing care for a child with alteration in intracranial regulation including the identification of risks, signs, symptoms, treatments, priority interventions, and complications. Key Terms: Intracranial regulation: collective bone structure (skull) that encloses the brain and the compliance and maintaining a balance to promote an environment that is conducive for optimal brain functioning Neurotransmission: process of sending signals from nerve to nerve across a synapse Neurotransmitter: chemical that transmits signals from a neuron to a target cell Regulation: maintenance of balance to promote an environment conducive to optimal brian fx Increased Intracranial Pressure: pathological condition or trauma causes pressure within the cranial vault to increase Critical Assessment finding ICP monitor to measure (do not change dressing daily) Cushing’s Triad: relationship between blood pressure, pulse rate, and ICP As symptoms increase, S/S become more pronounced, and LOC deteriorates Impaired Perfusion: There must be consistent flow of blood to the brain Interrupted/reduced blood flow impairs brain fx and can cause tissue death Compromised Neurotransmission: must be adequate transmission of nerve impulse across neuronal synapses by NTs for optimal brain fx 3 Major Consequences of Impaired Intracranial Regulation: Cerebral Edema: impacts brain size Increased Intracranial pressure: impacts sustained pressures Decreased Cerebral Perfusion Pressure: decreased perfusion of the brain Notice Risk Factors: Elderly: Degenerative pathological conditions Injuries (falls) Stroke: ischemic or hemorrhagic Adolescents and Young Adults: traumatic injury Bleed or hydrocephalus Glasgow Coma Scale: 3 part assessment Eye opening Verbal response Motor response Score of 15 = unaltered LOC Score of 8 or lower = coma Score of 3 = extremely decreased LOC (worst possible score) Primary Prevention of ICR: Healthy lifestyle to minimize cerebral artery disease Diet, exercise, tobacco products Injury prevention measures Immunization Hygiene Infection control Cerebral Trauma: head injury three major causes of brain damage Falls Motor vehicle injuries Bicycle or sports-related injuries Pathophysiology of Head Injury: Force of intracranial contents cannot be absorbed by the skull and musculoligamentous support of the head The head is especially vulnerable to acceleration-deceleration injuries A child’s response is different because of large head size in relation to body and because of insufficient musculoskeletal support Primary Head Injuries: occur at the time of physical trauma Skull Fracture: an immature skull can withstand a greater increase in deformities before fracture (infant force must be extreme) Linear, depressed Comminuted: split into multiple pieces Basilar: serious anterior-posterior Battle Sign: Bleeding posterior beck, Mastoid area Racoon eyes Leakage of CSF Bleeding from ears Clear drainage from nose Open Diastatic: transverses the sutures, widening the sutures Contusions/Lacerations: Contusion: visible bruising Laceration: tearing of tissue Coup: bruising at point of impact Contrecoup: bruising at a site far removed from point of impact Possibility of multiple sites of injury Intracranial hematoma Diffuse injury Concussion: alteration in neurological or cognitive function with out without loss of consciousness Transient and reversible Results from trauma to the head Generally followed by amnesia and confusion Complications of Head Trauma: Epidural Hemorrhage: bleeding between the skull and dura Subdural hemorrhage: bleeding between the dura and the arachnoid membrane Cerebral edema: associated with TBI, increased ICP with herniation Diagnostic Evaluation of Head Trauma: Detailed history Assessment of airway, breathing, and circulation (ABCs) Evaluation for shock Neurologic examination: LOC assessment Assessment of VS Common Tests: MRI or CT scan Skull radiograph Electroencephalogram Brain biopsy Lumbar puncture Labs: CBC, ABG, Drug/Alcohol, Na, Ammonia, Glucose Neurologic Examination: Notice: Vitals, LOC, Skin, Eyes Motor function: strength, symmetry Posturing Reflexes: Primitive and Babinski Clinical Manifestations of Increased ICP in Infants Clinical Manifestations of Increased ICP in Infants: Irritability, poor feeding High-pitched cry, difficult to soothe Fontanels: tense, bulging Increase head circumference Cranial sutures separated Eyes: setting-sun sign Scalp veins: distended Clinical Manifestations of Increased ICP in Children: Headache Forceful vomiting Nausea Seizures Irritability Drowsiness, lethargy Diminished physical activity Inability to follow simple commands Late Signs of Increasing ICP: Bradycardia Decreased motor response to a command Decreased sensory response to a painful stimuli Alterations in pupil size and reactivity Extension or flexion posturing Cushing’s triad: decreased HR RR, widening or increased BP Irregular, decreased respirations Widening pulse pressure Decreased consciousness Coma Level of Consciousness: in descending order Full consciousness Confusion: impaired decision making Disorientation: to time and place Lethargy: sluggish speech Obtundation: arouses with stimulation Stupor: responds only to vigorous and repeated stimulation Coma: no motor or verbal response to noxious stimuli Persistent vegetative state: permanent loss of function of cerebral cortex Documentation terms: AAO x 3, confused, lethargic, AAO x 2 Obtunded: responds to voice, respond to pain Instead of coma: unresponsive to verbal, pain, etc Unresponsive Stages of Brain Herniation: Diencephalic stage: obeying commands, bilateral Babinski reflexes, paratonia, grasp reflex, decortication Later as the midbrain and upper pons are involved Patient becomes decerebrated As lower pons and upper medulla are involved the patient becomes flaccid Nursing Care of Unconscious Child: Outcome and recovery may depend on the level of nursing care and observational skills Emergency management: Airway Neck stabilized Reduction of ICP: keep head midline Treatment of shock Gag and cough reflexes may be minima: protect airway Increased risk of aspiration of secretions Nothing administered orally at first Care in hospital if severe injuries, LOC for several minutes, prolonged/continued seizures Assessment in the Comatose Child: astute nursing observation Signs of pain: increased agitation and rigidity ICP increased by pain Alterations in vitals: Increased HR, RR, BP Decreased O2 Cushing’s Triad (BIP): Bradycardia Irregular Resp Widening Pulse Pressure (increased BP) Pupillary Changes Do not withdraw pain meds and sedation completely to assess May have ICP probe, bolt, or drain - DO NOT change dressings daily Nursing Activities for Increased ICP: Patient Positioning: Avoid neck vein compression Provide alternating-pressure mattress Elevate HOB 30 degrees Avoid activities that may increase ICP: crying, positioning, suctioning, coughing, straining CO2 Eliminate or minimize environmental noise Quiet, dimly lit environment Reduce suctioning Stool softener to reduce strain Thermoregulation Pain Strict I&O: fluid overload makes it worse because increased cerebral edema, possible foley Other care: Hygienic care Exercise: ROM (use it or lose it) Family support Long term care Prevention Medications: Acute Care: Antiepileptics: phenytoin Sedation or amnesic anxiolytics Barbiturates (controversial) Paralytic agents: all you have is vitals and pupils to assess (no neuro assessment) Analgesics: pain meds Antibiotics for infectious processes: open wounds Monitor for S/S of infection Watch for glucose changes and other steroid side effects Monitor for sodium loss Injury to pituitary gland can lead to SIADH Look for CBC changes and Steven Johnson with other drugs (Tegretol) First 72 hrs is the swelling so try and keep BP from getting high, keep them calm Class Drug Rationale Osmotic diuretic Mannitol For cerebral edema Sedative Propofol Lorazepam Minimize risk of second injury Analgesia Morphine Fentanyl Pain Anti-epileptics Phenytoin Valproic Acid Seizure prevention/precaution Glucocorticoid Steroid Reduce ICP Possible Sequelae to TBI: Post concussion syndrome Post-traumatic headache Post-traumatic seizures Hydrocephalus Hydrocephalus: caused by an imbalance in the production, flow, and/or absorption of CSF Pathophysiology: Impaired absorption of CSF within the subarachnoid space Obstruction through ventricular system Communicating and non-communicating hydrocephalus Result of developmental defects Usually apparent in early infancy Other causes: neoplasms, infection, and trauma Often associated with myelomeningocele Therapeutic Management: Relief Treatment of complications Assessment of problems related to the effects of motor development Treatment: most often surgical VP Shunt: Complications: infection and occlusion Know positioning Treatment: Shunt: best and most effective treatment Surgical procedure in which a flexible tube is place into the child’s CSF system which diverts the flow of CSF from the ventricles into another region of the body (peritoneal cavity or atrium of the heart) VA Shunt: do not want VA because you cannot grow with it because tubing is not long enough Drawings fluid from brain ventricles into the atria of heart usually used when abdominal surgery has been done VP Shunt: preferred; longer tubing can be inserted and allows for growth Drains fluid from brain ventricles into peritoneum Shunt tube: ⅛ inch diameter, silastic: soft, pliable plastic Shunt Systems: catheters (tubing), flow control mechanism (one-way valve) Preoperative: elevate head to facilitate drainage Postoperative: head flat to prevent rapid drainage; gradually elevate head Shunt care: revisions with growth; do not pump Shunt Parts: named according to where they are placed in the body Ventricular catheter: portion of tube which is inserted into ventricles Peritoneal catheter: portion of tube which passes the CSF into the abdomen (peritoneal cavity) Atrial catheter: portion of tube placed in the right atrium of heart Valve: regulates pressure of the CSF flow and prevents backward flow of spiral fluid toward the ventricles Some have on/off control device Pressure: low, medium, high Reservoir: flexible flushing chamber which may be within the same unit as the valve or be a separate unit along the shunt Reservoir Purposes: Permits MD to remove CSF samples from the shunt with a needle and syringe and to inject the chamber for testing shunt function and for treatment Allows the shunt to be flushed or pumped flushing the chamber sometimes allows your MD to determine whether the shunt is functioning properly SHOW ACTUAL SHUNT Patients and families should know the name and model of shunt Know if shunt has on/off valve Shunt Infection: period of greatest risk 1-2 months after placement Treatment: Massive dose antibiotics Shunt externalization Shunt removal S/S: Septicemia Bacterial endocarditis Wound infection Meningitis Fever Altered ICP = irritable, seizures Intracranial Infections: CNS has limited response to injury Difficult to determine cause from clinical manifestations Lab studies required to identify causative agent Inflammation: can affect meninges, brain, or spinal cord Bacterial Meningitis: acute inflammation of meninges and CSF Can be caused by various bacterial agents: Streptococcus pneumoniae Neisseria meningitidis Group B strep Staph. aureus E. coli Infant Clinication Manifestations: Poor feeding Vomiting Irritability Bulging fontanel Seizures Child Clinical Manifestations: Fever, chills Nuchal rigidity Photophobia + Brudzinki’s sign (left) + Kernig’s sign (right) Signs of increased ICP Characteristic of CSF Normal Bacterial Viral Appearance of CSF Clear Cloudy, turbulent, purulent Clear or slightly cloudy Pressure 70-000 mmH2O Elevated Normal or elevated WBC in CSF Normal: Newborn: 0-15 Infant: 0-8 Child: 0-5 Elevated 1,000 or more cells Polymorphonuclear cells predominate Elevated, generally below 500 cells but may be several thousands. Early: Polymorphonuclear cells predominate After 24 hrs: Mononuclear cells (lymphocytes) predominate Protein in CSF Normal: Newborn 20-120 Infant/child 15-45 Elevated 100-500 Normal or slightly elevated 30-100 mg/dl Glucose in CSF Normal: Newborn: 20-40 Infant/child: 70-90m Decreased 0-15 ~40% of serum glucose Normal ~66% of serum glucose Bacterial culture Negative Positive Negative Treatment: Droplet isolation from nasopharyngeal secretions Antibiotic IV therapy Control fever Ensure proper hydration Pain management: meds, positioning, decreased stimulation Reduce ICP Treat complications: ICP, seizures, subdural effusions Prevent the initial occurrence Appears as extension of other bacterial infection through vascular dissemination Organisms then spread through CSP Increased risk with the number of contacts Seasonal variation: late winter, early spring Immunize children for H flu! Aseptic Meningitis: nonbacterial Causative agents are viruses Arbovirus, herpes, cytomegalovirus, HIV, adenovirus Diagnosis is based on CSF findings Onset is abrupt or gradual Manifestations: Headache Fever Malaise Treatment is symptomatic Encephalitis: inflammatory process of CNS with altered function of brain and spinal cord Causative agents: Direct invasion of CNS by virus Post-infectious involvement of CNS after viral disease Vector reservoir: mosquitoes and ticks S/S: “girl on fire” Malaise Fever headache/dizziness Stiff neck Nausea/vomiting Ataxia Speech difficulties Seizure Disorders: caused by excessive and disorderly neuronal discharges in the brain Determined by site of origin Most common neurologic dysfunction in children Symptoms of underlying disease process (name 4) Epilepsy: two or more unprovoked seizures, caused by a variety of pathologic processes in the brain, needing accurate diagnosis and determination of cause Causes: Acute symptomatic: Head trauma Meningitis Remote symptomatic: Prior brain injury (encephalitis or stroke) Cryptogenic: no clear cause Idiopathic: genetic Defect in brain structure or function Seizure Classification: Focal: partial Local onset and involves a relatively small location of the brain Focal without impaired awareness (Simple Partial) Focal with impaired awareness (Complex Partial) Generalized: Grand Mal - Tonic-clonic Involved both hemispheres without local onset Atonic (drop) Myoclonic Absence: Petit Mal Momentary loss of consciousness: 5-10 seconds Appears daydreaming Poor school performance Posture is maintained Minor movements Age: 4-12 years Febrile: transient disorder of childhood Ages: 6 months to 3 years Raree after 5 y/o Twice as frequent in boys Treat fever with scheduled medication if needed Cause: uncertain - viral infection Infantile Spasms: West Syndrome Jerking of tummy, raising arms, blinks Occur every 3-5 seconds lasting up to 30 minute clusters See the signs: Clusters of sudden, repeated Uncontrolled movements (head bobs or body crunching) Take a Video: record the symptoms and talk to doctor immediately Obtain diagnosis: confirm an irregular brain wave pattern with EEG Prioritize treatment: end spasms to minimize developmental delays Treat with: ACTH (steroid): monitor immunosuppression, glucose, HTN Ketogenic diet: monitor ketones and glucose Vigabatrin Combination therapy Unclassified Treatment of Seizure Disorders: Goal: control seizures or reduce the frequency and severity Discover and correction of cause Management: Drug therapy Keto diet Vagus nerve stimulation Surgical therapy Corpus callosotomy: removal of corpus callosum Nursing Care of Seizures: Be attentive to aura Time seizures Apnea, cyanosis, pupils dilated and on-reactive, incontinence Keep patent safe (ABCs) Oxygen Seizure precautions, aspiration precautions, do not restrain Medication Therapy for Seizure Disorders: Rescue meds: Diazepam: oral, buccal, rectal, IV Ativan: lorazepam Fosphenytoin (Dilantin): Should not be taken with milk Causes gum hyperplasia Phenytoin: irritating and crystallized with glucose Sedation for Status Epilepticus: Phenobarbital (watch for SJS) Requires adequate Vitamin D and folic acid intake Propofol Levitarecetem (Keppra): watch for rash and mood swings Tegretol: watch for SJS Valproic Acid: can cause liver toxicity with young kids MUST GIVE MEDS ON TIME Spina Bifida: child with cerebral dysfunction Neural tube defect: Prevention: folic acid begin 3 prior to pregnancy and continue in 1st trimester Malformation can occur anywhere along spine, determine severity Sac like protrusion from spine: Myelomeningocele: more severe Spinal canal open: contains CSF, spinal cord, and nerves Abnormal body function: Malformations at or below lesion Mingocele: Meninges protrude through a spinal opening May have incomplete paralysis with urinary and bowel dysfunction Nursing Care of Infant: Keep sac free of stool and urine - cover with sterile moist gauze Positioning: Keep sac free from pressure, infection, and drying (sterile, saline soaked gauze) Knees flexed under or elevate foot of bed Assess neurological function Promote parent-infant bonding Keep baby NPO for surgery No rectal temps Monitor closely Post-op care Latex precautions Long Term Care: Set realistic goals Ambulation and ROM GU-Catheterization program GI-Bowel program Other complications: Skin, respiratory, neurological, musculoskeletal Collab with respiratory therapy, PT, OT, wound care Developmental delays Increased ICP → neuro damage Cranial Deformities: Craniosynostosis - bones join together too early Suture and fontanel ossification - normal 8 wks: posterior fontanel closed 6 months: fibrous suture lines and interlocking of serrated edges 18 months: anterior fontanel closed After 12 y/o: sutures unable to be separated by increased ICP Basically bones join together too early Surgery complications: Edema, bleeding, infection Supportive Care for Parents Reye’s Syndrome: Cause: giving aspirin to children with a viral infection Symptoms: Cerebral edema Liver involvement Vomiting Lethargy Diagnostic: liver biopsy Therapeutic Management: Early diagnosis and aggressive therapy for cerebral edema and symptoms Prognosis: recovery - good, in view of the gravity of the disease Complications: Coma, brain herniation, death MODULE 6: CARDIAC PERFUSION (10) Discuss nursing care of children undergoing cardiac diagnostic testing. Discuss clinical manifestations and care of children with congenital heart disease. ASD, VSD, PDA, Coarctation of Aorta, Pulmonic Stenosis, Tetralogy of Fallot, Transposition of Great Arteries Discuss clinical manifestations and care of children with acquired heart disease. Bacterial Endocarditis Kawasaki Disease Discuss the clinical manifestations and care of shock in children. Discuss the clinical manifestations and care of congestive heart failure in children. Types of Heart Defects: Congenital: Anatomic Abnormal function present at birth Clinical Consequences: heart failure and hypoxemia Acquired: Disease process - seen in normal heart or heart with CHD Infection Autoimmune response Environmental factors Familial Congenital Heart Defects: Heart is formed by 8 weeks, often before the mother realizes she is pregnant Incidence: 5-8 per 1000 live births About 2 or 3 of these cases are symptomatic in first year of life The major cause of death in first year of life (after prematurity) Most common anomaly is VSD (ventral septal defect) Cause thought genetic and environmental Often associated with other anomalies (trisomies 21, 13, 18) Maternal Risk Factors: Chronic illness (diabetes) Alcohol consumption, substance abuse Exposure to environmental toxins Infections Family history History and Physical: Activity/Exercise intolerance Developmental delay Frequent respiratory tract infections and difficulties Prenatal history Delivery history/Apgars Family History of Cardiac Disease Parent hx – may not notice – subtle changes – “baby has always been fussy” Child not being able to keep up with friends on the soccer field. Did the mother receive prenatal care? Maternal risk factors: Chronic illnesses (diabetes, poorly controlled phenylketonuria), alcohol consumption, exposure to environmental toxins and infections. – CMV, toxoplasmosis, viral illnesses Rubella in first 7 weeks of pregnancy – 50% risk of defects incl PDA HIV – (cardiomyopathy) IDMs (Insulin Dependent Mothers – 10% risk of CHD (VSD, cardiomyopathy, TGA, PDA) Teratogenic medications – ex: phenytoin (Dilantin). Maternal alcohol or drug use. Maternal age → Trisomy 21 Teratogens: Amphetamine VSD, PDA, ASD, TGA Valproic acid ASD, VSD, AS, CoA, PA/IVS Alcohol ASD, VSD Oral contraceptives VSD, TGA, TOF Infants with IUGR: more likely to have congenital anomalies High birth weight infants History of fetal loss, sudden infant or adult death. Family history of Cardiac defects (parents or siblings increases likelihood) Ex: Marfan, some cardiomyopathies hereditary Vital Signs: O2 saturations: preductal and postductal BP from different extremities: 4 extremities BPs Respiratory rate and status Heart rate Assessment: observe general appearance Nutritional status: FTT, poor weight gain Features - dysmorphic Color: cyanosis, pallor r/t poor perfusion Causes of cyanosis: Primary parenchymal lung disease Pneumonia, meconium aspiration syndrome Problem is with O2 diffusion Responds to increased FiO2 Primary cardiac disease Combination of decreased pulmonary flow and intracardiac mixing of “blue” and “pink” blood Doesn’t respond much to increased FiO2 Chest deformities: enlarged heart distorts chest Unusual pulsations: visible pulsations in neck Respiratory Status: excursion, effort, rate, tachypnea, dyspnea, expiratory grunt Clubbing of fingers Assessment: palpation, listen, percussion Palpation, percussion: Chest: heart size, thrills Abdomen: hepatomegaly, splenomegaly Peripheral pulses: rate, regularity, strength Auscultation: Heart rate and rhythm: tachy, brady, irregular Character of heart sounds: Distinct or muffled, murmurs, additional sounds Concerned with: Diminished femoral pulses Bounding pulses Difference in pre and post ductal O2 sats Heart murmur in delivery room Irregular rhythm Cardiac Diagnostic Testing: EKG/ECG - Electrocardiogram / Electrocardiography - displays electrical activity of the heart. 3-5 lead CP monitor – electrodes placed on the chest. Offers continuous monitoring of heart electrical activity and respirations. 12 lead ECG - electrodes placed across the chest and on each arm and leg Holter monitor – continuous recording. Often for 24 or 48 hours to look for any irregularities. Children are able to take the Holter home. Report activities on log. Report will look for correlation of log to events. Example, feeding, bathroom, play, running, crying, sleeping, any periods of cyanosis or shortness of breath. Event recorder - a long-term monitoring device, similar to a Holter. The device works by the patient or caregiver activating the monitor to record the heartbeat when certain events are felt or noted. Assess the patient, not the monitor! Compare pulses and apical heart rate on auscultation to rate and rhythm on monitor. Potential pitfalls: A patient jumping up and down or a parent burping a baby can look like a life threatening arrhythmia or extreme tachypnea A child that pulls 1 or more of their leads off and shakes them can look like a life threatening arrhythmia. PEA (Pulseless Electrical Activity) looks great, however, there is only electrical activity, no pulse, no perfusion. Respiratory count is particularly unreliable on a CP monitor with an active child. ECHO - Echocardiogram An ultrasound: shows the structure, size, and blood flow through the heart. Transthoracic echocardiography (TTE): Typically outpatient, gel placed on the patient's chest, transducer captures images of the heart. Transesophageal echocardiography (TEE): Typically done in the cath lab. Heart images viewed through a transducer placed in the patient's esophagus. Advantage of TEE over TTE: Image is usually clearer The heart rests on the esophagus so the ultrasound only has to go between millimeters of tissue vs the chest wall. Intracardiac echocardiography - Typically done in the cath lab. Procedure where cardiologist / interventional cardiologist places a catheter with camera directly into the heart. Often used to display images of the heart during procedures. Fetal echocardiography - Ultrasound image of the heart in utero. Referral from OB. Can be done as early as the first trimester. Exercise or Stress Test: Monitors for changes during a period of exercise. Often done with a treadmill or stationary bike. Start slowly, speed is increased to patient tolerance. EKG, Blood Pressure, O2 sats Respiratory Rate is measured. Spirometry may be used to measure Pulmonary function and tolerance. Syncope/Tilt Table Test: Diagnostic test to determine the cause of fainting. The child lies on a table that moves from a horizontal to a vertical position (usually 60-80 degrees). EKG, BP and patient symptoms (feeling of passing out, passing out) are monitored throughout for changes. The test typically lasts 20-60 minutes, and concludes when determined positive. Normal test - average blood pressure stays stable as the table tilts upward and your heart rate increases by a normal amount. Positive test: patient passes out, sudden period of hypotension, and/or bradycardia or tachycardia. Patients may faint or feel lightheaded. Corrected when tilted back to the flat position. Potential reasons: The patient’s heart rate may not compensate for the change or prolonged period in vertical position or the blood vessels do not constrict enough to support the BP. Cardiac MRI: Cardiac magnetic resonance imaging (MRI) creates both still and moving pictures of the heart and major blood vessels. MRI images of a beating heart help to analyze the heart structure and function. Cath - Cardiac catheterization Diagnostic – to diagnose prior to surgical repair Right and/or Left Right through the vein Left through the artery Helps plan for procedures Angiogram: to visualize the arteries surrounding the heart Contrast dye is used along with X-ray images Interventional – repair or other intervention Structural repair Electrophysiology studies: evaluate and destroy (ablate) accessory pathways that cause some tachydysrhythmias Nursing Care Before Cardiac Cath: Pre-procedure: Age appropriate teaching NPO at least 4-6 hrs Assessment: Vitals Baseline cardiopulmonary Perfusion Assess and mark pulses (tibial and dorsalis pedis) Height and weight O2 sat Sedation: oral or IV Nursing Care After Cardiac Cath: post-procedure Vitals Monitor blood glucose Consider adding to IVF if NPO prolonged or infant Frequent dressing checks: If bleeding, apply pressure at cardiac catheter entry site (2.5cm above entry site) Continuous cardiac monitoring and pulse ox Continuous or unit routine Assess rhythm (potential arrhythmias) EKG 12 lead, Chest C-ray, Echo post-op Assess perfusion of extremities and insertion site Pulse: normal to be weaker in affected extremity for a few hours Distal extremity pulses Color and perfusion Every 15 minutes x4 Then every 30 minutes x4 Then every hour x3 Then routine per unit Maintain affected leg straight for at least 2 hours May order led straight 4-8 hrs (depends) Then limited ambulation until tomorrow morning Bedrest 4-6 hours (strict) Progress from clear liquid to regular diet Monitor I&O Remove pressure dressing in 24 hrs, keep covered with bandage strip Tylenol or ibuprofen for comfort Avoid strenuous activity and tub baths for a few days Notify provider: Redness, swelling, bleeding, hematoma, arrhythmia, or fever Cardiac Physiology: CO = SV x HR Cardiac Output: amount of blood ejected in 1 minute From LV through aorta to body Adult has 5L of blood Stroke Volume: amount of blood in 1 good pump Preload: Volume of blood returning to the heart Circulating blood volume, CVP The stretch, just before a contraction Afterload: Systemic vascular resistance and pulmonary vascular resistance Systemic BP is some indication Force that opposes the ejection of blood out of the heart Resistance can be affected by constricting/dilating blood vessels Contractility: Efficiency of the myocardial muscle shortening Strength of the ventricle Fetal Circulation: Fetus has patent ductus arteriosus and foramen ovale. At delivery, the baby takes the first breath causing changes in pressures systemically and pulmonary. The PDA and foramen ovale will close 24-48 hrs after birth Sometimes, it is when these close that you might notice the patient decompensate, requiring evaluation for cardiac defect. Classification of CHD: based on hemodynamic characteristics Increased pulmonary blood flow Atrial Septal Defect (ASD) Ventricular Septal Defect (VSD) Patent Ductal Arteriosus (PDA) Obstruction of blood flow (out of the heart) Coarctation of Aorta Pulmonic Stenosis Aortic Stenosis Decreased pulmonary blood flow → cyanosis Tetralogy of Fallot (TOF) Mixed blood flow: saturated and desaturated blood mix Transposition of Great Arteries/Vessels (TGA) Cyanosis varies depending on degree of mixing and pulmonary blood flow Patient can be cyanotic with either Left to right shunt: higher pressure left side to lower pressure right side Leads to increased pulmonary blood flow and heart failure Left side obstruction: heart failure Right side obstruction: cyanosis Clinical Consequences: Heart Failure: Defects with left to right shunting Volume overload: septal defects Pressure overload: coarctation of aorta Decreased contractility: cardiomyopathy, drugs, electrolytes, arrhythmia Cyanosis: Defects with decreased pulmonary blood flow Defects with mixed blood flow Increased Pulmonary Blood Flow: ASD, VSD, PDA Characteristics Clinical Manifestations Abnormal connection b/n 2 sides of heart ↑ blood volume on R side Left to right shunt ↑ pulmonary blood flow ↓ systemic blood flow If small, may be asymptomatic ↑ fatigue Heart murmur ↑ risk endocarditis CHF Growth delay Atrial Septal Defect: Abnormal opening between atria Left to right shunt → INCREASED pulmonary blood flow RA and RV enlargement Often asymptomatic May develop HF in 30s or 40s if not repaired Clinical Manifestations of ASD If small, may be asymptomatic ↑ fatigue Murmur: systolic with a fixed split second heart sound (may be diastolic) ↑ risk for endocarditis CHF: mild HF Growth delay Possible enlarged right atrium Atrial dysrhythmias due to stretch and atrial enlargement Pulmonary vascular obstructive disease Emboli formation later r/t chronic ↑ pulmonary blood flow Treatment of ASD: Surgical patch: Pericardial/Dacron For moderate to large defects Non-Surgical: Amplatzer Septal Occluder placed by cardiac cath Low dose ASA x 6 months Prognosis of ASD: Operative mortality < 1% Ventricular Septal Defect: Abnormal opening between the right and left ventricles Size varies, often associated with other CHDs May close spontaneously in first year Left to right shunt Increased blood flow to PA may lead to increased pulmonary vascular resistance and HF Clinical Manifestations of VSD Heart failure Murmur: loud, harsh Holosystolic: best heard at left sternal border Failure to thrive May be asymptomatic if small At risk for bacterial endocarditis At risk for pulmonary vascular obstructive disease Poor feeding FTT/poor weight gain Activity intolerance Developmental delays Treatment of VSD: Palliative: PA banding to decrease pulmonary blood flow Surgical: Patch or suture (preferred) Required cardiac bypass Non-Surgical: Device closure with catheterization, more risk than ASD Patent Ductus Arteriosus (PDA): Failure of the fetal ductus arteriosus to close Allows left to right shunt from the aorta to the pulmonary artery Increased workload of left heart, pulmonary vascular congestion Possible right sided pressure and hypertrophy Ductal-dependent lesions: HLHS/HRHS Critical; aortic stenosis Severe COA Interrupted Aortic Arch Critical pulmonic stenosis Clinical Manifestations of PDA Range: asymptomatic to HF Murmur Widened pulse pressure Bounding pulses Treatment: Indomethacin (Indocin) or Ibuprofen In newborns closes PDA (prostaglandin inhibitors) Coiling procedure in cath lab Ligation of PDA with surgery (thoracotomy) Thoracoscopic placement of clip in some centers Meds keep a PDA open: Low pO2/hypoxia Prostaglandins Nitric Oxide What constricts a PDA: O2 Norepinephrine, acetylcholine, bradykinin Indomethacin ASA If small, clot at > 1 year: follow up yearly If large: surgery Prognosis: Under 1% mortality surgical and non-surgical Obstructive Defects: anatomic narrowing of blood vessel exiting the heart Coarctation of the Aorta: localized narrowing near the insertion of the ductus arteriosus Increased pressure proximal to defect (upper extremities) Decreased pressure distal to the defect (lower extremities) Clinical Manifestations of COA HTN, bounding pulses in UEs Weak or absent LE pulses, cool LEs HF (acidosis and hypotension) if severe Older children: dizziness, fainting, headaches, epistaxis from HTN Risk for: stroke, HTN, aortic aneurysm Treatment of COA: Initially, may need Prostaglandin E to maintain PDA (palliative) Balloon angioplasty Surgical: End to end anastomosis with prosthetic graft or portion of L subclavian artery Bypass not required, thoracotomy incision Must manage HTN post op with meds (Nipride or ACEI) May need angioplasty or stents later to maintain Prognosis: Mortality < 5% with isolated COA Goals: Improve ventricular function Restore blood flow to the lower body Aortic stenosis Pulmonic Stenosis Decreased Pulmonary Blood Flow: obstruction of pulmonary blood flow and an anatomic defect Tetralogy of Fallot: VSD Overriding aorta Pulmonic stenosis Right ventricular hypertrophy Clinical Manifestations of Tet Mild to severe cyanosis Murmur Clubbing polycythemia/clot formation FTT Tet spells: Anoxia with crying, feeding Risk for emboli, seizures, LOC, death Try to calm baby during episodes Surgical Treatment: Palliative: BT shunt Complete repair: Close VSD Resect pulmonary stenosis and patch pulmonary valve annulus Valve surgery when older because valve becomes incompetent Before surgical: walk and squat to increase venous return to heart Infant: bring legs up to the body to help pt increase blood return to heart Try to calm baby during episodes Mixed Defects: multiple complex defects Transposition of the Great Arteries (TGA) PA leaves the LV Aorta leaves the RV Must have other defects to allow for mixing (PFO, PDA, VSD) Clinical Manifestations of TGA Severe cyanosis at birth with minimum communication Sx of HF but less cyanosis with large VSD or PDA Murmur (+/-) Cardiomegaly within a few weeks Treatment of TGA: Ensure missing and improve arterial O2 sat Prostaglandin E to maintain PDA and O2 ≥ 75% Major side effect: apnea (likely to intubate/vent) CathLab: balloon ASD Balloon Artial Septostomy (BAS) Surgery: Arterial switch in first few weeks Treat CHF Progranosis: Mortality < 2% Hypoplastic Left Heart Syndrome (HLHS) Family Education: (for HLHS so might not need to know) Caution with illness, poor feeding Monitor for clinical manifestations of HF Medications Know how to contact cardiologist Maintain and information sheet/notebook Activity restrictions: require more calories because higher metabolic demand Go up to 28 cal/oz - teach them how to mix the formula or add formula to breast milk Diet/Nutrition Wound care/Bathing Sternotomy: scoop, don’t tear incision (6-8 wks) Maintain discipline/consistent limits Support groups Congestive Heart Failure: Inability of the heart to pump an adequate amount of blood into the systemic circulation Reasons: Structural abnormalities: more frequent in children r/t blood volume and pressure in heart Myocardial failure Cardiomyopathy, dysrhythmias, electrolyte imbalance (severe) Sepsis Anemia (severe) Heart muscle: may become damaged if left untreated (right or left side) Signs and Symptoms of HF: Impaired Myocardial function: Weakness, fatigue, restlessness, anorexia, FTT Tachycardia, gallop, diaphoresis, decreased UOP, pallor or mottling, cool extremities, weak pulses, delayed cap refill, cardiomegaly Pulmonary Congestion: Tachypnea, dyspnea, orthopnea, respiratory distress, activity intolerance Crackles, cough, retractions, flaring, grunting, wheezing, cyanosis Systemic Venous Congestion: Hepatomegaly, weight gain, edema (peripheral edema, periorbital edema), ascites, neck vein distension Weigh and chart - big jump = concern for fluid retention Goals of Treatment: Improve cardiac function Promote fluid loss - remove accumulated fluid and sodium Decrease cardiac demands Nutrition Improve tissue oxygenation and decrease oxygen consumption Improve Cardiac Function: Digoxin Improves contractility Increases force of contraction (+ inotrope) Decreases HR (- chronotrope) Increases renal perfusion Monitor apical pulse: Hold if HR under 70 bpm in older child or 90-100 in young child/infant Each child individualized and nurse should evaluate baseline MOnitor K+ level due to increased risk Dig toxicity Dig toxicity: Low HR, N/V, visual changes (halos), poor feeding, dysrhythmias Do not repeat dose if vomiting, notify health care team if misses more than 2 doses Keep in locked cabinet Give water after dosing to prevent tooth decay Improve Cardiac Function: ACE Inhibitors Captopril, Enalapril, Lisinopril Vasodilation decreases PVR and SVR Decreased BP and afterload Reduces aldosterone which prevents fluid retention Side effects: cough, hypotension Care: check BP before and after administration Promote Fluid Loss: Diuretics Eliminate excess water and salt Lasix, Chlorothiazide, Spironolactone (potassium sparing) Monitor Potassium S/S hypokalemia: muscle weakness, irritability, drowsiness, tachy/bradycardia Daily weights May be on fluid restriction K+ rich foods: bran cereals, potatoes, tomatoes, bananas, oranges Decrease Cardiac Demands: nursing interventions Conserve energy for feeding Organize care to provide uninterrupted sleep Avoid stressing child Watch for hyperthermia or hypothermia (neutral thermal environment) Radiant warming bed Prevent skin breakdown Oxygen therapy - caution in some Know pt’s normal saturations Nutrition: Caloric needs increased Fatigue may limit ability to take in calories Avoid crying/hunger, maintain consistent schedule Preemie nipple or enlarge opening Allow at least 30 min for feedings May need gavage feedings May need increased in kcal formula May be fluid restriction and strict schedule Improve Tissue Oxygenation, Decrease Oxygen Consumption: Reduce respiratory distress Assessment O2 Elevate HOB Acquired Heart Disease: Rheumatic fever Infective endocarditis Kawasaki Disease MIS-C Acute Rheumatic Fever: Inflammatory condition impacting joints, heart, brain, and skin 2-6 weeks after a group A streptococcal infection If remain untreated, antibodies for the infection cause damage to the valves May result in permanent cardiac valve damage (Rheumatic HD) Mitral valve is common valve involved Management: Antibiotics ASA - aspirin to prevent clotting, control inflammation, reduce fever and discomfort Bedrest initially followed by quiet activities Good nutrition May require heart valve repair or replacement Primary Prevention: Early diagnosis and treatment of GAS Penicillin is preferred Secondary Prevention: after recovery IM penicillin every 28 days Penicillin or erythromycin BID Nursing Care: Education regarding antibiotic prophylaxis compliance Management of chorea - one of the most disturbing symptoms Gradual onset and last for months after illness Abnormal movements, incoordination, weakness Movements will disappear on their own Rest Nutrition Bacterial Endocarditis: Infection of valves and inner lining of heart Potentially damages or destroys valves Most common in children with CHD but can occur without underlying HD Agents enter blood from any localized infection, brushing teeth, flossing, chewing, invasive procedure Most common agents: Streptococcus viridans and staphylococcus aureus Enterococcus Candida albicans Clinical Manifestations: Fever, malaise New/changed murmur, arthralgias Headache, weight loss Splinter hemorrhages: thin, black lines under the nails Osler nodes: red, painful intradermal nodes found on pads of phalanges Janeway lesions: painless hemorrhagic areas on palms and soles Petechiae on oral mucous membranes Secondary to extracardiac emboli - Vegetations on ECHO Possible HF, dysrhythmias +/- blood cultures, increased ESR, leukocytes Treatment: High dose antibiotics for 2-8 wks (PICC line) Consult ID Monitor blood cultures Prophylaxis to high risk patients prior to dental procedures (Amoxicillin most common) Preventive antibiotics are no longer recommended for any other congenital heart disease or conditions than these: Prosthetic heart valves or valve repair with prosthetic material History of endocarditis Heart transplant with history of abnormal heart valve function Cyanotic congenital heart disease that has not been fully repaired. includes children who have surgical shunts and/or conduits. A CHD that has been completely repaired with prosthetic material or a device for the first six months after the repair procedure. Repaired congenital heart disease with residual defects such as persisting leaks or abnormal flow at or adjacent to a prosthetic patch or prosthetic device. Caution with illness See provider for fever, illness, malaise, lethargy Kawasaki Disease: ​​Vascular Dysfunction Acute systemic vasculitis of unknown cause Inflammation of small and medium sized blood vessels, coronary arteries most susceptible to damage Acute stage- inflammation of capillaries, arterioles, venules and pancarditis Progression to muscular arteries can lead to coronary artery aneurysms Vessels reach maximum enlargement in 4-6 weeks from onset of fever Cause unknown 76% of patients < 5 years, most ethnic groups Self-limiting and resolves in 6-8 weeks Cardiac sequelae in 20% of children without treatment (coronary dilation or aneurysm) Treatment: Monitor labs: elevated ESR and CRP, anemia, and leukocytosis Baseline ECHO High dose IVIG: Frequent VS Monitor for anaphylaxis Monitor same as blood product Monitor cardiac status due to high volume of fluid Salicylate therapy: Anti-inflammatory Reduce clot formation secondary to thrombocytosis Plavix, lovenox, or warfarin may be indicated Nursing Care: VS, report any recurrent fever EKG monitoring Strict I&O Daily weight Monitor for S/S of HF Oral car/lip lubrication Lotion Cool compress to skin Soft comfortable clothing ROM for arthritis Quiet environment Parental support to irritable child Respite care for parents Discharge Teaching: Irritability for up to 2 months Easy bruising with ASA therapy - avoid contact sports Avoid exposure to chickenpox and flu while taking ASA to avoid Reyes Avoid Measles and Varicella vaccines for at least 11 months after IVIG admin Skin peeling in 2nd and 3rd weeks Recurrent fever: retreat with IVIG, notify HCP Most children recover without complications If large aneurysms present, risk for MI MIS-C: Complication of COVID-19 Similar to presentation of Kawasaki Disease or TSS Pathophysiology not well understood Onset is 2-6 wks after COVID-19 infection Symptoms: Fever Abdominal pain, vomiting, diarrhea Rash Conjunctivitis Red swollen lips, strawberry tongue HA, lethargy confusion Respiratory symptoms Sore throat Myalgia Swollen hand/feet Lymphadenopathy Treatment: Treat shock: treated with fluids, may require vasopressors (epinephrine and norepinephrine) IVIG ASA if S/S of coronary artery involvement Glucocorticoids Antibiotics often ceftriaxone and vancomycin Antiviral - may give remdesivir if showing s/s of concurrent covid-19 infection Pediatric Shock: Inadequate tissue perfusion to meet the metabolic needs Results in cellular dysfunction and eventual organ failure Physiologic consequences Hypotension Tissue hypoxia Metabolic acidosis Types of Shock: Hypovolemic: Blood loss secondary to trauma - GI bleed, intracranial bleed Plasma loss - increased capillary permeability associated with sepsis and acidosis Extracellular fluid loss: N/V/D Distributive: Anaphylaxis Sepsis Cardiogenic: Post op CHD - secondary to surgery Primary pump failure - myocarditis, myocardial trauma, CHF Dysrhythmias - SVT, AV block, ventricular dysrhythmias Clinical Manifestations of Shock: Three phases: compensated, decompensated, irreversible Differentiated by: Degree of tachycardia Perfusion to extremities LOC BP Initially symptoms are subtle - if we do not intervene → progress and worsen Apprehensiveness (early) Confusion (later) Tachycardia Tachypnea Thirst Pallor Decreased urinary output Decreased capillary refill Cool skin Management of Shock: Ventilatory support: May progress to respiratory failure Early endotracheal intubation CV support: IVF: boluses of 20 mL/kg push ND or lactated ringers Albumin Reassess after each bolus Meds: Dopamine Epinephrine Nursing Care of Shock: Ensure adequate tissue perfusion Establish IV access Monitor I&O VS every 15 minutes Lab work: H&H, ABGs, electrolytes Support family Textbook: most info is above from the ppt slides Box 23.1 (ASD, VSD, PDA), Box 23.2 (CoA), Box 23.3 (Tetralogy), Box 23.4 (TGA) MODULE 7A: CELLULAR PERFUSION (5) Determine the appropriate nursing care for a child with alterations in perfusion related to hematological function including: identifying risks, signs, symptoms, treatments, priority interventions, and complications Describe the clinical manifestations and care of children with blood disorders. Sickle Cell Disease Hemophilia Sickle Cell: 3 types - autosomal recessive Sickle Cell Anemia (SCA): HgbSS Most common form in US in AA 1 in 365 black births Homozygous form Hgb SS Sickle Cell C Disease (HgbSC) Heterozygous HbS and HbC Sickle Thalassemia Disease Pathophysiology HgbS changes molecular structure from a pliable disk to a crescent or sickle shaped RBC Caused by hypoxia, dehydration, and acidosis Results in obstruction from adhesion and inflammation Premature RBC destruction Shorter life span of HgbS SCA Diagnosis: Manifestations usually not common before 4-6 month/age due to fetal Hgb Mandatory screening Sickledex: detects HgbS but cannot differentiate trait from disease Hgb electrophoresis: separates various forms of Hgb, definitive diagnosis CBC: anemia Sickle Cell Crisis: Leads to a crisis: Anything that increases body’s need for oxygen or alters transport of oxygen Hypoxia Trauma Infection, fever Increased blood viscosity caused by dehydration Physical and emotional stress General Manifestations: Possible growth retardation and delayed sexual maturation Chronic anemia (Hgb 6-9 g/dl) Marked susceptibility to sepsis Vaso-Occlusive Crisis: ischemia and pain Painful joints and hands/feet (dactylitis Abdominal pain Stroke or visual changes Acute chest syndrome Hematuria Priapism Sequestration Crisis: Clinical Manifestations Hepatomegaly Splenomegaly Circulatory collapse Chronic Vaso-Occlusive Phenomena: Clinical Manifestations Cardiomegaly and systolic murmur from chronic anemia Decreased pulmonary function and increased infection Progressive renal failure Splenomegaly (fibrosis), susceptibility to infection Progressive retinal detachment and blindness AVN of hip or shoulder, lordosis or kyphosis, leg ulcers Hemiparesis Seizures Priapism Dactylitis General Solutions: Management Goals Prevent sickling, promote perfusion Hydration Hydroxyurea: increase Hgb F New: oxy bryta Prevent Complications: Penicillin prophylaxis: 2 months to 5 years minimum Administration of PRBCs or exchange transfusion Chelation therapy needed for chronic transfusions Transcranial Doppler recommended annually for children 2-16 y/o HSCT is only potential for cure: stem cell transplant Pain Reduction General Solution of Sickle Cell Crisis: Treat the medical emergencies of sickle cell crisis Rest Hydration: oral or IV therapy Electrolyte replacement Pain management Blood replacement Antibiotics for infection Monitoring of reticulocyte count regularly to evaluate bone marrow function Blood transfusion if needed If given early in crisis, may reduce ischemia Acute Pain Related to Tissue Anoxia: Mild to moderate: acetaminophen, ibuprofen, codeine, tordol Severe: immediate and sustained release of morphine, oxycodone, hydromorphone, methadone PCA: patient controlled analgesia Avoid Demerol due to risk for seizures and breakdown products Possible GI bleed for chronic NSAID use High doses may be needed Apply heat or massage affected area Avoid cold compresses Let child determine activity tolerance Believe their pain! Generate Solutions Deficient Fluid Volume Drink appropriate amounts of fluids Give patient/family specific amounts Increase fluid requirements during physical activity, stress, or crisis Monitor for and teach family s/s of dehydration Monitor I and O IV fluids Generate Solutions: Risk for Infection Occurs due to functional asplenia as spleen atrophies due to fibrosis by age 6 Notify HCP if temp > 38.5C Good nutrition Minimize sick contacts Hygiene Vaccines: Hib, pneumococcal, meningococcal, influenza Oral penicillin prophylaxis by 2 months/age Seek medical attention immediately if sick IV antibiotics to treat infection Generate Solutions: Deficient Knowledge Explain S/S of complications: Fever Pallor Respiratory distress Persistent Headache Pain Educate the school and teachers Wear medical ID band Frequent rest with physical activity Avoid contact sports with splenomegaly Genetic counseling Generate Solutions: SCA Emergencies Acute Chest Syndrome (ACS): Report chest pain, fever of 38.5C or higher, congested cough, tachycardia, dyspnea, retractions, decreased O2 sat Call provider immediately Cerebral Vascular Accident (CVA): Report seizures, abnormal behavior, weakness or inability to move an extremity, slurred speech, visual changes, vomiting, or severe headache If hx of CVA, child may get chronic transfusions for prevention Follow up with monthly blood transfusions on an individual basis Transcranial Doppler yearly for prevention or early detection Generate Solutions: Anemic or Aplastic Crisis CBC Reiculute count Type and cross PRBC’s Exchange transfusion Beta Thalassemia: Anemia: not pain Treatment chronic blood transfusions Goal: maintain Hgb above 9.5 Hemosiderosis and hemochromatosis: iron deposits with/without tissue damage Treatment for the build up of iron Oral chelation agents (deferasirox) Deferoxamine IV or subcut over 8-10 hrs (during sleep) Possible splenectomy - risk for infection HSCT up to 80% cure (hematopoietic stem cell transplant) Generate Solutions: Nursing Care Promote compliance with transfusion and chelation therapy Assist with anxiety/coping Assist with child and family adjustments to illness Observe for complications of multiple transfusions Genetic counseling Hemophilia Bleeding disorder from a congenital deficiency of specific coagulation proteins Most Common Forms: Factor VIII Deficiency (Hemophilia A) 1 in 5,000 people 80% of all cases Factor IX deficiency (Hemophilia B or Christmas disease) 1 in 50,000 people X-linked recessive disorder (60%) Gene Mutation: no family history Notice Hemophilia: Diagnosis: Factor VIII and Factor IX assays Severity: Factor VIII activity Severe (< 1%): spontaneous bleeding without trauma 60-70% of children are severe Moderate (1-5%): bleeding with trauma Mild (5-40%): bleeding with severe trauma or surgery Clinical Manifestations: bruising/bleeding Hemarthrosis: bleeding in joints Can → nony changes and deformity Early signs: stiffness, tingling, ache Obvious signs: Warmth, redness, swelling, pain, loss of movement Hematuria Epistaxis Subcutaneous and IM hemorrhages Generate Solutions: Care of Child with Hemophilia Replace missing clotting factors Factor VIII Aggressive replacement therapy with factor concentrate Home infusion Desmopressin (DDAVP): IV administration or nasal spray Causes 2-4 times increase in factor VIII activity Used for mild hemophilia Aminocaproic Acid Prevents clot destruction Prevent Bleeding: Prevent injury: age appropriate activity Regular exercise and PT may reduce bleeding episodes Sports: golf, swimming, jogging, fishing, bowling Soft bristled toothbrush Electric shavers Subcut instead of IM injections when possible Avoid aspirin or ibuprofen Medical ID bracelet Control Bleeding: Factor replacement RICE Keep cold packs ready Only active ROM Education: Factor VIII infusions Teach family and child Primary prophylaxis Injection Factor VIII 3x/week or every other day in severe disease Butterfly needles or SQP Secondary prophylaxis MODULE 7B: HORMONAL REGULATION (6) Examine the concept of hormonal regulation related to the care of the pediatric patient. Determine the appropriate nursing care for a child with an alteration in endocrine function including identification of risks, signs, symptoms, treatments, priority interventions, and complications. DM Type 1, Inborn Error of Metabolism, Childhood Obesity Discuss the differences in hypo and hyperglycemia including treatment. Diabetes in Children: Type 1 DM: Destruction of pancreatic beta cells Absolute insulin deficiency Type 2 DM: Insulin resistance Relative insulin deficiency Risk Factors: Genetics Toxins and viruses may destroy beta cells Obesity, sedentary lifestyle, high triglycerides, HTN Clinical Manifestations: typically hyperglycemia symptoms Polydipsia, polyuria, polyphagia Hyperglycemia Weight loss Fatigue/lethargy Enuresis in previously trained child Vaginitis (candida) in females Fruity odor to breath Delayed wound healing Blurred vision Changes in LOC Diagnosis: Fasting BG > 126 mg/dL Random BG of > 200 with chronic signs of diabetes OGTT > 200 mg/dL in the 2 hour sample Prep for OGTT: Balanced diet 3 days prior to test NPO for 8 hrs prior Fasting BG drawn at start of test, the client consumes a specific amount of glucose BG levels drawn q30 minutes x 2 hrs HbA1C: 4-6% or controlled < 7.5% determines long term control for children 6-12 y/o Diet with Diabetes: Meal planning weight/measure food for 3 months with progression to estimation of portion sizes 3 well balanced meals at regular intervals, afternoon, and night time snack Allow child to participate in food choices Extra food needs to be conceded for increased activity 10-15g of carbs for 30-45 min of activity Avoid concentrated sweets Sugar substitutes in moderation Monitoring: Target HbA1C of < 7.5% Self-monitoring should be done before meals (ac) and at bedtime (hs) at minimum Keep records of results with food intake and any other events such as increased activity or illness that may alter BG Follow proper procedure for BG monitoring Check strips for expiration Choose a machine needing small amount of blood Insulin Administration: School-aged children can be taught to administer insulin Rapid, short, intermediate, and long acting Allow children to do as much care as they can on their own to promote independence Parents taught as well Usually before breakfast and dinner, rapid/intermediate mix Use “pinch” technique Develop a schedule for site rotation Can use syringe: loaded injector or preloaded pen to avoid conventional vials/syringes Draw shorter acting insulin first May use insulin pump Some prefer it because it delivers a small amount SC and continuously If it malfunctions or becomes dislodged - need insulin soon Continuous glucose monitoring technology Exercise: Develop a plan Children with active teams sports require snack 30 minutes prior Check BG before exercise Children often need to increase food in summer with increased activity and decrease food when returning to school May need to decrease dose of insulin when exercising or a snack With consistent exercise –: dose of insulin may need to be decreased but never eliminated Safety/Developmental Issues: Child should wear Medic-Alert Bracelet Make sure the school knows the child has diabetes Child may need to eat during class Take supplies on school trips Plan for travel Adolescents may have trouble accepting/complying Hypoglycemia - Low Blood Sugar Hyperglycemia - High Blood Sugar Sweating Trembling Dizziness Mood changes Hunger Headaches Blurred vision Extreme tiredness Dry mouth Extreme thirst Frequent urge to urinate Drowsiness Frequent bed wetting Stomach pain Management of Hypoglycemia: S/S: Shakiness, dizziness Anxiety, nervousness Chills Headache Confusion Lethargy Difficulty focusing Hunger Pallor Palpitations If possible, confirm with BG check Administer rapid-releasing glucose immediately, followed by a complex carb + protein ½ cup orange juice or carbonated beverage (sweet) 8 oz milk Small box of raisins 3-4 hard candies or Life Savers 1 tsp of sugar or honey 1 candy bar Give extra snack if next meal is not planned If unconscious, squeeze cake frosting or glucose paste onto gums Glucagon may be required Management of Hyperglycemia: S/S: Lethargy Confusion Thirst N/V Abdominal pain Dehydration Rapid respiration Fruity breath Encourage oral fluid intake Administer insulin as prescribed Test urine for glucose and ketones Notify HCP if symptoms progress Observe signs for DKA (can be life threatening) Diabetes and Illness (Sick Days): May need ↑ or ↓ insulin Illness, infection, and stress usually ↑ need for insulin Monitor blood glucose and urine for ketones every 3 hours when sick Continue to take baseline insulin Stay hydrated: sugar free, non-caffeinated beverages to keep hydrated Meet carbohydrate needs by eating soft foods if possible Rest Call HCP if: BG > 240 Fever higher than 38.9℃, fever does not respond to acetaminophen, or lasts over 12 hrs + urine ketones Disoriented or confused Rapid breathing Vomiting more than once Diarrhea over 5 times or more than 24 hrs Unable to tolerate liquids Illness > 2 days Hydration and glucose management are key Functions of the Pituitary: Control center of the hormone releasing glands but most specifically growth hormone Major concern for children: affects growth and development Concentrate on growth hormone deficiency and thyroid insufficiency There can be under or over secretion of hormones or irregular timing of hormone production Other issues can lead to: Producing increased growth Decreased growth Early or late sexual development Adrenal insufficiency or excess Hyper/hypothyroidism Growth Hormone Deficiency: GH is essential for: Growth, development, and cellular metabolism Congenital or acquired Idiopathic Head trauma Tumor Radiation/surgical damage Clinical Manifestations: Short stature (proportional height and weight) Delayed epiphyseal closure Increased insulin sensitivity Delayed dentition Underdeveloped jaw Delayed sexual development Evaluated/managed by endocrinologist Evaluate growth curve Diagnostic Testing: IGF-1 and IGFBP-3 levels done fasting Draw baseline sample at 6-8am Administer med that triggers release of GH (GH releasing hormone or arginine) Obtain blood samples q30 minutes x 3hrs GH Stimulation testing: NPO and limit activity 10-12 hrs prior to test Bone age: X-ray of left hand/wrist to predict remaining growth potential CT/MRI and skull x-rays to determine presence of tumor or other structural defects Growth Hormone Deficiency: Nursing Care Assess height and weight velocity on growth charts Evaluate family patterns of height GH replacement therapy (Somatropin) Subcutaneous injections 6-7 days/week Best given at bedtime as mimics normal patterns of GH release 45-90 mins after sleep Give until bone age > 14 in girls and > 16 in boys or when growth is under 1 in/yr Growth velocity increases most in first year Psychosocial concerns Height is usually attained slower than peers Realistic expectations Final height is likely to remain less than norm Management of other pituitary dysfunction Complications: if not treated GHD → Disrupted vertical growth Delayed epiphyseal closure Delayed bone age Delayed sexual development Premature aging in later life Hypothyroidism: Thyroid hormone regulates basal metabolic rate and controls processes of growth and tissue differentiation TH released from thyroid Depends on dietary iodine and tyrosine Thyroid Stimulating Hormone (TSH): released from anterior pituitary Congenital or acquired Thyrotropin-releasing factor from hypothalamus as negative feedback response Congenital Hypothyroidism: Most common: thyroid dysgenesis from unknown cause 1 in 3000 to 1 in 4000 newborns Screen mandated in US Early detection and prompt treatment are vital Affects intellectual development and growth If treatment begins: 0-3 months/age: IQ mean 89 3-6 months/age: IQ mean 71 After 6 months: IQ 54 Birth 6-9 weeks Older Child Poor feeding Lethargy Constipation Bradycardia Respiratory difficulty Large fontanels Depressed nasal bridge Short forehead Puffy eyes Large tongue Thick dry skin Coarse hair Abdominal distension Hypotension Short Obese Abnormal reflexes Awkward mobility Intellectual disability Treatment/Nursing Care: Congenital Hypothyroidism If screen is positive, further blood studies done Refer to pediatric endocrinologist Treated with levothyroxine (Synthroid) Keep TSH levels between 0.5-2 mU/L Usually treated for life Parental education and compliance S/S of overdose/toxicity: Tachycardia Dyspnea Irritability Insomnia Fever Sweating Weight loss Nurse should ensure all newborn screened Childhood Obesity: BMI: 85-95% overweight BMI: 95% or greater obese BMI correlated well with DXA measures of body fat in overweight youth BMI may be high in very fit individuals with muscle mass Why the Concern with Childhood Obesity? Persists in adulthood and linked to subsequent morbidity/mortality Now obesity associated with physical conditions being reported in children Psychosocial consequences: Low self-esteem Depression Social isolation Obesity markedly lessens life expectancy Obesity expected to become the leading cause of preventable death (overtaking tobacco) Costly Increased incidence of obesity-associated chronic conditions: Type 2 diabetes mellitus Apnea HTN Dyslipidemia Asthma Gallbladder disease Severely obese 20-30 y/o have a reduced life expectancy 5-20 years Obesity is a common risk factor for: CV disease Type 2 DM Non-pharmacological programs: Behavioral and lifestyle modifications Minimal knowledge regarding youth and the non-pharmacological programs More obese adults in the south Health Consequences of Youth Obesity: Diabetes HTN Dyslipidemia Insulin Resistance Syndrome (Metabolic Syndrome) Sleep apnea Gallbladder disease Asthma Exacerbations Economic consequences: hospital costs, etc Assessment Recommendations: Body circumferences Headaches and blurred optic disk margins, which may indicate pseudotumor cerebri Sleep apnea: Snoring, breathing difficulties, daytime somnolence Acanthosis nigricans and skin tags Hirsutism, striae, oligomenorrhea: PCOS or Cushings Hypothyroidism Stress incontinence: bladder pressure Hepatic steatosis and renal insufficiency Gallbladder disease Early puberty is common Blount’s disease Diagnostic testing aids to identify common comorbidities: Dyslipidemia Platelet dysfunction Fatty liver disease Glucose intolerance Type 2 DM Thyroid levels (TSH) Lipid profile with total, LDL, and HDL Cholesterol levels Liver profile Fasting glucose Insulin C-peptide Portion Sizes: Have increased over the years Assessment: determine the degree of obesity and patient’s risk status based on other factors Management: includes both weight control or reducing excess body weight and maintaining that weight loss as well as instituting other measures to control associated risk factors Obesity is a chronic disease Examples of Initial Education: Participate in some form of psychical activity daily (60 minutes) Choose low-fat, low-sugar foods and snacks Eliminate, good, and better choice given for food groups (red light - green light) Limit soft drinks, juice, pre-packaged foods.meats, salt, sweets Eat slow and smaller portions Limit TV, video game, computer time Eat only at the table without other distractions like TV or reading a book Use fruits for desserts Use smaller plates, plastic utensils, put fork down between bites Limit “sit time” at computer, TV, video games to < 2 hrs/day Parent model exercise and eating habits Get out and be physically active together Evidence for Treatment Management: Multidisciplinary approach Family involvement Behavioral/Lifestyle remains key component Medication can be used as adjunct (Metformin if insulin resistance, Phentermine) Bariatric surgery: last resort Comorbidities: Metformin and other insulin-lowering drugs Lipid-lowering drugs High blood pressure medicine MODULE 8A: MOBILITY (11) Examine the concept of mobility related to the care of the pediatric patient. Determine the appropriate nursing care for a child with an alteration in mobility including the identification of risks, signs, symptoms, treatments, priority interventions, and complications. Recognize the effects of immobility in the hospitalized child. Effects of immobility of children Muscular system: atrophy which leads to decreased strength and endurance Skeletal: decreased bone density and contracture of joints Metabolism: decreased metabolism & decreased nutrition which decreases healing Cardiovascular: DVT and decreased cardiac output Respiratory: loss of respiratory muscles and decreased O2 demand GI: constipation, difficulties voiding, feeding difficulties due to positioning Urinary: difficulties voiding Integumentary: skin breakdown, decreased hygiene (cleansing around casts) Supporting Musculoskeletal System Proper positioning: Proper alignment Turn every 2 hours Skin care Range of Motion: Active or passive Nutrition Medication: Analgesics Anti inflammatories Muscle relaxants Supplements Diagnostics: Physical assessment is the key to diagnosis Assessing pain, ROM, tenderness, and erythema X-rays: can be difficult due to localized swelling CT scand and MRI: find injuries, areas of infection, and cacner Lab Values: Calcium: levels can vary due to bone breakdown and immobility Hydration is crucial to prevent this WBC and blood cultures: Alkaline phosphate ESR Bone and Muscle injuries: Contusions: damage to the soft tissue, subQ tissue, and muscle Escape of blood into tissues causes echmosis (black and blue discoloration) Dislocation: displacement of normal positions of opposing bone ends or bone ends to socket Strain: microscopic tear in the muscles and tendons Sprain: ligament is torn or partially torn Separation Tendons connect muscle to bone Ligaments connect bone to bone Management of Bone and Muslce Injuries: RICE: Rest, Ice, compress, elevate ICES Fractures: Plastic deformation: bone is bent but not broken Buckle: appears raised or bulging Very common injury in children Methods of treatment are different in children than in adults Rare in infants!!! Warrants investigation Distal forearm: most frequently broken bone in childhood School age: most likely from bike related or sports injury Simle or closed: does not produce a break i nthe skin Open or compound: bone protrudes through the skin Complicated: bone fragments have damaged other organs or tissue Comminuted: small fragments of bone are broken from fractures shaft and lie in surrounding tissue Epiphyseal Injury: Type I: separation of growth plate Type 3: may require surgery These need careful, frequent surveillance Direct management is needed to ensure that bone growth is not affected 2 Weakest point of the bone: the cartilage growth plate (epiphyseal plate) Frequent site of damage during trauma May affect future bone growth Treatment: open reduction and internal fixation to prevent growth disturb NEVER apply heat to a fracture- could lead to blood loss Use ice and splinting instead Goals of Management Regain alignment (reduction) Retain alignment (immobilization) Restore function Prevent deformity or injury Traction Fatiugues muscles to reduce spasms Realignment Helps with positioning and preventing deformity Bone Healing and Remodeling Typically rapid healing in children Neonatal period: 2-3 weeks Early childhood: 4 weeks Later childhood: 6-8 weeks Adolescence: 8-12 weeks Assessment for injured extremity: Box 29.3 Pain Pulse Pallor Parethesia Paralysis Pressure Edema, coolness, or warmth Interventions for fracture: immobilize, cover wounds, elevate, apply cold triage Interventions for compartment syndrome: fasciotomy, do not elevate, administer fluids if needed Casting: Dries in 2-48 hours for plaster of paris- handle with palms Fiberglass dries in 2 ish hours Elevate initially Check the P’s Pain, pulse, pallor, paresthesia, paralysis, pressure Encourage the use of unaffected muscles No coat hangers or straws to itch Keep it cool If using crutches- use hand grips for support Purpose of traction: Relieve fatigue in involved muscles Position distal and proximal bone ends Immobilie fracture site Prevent deformity Immobilize healing bone and prevent further injury Reduce muscle spasms Traction: Forward force produced by attaching weight to distal bone fragment Skin: Buck’s and Bryant’s (DDH) Skeletal: 90 degrees is common or halo vest External fixation Interventions: Diazepam (msucle spasms) Skin Pin site care Adjust by adding or subtracting weight Countertraction: backward force provided by body weight Increase by elevating foot of bed Frictional force: provided by patient’s contact with the bed NURSES DO NOT LIFE IS DISCONTINUE WEIGHTS Compartment syndrome: Do NOT elevate Bivalve cast Fasciotomy Administer fluids if needed Would you need to notify the provider? Review questions: Congenital Dislocation of Hip & Developmental Dysplasia of Hip (DDH) Diagnosis is made after birth Tests are reliable from birth Barlow If pressure from the front femoral had slips out over posterior lip of acetabulum then slips back when pressure is released the hip is unstable Ortolani sign: disappears when adduction contractures develop at about 6-10 weeks Middle finger over greater troch and thumbs opposite of lesser torch, abduct knees and put forward pressure behind tronchator than backward pressure If femoral slips forward into acetabulum it is dislocated Limb shortening Asymmetric thigh and gluteal folds 3 degrees of DDH pg. 986 Acetabular dysplasia Mildest form Neither subluxation nor dislocation is present Delay in acetabular development Femoral head remains in the acetabulum Subluxation: Largest percentage of DDH Implies incomplete dislocation of the hip Femoral head remains in the acetabulum but a stretched capsule and ligamentum teres cause the head of the femur to be partially displaced Dislocation: Femoral head loses contact with the acetabulum and is displaced posteriorly and superiorly over the fibrocartilaginous rim Ligamentum teres is elongated and taut Pavlik Harness Treatment is started as soon as diagnosis is made Early treatment leads to a favorable outcome Delayed treatment leads to complications Pavlik harness for infants Traction Closed reduction or open reduction Difficulty healing past age 4 years Therapeutic management of DDH Importance of early intervention Birth- 6 months: pavlik harness for abduction of hip Ages 6-24 months: dislocation unrecognized until child begins to stand and walk Treatment: closed reduction and spica cast Older child: operative reduction, tenotomy, osteotomy Correction is very difficult after 4 y/o- may use Bryant’s traction Club Foot Casting is started soon after birth Involves manipulation of foot for gradual stretching of skin and ligaments on the medial side of the food Correction by casting may be done every few days at first- then every 1-2 weeks Extremity remains in cast until maximum correction is achieved: 8-12 weeks X-rays: evaluate the alignment of the bones Surgery: 6 months- 1 year if casting fails 3 stages of management Correction of deformity Maintain correction until muscle balance is regained Frequent follow up care to monitor for recurrence of deformity Slipped Capital Femoral Epiphysis: Occurs mostly in boys ages 12-13 years during rapid growth period Increased weight causes movement of femoral neck from the femoral head Symptoms: limp, pain, altered gait, loss of abduction, internal rotation, limb shortening groin Treatment: bed rest to avoid further damage and buck’s traction Surgery: should be done within 24 hours to prevent avascular necrosis Post operative care: non weight bearing initially Scoliosis: Spinal deformity involving: Lateral curve Spinal rotation Thoracic hypokyphosis Congenital Screen in school age (during preadolescent growth spurt) Can develop in conjunction with other diseases: spina bifida and cerebral palsy Body image is important to the adolescent experiencing this Early stage: may have NO pain Degrees of scoliosis: 10 degrees postural variation 20 degrees or less:may not require intervention 40 degrees or more: typically require surgery Scoliosis Diagnosis: Scoliosis screening: school age With child in only underwear- observe the back Asymmetry of hips, scapula, shoulder height Child bends at waist- trunk parallel to floor and arms hanging free Asymmetry of rubs, flank, or widened angle between arm and body is noted Hold hips stable and have child turn left/ right Flexibility of curve is assessed X-rays: determine skeletal maturity Degree of curvature- not all spinal curves are scoliosis Treatment of Scoliosis Exercise Bracing (skin care) Boston, TLSO, Milwaukee (used for kyphosis) Braces are worn 23 hours per day Not curative Bracing slows or stops the progression of the curvature until child reaches skeletal maturity Surgery: Depends on degree of curvature When exercise and brace management fails Posterior and anterior approaches Instrumentation TLSO brace Surgical Preparation for Scoliosis: Major surgery Usually done during the summer Activity restriction: spine is fused and rods may be used Spinal fusion is more common and less ins=vasive than rod insertion Inform child about procedure and what to expect postop Bed rest Log rolling PCA/ pain control SCDs Frequent assessment Foley occasionally Best outcomes occur with early mobility Brace may or may not be used VEPTR: done for younger children with sever scoliosis Lengthens ribs by using a prosthetic rib that can be lengthened as the child grows Post op complications: bleeding, wound infection, duodenal compression Osteomyelitis- could be complication of fracture Infection: acute hematogenous osteomyelitis S. Aureus most common (presents as Group B strep in neonates) H.flu: less common bc of vaccine Salmonella in sickle cell children Neisseria gonorrhea in sexually active adolescents Occurs at any age- more common in 10 y/o or younger Severe pain- child will guard extremity, refuse to walk or move arm Fever, irritability Tenderness at site Signs of local inflammation may be present Symptoms may be menial in infants Labs to monitor: CBC: elevated Diagnostic tests: x-rays, CT, MRI, CBC, aspirate, bone scans Management of Osteomyelitis Acute stage: child is on bed rest, affected limb immobilized Pain meds, sedation, temperature, and anorexia Return of appetite coincides with resolution of the acute phase Need to occupy child's day as they will continue bed rest IV antibiotics: PICC lines Can go home with PICC line Surgical debridement of dead bone Drain to instill ATB directly to area CT scans: can show increase blood flow to site and later soft tissue Juvenile Idiopathic Arthritis (JIA/ JRA) Etiology is unknown Autoimmune more common in girls Inflammation begins in joints Pain, swelling, scar tissue (limited range of motion) Classified into a group of diseases depending on joints affected, systemic factors, and presence or absence of rheumatoid factors Rash, fever, lymphadenopathy, splenomegaly, hepatomegaly, limp, favoring of extremity, pain, uveitis New Nomenclature of JIA 16 years or younger Symptoms longer than 6 weeks Only 10% have positive rheumatoid factor JIA Pharmacological treatments: NSAIDS- Ibuprofen and Aspirin Methotrexate Corticosteroids Biologic agents: Etanercept and Adalimumab Avoid viral illness because using aspirin with a viral illness can cause Reye’s syndrome JIA Management Goal: preserve function, limit abnormalities, and relieve symptoms PT, relieve pain, use moist heat, exercise, promote general health and nutrition Remissions and exacerbations Early onset, better long-term prognosis 60% have total remission by adulthood Muscular Dystrophy: Box 49.8 and 49.9 S/S: fatty muscles, push themselves to get off floor (Gower’s sign) Many children are in wheelchair by school age X-linked disease, gradual, progressive degeneration of the muscle fibers Causing progressive weakness and muscle wasting Duchene: most severe type with bad outcomes S/S: waddles, lordosis, frequent falls, Gower’s sign Mild to moderate mental impairment Decrease in verbal skills r/t oher children Higher emotional disturbance Delayed motor development Muscular Dystrophy continued: Onset usually 3-5 y/o Ambulation impossible by approximately age 12 Confined to wheelchair Complication include: Contractures, obesity, respiratory, cardiopulmonary, disuse atrophy Terminal stages: respiratory, oropharyngeal, and facial muscles are affected Resp infection or heart failure is the cause of death Muscular Dystrophy Diagnostic tests: DNA analysis Serum CK: elevated levels in first 2 years of life EMG Biopsy Nursing Care of Muscular Dystrophy: Prevent contracture deformities Keep active as long as possible (allow for rest periods as needed) Avoid wheelchair confinement in early stages Then do passage ROM, soft casts, surgery Position changes when in wheelchair: allow for time to lay flat Use a multidisciplinary approach Family needs support: Encourage normalcy As condition progresses- make changes EX: don’t make child use wheelchair while they are still able to walk DMD: chronic fatal disease (family will be grieving) Guild from passing on disease to child Genetic counseling for female relatives and siblings Management of MD is symptomatic to assist with maintaining the highest level of mobility and prevention of complications as the disease progresses. Other limb defects: Genu varum: knees apart (bowleg) Refer if continues after 2 y/o or worsens after 1 y/o Genu valgum: knees together (knock knees) Treatment: exercise, splint or cast Refer is persists after 7 years Osteogenesis Imperfecta Disease of connective tissue S/S: scoliosis, kyphosis, bowed extremities Type 1a Blue sclera Hearing loss Type 2 Still born Fractures at birth Type 3 Severe bone fragility leading to deformities No cure: bone marrow is only experimental Manage fractures and educate parents Stress is caused by the confusion with child abuse Autosomal dominant type 1 or autosomal recessive Nonaccidental trauma needs to be ruled out Therapeutic management of Osteogenesis: Primarily supportive care IV bisphosphonate therapy May rule out osteogenesis imperfecta if multiple fractures occur Rehabilitative approach for prevention of further complications Positional contractures and deformities Muscle weakness and osteoporosis Malignant of lower extremity joints Legg- Calve Perthes Box 48-7 Self timing deterioration/ necrosis of femoral head- goal is to keep femoral head in acetabulum Effects ages 2-12 years of age Can resolve by 18 months to several years This can alter their physical activities Come activities can be resumes with use of a brace Presents with mild hip pain Limping, limited ROM, pain Diagnosed through x-ray Treated with rest, braces to displace femoral head and allow to heal, NSAIDS, home traction Stages Avascular stage: may need brace and immobilization Revascularization Reossification Regenerative Osgood Schlatter Disease Typical: very active adolescent, pain at tibia tubercule- knee pain Self limiting- rest, ice, heat, NSAIDs Hamstring stretches PT Practice Questions: Which of these nursing interventions are important to prevent complications in a child with a femur fracture ? Use an incentive spirometer every 2 hours. Limit caloric intake. Take a stool softener daily. Adjust weights to sustain traction. \ ROM on unaffected extremities 4 times a day. What is the appropriate action for a client with a long leg cast whose assessment reveals that the foot is pale and cool to touch, right leg pain severe unrelieved by acetaminophen with codeine? Apply heating pad to right toes. Administer IV pain medication. Remove the cast immediately. Notify the provider. MODULE 8B: INTELLECTUAL DISABILITY (3) Illustrate the use of care collaboration for the pediatric patient and family. Develop a basic plan of care for a child with a multisystem complex disorder. Create a plan of care to address the special health needs of a child. Begin to apply leadership and care coordination in the planning of care. Children with Special Health Care Needs (CSHCN): Increased risk for chronic physical, developmental, behavioral, or emotional condition Also require health and related services beyond that required by children generally Cerebral Palsy: non-progressive injury to the motor centers of the brain causing neuromuscular problems of spasticity or dyskinesia May be associated with intellectual disabilities and seizures: Anoxic injury before, during, or after birth Maternal infections Kernicterus: high levels of unbound bilirubin Yellow staining of brain cells may result in encephalopathy Low birth weight Trauma Upper motor neuron disease Risk factors: Prematurity (most common) Anoxic injury Intrauterine insults Perinatal infection (i.e. TORCH) Meningitis Brain malformations NAT Diagnosis: first 1-2 years of life Assessment Findings: Dyskinetic: Twisting movements Tremors, inconsistent muscle tone, impaired voluntary muscle control Exaggerated posturing Spastic: Rigidity, hypertonia Exaggerated DTR, primitive reflexes, contractures, and spinal deformities Nursing Assessment: Persistent neonatal reflexes after 6 months Moro, tonic neck Delayed developmental milestones Apparent early preference for one hand Poor suck/tongue thrust Spasticity: trouble diapering Scissoring of legs Involuntary and/or asymmetrical movements Seizures Could have normal intelligence - know your patients’ baseline Nursing Care and Interventions: Identify high risk groups Education regarding long term care Multidisciplinary interventions Speech, PT, occupational, surgery, med, care coordination, orthotics Maximize abilities (early intervention) Prevent intervention Medications: look up rationales Baclofen group A Diazepam group B Botulism toxin A group C Anti-epileptics group D, E Orthotics Concerns: Contractures Fractures Upper extremity deformities Hip subluxation/dislocation Spinal deformities Foot deformities Gait disorders Personnel: Orthopedic surgeon NP, research nurse PT, OT, speech Adaptive equipment specialist Social work Seating and mobility specialists Orthotist Sensory Impairment in Children Hearing Impairment: From slight to profound Deaf: a person whose hearing disability precludes processing linguistic information with or without hearing aid Hard of hearing: generally able to hear with hearing aid For children: 6-19 y/o Profound deafness likely to be diagnosed in infancy Entry into school Concerns with speech development Prevention of Hearing Loss: Treatment and management of recurrent otitis media (ear infections) Prenatal preventative measures Avoid exposure to loud noise Early screening Promoting Communication: Lip reading Cued speech Sign language Speech language therapy Socialization Additional aids Reassess understanding of instructions given Supplement with visual and tactile media Communication devices: Picture board Common words and needs (food, water, toilet) Visual Impairment: Visual loss that cannot be corrected with regular prescription lenses Classification: Partially sighted: Acuity of 20/70 to 20/200 Education usually in public school system Legal blindness: Acuity of 20/200 or less Legal as well as medical term Etiology: Perinatal or postnatal infections Gonorrhea, Chlamydia, rubella, syphilis, toxoplasmosis Retinopathy or prematurity Perinatal or postnatal trauma Other disorders and unknown causes Other Visual Impairments: Refraction: bending of light rays through the lens of the eye Myopia: nearsighted Hyperopia: farsighted Strabismus: Testing important in children: cover uncover, corneal, light reflex Treatment: vision occlusion therapy, surgery Amblyopia Astigmatism: unequal curves Nursing Assessment: Identify children at risk Observe behaviors that indicate vision loss Screen all children for visual acuity Nursing Alert: Suspect visual impairment in a child for any age whose pupils do not react to light Care Management: Promote parent-child attachment Promote optimal development Development and independence Play and socialization Educate: Braille Audio books and learning material Provide a safe environment Provider reassurance during all treatments Orient child to surroundings Encourage independence Treatment team members should be consistent Prevention Periodic screening of all children, Rubella immunizations Intellectual Disability (ID): Significant limitations in intellectual functioning and adaptive behavior (conceptual, social, practical) Before age 22 Functional impairment in 2 or more areas: Communication Self-care Home living Social skills Use of community resources Self-direction Health and safety Functional academics Leisure Work IQ Scores: Educable/mild: 50-75 Trainable/moderate: 36-49 Severe: 20-35 Profound: < 20 Diagnostic Tests for Development: Bayley scales of infant development Mullen scale of early learning Denver developmental screening Harris infant Neuromotor (HINT) Wechsler intelligence scale and Kaufman Assessment Battery from toddlerhood through school age Leiter international performance scale for children who speak a different language, nonverbal children, or those with significant language or motor impairment Individually tested by specially trained clinicians Developmental Disability Definition severe , chronic disability which: Originated at birth or childhood Expected to continue indefinitely Substantially restricts the individual’s functioning in several major life activities Manifested before age 22 Results in substantial functional limitations in 3 more of the following major life activities Self care Receptive and expressive language Learning Mobility Self direction Capacity for independent living Economic self sufficiency Etiology of ID: familial , social, environmental, organic, and unknown causes Prenatal chromosomal disorders Down syndrome Fragile x syndrome Prenatal toxin exposure, fetal alcohol syndrome, infection Perinatal: prematurity or birth injury Postnatal CNS infection, brain injury, environmental factors Other identifiable disorders or syndromes: Severe cerebral palsy Microcephaly Primary prevention of ID Avoidance of prenatal rubella infection- keep immunizations up to date Genetic counseling, especially regarding risk of down or fragile x syndrome Use of folic acid supplements Education regarding the dangers of smoking or alcohol use during pregnancy Reduction of head injuries Future gene therapy for genetic disorders, such as PKU Secondary Prevention of ID: Early identification to initiate treatment to avert damage Prenatal diagnosis or carrier detection of disorders such as down syndrome Newborn screening for treatable inborn errors of metabolism Congenital hypothyroidism Phenylketonuria (PKU) Galactosemia Continued monitoring of developmental milestones Tertiary prevention of ID: Treatment to minimize long term consequences Early ID of therapies and rehabilitation services Treatment of coexisting problems Programs for infant stimulation, parent training, preschool education Counseling services to preserve the family unit ID and Co- morbidities: Seizure disorders ADHD Psychiatric disorders Behavior disorders Depression Atypical psychosis FTT, GI, and respiratory disorders GERD Aspiration pneumonia Swallowing problems leading to poor oral intake Vision impairments Communication disorders Diagnostic Evaluation to identify cause of ID Genetic testing Metabolic screening CT MRI Functional MRI PET scan Heavy metal assay (lead, mercury, arsenic) Early behavioral signs of cognitive impairment or ID No response to contact, voice, movement Irritability poor/ slow feeding Poor eye contact during feeding Diminished spontaneous activity Persistent primitive reflexes Interpret and respond: Development of communication Receptive skills Expressive skills Sign language Message boards Computer Discipline: Simple, consistent, limit setting Must be appropriate for child’s mental age Control measures based on specific behavior Behavior modification Reinforcement of desired actions Use of time out Play, exercise, and socialization Benefits of exercise Development of coordination Cardiovascular fitness Weight management May not be able to initiate appropriate play activities on their own Enhance socialization Acquisition of social skills Active rehearsal Positive reinforcement for desired behavior Sexuality: parental concern Promoting independence Parents may need assistance to promote independent self- help skills Feeding, toileting, dressing, and grooming Socialization Early intervention programs Promote skills Care of child during hospitalization Detailed Hx with special focus on self care abilities Assess developmental age Communication with child appropriate to cognitive or developmental level Involve parents Make efforts to involve the child Utilize child life Down Syndrome: Down syndrome- Care coordination Phenotypic pattern in neonates Poor moro reflex Hypotonia Slanted palpebral fissures Hypoplasia of fifth finger Brushfield’s spots Simian crease Cardiac abnormality (40-50%) Etiology is unknown Most common chromosome abnormalities (1 in 800-1000 births) Most common genetic cause of cognitive impairment (CI) Maternal age plays a factor 80% of infants with DS born to mothers < 35 y/o Age 35+ incidence approximately 1 in 350 Age 40+ incidence approximately 1 in 100 Paternal age as factor Less than 5% of cases extra chromosome is from the father if > 55 y/o Down syndrome IQ: Wide variation from severely CI to low average intelligence Generally mild to moderate CI Initial development may appear normal Social development may be 2-3 years> mental age May be related to parental IQ Often initial development appears near- normal Notice- DS Congenital Anomalies Renal, Hirschsprung, tracheoesophageal fistula Altered immune function Musculoskeletal: Muscles: hypotonic Joints: hyperextensible Atlantoaxial instability: 15-20% Instability of 1st and 2nd cervical vertebrae Concern with sports activities Stress on head and neck= spinal cord compression May require surgical intervention Sensory up to 75% hearing issues and visual issues Growth: weight vs. height- Please regularly plot height and weight on charts Sexual development Rule out: Congenital heart defects: 50% of children with DS Most common: septal defects Hypothyroidism: 10% Small intestinal blockage: 4% Rare form of leukemia: 1% Altered immune function= early senescence (aging) Life expectancy for DS: 80% to age 55+ Remains lower than the general population Assist the family in preventing physical problems positioning : poor muscle tone Feeding Attachment Upper respiratory tract and ear infections Nutrition Classic manifestations of DS upward/ outward slant to eyes Palmar creases Small nose with flat nasal bridge High arched palpate Excess skin in neck folds Plantar crease between big and 2nd toe Wide space between big and 2nd toe Hyperflexible hypotonia/ muscle weakness Newborn Assestment: Separated sagittal suture Oblique palpebral fissures Depressed nasal bridge Protruding tongue Transverse palmar creases Autism: Expected findings: Delays in social interaction, communication, and imaginative paly before age 3 years Impaired communication Avoidance of eye and physical contact Short attention span Rhythmic movements Attached to routines Autism Nursing Care Screening Early intervention Behavior modification Decreased environmental stimuli Introduce new situations slowly Involve parents Promote consistency in care giving Ensure safety Learning Disability (LD): Heterogenous group of disorders with difficulties in acquisition and sue of listening, speaking, reading, writing, reasoning, math, and/ or social skills Includes dyslexia, dysgraphia, dyscalculia, right/left confusion, and short attention span Diagnostic testing for ADD, ADHD, and LD IQ Hand eye coordination Visual and auditory perception Comprehension Memory Management: Primarily education intervention Wide variation of diagnostic severity ADHD: Attention deficit hyperactivity disorder: Etiology unknown- probably multifactorial Inattention, impulsiveness, hyperactivity Typical onset before age 7 Diagnostic criteria for ADHD Therapeutic Management of ADHD Classroom Control overstimulation Vary learning activities Family education and counseling Behavioral therapy and or psychotherapy for child Environmental manipulation: not too many distractions Medication Medication for ADHD Not all children benefit from pharmacologic therapy Stimulants: Dexedrine, Adderall Ritalin, Methylphenidate Side effects: Insomnia Anorexia Weight loss Hypertension Long term use may suppress growth Developmental Delay (DD): Any significant lag or delay in physical, cognitive, behavioral, emotional, or social development Most common delays: Language and cognitive skills Fine and gross motor skills Emphasize abilities, environments, supports, and empowerment Classification of support required Intermittent, limited, extensive, or pervasive The underlying assumption- function improves with support GOAL: to promote optimum social, physical, cognitive, adaptive development as individuals within a family and community