Pediatrics Unit 2 Study Guide Respiratory infection Described according to anatomic area of involvement Upper respiratory tract consists

onsists of oronasopharynx, pharynx, larynx, and upper part of trachea Lower respiratory tract consists of lower trachea, mainstem bronchi, segmental bronchi, subsegmental bronchioles, terminal bronchioles, and alveoli Infections of epiglottis and larynx categorized as croup syndromes

Often spread from one structure to another due to contiguous nature of mucus membrane lining entire tract Account for majority of acute illnesses in children Infants are nose breathers < 6 years old most common breathing pattern = diaphragmatic RSV can cause severe respiratory compromise in infants 5 years old = very susceptible to upper respiratory infections (URIs) 6 years old = bacterial infections Size Smaller child = smaller airway Diameter of airways is smaller in young children = risk for considerable narrowing from edematous mucus membranes and production of secretions Distance between structures within respiratory tract is shorter in young child organisms may move rapidly down respiratory tract = extensive involvement Relatively short and open eustachian tube in infants and young children allows pathogens easy access to middle ear Resistance Deficiencies of immune system place child at risk for infection resistance due to malnutrition, anemia, fatigue, and chilling of body Allergies, preterm birth, broncho-pulmonary dysplasia (BPD), asthma, history of RSV infection, cardiac anomalies, cystic fibrosis (CF) Day care attendance Smoke in home Seasonal Variations Most common season for URI = late fall Most common respiratory pathogens appear during winter and spring months Mycoplasmal infections occur more often in autumn and early winter Infection-related asthma occurs more frequently during cold weather RSV typically occurs during winter and spring

Most infections caused by viruses respiratory syncytial virus (RSV), adenoviruses, parainfluenza viruses, pneumonia

Etiology influenced by age of child, season, living conditions, and preexisting medical problems Age

< 3 months = infection rate vs. older children due to protective function of maternal antibodies 3 6 months = infection rate (maternal antibodies disappear and infant develops own antibodies) Viral infection rate remains during toddler and preschool years

Manifestations infants and young children, especially between 6 months 3 years, react more severely to acute respiratory tract infection vs. older children Respiratory Sounds Sore Throat Frequent complaint of older children Young children are unable to describe symptoms may not complain even when highly inflamed Child will often refuse to take oral fluids or solids Anorexia Common with most childhood illnesses Frequently 1 evidence of illness Persists to a greater or lesser degree throughout febrile stage of illness Often extends into convalescence
st

Sounds associated with respiratory disease hoarseness, grunting, stridor, wheezing, cough Common feature = cough may be evident only during acute phase and can persist several months after disease Auscultation sounds wheezing, crackles, absence of breath sounds

Vomiting Typical with small children during illness clue to onset of infection (may precede other signs) Usually short-lived but may persist during illness Frequent cause of dehydration if fluid intake is impaired Nasal Small nasal passages of infants easily blocked by mucosal swelling and exudation Blockage can interfere with respiration and feeding in infants and may contribute to development of otitis media and sinusitis Frequent occurrence of nasal discharge Discharge may be thin and watery (rhinorrhea) or thick and purulent depends on type or stage of infection Nasal discharge is associated with itching and may irritate upper lip and skin surrounding nose Nursing interventions

Diarrhea Usually mild, transient diarrhea but may become severe Often accompanies viral respiratory infections Frequent cause of dehydration Meningismus Meningeal signs without infection of meninges Occurs with abrupt onset of fever Signs and symptoms = headache, pain + stiffness in back and neck, Kernig and Brudzinski signs Kernig sign flexing o patients hip 90 then extending patients knee causes pain Brudzinski sign flexing patients neck causes flexion of patients hips and knees

Abdominal Pain Common complaint sometimes indistinguishable from pain of appendicitis May be caused by mesenteric lymphadenitis Can be caused by muscle spasms from vomiting Fever May be absent in newborn infants Greatest at ages 6 months 3 years Temperature may reach 103 105 F even with mild infections Often appears as 1 sign of infection May be listless and irritable or activity Tendency to develop high temperatures with infection in certain families May precipitate febrile seizures uncommon after 3 4 years old
st

Subsides as temperature

Monitor for signs of respiratory distress = tachypnea, stridor, coughing, wheezing, retractions, grunting, nasal flaring, accessory muscle usage Assess O2 levels pulse oximetry, color (cyanotic, pallor) Assess childs LOC, response to stimuli, and hydration status (insatiable loss from breathing) Respirations Rate rapid (tachypnea), normal, or slow for particular child Depth normal depth, too shallow (hypopnea), too deep (hyperpnea) Ease effortless, labored (dyspnea), orthopnea (difficult breathing except in upright position), associated with intercostal or substernal retractions, flaring nares, head bobbing, grunting, or wheezing Labored breathing continuous, intermittent, becoming steadily worse, sudden onset, at rest or on exertion, associated with wheezing or grunting, associated with pain Rhythm variation in rate and depth of respirations Other Observations Evidence of infection look for temperature, enlarged cervical lymph nodes, inflamed mucus membranes, purulent discharges from nose, ears, or lungs (sputum) Cough under what circumstances cough is heard (e.g., night only, on arising), nature of cough (paroxysmal with or without wheeze, croupy or brassy), frequency , associated with swallowing or other activity, character of cough (moist and dry), productivity Wheeze expiratory or inspiratory, high-pitched or musical, prolonged, slowly progressive or sudden, associated with labored breathing Cyanosis distribution (peripheral, perioral, facial, trunk, and face), degree, duration, associated with activity Abdominal pain seen with preschooler and school-age children, referred pain from chest Chest pain seen with older children, localized or generalized, referred to base of neck or abdomen, dull or sharp, deep or superficial, associated with rapid, shallow respirations or grunting Sputum volume, color, viscosity, and odor Bad breath may be associated with some lung infections

Ease respiratory efforts warm or cool mist is a common therapeutic measure for symptomatic relief of respiratory discomfort Moisture soothes inflamed membranes and is beneficial with hoarseness or laryngeal involvement Cool vaporizers in home is better than warm vaporizers due to risk of burns from steam Can also run hot water shower to produce warm mist

Promote rest acute febrile illness = place on bed rest Promote comfort saline nose drops, nasal irrigation, vasoconstrictive nose drops or sprays DO NOT administer nose drops or sprays for > 3 days can cause rebound nasal constriction Prevent spread of infection use a tissue or hand to cover nose and mouth when coughing or sneezeing, dispose of tissues properly, frequent handwashing, do NOT share cups/washcloths/towels temperature acetaminophen (or ibuprofen if child > 6 months) and administer cool liquids Promote hydration dehydration is a potential complication when children have respiratory tract infections and are febrile or anorexic, especially when vomiting or diarrhea is present Infants are especially prone to fluid and electrolyte with respiratory illness Rapid respiratory rate precludes adequate fluid intake Fever total body fluid turnover in infants Nasal secretions can block narrow nasal passages when infant reclines to feed stops compensatory mouth breathing effort = intake of fluids Offer oral rehydration solutions Infalyte or Pedialyte (for infants) or Gatorade (for older children)

AVOID high-calorie liquids (soda, fruit juice), fluids with caffeine (tea, coffee)

Breastfeeding infants should continue to be breastfed because human milk confers some degree of protection from infection Fluids should NOT be forced Assess child's level of hydration by observing frequency of voiding

Provide nutrition loss of appetite is characteristic of children with acute infections

Nasopharyngitis upper respiratory tract infection equivalent of common cold Most common respiratory illness Commonly caused by virus rhinovirus, RSV, adenovirus, influenza virus, and parainfluenza virus Symptoms are more severe in infants and children vs. adults Fever, irritability, restlessness, sneezing, coughing, nasal congestion, anorexia, general malaise Antipyretics for mild fever and discomfort NO aspirin! Rest recommended until child is free of fever for at least 1 day Decongestants may be used for children > 5 years of age to shrink swollen nasal passages Affect ALL vascular beds give with caution to children with diabetes DO NOT give oral decongestants to infants < 6 months Used for MAXIMUM of 3 days Treatment managed at home

Cough suppressants may be prescribed for a dry, hacking cough in older children Antihistamines ineffective in treatment of nasopharyngitis Prevent spread of infection frequent handwashing, avoid contact with affected persons Hydration essential to maintain adequate fluid intake Earache Fever > 38.3 C (101 F) irritability, crying Respirations > 50 60 breaths/min, wheezing Persistent cough for 2 days Restlessness and poor sleep patterns

Early evidence of respiratory complications

Tonsillitis inflammation of palantine tonsils (masses of lymphoid tissue located in pharyngeal cavity) Tonsils filter and protect respiratory and alimentary tracts from invasion by pathogenic organisms and play a role in antibody formation Children generally have much larger tonsils than adolescents or adults Proximity to nares and eustachian tubes causes difficulties in instances of inflammation Etiology tonsillitis often occurs with pharyngitis Can be caused by virus or bacteria Tonsils and pharynx may be inflamed and covered with exudates Palatine tonsils enlarge from edema = obstructs passage = difficulty swallowing and breathing Adenoids enlarge = space behind posterior nares becomes blocked = difficult or impossible for air to pass from nose to throat = child must breathe through mouth Throat culture should be performed to rule out GABHS (Group A Beta Hemolytic Streptococcus) GABHS infection of upper airway (aka strep throat) = risk for rheumatic fever (lead to heart and joing complications) and acute glomerulonephritis (kidney infection) Manifestations caused by inflammation

Nodes are often tender (due to pharyngitis) pain can be relatively mild to severe enough to make swallowing difficult Thick, nasal voice + pain Administration of antibiotics, if bacterial in origin children should NOT return to school until they have been taking antibiotics for a full 24 hours Oral erythromycin is indicated for children allergic to penicillin Azithromycin, clarithromycin, oral cephalosporins, amoxicillin, and amoxicillin with clavulanic acid Advise parents that entire dose of antibiotics MUST be completed Discard toothbrush and replace it with a new one after taking antibiotics for 24 hours Prevent sharing of drinking or eating items to prevent spread of infection

Treatment tonsillitis is usually self-limiting, treat symptoms

Warm saline gargles offer relief of throat discomfort Tonsillectomy = surgical removal of palatine tonsils Indicated for recurrent tonsillitis (2 3 occurances in a year), peritonsillar abcess, airway obstruction, sleep apnea, or recurrent ear infections Recommended for hypertrophied adenoids that obstruct nasal breathing, recurrent adenoiditis and sinusitis, otitis media (OM) with effusion, sleep apnea, and recurrent rhinorrhea

Adenoidectomy = surgical removal of adenoids (aka pharengeal tonsils)

Nursing interventions provide comfort and activites or interventions that precipitate bleeding Soft to liquid diet is preferred Cool-mist vaporizer keeps mucus membranes moist during periods of mouth breathing Opioids may be needed to pain for child to drink Preoperative interventions Teach what to expect after surgery, preoperative requirements, lab work Check for any loose teeth poses aspiration risk if knocked loose during surgery Place child on abdomen or side until fully awake facilitates drainage of secretions Careful suctioning only when needed AVOID trauma to oropharynx AVOID gargling, coughing, clearing throat, or blowing nose aggravates operative site Assure parents that dark brown (old) blood is usually present in emesis, in nose, and between teeth Anticipate low grade fever, slight ear pain, and mouth odor for 1 few days (due to healing process)
st

Postoperative interventions

Throat will be sore after surgery provide analgesics at regular intervals (tendency is to undermedicate kids), ice collar, anesthetic pops Food and fluids are restricted until children are fully alert and there are no signs of hemorrhage fluids = healing (keep child hydrated!) Start with liquids the move to soft solids NO citrus, spices, hot food, irritating foods AVOID any red or brown colored fluids (need to be able to distinguish fresh or old blood) Milk, ice cream, and pudding are usually NOT offered coat mouth and throat with mucus and may cause child to clear throat = can initiate bleeding Observe throat directly for evidence of bleeding first 24 hours after surgery, then again 7 10 days after surgery (when scab falls off) Most obvious early sign of bleeding = child's continuous swallowing of trickling blood Signs of hemorrhage tachycardia, pallor, frequent clearing of throat, vomiting of bright red blood, restlessness, blood pressure (late sign of shock) Airway obstruction can occur due to edema or accumulated secretions Look for signs of respiratory distress stridor, drooling, restlessness, agitation, respiratory rate, and progressive cyanosis

Monitor vital signs look for signs of bleeding and shock

Otitis Media inflammation of middle ear Incidence is highest in winter months many cases of bacterial OM are preceded by a viral respiratory infection (RSV or influenza) Etiology one of most prevalent diseases of early childhood Most episodes occur between 6 months 2 years old incidence with age, except for a small at age 5 6 years when children enter school Occurs infrequently in children > 7 years old

risk for boys, children in large households, presence of smoke in home, siblings with history of OM Can be caused by bacteria or by blocked eustachian tubes from edema, URIs, allergic rhinitis, hypertrophic adenoids Relationship with infant feeding methods Breast milk = incidence vs. formula-fed infants Semivertical feeding position = incidence incidence of OM if bottle left in bed with infant

Pathophysiology primarily a result of bacteria and/or malfunctioning eustachian tubes which causes obstruction Diagnosis is made via visual inspection of tympanic membrane Serous or purulent discolored fluid line (effusion) Membrane is bulging or full, opacified, reddened, immobile and bony landmarks are not visible Acute Otitis Media Definition Causative Agent Manifestations Sudden, rapid onset Lasts 3 weeks Strep, RSV, influenza frequently occurs after URIs Eustachian tube dysfunction Irritability, otalgia (earache), high fever Pulling at ear, drainage Purulent discharge (otorrhea) may or may not be present Poor feeding Hearing loss (most frequent) Difficulty communicating Feeling of fullness in ear Tinnitus, vertigo Frequently an extension of an acute episode of OM Chronic Otitis Media Lasts > 3 weeks

Treatment most are viral in nature, therefore there is movement away from giving antibiotics Broad spectrum antibiotics given amoxicillin, Septra, erythromycins take all doses! Should wait up to 72 hours for spontaneous resolution before taking antibiotics ALL cases of AOM in infants < 6 months old should be treated with antibiotics due to infant's immature immune system NOT recommended for children < 2 years old who have persistent acute symptoms of fever and severe ear pain

Analgesic-antipyretic (acetaminophen or ibuprofen if > 6 months old), warm compress to ear, lying on affected side to promote drainage Antihistamines and decongestants are NOT recommended Myringotomy surgical incision of eardrum used to provide continuous drainage of infected middle ear fluid Can be done with or without tympanostomy tube placement and/or adenoidectomy NOT recommended for children < 3 years old unless there is pathology NOT used for initial management of OM! Can cause child to hear much better, but sounds can be very loud talk in muted voice so child is not scared Child will have low-grade fever (< 100.4 F) for 24 48 hours postoperatively Advise parents of grommet appearance (usually a tiny, white, plastic spool-shaped tube) so they can recognize it if it falls out normal, requires no immediate intervention (but should still advise HCP)
o

Croup syndrome croup = general term used for symptom complex that can affect larynx, trachea, bronchi, and epiglottis Characterized by Hoarseness Resonant cough described as barking or brassy (croupy) Varying degrees of inspiratory stridor Varying degrees of respiratory distress resulting from swelling or obstruction in larynx Laryngotracheobronchitis and epiglottitis DO NOT occur together Epiglottitis Laryngotracheobronchitis

Acute infections of larynx in infants and small children = risk for significant narrowing with inflammation

Definition

Serious obstructive inflammation of epiglottis that covers airway Medical emergency requires IMMEDIATE attention! Obstruction is supraglottic

Most common croup syndrome Usually preceded by a URI gradually descends to adjacent structures Typically occurs and/or worsens during night Subglottic obstruction of laryngitis

Epiglottitis Age Group Etiology Onset Signs & Symptoms 2 8 yr Usually H. influenzae Bacterial or viral Abrupt Can rapidly progress to severe respiratory distress NO spontaneous cough + NO hoarseness High fever (> 102 F) + toxic appearance Restlessness + irritability Thick, muffled voice + frog-like croaking sound on inspiration (stridor when supine) Drooling + red and inflamed throat + distinctive large, cherry-red, edematous epiglottis + pain on swallowing (dysphagia) Tripod position child insists of sitting upright and leans forward with chin thrust out (to expand neck), mouth open, tongue protruding Respiratory obstruction can appear suddenly Hypoxia, hypercapnia, acidosis followed by muscle tone level of consciousness Sudden death Keep child quiet, calm, and comfortable avoid invasive procedures Keep child NPO DO NOT inspect throat or stick anything into mouth if child is suspected to have epiglottisis! can cause complete airway obstruction (have intubation tray at bedside) DO NOT take child to x-ray Fluids + humidified O2 (cool mist) Monitor airway pulse ox, respiratory status Antibiotic therapy (if bacterial) 7 10 days High dose corticosteroids used to edema PO or nebulized (racemic) epinephrine
o

Laryngotracheobronchitis < 2 yr (boys > girls) Parainfluenza virus, RSV, Influenza A and Bi Mycoplasma pneumonia (microbacteria) Gradual onset Slowly progressive Barky, brassy cough (seal-like) + hoarseness Low-grade fever (< 100.5 F) + does NOT look toxic Restlessness + irritability Inspiratory stridor problem getting air IN vs. wheezing, which is problem getting air OUT Suprasternal retractions due to narrowing of airway from inflamed mucosa (dyspnea) Stage 1 (hoarseness, croupy cough, some agitation) can be treated at home need to treat in hospital if advances to stage 2 4 Respiratory distress nasal flaring, intercostal retractions, tachypnea, and continuous stridor (stage 2) Hypoxia, anoxia, hypercapnia, acidosis (stage 3) Cyanosis, respiratory failure (stage 4) Keep child calm Elevate head of bed ( breathing ability), maintain airway, and provide adequate respiratory exchange Continuous, vigilant observation + accurate assessment of respiratory status High humidity + cool mist Nebulized (racemic) epinephrine upper airway bronchodilator used for severe disease, stridor at rest, retractions, or difficulty breathing Corticosteroids dexamethasone Fluids cool, clear liquids Educate parents on how to make child more comfortable
o

Complications

Treatment

Bronchiolitis most common acute viral infection that mainly affects bronchiolar level (lower airways) Etiology most cases caused by respiratory syncytial virus (RSV) also caused by adenoviruses and parainfluenza Primarily occurs in late fall through spring Spread by hand nose contact Mostly occurs in children < 2 years old rare in children > 2 years old Peak age = 6 months By age 3 years most children have been infected at least once with RSV severe RSV infections in st 1 year of life = risk for development of asthma up to age 13

Pathophysiology bronchiolar mucosa swells = lumina filled with mucus and exudate Obstruction in small air passages = hyperinflation, obstructive emphysema, and patchy areas of atelectasis Dilation of bronchial passages on inspiration allows for adequate intake of air (can breathe in) Narrowing of passages on expiration prevents air from leaving lungs air is trapped distal to obstruction = causes progressive overinflation (emphysema)

Manifestations usually begins with a URI Infants can have URI symptoms, slight lethargy, poor feeding, or irritability apena may be 1 recognized indicator of RSV infection in very young infants Initial Symptoms Rhinorrhea (thick clear to white discharge) Pharyngitis Productive cough + sneezing Wheezing (work of breathing) Intermittent low-grade fever (< 100.5 F) Possible otitis media and conjunctivitis o can cause fever > 100.5 F
o st

Progression of Illness coughing and wheezing Tachypnea, dyspnea Retractions Crackles Cyanosis

Severe Illness Tachypnea > 70 breaths/min Listlessness Apneic spells Poor air exchange breath sounds

Diagnosis tests done on nasal or nasopharyngeal secretions ELISA techniques used for RSV antigen detection Treatment manged at home with cool humidified O2, adequate fluid intake, airway maintenance via saline nose drops + nasal suction, rest, and medications Hospitalization usually recommended for children: 6 months old due to being obligate nose breathers (can cause apnea) With respiratory distress Who cannot maintain adequate hydration Have underlying lung or heart disease Whose home environment cannot adequately manage disease (exhausted parent)

Medical therapy for bronchiolitis is primarily supportive aimed at airway hyperresonance and inflammation and adequate fluid intake Bronchodilators may provide short-term benefits Corticosteroids and antihistamines NOT recommended for routine use Antibiotics are NOT part of treatment of RSV unless there is a coexisting bacterial infection (otitis o media, conjunctivitis) that causes temperature > 100.5 F If respirations > 60 breaths/min child is kept NPO due to risk for aspiration Drug is aerosolized and delivered via a small-particle aerosol generator Administered by mist tent for 18 hours/day for 7 10 days Teratogenic drug no pregnant women can be around it

Ribavirin only antiviral agent approved for hospitalized children

Prevention of RSV infection RSV is very contagious contact and standard precautions are used Frequent handwashing NO touching nasal mucosa or conjunctiva Use gloves and gowns when entering patient's room Make patient assignments so that nurses assigned to children with RSV are NOT caring for other patients who are considered high risk Children suspected RSV infection may be assigned separate rooms or grouped with other RSVinfected children RSV-IG IV med given monthly for 8 months Synagis IM med given monthly for 8 months

Prophylactic medications only given to children who are high risk (cardiac, premies, immunocompromised)

Asthma chronic inflammatory disorder of airways Most common chronic disease in children Etiology allergy and viral respiratory infections (RSV in infants) influence persistence and severity of disease Boys are affected more frequently than girls until adolescence, when trend reverses 1 asthma attack frequently occurs < 5 years old
st

Pathophysiology asthma results from complex interactions mediated by mast cells, eosinophils, histamine, and T lymphocytes present in airways Exaggerated inflammatory response to stimuli (hyperresponsiveness of airway) Allergens and environmental factors Physical, chemical, pharmacological agents Infection, URIs Stress psychsocial factors Exercise Hereditary runs in families, but NOT considered a genetic disease

Hyper-responsiveness of airways = bronchospasm shortening and narrowing of airway Spasm of smooth muscle of bronchi and bronchioles = caliber of bronchioles

Inflammation = airway edema + secretion of mucus Air and mucus becomes trapped in distended aveoli Hyperinflation of alveoli = effect of cough respiratory effort during expiratory phase of respiration (wheeze) Trapped O2 takes up lung space actelectasis causes no sound Person fights to inspire sufficient air due to trapped air Causes fatigue, respiratory effectiveness, and O2 consumption alveolar ventilation = CO2 retention, hypoxemia, respiratory acidosis, and eventually respiratory failure

Chronic inflammation may cause permanent damage to airway structures airway remodeling Provide a stepwise approach to pharmacologic management, environmental control, and educational interventions needed for each category Intermittent Mild Persistent > 2 days/week NOT daily < 2 times/month None < 2 days/week 3 4 times/month Minor limitation > 2 days/week NOT daily 2 3 times/week NOT nightly Some limitation Daily Moderate Persistent Daily Severe Persistent Continual symptoms despite taking meds Frequent Nightly Extremely limited (due to pulmonary function) Multiple times/day

Classified into 4 categories based on symptom indicators of disease severity

Frequency of Day Symptoms Frequency of Night Symptoms Interference with Normal Activity Use of Meds for Symptom Control

< 2 days/week

Manifestations main symptoms are dyspnea, wheezing, and coughing Cough Respiratory-Related Signs Shortness of breath expiratory phase (takes longer to breath out) with audible wheeze Malar flush, red ears, with lips deep dark red color (due to hypoxia) Possible progression to cyanosis of nail beds or circumoral cyanosis Restlessness, apprehension sweating as attack progresses May sit in tripod position upright with shoulders in a hunched-over position, hands on bed or chair, and arms braced Speak in short, panting, broken phrases Chest Hyperresonance on percussion (hollow drum) Coarse, loud breath sounds Bilateral wheezes throughout lung fields Prolonged expiration Crackles (with URI pneumonia) Retractions With Repeated Episodes Barrel chest AP diameter transverse diameter Elevated shoulders Use of accessory muscles of respiration (causes stomache to hurt) Facial appearance: flattened malar bones, circles beneath eyes, narrow nose, big upper teeth

Initially dry, hacking, nonproductive Paroxysmal attack comes on suddenly Irritative Becomes rattling Can eventually produce frothy, clear, gelatinous sputum

Retractions Mild Distress Isolated intercostal Moderate Distress Anything that starts with an S Subcostal, suprasternal, and supraclavicular Severe Distress Retractions + use of accessory muscles

Diagnosis determined primarily on basis of clinical manifestations, history, physical examination, and some labs Chronic cough in absence of infection or diffuse wheezing during expiratory phase of respiration is sufficient to establish a diagnosis Chest x-rays show infiltrated and hyperexpansion of airways, pneumonias or respiratory tract infections Pulmonary function tests (PFTs) evaluates presence and degree of impact on lung fields Peak expiratory flow rate (PEFR) measures maximum flow of air that can be forcefully exhaled in 1 second Early recognition of symptoms is crucial Overall goals of asthma management: Maintain normal activity levels and pulmonary function Prevent chronic symptoms and recurrent exacerbations avoid triggers and allergens + use meds Provide optimal drug therapy with minimal or no adverse effect used to underlying inflammation and or prevent symptomatic airway narrowing Patient and family education + monitoring severity of disease = correct course of therapy Explain pathophysiology to parents used of steroid + drink fluids = inflammation and mucus

Treatment continuous care approach with regular visits (at least every 1 6 months) to HCP

Medications used to prevent and control asthma symptoms, frequency and severity of asthma exacerbations, and reverse airflow obstruction Therapy is directed toward long-term suppression of inflammation Administered as PO meds or by inhalation with a nebulizer or a metered-dose inhaler (MDI) MDI (method of choice for kids) should always be attached to a spacer when: An inhaled corticosteroid is given to prevent yeast infections in mouth Children have difficulty coordinating or learning proper inhalation technique

2 general classes often used in combination Preventive Medications Long-term control medications Used to achieve and maintain control of inflammation Inhaled corticosteroids, cromolyn sodium and nedocromil, long-acting 2-agonists, methylxanthines, and leukotriene modifiers Indication 1 line drug used for treatment of acute exacerbations and prevention of exercise induced asthma Binds with 2-receptors to relax bronchial smooth muscle, dilate airways, and eliminate bronchospasm 1-effects ( heart rate and GI disturbances) have been minimized Bronchodilator used for long-term prevention of symptoms especially nighttime symptoms and exercise-induced bronchospasm Typically added to antiinflammatory therapy Antiinflammatory drug 2 line drug used to control chronic or acute inflammatory process Treats reversible airflow obstruction and bronchial hyper-responsiveness Inhibits mast cells + inhibits activation and release of mediators from eosinophil and epithelial cells Inhibits bronchoconstrictor response to inhaled antigens Used as daily maintenance therapy Bronchodilator + CNS stimulant Mainly used for adult COPD treatments NOT used as mainline management neurotoxic and can cause hypertension Mediate inflammation that cause in airway hyper-responsiveness Block inflammation and bronchospasms Used for long-term control and prevention of mild persistant asthma Used for relief of acute bronchospasm Significantly improves lung function
nd st

Rescue Medications Quick-relief medications Used to treat symptoms and exacerbations Short-acting 2-agonists, anticholinergics, and systemic corticosteroids Nursing Interventions Administered PO or via inhalation (MDI or aerosol) inhaled has more rapid onset of action (within minutes) Inhaled -adrenergic agents should NOT be taken > 3 4 times/day for acute symptoms Side effects irritability, tremor, nervousness, insomnia, tachycardia (except with Xopenex) Used twice daily (q12h) NOT used in children < 12 years old NOT used for acute symptoms or exacerbations Administered IV, PO, or by inhalation takes about 2 6 hours for onset Given in bursts over several days Frequently monitor growth of children and adolescents taking corticosteroids to assess systemic effects of these drugs Administered via nebulizer or MDI Minimal side effects occasional coughing from inhalation of powder NOT effective for acute exacerbation long onset of action time NOT used in children < 5 years old Used primarily in ER when child is not responding to maximal therapy MUST obtain therapeutic levels with this drug has a narrow therapeutic window Administered in combination with -agonists and steroids NOT used to treat acute episodes Montelukast for 12 months old Zafirlukast for children 7 years old Side effects drying of respiratory secretions, blurred vision, and cardiac + CNS stimulation (except for ipratropium)

Drug Short Acting -Adrenergic Agonists Albuterol Epinephrine levalbuterol (Xopenex) Terbutaline

Long Acting -Adrenergic Agonists salmeterol (Serevent)

Corticosteroids budesonide fluticasone

Nonsteroidal AntiInflammatory Drug (NSAID) cromolyn sodium (Intal) nedocromil sodium (Tilade) CNS Stimulant theophylline

Leukotriene Modifiers zafirlukast (Accolate) montelukast sodium (Singulair) Anticholinergics Atropine ipratropium (Atrovent)

Exercise induced bronchospasm (EIB) acute, reversible, usually self-terminating airway obstruction that develops during or after vigorous activity Reaches its peak 5 10 minutes after stopping activity Episode typically ends after another 20 30 minutes Manifestations cough, shortness of breath, chest pain, tightness, wheezing, endurance problems during exercise Does NOT usually occur during activities that require short bursts of energy baseball, sprints, gymnastics More common during activities that involve endurance exercise soccer, basketball, distance running Swimming is well tolerated by children with EIB breathing air fully saturated with moisture

Diagnosis requires exercise challenge test in a laboratory setting Medical emergency can result in respiratory failure and death Rapid onset + immediate progression Often coincides with complicating conditions pneumonia, respiratory virus, exposure to triggers Most important treatment goals ventilation airway resistance Relieving bronchspasm Correcting dehydration and acidosis child and parent anxiety Treating any concurrent infection

Status asthmaticus continued respiratory distress despite vigorous therapeutic measures

Treatment usually requires hospitalization

Place child in position of comfort = high Fowlers expands airway Humidified O2 administed to maintain O2 saturation > 90% Inhaled aerosolized short-acting 2-agonists (albuterol) Systemic corticosteroid used to effects of inflammation IV flids for hydration and to administer medications Can also give IV magnesium sulfate potent muscle relaxant that acts to inflammation and pulmonary function and peak flow rate Antibiotics should NOT be used to treat acute asthma attacks EXCEPT with an underlying bacterial infection

Cystic Fibrosis genetic disease that causes viscosity of exocrine (mucus producing) gland secretions Autosomal recessive trait affected child inherits defective gene from both parents Progressive and incurable disease Pathophysiology affects multiple organ systems of sweat electrolytes (Na and Cl ) Chloride channel impeded Salty taste on skin
+ -

Mucus glands produce a thick mucoprotein that accumulates and causes gland to dilate viscosity of secretions = mechanical obstruction in organs Obstruction = secretions precipitate or coagulate to form concretions in glands and ducts Affects organ defined by mucus production Pulmonary = lungs Gastrointestinal = liver, pancreas, intestines, saliva, stomach Sexual organs

Develop abnormalities in autonomic nervous system function

Manifestations Meconium Ileus Small intestine blocked with meconium = earliest postnatal manifestation in newborn Abdominal distention Vomiting Failure to pass stools Rapid development of dehydration Gastrointestinal Tract Large, bulky, loose, frothy, extremely foul-smelling stools Abdominal distention Weight loss, failure to grow Marked tissue wasting, thin extremities Sallow skin, anemia Deficiency of fat-soluble vitamins (A, D, E, and K) Pulmonary Function Wheezing + dry, nonproductive cough (initially) dyspnea, paroxysmal cough Obstructive emphysema and patchy areas of atelectasis Overinflated, barrel-shaped chest Hypoxia, cyanosis, hypercapnia Clubbed fingers Repeated episodes of bronchitis and bronchopneumonia

Possible complications as disease progresses Pancreas thick secretions block ducts = causes pancreatic fibrosis Prevents essential pancreatic enzymes from reaching duodenum = digestion and absorption of nutrients Formation of bulky stools that are frothy from undigested fat (steatorrhea) and foul smelling from putrefied protein (azotorrhea) incidence of insulin dependent cystic fibrosis related diabetes (CFRD)

Liver biliary obstruction and fibrosis Rectum prolapse occurs in infancy and childhood Related to large, bulky stools, malnutrition, and intraabdominal pressure secondary to paroxysmal cough Intestinal obstruction from inspissated or impacted feces Secretions are difficult to expectorate gradually obstruct bronchi and bronchioles Causes scattered areas of bronchiectasis, atelectasis, and hyperinflation Stagnant mucus offers a favorable environment for bacterial growth = risk for passing infection to other CF children

Pulmonary destruction of lung tissue and respiratory failure

Most children show respiratory symptoms before 1 year old

Reproductive systems inhibited fertility or sterility Growth and development restricted as a result of absorption of nutrients, including vitamins and fat

Diagnosis family history, absence of pancreatic enzymes, chest radiography (shows patchy atelectasis and obstructive emphysema), chronic pulmonary involvement Can also be diagnosied via newborn screening, DNA identification of mutant genes, and abnormal nasal potential difference measurement Gold standard = positive sweat chloride test Chloride concentration > 60 mEq/L is diagnostic of CF (normal = 35 40 mEq/L)

Therapeutic management requires multidisciplinary approach to treatment Goals of CF therapeutic management: Prevent or pulmonary complications Ensure adequate nutrition for growth Encourage appropriate physical activity Promote a reasonable quality of life for child and family Manage Gastrointestinal Problems Administration of fat-soluble vitamins (A, D, E, and K) + multivitamin + pancreatic enzymes with meals and snacks Well-balanced, high-protein, high-caloric diet due to intestinal absorption 3 daily meals + 3 snacks recommended to meet energy and growth requirements Experience frequent anorexia nighttime gastrostomy feedings or parenteral alimentation to supplement salt intake, especially in during activities and in hot weather Monitor growth treatments working if child still has stools (but no steatorrhea) and child is growing Manage Endocrine Problems Management of CFRD is critical Monitor blood glucose Administer oral glucoselowering agents or insulin injections Diet and exercise management Ketoacidosis rare with CFRD due to no fat absorption Assessment of bone health to detect and prevent osteoporosis or osteopenia

Manage Pulmonary Problems Prevention and treatment of pulmonary infection remove mucopurulent secretions, ventilation, antibiotics Recurrent pulmonary infections = damage to small airways = bronchiectasis Daily routine of chest physical therapy (CPT) to maintain pulmonary hygiene Medications bronchodilators, expectorants, mucolytics Most children will have central venous line O2 administration for acute episodes CO2 retention in CF patients can lead to bronchial cysts and emphysema if cysts rupture = pneumothroax (tachypnea, tachycardia, dyspnea, pallor, cyanosis)

Nursing interventions most patients with CF require hospitalization only for treatment of pulmonary infection, uncontrolled diabetes, or a coexisting medical problem Standard precautions with meticulous handwashing should be implemented to nosocomial spread of organisms to CF patient and between hospitalized CF patients Evaluate effectiveness of aerosol therapy, chest percussion therapy, and postural drainage CPT should NOT be performed before or immediately after meals Provide supplemental O2 therapy and evaluate effectiveness pulse oximetry, observation of respiratory pattern, work of breathing, and lung auscultation Educate parents in breathing exercises and techniques for removal of mucus

Cardiovascular disorders Divided into 2 major groups: Congenital Heart Disease (CHD) Anatomic abnormalities that result in abnormal cardiac function Present at birth Clinical consequences = congestive heart failure (CHF) and hypoxemia Acquired Heart Disorders Result from infection, autoimmune responses, environmental factors, and familial tendencies Disease processes or abnormalities that occur AFTER birth Can be seen in normal heart or in presence of congenital heart defects

History and physical examination Ask details about mother's health history, pregnancy, and birth history + family health history Chronic maternal health conditions (diabetes type 1, lupus) Exposure to infections (rubella) Medication use during pregnancy (seizure meds) that could be teratogenic to fetus Maternal alcohol or illicit drug use Maternal age > 40 years old Low birth weight = risk for CHD High birth weight = risk for heart disease incidence of congenital cardiac defects if either parent or a sibling has a heart defect Congenital heart defects occur with chromosomal defects Down and Turner's syndromes Genetic predisposition to cardiomyopathies

Observations during physical examination Nutritional state failure to thrive, poor weight gain, poor feeding Skin/Color cyanosis, pallor (due to poor perfusion), sweating (especially unusual in infants) Chest deformities enlarged heart can distort chest configuration Unusual pulsations visible pulsations of neck veins seen in some patients Respiratory excursion tachypnea, dyspnea, expiratory grunt, frequent URIs, shortness of breath Clubbing of fingers associated with cyanosis Abdomen hepatomegaly or splenomegaly Peripheral pulses discrepancies in rate, regularity, and amplitude (strength) Heart tachycardia, bradycardia, irregular rhythms, murmurs, and additional heart sounds

Diagnosis Chest x-ray provides information on heart size and pulmonary blood flow patterns EKG graphic measure of electrical activity of heart, heart rate, and rhythm Echocardiogram produces image of cardiac structures Cardiac catheterization radiopaque catheters placed in a peripheral blood vessel and advanced into heart to measure pressures and O2 levels in heart chambers and visualize heart structures and blood flow patterns Potential complications = cardiac irregularities, venous spasm, hemorrhage Preprocedural Care Accurate height and weight determines correct size and length of catheter History of allergic reactions to iodine betadine, shellfish Assess for signs and symptoms of infection Assess diaper area diaper rash risk for infection if femoral access is required Assess, mark, and document pedal pulses (dorasalis pedis, posterior tibial) for later comparison after procedure Record baseline O2 levels Educate child and family on procedure (based on developmental level) Child is kept NPO for 4 6 hours if sedated Postprocedural Care Vital signs taken every 15 minutes blood pressure, heart rate (for 1 FULL MINUTE), temperature, respirations, O2 levels (pulse ox) Compare pulses distal to catheterization site with baseline values Assess temperature and color of affected extremity coolness or blanching may indicate arterial obstruction Monitor dressing site for signs of bleeding Monitor fluid intake to ensure hydration Immobilize and straighten affected extremity Child is encouraged to void to clear contrast material from blood Pain control acetaminophen or ibuprofen

Blood flow during fetal life blood carrying O2 from placenta enters fetal system through large umbilical vein 1) Blood travels to inferior vena cava and enters heart through right atrium 2) pressure of blood entering right atrium = blood directed posteriorly in a straight pathway across right atrium and through foramen ovale to left atrium 3) Better-oxygenated blood enters left atrium and ventricle to be pumped through aorta to head and upper extremities 4) Blood from head and upper extremities enters right atrium from superior vena cava is directed downward through tricuspid valve into right ventricle 5) Blood is pumped through pulmonary artery, where major portion is shunted to descending aorta via ductus arteriosus bypasses fetal pulmonary system (only a small amount of blood flows to and from nonfunctioning fetal lungs)

Changes to blood flow after birth cessation of placental blood flow from clamping of umbilical cord and expansion of lungs at birth causes hemodynamics of fetal vascular system to undergo pronounced and abrupt changes With 1 breath lungs are expanded, and O2 causes pulmonary vasodilation Pulmonary pressures start to as systemic pressures start to Foramen ovale closes as pressure in left atrium > pressure in right atrium Ductus arteriosus starts to close in presence of O2 concentration in blood and other factors Blood flows in response to pumping action of heart: From area of pressure to area of pressure L side pressure > R side pressure Toward path of least resistance pulmonary system resistance < systemic circulation pressure gradient = rate of flow resistance = rate of flow Abnormal connection between heart chambers (septal defect) causes blood to flow from an area of pressure (left side) to one of pressure (right side) = left-to-right shunt Anomalies resulting in cyanosis may result from a change in pressure blood is shunted from right to left side of heart (right-to-left shunt)
st

Congenital heart disease (CHD) incidence of CHD in children is approximately 5 8 per 1000 live births major st cause of death (other than prematurity) in 1 year of life Most common heart anomaly = ventricular septal defect (VSD) Main consequences of CHD = congestive heart failure (CHF), hypoxia ( O2), polycythemia ( RBCs) Categories based on hemodynamic characteristics (blood flow patterns within heart):
Acyanotic Cyanotic

Left-to-right shunt = pulmonary blood flow

Obstruction to blood flow out of ventricles

Pulmonary blood flow

Mixed blood flow

Atrial septal defect Ventrical septal defect Patent ductus arteriosus Atrioventricular canal

Coarctation of aorta Aortic stenosis Pulmonic stenosis

Tetralogy of Fallot Tricuspid atresia

Transposition of great arteries Total anomalous pulmonary venous return Truncus arteriosus Hypoplastic left heart syndrome

CHF

Left side obstruction = CHF

Right side obstruction = Cyanosis

Defects with pulmonary blood flow blood flows from pressure LEFT side of heart to pressure RIGHT side of heart Caused by abnormal connection between septum wall of heart or abnormal connection between great arteries blood volume on right side of heart = pulmonary blood flow at expense of systemic blood flow () Patients show signs and symptoms of CHF Atrial septal defect (ASD) abnormal opening between atria Pathophysiology blood flows from pressure LEFT atrium into pressure RIGHT atrium = flow of O2 blood into right side of heart There is right atrial and ventricular enlargement, but cardiac failure is unusual in an uncomplicated ASD Manifestations least symptomatic Characteristic systolic murmur fixed split second heart sound At risk for atrial dysrhythmias due to right atrial enlargement and stretching of conduction fibers to cause electrical system changes

Complications pulmonary vascular obstructive disease and emboli formation later in life from chronically pulmonary blood flow Treatment surgical patch closure or closure with a device during cardiac catheterization

Ventricular septal defect (VSD) Abnormal opening between right and left ventricles Frequently associated with other defects pulmonary stenosis, transposition of great vessels, patent ductus arteriosus (PDA), atrial defects, and coarctation of aorta Many VSDs close spontaneously during 1 year of life Pathophysiology blood flows through defect into pulmonary artery (area of least resistance) = blood volume pumped into lungs = pulmonary vascular resistance Left-to-right shunting + pulmonary resistance + back up of blood at vena cava = pressure in right ventricle Right side works harder to push blood = right ventricle hypertrophy can lead to right sided heart failure Characteristic systolic murmur loud, booming sound
st

Manifestations CHF is common Complications at risk for bacterial endocarditis and pulmonary vascular obstructive disease Treatment pulmonary artery banding (palliative), closing of defect with sutures or patch (complete repair), or device closure during cardiac catheterization

Patent ductus arteriosus (PDA) failure of fetal ductus arteriosus (artery st connecting aorta and pulmonary artery) to close within 1 weeks of life (most common in premies) Pathophysiology systemic pressure > pulmonary pressure = blood shunts from pressure aorta across duct to pressure pulmonary artery = left-to-right shunt Additional blood is recirculated through lungs returned to left atrium and left ventricle amount of blood flow to pulmonary system = pulmonary resistance + vascular congestion workload on left side of heart and right ventricular pressure and hypertrophy Characteristic machinery-like murmur pulse pressure + bounding pulses due to runoff of blood from aorta to pulmonary artery

Manifestions may be asymptomatic or show signs of CHF

Complications at risk for bacterial endocarditis and pulmonary vascular obstructive disease in later life from chronic excessive pulmonary blood flow Treatment administration of indomethacin (prostaglandin inhibitor), surgical division or ligation of patent vessel, or placement of coils to occlude PDA via cardiac catherization

Obstructive defects blood exiting heart meets an area of anatomic narrowing (stenosis) = obstruction to blood flow pressure in ventricle and great artery before obstruction + pressure in area after obstruction = pressure load on ventricle + cardiac output Coarctation of aorta localized narrowing near insertion of ductus arteriosus (right after great vessels) Pathophysiology effect of a narrowing within aorta = blood pressure proximal to defect (head and upper extremities) and blood pressure distal to obstruction (body and lower extremities) Manifestations dizziness, headaches, fainting, and epistaxis resulting from hypertension High blood pressure + bounding pulses in arms Weak or absent femoral pulses Lower blood pressure + weak pulses in lower extremities

Complications at risk for hypertension, ruptured aorta, aortic aneurysm, and stroke Treatment surgical repair by resection of coarctated portion with an end-to-end anastomosis of aorta, enlargement of constricted section using a graft, or balloon angioplasty Causes resistance to blood flow in LEFT ventricle, cardiac output, LEFT ventricular hypertrophy, and pulmonary edema Left ventricular wall hypertrophy leads to pulmonary venous and pulmonary arterial hypertension Obstruction tends to be progressive and can cause sudden episodes of myocardial ischemia Pathophysiology stricture in aortic outflow tract = resistance to ejection of blood from left ventricle Extra workload on left ventricle causes hypertrophy Left ventricular failure = left atrial pressure = pressure in pulmonary veins = pulmonary vascular congestion (pulmonary edema)

Aortic stenosis narrowing or stricture of aortic valve

Manifestations faint pulses, cardiac output, hypotension, tachycardia, poor feeding, exercise intolerance, chest pain, and dizziness Complications at risk for bacterial endocarditis, coronary insufficiency, and ventricular dysfunction Treatment balloon dilation, aortic valve replacement Pathophysiology resistance to blood flow = RIGHT ventricular hypertrophy + pulmonary blood flow Right ventricular failure = right atrial pressure = reopening of foramen ovale Causes shunting of unoxygenated blood into left atrium + systemic cyanosis Loud systolic ejection murmur

Pulmonic stenosis narrowing at entrance to pulmonary artery

Manifestations cyanosis, CHF, cardiomegaly Complications at risk for bacterial endocarditis Treatment balloon angioplasty, pulmonary valvotomy

Defects with pulmonary blood flow obstruction of pulmonary blood flow + an anatomic defect (ASD or VSD) between right and left sides of heart Blood has difficulty exiting right side of heart via pulmonary artery = R side pressure > L side pressure O2 saturated blood mixes with desaturated blood Allows desaturated blood to shunt right-to-left = desaturation in left side of heart and in systemic circulation = child is hypoxemic and appears cyanotic Survival postnatally depends on mixing of lood with heart chambers Pathophysiology alteration in hemodynamics Ventricular septal defect is usually large = equal pressures in right and left ventricles Shunt direction depends on difference between pulmonary and systemic vascular resistance Pulmonary resistance > systemic resistance = right-to-left shunt Pulmonary resistance < systemic resistance = left-to-right shunt Tetralogy of Fallot combination of 4 cardiac defects

Pulmonic stenosis = blood flow to lungs = amount of O2 blood returning to left side of heart Back up of blood = RIGHT ventricular hypertrophy Overriding aorta aortic valve is off normal position = blood from both ventricles may be distributed systemically Characteristic systolic murmur moderate in intensity Stress can cause acute episodes of cyanosis and hypoxia blue spells or tet spells O2 requirements > blood supply to heart and system Obstruction of blood flow related to infundibular spasm right-to-left shunt = pulmonary flow Mostly seen in 1 year of life relationship to feeding, stooling, crying, stress Treatment morphine sulfate (to relieve spasm) + knee-to-chest position ( resistance in system to O2 levels)
st

Manifestations cyanosis (blue babies)

Complications at risk for emboli, seizures, loss of consciousness or sudden death following an anoxic spell Treatment palliative shunt, surgical repair and patches of defects

Congestive heart failure (CHF) inability of heart to pump an adequate amount of blood to systemic circulation at normal filling pressures to meet body's metabolic demands (major consequence of congential heart disease Pathophysiology 2 categories: right-sided and left-sided failure typically when one side fails, other fails too LEFT-Sided Failure Left ventricle unable to pump blood effectively into systemic circulation Results in pressure in left atrium and pulmonary veins Lungs become congested with blood = pulmonary pressures + pulmonary edema RIGHT-Sided Failure Right ventricle unable to pump blood effectively into pulmonary artery Results in pressure in right atrium and systemic venous circulation = systemic congestion Systemic venous hypertension = hepatosplenomegaly + edema

Heart muscle becomes damaged heart becomes unable to maintain an adequate cardiac output blood flow to kidneys = Na and H2O reabsorption Leads to fluid overload, workload on heart, and congestion in pulmonary and systemic circulations
+

Most frequently occurs secondary to structural abnormalities (septal defects) that result in blood volume and pressure within heart Other causes cardiomyopathy, dysrhythmias, severe electrolyte disturbances, or with excessive demands on a normal heart muscle (such as sepsis or severe anemia) Impaired Myocardial Function Tachycardia, cardiomegaly, cardiac output blood pressure, weak peripheral pulses Sweating (inappropriate) Pale, cool extremities urinary output Fatigue, weakness, restlessness, anorexia Pulmonary Congestion work of breathing Tachypnea, dyspnea, flaring nares Retractions (in infants) Exercise intolerance, orthopnea Cough, hoarseness Cyanosis, wheezing, grunting, moist crackles Systemic Venous Congestion Weight gain Hepatomegaly Peripheral edema, especially periorbital Ascites Neck vein distention (children)

Manifestations

Drug

Diagnosis made on basis of clinical symptoms, chest x-ray (shows cardiomegaly), ECG (displays ventricular hypertrophy), and echocardiogram (determines if cause is congential heart defect or poor ventricular function) Treatment 4 goals: Improve cardiac function achieved through medication administration Indications Used to cardiac output, heart size, venous pressure, and edema Nursing Interventions Rapid onset drug in liquid form given q12h, 1 hr before or 2 hrs after meals, do NOT mix with liquids, do NOT repeat dose Monitor K levels hypokalemia = risk for digoxin toxicity (bradycardia, dysrhythmias, nausea, vomiting, halos) Dose calculated in micrograms > 1 mL of med = TOO MUCH! ALWAYS check apical pulse for 1 FULL MINUTE before dose DO NOT give digoxin if pulse is < 90 110 beats/min (infants and young children) or < 70 beats/min (older children)
+ +

Digitalis Glycosides digoxin (Lanoxin)

ACE Inhibitors captopril (Capoten) enalapril (Vasotec) Drug K Wasting Diuretic furosemide (Lasix)
+ +

Blocks aldosterone and conversion of angiotensin I to II in kidneys = vasodilation

+

DO NOT give additional K supplements or spironolactone (Aldactone), if using a diuretic may cause hyperkalemia Results in pulmonary and systemic vascular resistance, BP, in afterload Common side effects hypotension, cough, renal dysfunction

Remove excess fluid and Na diuretics + possible fluid restriction (except for infants and poor feeders) + + possible Na restriction (make game of restriction for older children) Indications Blocks reabsorption of + Na and H2O in proximal renal tubule Nursing Interventions Drug of choice in severe CHF Causes of electrolytes encourage foods high in K or give K supplements (especially if on digoxin) Keep strict I & O and monitor for dehydration Takes several days to achieve maximum effectiveness DO NOT administer K supplements can cause hyperkalemia Side effects skin rash, drowsiness, ataxia, hyperkalemia
+ + +

K Sparing Diuretic spironolactone (Aldactone)

Blocks action of aldosterone to excrete + + Na and retain K

cardiac demands minimize metabolic needs of child Limit physical activity (bed rest) Maintain body temperature Treat any infections Cluster nursing care so as not to tire child Semi-Fowlers position ( effort of breathing) Possibly medicate an irritable child Provide O2 cautiously make sure O2 is warm Caloric needs > average infant (due to metabolic rate), yet their ability to take in adequate calories is hampered by their fatigue Instruct family on feeding techniques that cardiac demands Always hold an infant for feedings feed in a semiupright position Use a softer nipple or make nipple hole larger to sucking effort

Maintain nutritional status especially for poor feeders

Higher caloric content = less volume needed NG supplemental feedings

Hypoxia = tissue oxygenation that is caused by low O2 saturations and PaO2 (arterial O2 tension) Results in impaired cellular processes Cyanosis Blue discoloration in mucus membranes, skin, and nail beds of child with O2 saturation Results from presence of deoxygenated hemoglobin (hemoglobin NOT bound to O2) Usually apparent when arterial O2 saturations are 80% 85% Heart defects that cause hypoxemia and cyanosis result from desaturated venous blood (blue blood) entering systemic circulation without passing through lungs Polycythemia number of RBCs due to chronic hypoxia O2-carrying capacity of blood Anemia may result if iron is not readily available for formation of hemoglobin viscosity of blood + crowd out clotting factors = risk for clotting = risk for stroke if dehydrated Clubbing Thickening and flattening of tips of fingers and toes Occurs due to chronic tissue hypoxemia and polycythemia

Bacterial endocarditis (BE) infection of heart valves and inner lining of heart Most often a sequela of bacteremia in child with acquired or congenital anomalies of heart or great vessels Affects children with valvular abnormalities, prosthetic valves, shunts, recent cardiac surgery with invasive lines, and rheumatic heart disease with valve involvement Most common causative agent = Streptococcus viridians

Pathophysiology organisms enter bloodstream (from any site of localized infection or from interruption of skin integrity, such as dental work, cardiac surgery, long-term indwelling catheters) to grow on heart endocardium Lesion may invade adjacent tissues, such as aortic and mitral valves, and may break off and embolize elsewhere, especially in spleen, kidney, and CNS Unexplained fever (low grade and intermittent) Anorexia, weight loss, malaise Prevention = administration of prophylactic antibiotic therapy 1 hour before any procedures known to risk of entry of organisms in very high risk patients dental work Educate parents to report any unexplained fever, weight loss, or change in behavior (lethargy, malaise, anorexia) to HCP

Manifestations onset usually insidious

Treatment started immediately with high doses of appropriate IV antibiotics for 2 8 weeks

Rheumatic fever inflammatory disease that occurs after infection with Group A -hemolytic streptococcal (GABHS) bacteria Self-limited illness that involves joints, skin, brain, serous surfaces, and heart cardiac valve damage (rheumatic heart disease) is most significant complication Etiology relationship between URI infection (pharyngitis) with GABHS and subsequent development of rheumatic fever (usually within 2 6 weeks) Manifestations fever, carditis, tachycardia, cardiomegaly, chest pain, polyarthritis, inflamed joints Treatment goals of medical management Eradication of hemolytic streptococci use of penicillin or erythromycin Prevention of permanent cardiac damage Palliation of other symptoms salicylates used to control inflammation and fever and discomfort Prevention of recurrences of rheumatic fever prophylactic antibiotics

Sickle cell anemia (SCA) normal adult Hgb (Hgb-A) is partly or completely replaced by abnormal sickle Hgb (Hgb-S) Sickle cell disease (SCD) includes all hereditary disorders with clinical, hematologic, and pathologic features that are related to presence of Hgb-S Characterized by vasoocclusion, infarcts, hemolysis, anemia, pain, ischemia, and hypoxia RBCs are hard, inflexible, and sickle-shaped

Etiology autosomal recessive disorder = 25% chance of producing offspring with SCA if both parents are carriers Primarily affects African-Americans Newborn with SCA is generally asymptomatic due to Hgb-F concentrations > Hgb-S concentrations

Pathophysiology primarily result of obstruction caused by sickled RBCs and RBC destruction (7 10 day life span) Abnormal adhesion, entanglement, and enmeshing of rigid sickle-shaped cells with one another block microcirculation = causes vasoocclusion blood flow to adjacent tissues = local hypoxia = leads to tissue ischemia and infarction (cellular death) Accumulation of RBCs in organs cause enlargement infarction with ischemia + repeated destruction of organ fibrous scarring tissue formation infarction in spleen due to sequestration = makes childs spleen nonfunctional by 6 years of age

Manifestations PAIN, immune response = risk for infections, chronic anemia (Hgb 6 9 g/dL), failure to grow Most acute symptoms of disease occur during periods of exacerbation (crises) Vasoocclusive crisis most common distal ischemia and pain Sequestration crisis pooling of blood in liver and spleen with blood volume and shock Aplastic crisis profound anemia from RBC production in bone marrow Hyperhemolytic crisis anemia, jaundice, and reticulocytosis from rate of RBC destruction Dactylitis painful swelling of hands and feet usually 1 crisis in toddlers
st

Crisis precipitated by infections, cold exposure, stress, dehydration, changes in altitude, and vigorous activities

Complications stroke (CVAs), chest syndrome (looks like pneumonia), avascular necrosis (mostly in hip joints and shoulders), priapism (painful, constant penile erection) Diagnosis sickle-turbidity newborn screening test (Sickledex), Hgb electrophoresis Early identification is crucial to initiate supportive care administer prophylactic antibiotics REST to minimize energy expenditure and O2 use fluids either PO or IV therapy to prevent dehydration, which precipitates a crisis Oral intake should be at least 2x what others drink extra fluids should be given with in exercise and in hot weather fluids + impaired kidney function = problem of enuresis (bed-wetting) Treatment aimed at preventing sickling phenomena and treating medical emergencies of sickle cell crisis

Electrolyte replacement hypoxia results in metabolic acidosis, which promotes sickling Analgesics for severe pain from vasoocclusion Start with ibuprofen or acetaminophen (Tylenol) if ineffective, add codeine (titrated to therapeutic level) progress to opioids, such as morphine, oxycodone, hydromorphone (Dilaudid), and methadone Patient-controlled analgesia (PCA) Warm compress to pain cold compresses are NOT applied ( sickling and vasoconstriction) Can cause iron overload kidneys and heart can fail Need to supplement with iron chealtor drug to break down iron Prolonged therapy with supplemental O2 can depress bone marrow erythropoiesis = anemia Administer pneumococcal, meningococcal, and influenza vaccines due to susceptibility to infection as a result of a functional asplenia (absence of normal spleen function) Prophylactic antibiotics till 5 6 years old

Blood replacement to treat anemia and viscosity of sickled blood

Short term O2 therapy to prevent hypoxia, which can cause massive systemic sickling Antibiotics to prevent (most important) or treat any existing infection

Educate family and child seek early intervention for fevers, give antibiotics as ordered, monitor for signs of hypoxia and respiratory problems Medications Folic acid stimulates erythropoesis in bone marrow Hydroxyurea adjunctive chemotherapeutic agent that stimulates production of fetal Hgb-F

Hemophilia group of bleeding disorders in which there is a deficiency of a factor necessary for coagulation of blood Common forms of disorder Factor VIII deficiency = hemophilia A (classic hemophilia) Accounts for 80% 85% of all cases Factor IX deficiency = hemophilia B, or Christmas disease Von Willebrand disease = deficiency, abnormality, or absence of protein called von Willebrand factor (vWF) and a deficiency of factor VIII (affects both males and females)

Etiology X-linked recessive disorder that mostly affects males Pathophysiology basic defect of hemophilia A = deficiency of antihemophilic factor (factor VIII) Produced by liver needed for formation of thromboplastin in blood coagulation Still produce platelets results in bleeding for longer periods of time, NOT at a faster rate Prolonged bleeding anywhere in body Excessive bruising SubQ and IM hemorrhages Spontaneous hematuria Hemarthrosis (bleeding into joint cavities) especially knees, ankles, and elbows Hemorrhage from any trauma cuts, circumcision, epistaxis, injections

Manifestations

Diagnosis history of bleeding episodes, evidence of X-linked inheritance, and laboratory findings (PTT, PT) Treatment replacement of missing clotting factor and prevention of injuries Factor VIII concentrate from pooled plasma or a genetically engineered recombinant Given with any trauma or prophylactically can risk for brain and GI bleeds Treatment of choice in mild hemophilia and vWD if child shows an appropriate response NOT effective in treatment of severe hemophilia A, severe vWD, or any form of hemophilia B DDAVP synthetic form of vasopressin that plasma factor VIII and vWF levels

Vigorous physical therapy is instituted to prevent chronic crippling effects from joint bleeding

Nursing interventions limit procedures that may cause bleeding Encourage use of soft toothbrush at home SubQ route is substituted for IM injections whenever possible Venipunctures for blood samples (instead of finger or heel sticks) are preferred NO aspirin or any aspirin-containing compound should be used substitute with acetaminophen for controlling pain at home

Leukemia cancer of blood-forming tissues (bone marrow and lymphatic system) Etiology most common form of childhood cancer More common in males and Caucasians Peak age of onset = 2 6 years old

Pathophysiology unrestricted proliferation of immature WBCs in blood-forming tissues of body Leukemic cells demonstrate same neoplastic properties as solid cancers Pathologic condition caused by infiltration and replacement of tissue with nonfunctional leukemic cells Most severely affects highly vascular organs (spleen, liver)

Leukocyte count is low Cellular destruction takes place by infiltration and subsequent competition for metabolic elements compete with all good cells for resources Proliferating cells production of formed elements of blood in bone marrow by competing for and depriving normal cells of essential nutrients for metabolism Acute lymphoid leukemia (ALL) Groups Affected Peak Age Survival Rate White males 4 years old 75% survival Acute nonlymphoid (myelogenous) leukemia (ANLL or AML) All ethnic groups No peak age < 40% survival

2 major forms of leukemia

Manifestations PAIN, pallor, fatigue, lethargy, malaise, fever, hemorrhage (petechiae), bruising, anorexia Most frequent presenting signs and symptoms of leukemia = result of infiltration of bone marrow Anemia from RBCs Infection from neutropenia (too many immature WBCs) Bleeding from platelet production (thrombocytopenia) Due to blasts in bones Gradually causes a weakening of bone and a tendency toward fractures
th

Bone and joint pain especially in long bones

Leukemic cells invade periosteum = pressure = severe pain If CNS involved headache, vomiting, papilledema, 6 nerve palsy (due to intracranial pressure)

Diagnosis based on the history, physical manifestations, and peripheral blood smear that contains immature forms of leukocytes + low blood counts (CBC) Bone marrow aspiration and biopsy = gold standard diagnosis shows hyper cellular bone marrow that contains primarily blast (immature) cells Lumbar puncture determines if leukemic cells are in brain and CNS

Treatment chemotherapeutic agents, with or without cranial irradiation, in 4 phases: Induction Therapy CNS Prophylactic Therapy Prevents leukemic cells from invading CNS Treat brain with chemo via lumbar puncture most chemo drugs do NOT cross blood brain barrier Intensification Therapy Consolidation eradicates residual leukemia cells Followed by delayed intensification prevents emergence of resistant leukemic clones Maintenance Therapy Maintains remission phase Reinduction if there is a relapse

Massive blast of chemo Achieves a complete remission or < 5% leukemic cells in bone marrow Most dangerous stage

Prepare child and family for what to expect during diagnostic and therapeutic procedures Relieve pain opioids titrated to child's needs and administered around clock for optimal pain control Nonpharmacologic strategies also used are NOT substitutes for pharmacologic management Prevent complications of myelosuppression infection, bleeding tendencies, and anemia due to number of blood cells Infection Hemorrhage Most bleeding episodes can be prevented or controlled with administration of platelet concentrates or platelet-rich plasma Skin punctures are avoided whenever possible Frequent mouth care is essential can result in gingival bleeding Avoid activities that might cause injury or bleeding Anemia May be profound from complete replacement of bone marrow by leukemic cells Blood transfusions may be necessary during induction therapy

Secondary to neutropenia Child in private room Restriction of visitors and health personnel with active infections Strict aseptic technique NO immunization using live attenuated viruses (measles, mumps, rubella, varicella, polio) Monitor for temperature

Nursing interventions use chemo precautions (handling chemotherapeutic agents may present risks to handlers) Chemotherapeutic drugs must be given through a free-flowing IV line Child must be observed for 20 minutes after infusion for signs of anaphylaxis cyanosis, hypotension, wheezing, severe urticaria Nausea and vomiting can be profound Serotonin-receptor antagonists (ondansetron, granisetron) + dexamethasone treatment of choice in the prevention of delayed emesis Most beneficial regimen = administration of antiemetic before chemotherapy NG tube feeds or total parenteral nutrition may be implemented Oral ulcers anorexia provide a bland, moist, soft diet, use Toothettes, provide frequent mouthwashes with normal saline, use local anesthetics chlorhexidine gluconate (Peridex) used to prevent or treat mucositis Stool softeners (due to bowel innervations), use of footboard (to minimize or prevent footdrop), safety measures during ambulation (due to weakness and numbness in extremities, providing soft or liquid diet (for severe jaw pain) fluid intake, void frequently, administer mesna (alkalizing agent to counteract acidity) Hair regrowth occurs in 3 6 months may be of a different color and texture

Manage problems related to chemotherapy

Anorexia loss of appetite is a direct consequence of chemotherapy or irradiation Mucosal ulceration mucosal cell damage can produce ulcers anywhere along GI tract

Neuropathy due to Vincristine use

Hemorrhagic cystitis due to chemical irritation to bladder Alopecia hair loss is common side effect protect scalp, use cotton head wear Moon face from short-term steroid use reassure child that normal facial shape returns Mood changes due to steroid therapy