Peds Exam 2 - Pediatric Nursing Review PDF

Week 3 CH 16 (ADHD) Discuss clinical characteristics of attention-deficit/hyperactivity disorder (ADHD). ​ ADHD: developmentally inappropriate degrees of inattention, impulsiveness, & hyperactivity 6+ symptoms (5+ for 17+years-old) must be present for at least 6 months: In...

Week 3 CH 16 (ADHD) Discuss clinical characteristics of attention-deficit/hyperactivity disorder (ADHD). ​ ADHD: developmentally inappropriate degrees of inattention, impulsiveness, & hyperactivity 6+ symptoms (5+ for 17+years-old) must be present for at least 6 months: Inattentive (IA) Hyperactive / Impulsive (HI) Difficulty sustaining attention, careless, Poor impulse control, difficulty sitting still, disorganized fidgeting, squirming Doesn’t play close attention to detail / make Fidgets / taps / squirms careless mistakes for tasks Can’t stay seated Problem focusing on activities Runs about or climbs where it is inappropriate Doesn’t appear to listen when spoken to Unable to play or do leisure activities quietly Doesn’t follow through on instructions / HW Always “on the go” Avoids talks that require sustained mental Talks too much effort Blurts out answers before question is finished Often loses things Difficulty waiting their turn while in line Forgets daily tasks Interrupts or intrudes others Easily distracted Discuss patient centered care for ADHD including nonpharmacologic and pharmacologic therapies. Nonpharm Pharm 1st-line tx: behavioral therapy Most effective/frequently used meds: Family education Stimulants -​ Organizational chart -​ Methylphenidate -​ Positive reinforcement -​ Dextroamphetamine -​ Rewards > punishment Counseling **** start small, gradually increase, avoid Environmental manipulation caffeine, monitor for tics, admin after meals -​ Consistency is KEY -​ Decreasing distractions when doing Nonstimulants (fewer S/E) tasks -​ NE reuptake inhibitors Psychotherapy -​ Adrenergic agonists Most effective: multimodal *** Both type of drugs (stimulant / nonstimulants) have potential for abuse -​ Should monitor all children for psychological dependence, tolerance, depression -​ Appetite loss, h/a, sleep disturbances, growth velocity Key things for care: Routine for security CH 30 (CEREBRAL DYSFUNCTION) **** Getting an objective baseline is very important + history Identify the clinical manifestations of increased intracranial pressure in infants and children *** Infants: -​ EARLY: intense bulging fontanel = larger head, high pitched cry -​ LATE: pupils change, bradycardia **** CSF can leak from either ears or nose Discuss the assessment of altered states of consciousness, including the use of the pediatric Glasgow Coma Scale. Pupil Assessment -​ Fixed Pupils dilated: narcotics / analgesics (poisoning / drugs ) // hypothermia (usually not) -​ A lot of ICP can also cause this → hernia → brain death -​ EMERGENCY, usually -​ Unequal pupils: compression on brain stem -​ Can lead to fixed pupil dilation -​ Pinpoint pupils: Might be on opioids Level of Consciousness: can pick up either improvement or deterioration in neuro stat Pediatric GCS: recognizes that expected verbal & motor responses must be related to child’s age -​ It does NOT assess verbal responses -​ Records smiling, crying, interaction Posturing (both bad) -​ A: Flexion (decorticate) -​ Rigid flexion, with arms held tightly to the body; flexed elbows, writs, fingers; plantar flexed feet; legs extended & internally rotated; possibly fine tremors or intense stiffness -​ B: Extension (decerebrate): WORSE  -​ Rigid extension & pronation of arms & legs, flexed wrist & fingers, clenched jaw, extended neck, possibly arched back Discuss the pathology and major complications of head trauma. **** Leading cause of death < 1 years old -​ Falls (#1) -​ MVA -​ Bicycle injuries -​ Assault (< 4yrs) PATHOLOGY -​ Child’s larger head size & insufficient musculoskeletal support = vulnerable to accel-decel injuries -​ Concussion: confusion, dizzy, h/a, amnesia (usually from sports) -​ Contusions & Laceration: more localized -​ Significant injury occur as brain changes shape in response to the force transmitted from impact to the skull -​ Coup: head moves forward (initial impact/force) = Brain crashes forward -​ Brain bleeds, blood vessels tear & rip -​ Brain moves back & forth = shearing, more damage to brain -​ Countercoup = backwards impact MAJOR COMPLICATIONS Epidural & Subdural Bleed Epidural bleeding Bleeding just above dura, under the skull (above, on top of) -​ Generally arterial bleed -​ Arteries = HIGH pressure (hence, fast bleed and  faster brain compressions) -​ Quick evidence (obvious injury) Subdural bleeding Bleeding below the dura -​ Hemorrhage between dura & arachnoid membrane 2 courses 1.​ Tearing of veins that bridge subdural space 2.​ Hemorrhage from cortex of brain d/t direct brain trauma Develops slowly & spreads widely -​ Slower onset of symptoms ** can be a birth injury *** worried about swelling (cerebral edema) → herniation **** Interventions: mannitol, pain & sedation meds, EVD’s (external ventricular drain), craniotomy, surgery Discuss the nursing care management of a child with a head injury and the child’s family Neuro assessment / evaluation q15 minutes -​ ID s/s of increased ICP, prevent complications -​ Frequent VS, LOC, neuro (Look for consciousness) -​ Note any abnormal posturing (whether continuously or intermittently) Goals -​ Maintain adequate ventilation, oxygenation, circulation -​ Monitor / treat increased ICP -​ Minimize cerebral oxygen requirements -​ Support child & family during recovery Bed Rest -​ HOB elevated slightly & head in midline position Safety -​ Side rails up & seizure precautions -​ Padded surface / restraints to prevent injury Provide sedation & analgesia -​ For pain, NOT decreased LOC  Quiet environment -​ Decreases restlessness & irritability DOCUMENT any draining w/ amount & characteristics -​ Bleeding from ear : basal skull fracture -​ Clear nasal draining : anterior basal skull fracture Clear liquid diet unless NPO Emotional & educational support to family Discuss the pathophysiology of submersion injury Drowning -​ 2nd leading cause of accidental death -​ Deprivation of O2 → brain injury (anoxic injury) -​ Aspirate fluid when they land in water → pulmonary edema / fluid in lungs -​ And possibly aspirated pneumonia -​ Affects pulmonary, cardiovascular, & neuro too -​ Treatment: treat hypoxia and provide oxygen asap Describe the pathophysiology, clinical manifestations, diagnostic evaluation, and complications of bacterial meningitis in children Bacterial Meningitis **** Bacterial is less common but is more serious than the viral  Hallmark sign: neck rigidity -​ Infants: poor sucking, fever, irritability, N/V, jaundice, cyanosis Precaution: must be under droplet precaution Interventions: abx, corticosteroids, analgesics, reduce environmental stimuli, monitor ICP Define epilepsy and discuss the etiology of acute and chronic seizures in children. Seizures Epilepsy: 2+ unprovoked seizures more than 24 hours apart Etiology (what causes it?) Acute seizure Fever (most common), trauma, infection, tumor, drugs, metabolic alterations (hypoglycemia), hemorrhage, acute cerebral edema Chronic seizure Idiopathic epilepsy Epilepsy secondary to trauma, anoxia, congenital defects **** medical tx needed if lasts > 5mins -​ Safety first when another seizure happens, note time, nothing in mouth, clear environment, having them on the floor, sidelying (to prevent aspiration) Discuss unclassified seizures seen in infants and children, including West syndrome and Lennox- Gastaut syndrome. West syndrome Infantile spasms Peaks @ 4-8months of age Cognitive Developmental impact ACTH (short-term therapy, hormone injected) -​ Doesn’t cure, but helps manage -​ Ketogenic diet helps too Can happen d/t genetics, structural abnormalities in brain Lennox-Gastaut Infantile spasms can develop into LGS Syndrome Onset: 1-8 years of age Always have significant cognitive impairment / developmental delay Multiple seizure types Not good prognosis Slow spike waves (EEG) Discuss the primary treatment options for seizures, including drug therapy, ketogenic diet, vagus nerve stimulation, and surgery **** Main goal of drug therapy: raise threshold of neuro electrical activity -​ Start one med at a time w/ lowest dose -​ If one is max dose → combo therapy -​ After 2 years of therapeutic, can slowly discontinue BUT need to be seizure FREE for 2 years FOCUS: Drug & A/E Carbamazepine Nausea, diplopia, blurry vision, dizziness, drowsiness, hypersensitivity syndrome **** dermatologic rxns: toxic epidermal necrolysis, Stevens-Johnson syndrome **** blood dyscrasias: anemia, agranulocytosis Phenytoin Rashes, sedation, nystagmus, ataxia, hirsutism, gingival hyperplasia, coarse features, folate def. **** sever hypoTN, cardiac arrhythmias w/ rapid infusion Valproic acid Nausea, tremor, weight gain, hair loss, thrombocytopenia, pancreatitis **** risk for hepatic failure → death Phenobarbital Sedation, inattention, hyperactivity, irritability, cognitive impairment, rash, rare hypersensitivity reactions Clonazepam Sedation, irritability, tolerance, ataxia, diplopia **** Concomitant use of opioids/benzos can cause profound sedation, respiratory depression, coma, death  Felbamate Anorexia, weight loss, nausea, insomnia, h/a, fatigue *** aplastic anemia, hepatic toxicity Gabapentin Somnolence, dizziness, ataxia, fatigue, diplopia, weight gain Lamotrigine Somnolence, dizziness, diplopia, tremor *** serious rashes: stevens-johnson syndrome topiramate Somnolence, anorexia, weight loss, fatigue, diff w/ concentration, kidney stones Tiagabine Dizziness, somnolence, h/a tremor, diff w/ concentration, depression oxcarbazepine Somnolence, h/a, dizziness, nausea, ataxia Levetiracetam Drowsiness, dizziness, bx problems AIRWAY COMES FIRST: so if ABCs closed d/t secretions, USE SUCTION Discuss the etiology and risk factors of febrile seizures and the management of a child experiencing this event **** Usually because of a viral infection -​ Focus on: Risk Factors -​ Antipyretics: not gonna prevent seizures bcuz brain reacts to sudden increase in body temp Discuss the pathophysiology and clinical manifestations of hydrocephalus and the therapeutic management of a child with this condition Hydrocephalus Pathophysiology Imbalance in the production & absorption of CSF in the  ventricular system -​ Causes extra fluid that accumulates in brain Causes: 1.​ Impaired absorption of CSF w/in subarachnoid space a.​ Nonobstructive or communicating hydrocephalus 2.​ Obstruction to the flow of CSF via ventricular system a.​ Obstructive or noncommunicating hydrocephalus ​ ​ ​ ​ ​ Fluid flows from lateral ventricles through the foramen of Monro to third ventricle, combines with fluid secreted into the third ventricle →​ CSF flows through the aqueduct of Sylvius into the fourth ventricle, where more fluid is formed →​ It then leaves the fourth ventricle by way of the lateral foramen of Luschka and the midline foramen of Magendie and flows into the cisterna magna →​ From the cisterna magna, CSF flows to the cerebral and cerebellar subarachnoid spaces where it is absorbed →​ A large portion is absorbed through the arachnoid villi, but the sinuses, veins, brain substance, and dura also participate in absorption. Clinical Manifestations Increase ICP -​ Bulging fontanels w/ head enlargement (predominant sign) -​ Scalp veins dilated -​ Macewen sign: crackpot sounds on percussion of skull -​ Frontal bossing: frontal protrusion -​ Setting-sun sign: eyes may be rotated downward Therapeutic Management Ventriculoperitoneal shunt (VP) - standard procedure -​ Greater allowance for excess tubing, which minimizes the number of revisions needed as the child grows -​ Tube into the brain → neck → chest cavity → drain abdominal cavity for it to be reabsorbed into circulation NO: kids w/ cardiac issues YES: kids w/ abdominal issues Shunt malfunction: dislodgement or obstruction -​ s/s: irritability, seizure, change in bx Dx: head circumference measurements, CT, MRI Week 4 CH 6 (Infectious Dz) Define key terms associated with immunizations. Immunization Process of inducing or providing active or passive immunity artificially Immunity Inherited or acquired state in which an individ is resistant to occurrence or effects of a specific dz Natural immunity Innate immunity or resistance to infection or toxicity Acquired immunity Immunity from exposure to invading agent Active immunity Immune bodies are activity formed against specific antigens -​ Naturally v. artificially​ Passive immunity Temporary immunity obtained by transfusing IgGs or mother to fetus via placenta antibody Protein found in serum that is formed in response to exposure to specific antigen antigen Foreign substance attenuate Reduce virulence by treating w/ heat or chemical or cultivating it on certain medium immunobiologic Antigenic substances or antibody-containing preparations -​ Used for active or passive immunization vaccine toxoid Modified bacterial toxin that as been made nontoxic antitoxin Solution of antibodies derived from serum of animals immunized w/ specific antigens Ig or IV Ig Sterile solution containing antibodies from large pools of human blood plasma -​ For routine maintenance of immunity of certain immunodef. -​ For passive immunization against measles & Hep A Herd immunity Majority of population is vaccinated and spread of certain dz spots Monovalent vaccine Vaccine designed to vaccinate against single antigen or organism Conjugate vaccine A carrier protein with proven immunologic potential combined with a less antigenic polysaccharide antigen to enhance the type and magnitude of immune response Combination vaccine Combo of multiple vaccines into 1 parental form Polyvalent vaccine Vaccine designed to vaccinate against 2+ antigen or organisms (multiple strain of the same organism) -​ The flu vax cocooning Strategy of protecting infants from pertussis by vaccinating all persons who come in close contact w/ infant +​ Mother, grandparents, health care workers Review recommendations for routine immunizations, including contraindications (C/I) and precautions (P/C). ****Don’t need to memorize exactly when they need to be received… Just know HepB @ birth and that most vaccinations are given at 2 months…. Also know when they get their first dose HepB Min. age: Birth Should be withheld for infants born prematurely & weighing < 2,000g if mother is negative for HepB C/I: anaphylactic allergy to yeast P/C: infant weight < 2kg RV (Rotavirus) Min. age: 6 weeks Doses: 2 (RV1) or 3 (RV5) Series shouldn’t be initiated for children 15 weeks+ C/I: hx of intussusception; SCID; immunocomp -​ LIVE VAX P/C: chronic GI dz, Spina bifida, bladder exstrophy, immunocompromised DTaP Min. age: 6 weeks (Diphtheria, Doses: 5 tetanus, acellular Dose 5 is not needed if dose 4 was given at 4y.o. Or older pertussis) C/I: encephalopathy w/in 7 days prior doses P/C: GBS, neuro d/o, uncontrolled seizures, acute illness Tdap; Td Min, age: 11 years Doses: One (Tdap) or q10Y (Td) Admin 1 dose to adolescents who are preggo regardless of timing of prev. TD/Tdap Booster w/ Td q10Y after admin of Tdap Hib (Haemophilus Min. age: 6 weeks influenzae type B) Doses: 4 (PRP-T) or 3 (PRP-OMP) Only 1 dose is recc for kids 15 months or +, & not immunized Admin different Hib vax for booster dose C/I: age younger than 6 weeks PCV13 Min. age: 6 weeks (Pneumococcal Doses: 4 conjugate) Follow recc for dual vax series for kids w/ high-risk conditions C/I: anaphylactic rxn to any vax containing diphtheria toxoid IPV (Inactivated Min. age: 6 weeks poliovirus) Doses: 4 Final dose on or after 4 y.o. C/I: anaphylactic rxn to neomycin/streptomycin/polymyxin B P/C: pregnancy IIV (Inactivated Min. age: 6 months influenza vax) Doses: Yearly Must be 2 y.o. Or + to receive live IIV (LAIV) P/C: GBS w/in 6wks prior; acute illness; egg allergy MMR (Measles, Min. age: 12 months mumps, rubella) Doses: 2  C/I: Pregnancy, immunodef. -​ LIVE VAX P/C: hx of thrombocytopenia/purpura; anaphylactic rxn to eggs/gelatin/neomycin; blood transf. + antibodies w/in 11 months prior; tuberculin skin testing; passive immunity VAR (Varicella) Min. Age: 12 months Doses: 2 C/I: pregnancy, anaphylactic rxn to gelatin/neomycin, immunodef. -​ LIVE VAX P/C: Blood transf + antibodies w/in 3-11 months prior; tx w/ antiviral med; tx w/ immunosuppressants (corticosteroids); cancer; ASA; passive immunity HepA Min. age: 12 months Doses: 2 C/I: severe allergy to neomycin/yeast MenACWY Min. age: 2 months or 9 months (Meningococcal Doses: 1 conjugate) MenB Min. age: 10 y.o (Meningococcal) Doses: 2 or 3 series Max age: 23 C/I: prior hypersensitivity; bexsero (latex allergy) P/C: use bexsero in pregnancy only if clearly indicated; trumenba safety not established for kids < 10 y.o. HPV Min. age: 9 y.o. Doses: 2 or 3 C/I: severe allergy to yeast P/C: pregnancy *** prevent cervical cancer *****Contraindication (C/I): never give vax *****Precaution (P/C): can cause a reaction; but need to weigh the risk/benefit ratio (determined on individual basis) General rule of thumb: don’t give children aspirin Describe the nursing care management of communicable diseases, including preventive measures. Suspecting communicable dz: Rash, sneezing, coughing, fever Nursing care of communicable disease Chickenpox (varicella) Maintain Standard, Airborne, and Contact Precautions if hospitalized until all lesions are crusted; for immunized child with mild breakthrough varicella, isolate until no new lesions are seen. Keep child in home away from susceptible individuals until vesicles have dried (usually 1 week after onset of disease), and isolate high-risk children from infected children. Administer skin care: give bath and change clothes and linens daily; administer topical calamine lotion; keep child's fingernails short and clean; apply mittens if child scratches. Keep child cool (may decrease number of lesions). Lessen pruritus; keep child occupied. Remove loose crusts that rub and irritate skin. Teach child to apply pressure to pruritic area rather than scratching it. Avoid use of aspirin (possible association with Reye syndrome). **** HIGHEST PRECAUTION BCUZ IT’S AIRBORNE Don’t need to know the stages of it **** child is contagious a day before rash appears & until vesicles are crusted Diphtheria Follow Standard and Droplet Precautions until two cultures are negative for C. diphtheriae; use Contact Precautions with cutaneous manifestations. Administer antibiotics in timely manner. Participate in sensitivity testing; have epinephrine available. Administer complete care to maintain bed rest. Use suctioning as needed. Observe respiration for signs of obstruction. Administer humidified oxygen as prescribed. Erythema Isolation of child is not necessary, except hospitalized child (immunosuppressed or with aplastic crises) suspected of parvovirus infection is placed on Droplet Precautions and Standard Infectiosum Precautions. (Fifth Pregnant women need not be excluded from workplace where parvovirus infection is present; Disease) they should not care for patients with aplastic crises. Explain low risk of fetal death to those in contact with affected children; assist with routine fetal ultrasound for detection of fetal hydrops. **** NOT GOING TO BE TESTED Exanthem Use Standard Precautions. Teach parents measures for lowering/. temperature (antipyretic drugs); ensure adequate parental Subitum understanding of specific antipyretic dosage to prevent accidental overdose. (Roseola If child is prone to seizures, discuss appropriate precautions and possibility of recurrent febrile Infantum) seizures. Mumps Maintain isolation during period of communicability; institute Droplet and Contact Precautions during hospitalization. Encourage rest and decreased activity during prodromal phase until swelling subsides. Give analgesics for pain; if child is unwilling to swallow pills or tablets medication, use elixir form. Encourage fluids and soft, bland foods; avoid foods requiring chewing. Apply hot or cold compresses to neck, whichever is more comforting. To relieve orchitis, provide hot or cold packs for analgesia and scrotal elevation. Measles Maintain isolation until fifth day of rash; if child is hospitalized, institute Airborne Precautions. (Rubeola) Encourage rest during prodromal stage; provide quiet activity. Fever—Instruct parents to administer antipyretics; avoid chilling; if child is prone to seizures, institute appropriate precautions. Eye care—Dim lights if photophobia present; clean eyelids with warm saline solution to remove secretions or crusts; keep child from rubbing eyes. Coryza, cough—Use cool-mist vaporizer; protect skin around nares with layer of petrolatum; encourage fluids and soft, bland foods. Skin care—Keep skin clean; use tepid baths as necessary RASH (But NOT transmitted via contact… AIRBORNE) Pertussis Maintain isolation during catarrhal stage; if child is hospitalized, institute Standard Contact and Droplet Precautions. (Whooping Obtain nasopharyngeal culture for diagnosis. Cough) Encourage oral fluids; offer small amount of fluids frequently. Ensure adequate oxygenation during paroxysms; position infant on side to decrease chance of aspiration with vomiting. Provide humidified oxygen; suction as needed to prevent choking on secretions. Observe for signs of airway obstruction (e.g., increased restlessness, apprehension, retractions, cyanosis). Encourage compliance with antibiotic therapy for household contacts. Encourage adolescents to obtain pertussis booster (Tdap) (see Immunizations section in this chapter). Use Standard Precautions and Droplet in health care workers exposed to children with persistent cough and high suspicion of pertussis. BACTERIAL, hence ABX Poliomyelitis Institute Contact Precautions. Administer mild sedatives as necessary to relieve anxiety and promote rest. Participate in physical therapy procedures (use of moist hot packs and range-of-motion exercises). Position child to maintain body alignment and prevent contractures or skin breakdown; use footboard or appropriate orthoses to prevent footdrop; use pressure mattress for prolonged immobility. Encourage child to perform activities of daily living to capability; promote early ambulation with assistive devices; administer analgesics for maximum comfort during physical activity; give high-protein diet and bowel management for prolonged immobility. Observe for respiratory paralysis (e.g., difficulty talking, ineffective cough, inability to hold breath, shallow and rapid respirations); report such signs and symptoms to practitioner.  Rubella Institute Droplet Precautions. Reassure parents of benign nature of illness in affected child. (German Use comfort measures as necessary. Measles) Avoid contact with pregnant woman. Monitor rubella titer in pregnant adolescent. Scarlet Fever Institute Standard and Droplet Precautions until 24 hours after initiation of treatment. Ensure compliance with oral antibiotic therapy; intramuscular benzathine penicillin G [Bicillin] may be given. Encourage rest during febrile phase; provide quiet activity during convalescent period. Relieve discomfort of sore throat with analgesics, gargles, lozenges, antiseptic throat sprays, and inhalation of cool mist. Encourage fluids during febrile phase; avoid irritating liquids (e.g., citrus juices) or rough foods (e.g., chips); when child is able to eat, begin with soft diet. Advise parents to consult practitioner if fever persists after beginning therapy. Discuss procedures for preventing spread of infection—discard toothbrush; avoid sharing drinking and eating utensils. Preventable measures -​ Hand washing -​ Practice → kids: after toileting & before eating -​ Reduce airborne transmission -​ Cover face when coughing or sneezing -​ Hygiene -​ Not sharing eating & drinking utensils Conjunctivitis: bacterial & viral S/S Common: inflamed eyes Bacterial -​ Most common -​ Pink eye -​ Purulent discharge -​ Crusting of eyelid -​ Tx: topical abx Viral -​ Usually w/ URI -​ Looks inflamed & red; clear and stringy discharge -​ Itching -​ Tx: self-limiting; remove secretions Management Prevent spread, keep eye clean & dry; ophthalmic meds Education Avoid compresses; separate washcloths/towels, discard used tissues; avoid touching eye, hand washing CH 26 Respiratory Dysfunction Describe the factors that influence the etiology and characteristics of respiratory infections in children. ++++ CHILDREN ARE NOSE BREATHERS Infectious agents Viruses Age Full term infant < 3 months have lower infection rate Rate increase from 3-6 months old Size Antomic diff influence response to respiratory tract infections -​ Diameter of airways is smaller in young kids -​ Less alveoli for gas exchange -​ Distance between structures w/in tract is shorter in young kids -​ Organisms move more rapidly down respiratory tract Resistance Ability to resist pathogen depend on several factors -​ Immune system -​ Conditions that decrease resistance: malnutrition, anemia, fatigue -​ Conditions that weaken defenses of respiratory tract: allergies, bronchopulmonary dysplasia, asthma, RSV, cardiac anomalies, CF Seasonal variations Most common respiratory tract pathogens appear in epidemics: winter & spring Mycoplasma infections: autumn & early winter Infection-related asthma: cold weather RSV: winter & spring List signs and symptoms of respiratory infections and symptoms of a respiratory emergency in infants and young children. **** ID respiratory distress: retractions, nasal flaring, tracheal tugging, bradycardia, apnea, anxiety, tripod (they want to sit up), lethargy Discuss the etiology, clinical manifestations, therapeutic management, and nursing care of children with acute streptococcal pharyngitis, tonsillitis, and influenza. Acute Streptococcal Pharyngitis Etiology Group A streptococci (BACTERIAL) → upper airway (strep throat) Permanent dmg can happen from sequelae, especially acute rheumatic fever  Clinical Manifestations Abrupt onset of pharyngitis -​ h/a, fever -​ abdominal pain -​ tonsils/pharynx may be inflamed & covered in exudate -​ anterior cervical lymphadenopathy occurs early​ Complications Rheumatic fever Kidney dz Abscess Scarlet fever may also occur -​ s/e: pharyngitis, erythematous sandpaper-like rash Therapeutic management PO penicillin (ampicillin / amoxicillin) IM penicillin G benzathine Nursing care Obtain throat swab Cold / warm compress to neck Warm saline gargles for throat relief Don’t force kid to eat -​ Encourage cool liquids or ice chips *NEVER administer penicillin G procaine/benzathine IV → may cause embolism or toxic reaction with ensuing death in MINUTES. Administer meds deep into muscle tissue to decrease localized reactions and pain* Tonsillitis *** looks like strep throat but the bad breath is key Etiology Tonsillitis often occurs w/ pharyngitis Common cause of illness in young children Viral or bacterial Clinical Manifestations Inflammation -​ Difficulty swallowing / breathing -​ Mouth breathing -​ Offensive mouth odor (halitosis) -​ Impaired sense of taste / smell -​ Persistent cough Therapeutic management Maintain NPO status Tonsillectomy Adenoidectomy Nursing care Viral: Rest, warm fluids, warm salt-water gargles Bacterial: abx therapy Soft to liquid diet -​ Cool water / ice chips  Avoid fluids w/ red or brown color Post-Surgery -​ After: suction carefully to avoid trauma -​ Discourage from coughing frequently, clearing throat, blowing nose -​ Monitor for bleeding -​ Swallowing a bunch (emergency) Influenza Etiology d/t influenza virus ( A & B ) Clinical Manifestations Sudden onset of fever & chills Dry throat & nasal mucosa Dry cough Flushed face Photophobia Myalgia fatigue Complication Pneumonia (want to monitor for it) Therapeutic management Antiviral meds Rest hydration Influenza Vaccine (prevention) Nursing care Promote increased fluid intake Rest Acetaminophen, ibuprofen for fever List factors that predispose children to the development of otitis media and discuss therapeutic management Otitis Media (Ear infection) ***** common in children Predisposing factors Eustachian tubes are short, wide, and straight (hence more susceptible) Exposure to smoke, frequent respiratory infections Therapeutic management Abx for bacterial infections Analgesics Placement of tympanostomy tubes recc after a total of 3-6 months of bilateral effusion from bilateral hearing def. Differentiate among the following upper airway conditions or croup syndromes in terms of etiology, defining characteristics, and nursing care management: acute epiglottitis, acute laryngotracheobronchitis, and acute spasmodic laryngitis. Acute spasmodic -​ etiology : allergy -​ s/s: ACUTE barky cough (Only at night) -​ NI: Cool mist humidifier -​ Treated at home ​ LTB (acute laryngotracheobronchitis) -​ Etiology: viral -​ s/s: GRADUAL barking cough, stridor -​ More s/s -​ NI:” nebulized tx, humidified air -​ May need Treatment @ hospital ​ Acute Epiglottis -​ Etiology: bacterial -​ s/s: ACUTE ( drooling, tripod, muffled speech) MEDICAL EMERGENCY  -​ NI: airway protection, avoid throat exams until secure (NOTHING IN THE MOUTH) because can aggravate Discuss the etiology, signs and symptoms, therapeutic management, and nursing care of children with bronchiolitis, specifically respiratory syncytial virus (RSV) RSV **** Inflammation → increased mucus production → airway obstruction *** SUCTION + GIVE OXYGEN ****NPO d/t Asirpation (especially if their RR is super high) *** monitor respiratory status Discuss the etiology, signs and symptoms, therapeutic management, and nursing care of children with severe acute respiratory syndrome Coronavirus 2 (SARS-COV-2): COVID-19 COVID-19 Etiology Caused by SARS-CoV-2 S/S Fever, cough, SOB, muscle aches, sore throat, h/a, N/V/D, loss of taste or smell Complication Post-infection: (MIS/C) Multi-system inflammatory syndrome in children Therapeutic M Case by case -​ Antiviral -​ Monoclonal antibody therapy Nursing care Provide info on isolation precautions Admin ibuprofen & acetaminophen: fever / discomfort Educate about illness progression -​ Difficulty breathing -​ Chest pain / pressure -​ Cyanosis in face / lips -​ Decreased UO -​ Confusion -​ Difficulty tolerating PO fluids Increase fluid intake Identify the pathophysiology, clinical manifestations, therapeutic management, and nursing care of children with allergic rhinitis and with asthma. Allergic Rhinitis **** KEY: itchy eyes/nose/throat Asthma **** difficulty w/ exhalation; (airway resistance) ***** We need to know the different types of asthma **** Need to know PEF (this is for diagnosing for the severity of asthma) -​ Know the action plan (Green, yellow, red - know percentage) Big thing to know: what is our first tx for acute exacerbation: albuterol (short acting beta agonist -SABA) -​ Steroids for inflammation Know how to treat status asthmaticus (MED EMERGENCY) -​ Diminished breath sounds, respiratory distress, accessory muscle use -​ Risk for cardiac/respiratory distress Identify the primary risk factors for the development of asthma, for experiencing asthma episodes, and for increased asthma mortality. Describe the etiology, physiology, clinical manifestations, for cystic fibrosis. Cystic Fibrosis Water → Na → Cl **** bulky stool / steatorrhea Vitamin A,D,E,K malnutrition Describe the diagnostic evaluation, screening, treatment, and nursing care management for cystic fibrosis *** know the chest XR one: patchy atelectasis/obstructive emphysema ACT + exercise = very good together +​ Prior to ACT: bronchodilators: to open airway Pulmozyme: mucolytic Abx: need higher dose and treat quickly GI management - pancreatic enzyme capsules: based on their stool + weight KHAN: Cystic Fibrosis -​ Genetic thing (recessive dz) - CFTCR gene -​ Most common in Caucasians -​ Defect w/ Cl & Na -​ Most affected organ: lungs -​ Lungs lubricated by mucus (made up by Cl & Na) -​ Defect → clog up lungs → infections -​ Pseudomonas very prominent & hard to treat  -​ O2 concentration low d/t to gunk; hard to cough up -​ Another affected organ: digestive tract ( GI & pancreas ) -​ Pancreas job: secrete enzymes to help digest food -​ CF kids: usually malnourished -​ Affected organ: liver (secretes bile which emulsify fats) -​ Bile can’t get out -​ Pancreas (insulin) -​ CF: diabetes CF Patho: -​ CFTCR : chloride channel - being blocked -​ Na affected d/t relationship w/ Cl -​ Cl (-) & Na (+) want to balance each other -​ Sweat glands -​ d/t malfunctioning chloride channel, Cl can’t be reabsorbed → Na won’t either -​ CF s/s: salty skin KHAN: Epiglottis Epiglottis: small lid that protects the airway when you swallow Arytenoid cartilage: control vocal chords Epiglottitis: can swell so much that it can close the airway -​ Tx: cricothyroidotomy -​ Can be really serious in children since their airway is fairly narrow -​ s/s: fever, sore throat, muffled voice, difficulty speaking / swallowing → drooling, stridor Week 5 CH 27 CARDIAC DYSFUNCTION Review basic cardiac structure and physiology for fetal and post birth circulation. Mediastinum Space between 2 pleural cavities Pericardium Double-walled membrane covering the heart Chordae tendineae Cordlike structures attached to papillary muscle & valve leaflets Atria 2 upper chambers Ventricles 2 bottom chambers Cardiac output HR x Stroke volume Volume of blood ejected by heart in 1 minute Stroke Volume Preload, afterload, contractility Preload Volume of blood returning to heart Afterload Ventricular ejection Determined by systemic vascular resistance & pulmonary vascular resistance Contractility Efficiency of myocardial fiber shortening or ability of cardiac muscles to act as an efficient pump 4th week: heart & circulatory begin to develop 8th week: completely developed Circulation Fetal Post-Birth O2 blood & nutritional via large umbilical vein Transition to high-pressure systemic (mom) circulation & low pressure pulmonary Higher pressure of blood entering RA from circulation (takes 6-8 weeks) IVC Patent ductus arteriosus close w/in 1st after 3 affected structures birth -​ Ductus arteriosus (pulmonary artery ​ → aorta) Heart is the 1st organ to receive blood w/ -​ Foramen ovale (connects atriums) each heartbeat; brain is next -​ Ductus venosus (most of blood goes -​ Depend most on adequate O2 levels into R. atrium → rest of body) to function *** After birth, these structures will close Right side: high pressure Left side: lower pressure Congenital Heart Dz (HF / Hypoxemia) -​ Consequences : Left-to-right shunt -​ Decreased pulmonary BF -​ NC: Cut-off for Digoxin: 90-110HR -​ Rarely up to 1mL for dig. For peds -​ ACE-I: monitor BP (hypotension) Discuss the causes of heart failure (HF) and major clinical manifestations of HF in children. Heart Failure Causes Volume overload (left-to-right shunts → RV hypertrophy) Pressure overload -​ d/t obstructive lesions (valvular stenosis / coarctation of aorta) Decreased contractility -​ d/t cardiomyopathy / MI from severe anemia or asphyxia / heart block / acidemia / low K, glucose, Ca, Mg High CO demands -​ d/t examples: sepsis / hyperthyroidism / severe anemia Clinical Manifestations Impaired myocardial function -​ Tachycardia, sweating, decreased UO, fatigue, weakness, anorexia, pale, weak pulse, decrease BP, gallop rhythm, cardiomegaly Pulmonary congestion (L. HF)  -​ Tachypnea, dyspnea, retractions, flaring nares, exercise intolerance, orthopnea, cough, hoarseness, cyanosis, wheezing, grunting Systemic venous congestion (R. HF) -​ Weight gain, hepatomegaly, peripheral edema, ascites, JVD Describe the four goals of treatment and nursing care management of a child with HF. Tx goals 1.​ Improve cardiac function -​ Increase contractility & decrease afterload 2.​ Remove accumulated fluid & sodium -​ Decrease preload & minimize fluid overload 3.​ Decrease cardiac demands 4.​ Improve tissue oxygenation & decrease O2 consumption NC Emotional support Minimize fluid overload -​ Record I/Os / body weight qday -​ Furosemide -​ Fluid restriction Improve cardiac function (uses digoxin) -​ Appropriate calculation -​ Watch for dig. Tox. (bradycardia, N/V) Reduce afterload -​ Monitor BP for hypotension & serum electrolyte levels -​ ACE-I by relaxing muscles Decrease Cardiac demands -​ Rest and energy conservation -​ Smaller feedings q3hrs (infants tire easily & sleep through feedings) Reduce respiratory distress -​ Position & oxygen -​ Hand hygiene / abx -​ Minimize too much action Maintain nutritional status Discuss hemodynamics, clinical manifestations, therapeutic management (including shunt procedures), and nursing care indications for infants and children with hypoxemia Hypoxemia Hemodynamics 1.​ Severe obstruction to pulm. BF & blood shunting from R. → L. side of heart (Tetralogy of Fallot) 2.​ Mixing of arterial & venous blood w/in chambers of heart itself (single ventricle) 3.​ Transposition of the great arteries -​ parallel of pulm & systemic circulations rather  than sequence Clinical manifestations Chronic: polycythemia & clubbing Mild: asymptomatic except cyanosis Severe: fatigue w/ feeding, poor weight gain, tachypnea, dyspnea, cyanosis, cool, respiratory distress signs Hypercyanotic spells “blue spells” Therapeutic management Cyanosis / ductus-dependent cardiac defects : Prostaglandins -​ Vasodilation Hypercyanotic spell: knee-chest position, morphine, O2 NC Teach parents tx for hypercyanotic spells Instruct parents on importance of adequate fluid intake & measures to prevent dehydration Hand hygiene -​ Prevent respiratory infections Shunt Procedures **** Helpful for cyanotic defect -​ Just know what it’s for… don’t need to know the different types of shunts Describe cardiac defects characterized by increased pulmonary flow and their pathophysiology, clinical manifestations, and treatment. Increased Pulmonary Flow (ACYANOTIC) Management of Increased Pulmonary BF -​ Close the defect -​ Surgical repair -​ Indomethacin to close PDA -​ Monitor for s/s of HF ******Indomethacin: close PDA (increased pulmonary BF)​ ******Prostaglandin: open PDA (decreased pulmonary BF) Describe cardiac defects characterized by obstruction and their pathophysiology, clinical manifestations, and treatment. Obstructive Defects (ACYANOTIC) *** big one to know: Coarctation of aorta -​ High pressure above blockage -​ Low pressure below blockage General management -​ Surgical repair: balloon angioplasty -​ Treat HTN (HTN for rest of life) -​ ACE-I -​ Beta Blockers -​ Monitor S/S of HF Describe cardiac defects characterized by decreased pulmonary flow Decreased Pulmonary Flow (CYANOTIC) Tetralogy of Fallot -​ Pulmonic stenosis → R. ventricular hypertrophy, aorta displaced around defect, ventricular septal defect Tetralogy of Fallot -​ “Tet spells” need to be treated asap -​ Tx: knees up to chest, O2/morphine + prostaglandins (keep PDA open) General Management of Decreased Pulmonary BF Prevent Dehydration Prevent Respiratory Infection Strict I/Os Hand washing Daily weight Avoid sick contacts gavage/IV fluid (if poor PO) Aggressive pulmonary hygiene Notify of fever, V/D Describe mixed cardiac defects and their pathophysiology, clinical manifestations, and treatment. Mixed Cardiac Defects Ones to know: Transposition (PRE-CYANOTIC) & Hypoplastic -​ Open PDA crucial General Management -​ Prostaglandin E1: open PDA -​ Surgical intervention -​ Transplantation : for hypoplastic left heart -​ Monitor s/s of HF / infection Describe the etiology, clinical manifestations, and diagnostic evaluation of rheumatic fever (RF) and therapeutic and nursing care management of RF in children. Rheumatic Fever Etiology Group A streptococci infection (usually pharyngitis) -​ Inflammation of CT: joints, skin, brain, heart -​ Mitral valve most affected If kid have 1 strep infection, they’re at greater risk for repeated / recurrent infections Clinical manifestations Asymptomatic Major manifestations: -​ Carditis (50-70%), polyarthritis, Chorea, SQ nodules, erythema marginatum Minor manifestations: -​ fever, Prolonged PR, ESR > 60, CRP > 3 Diagnostic Evaluations Jones criteria -​ Must have 2 major manifestations or 1 major & 2 minor manifestations for diagnosis Lab tests: rapid antigen detection + throat culture CBC, ESR, CRP ECG CXR Therapeutic Management Prompt dx & tx of strep throat infections -​ Oral penicillin Salicylates: anti-inflammatory (exception) Supportive care Mange HF in some cases NC Objective: prevention Goals: compliance w/ drug regimens, emotional support Interventions: rest, nutrition, management of symptoms Discuss the pathophysiology, clinical manifestations (including cardiac involvement), classic clinical criteria, and diagnostic evaluation of Kawasaki disease and its therapeutic and nursing care management in children. Kawasaki Disease Patho Acute systemic vasculitis of unknown case Self limiting & resolves in 6-8 wks -​ w/out tx → cardiac sequelae Widespread inflammation of blood vessels w/ coronary arteries being most susceptible to dmg Peak incidence: toddler, asian, season (late winter/early spring) Not contagious Clinical Manifestations 3 phases: + cardiac involvement 1)Acute -​ Abrupt onset of high fever (unresponsive to abx & antipyretics) -​ Bulbar conjunctiva become red, nonexudative -​ Strawberry tongue, cracked/red lips, rash (accentuated in perineum) - mucositis -​ Edematous hands & feet -​ Cervical lymphadenopathy, temporary arthritis (small joints) 2)Subacute -​ Begins w/ resolution of fevers -​ Periungual desquamation (peeling under fingertips/toes) -​ Temp arthritis (larger weight-bearing joints) 3)Convalescent -​ Clinical signs mostly resolved but labs abnormal -​ Arthritis may continue Cardiac involvement -​ Most dangerous: development of coronary artery aneurysms & potential MI -​ Symptoms subtle: abdo pain, vomiting, restlessness, inconsolable crying, pallor, shock Classical signs -​ Fever (at least 5 days) -​ Change in extremities, polymorphous exanthem, bilateral bulbar conjunctival w/out exudate, erythema & cracking of lips / strawberry tongue, cervical lymphadenopathy (unilateral) Dx No specific dx test -​ Based on clinical findings & assoc. Lab results -​ Baseline ECHO at time of diagnosis -​ RBC / albumin slightly decreased -​ Everything else CBC elevated Acute phase -​ Somewhat anemic & leukocytosis, elevated liver enzymes, CSF (aseptic meningitis), low albumin levels Subacute phase -​ Thrombocytosis w/ hypercoagulability -​ Elevated ESR & CRP for 6-8 wks Therapeutic Standard: high-dose IVIG along w/ salicylate therapy Management -​ Reduces duration of fever & incidence of coronary artery abnormalities Cyclosporine / anti-inflamm. Drugs -​ For sickest pt w/ developing aneurysms Aspirin -​ Mod - high dose NC Focus on symptomatic relief, emotional support, dx assistance, med administration, education -​ Monitor cardiac status carefully (esp. During IVIG infusion) -​ I/O’s, daily weight  IV fluids administered w/ care Assess for HF​ Apply cool cloths / soft loose clothing Mouth care (lips) Clear liquids / soft foods + monitor temp If arthritis: ROM exercise IVIG: monitor VS -​ Watch for allergy Infective Endocarditis (IE) -​ Causes: streptococci, staphylococci, fungal -​ Most at risk: immunocompromised, PICC / central lines -​ Clinical/M: insidious, malaise, low-grade fever -​ Emboli (hands/feets) -​ Osler’s nodes (painful… found on fingers/toes) -​ Murmurs -​ HF CH 28: HEMATOLOGIC / IMMUNOLOGIC DYSFUNCTION Discuss etiology, pathophysiology, clinical manifestations, and diagnostic evaluation of iron deficiency anemia Iron Deficiency Anemia Etiology Anemia: reduction in rbc / hgb -​ Iron def. Anemia: anemia d/t inadequate iron Cause: -​ inadequate supply (rapid growth, exclusive breastfeeding after 6M) -​ Inadequate iron stores @ birth -​ Impaired absorption (iron inhibitors, malabsorption disorders, chronic diarrhea) -​ Blood loss -​ Excess demands for iron required for growth (prematurity, adolescence, pregnancy) Patho Iron needed for hgb production -​ Iron def = decreased hgb, reduced O2 carrying capacity in blood​ Clinical M Underweight or overweight (d/t excessive milk ingestion) -​ Milk is poor source of iron Pale (porcelain-like) Fatigue Poor muscle devo, prone to infection, leakage of plasma proteins, irritability, tachycardia, glossitis, angular stomatitis, koilonychia  Dx Microcytic RBCs → low MCV Reticulocyte count elevated 3-4% in SEVERE cases Total iron-binding cap. ELEVATED Biochemical tests: ferritin, TIBC, SIC Discuss the therapeutic and nursing care management of children with iron deficiency anemia. Therapeutic M Prevention: optimum nutrition & appropriate iron supplementation -​ Only breast milk or iron-fortified for first 12 months Iron supplementations of 1mg/kg/day by 4-6months in full-term infants -​ Black tarry stool, stains teeth, constipation Limit amount of formula to no more than 1L/day Ascorbic acid (Vita C): facilitates absorption of iron NC Family education -​ Milk is def. In iron, V.C, zinc, fluorid -​ Overweight doesn’t equal healthy Iron medication: large amounts = toxic -​ Liquid preps may temporarily stain teeth -​ Small doses more tolerated & absorbed better Discuss the types of episodic sickle cell crises and the clinical manifestations of each Episodic Sickle Cell Crises Vaso Occlusive Painful episode or event -​ Most common type of non-life-threatening crisis -​ Ischemia → mild to severe pain that can last from minutes to days S/S -​ Localized / generalized pain (migratory) + low-grade fever -​ Acute abdo pain from visceral hypoxia / gallstones -​ Priapism, arthralgia, dactylitis Sequestration crisis / Acute Pooling of large quantities of blood , usually in spleen & infreq Splenic Sequestration in liver -​ Causes a decrease in blood volume → shock -​ Acute: most common in kids 2m/o - 5 y/o -​ Can result in death -​ Chronic: hypersplenism / splenomegaly Death from profound anemia & cardiovascular collapse Aplastic Diminished RBC production, usually triggered by infection w/ virus -​ Packed RBC transfusion is occasionally required Hyperhemolytic An accelerated rate of RBC destruction  -​ Anemia, jaundice, reticulocytosis Freq suggests other co-existing conditions Cerebrovascular Accident Sudden & severe complication, often w/ no related illness (stroke) -​ Sickled cells block major blood vessels in the brain → cerebral infarction → neuro impairment -​ Repeat CVA can cause progressive brain dmg who already experienced stroke & didn’t receive monthly transfusions Report immediately if: -​ Severe, unrelieved h/a -​ Severe vomiting -​ weakness/inability to move hands, feets, or legs -​ Stutter/slurred speech, strange bx, seizures Acute chest syndrome Clinically similar to pneumonia & results in pulmonary infiltrate (CXR) -​ Can be accompanied by chest pain, fever, cough, tachypnea, wheeze, hypoxia -​ Repeated episodes can cause restrictive lung dz & pulmonary htn Report immediately if: -​ Severe chest pain, back pain, or abdo pain -​ Fever > 101.3 -​ Dyspnea, tachypnea, declining O2 sat, retractions Infection related Overwhelming infection d/t defective splenic function -​ Strep. Pneumoniae & H. influenzae Repeated insults on splenic sinuses → impaired filtration /function → septicemia / poss. Subsequent death **** dactylitis: hand-and-foot syndrome -​ Infarction of short tubular bones; pan & swelling of soft tissue over hands and feet -​ Usually resolves spontaneously within days to weeks Discuss the therapeutic and nursing care management of children with sickle cell anemia Sickle Cell Anemia Occur d/t Dehydration, hypoxia, acidosis, temperature elevation Clinical Manifestation Possible growth retardation, chronic anemia, delayed sexual maturation, sepsis, exercise intolerance, anorexia, jaundice, gallstone, chronic leg ulcer Dx Stained blood smear Sickle turbidity test Hgb electrophoresis Newborn screening  Therapeutic M Prevent sickling phenomenon (responsible for pathologic sequelae) -​ Treat medical emergency of sickle cell crisis Bed rest -​ minimize energy expenditure & improve O2 utilization Hydration -​ Oral / IV therapy Electrolyte replacement -​ Hypoxia = metabolic acidosis = promote sickling Analgesia: severe pain from vaso-occlusion Blood replacement -​ Treat anemia & reduce viscosity of sickle blood Abx therapy -​ Treat existing infections Heat therapy not cold therapy NC Immunizations Penicillin prophylaxis: 2 months of age to prevent pneumococcal sepsis SCA screening programs -​ ID pt w/ abnormal hgb Assess -​ VS, neuro, vision, hearing, respiratory, GI, renal, MS, pain Minimize tissue deoxygenation -​ Frequent rest breaks -​ Avoid contact sports w/ splenomegaly (can cause rupture) -​ Avoid environments w/ low O2 concentration -​ High altitudes, non pressurized airplane flights -​ Avoid known sources of infection Promote hydration -​ Prevent sickling & delay vaso-occlusion & hypoxia-ischemia cycle Minimize crises -​ Infection is major cause of death -​ Adequate nutrition, freq med supervision, proper hand washing, isolation from known infection Promote supportive therapies -​ Aware of inadequate pain control -​ Reassure that opioids are medically indicated, high doses may be needed, and kids rarely become addicted -​ Heat to affected area is soothing -​ No cold d/t enhance vasoconstriction & occlusion -​ Passive ROM exercise → circulation -​ Let kids determine their own activity tolerance Weight taken for baseline for evaluating hydration Infants get it around 4 months because they’re born with HgbF Discuss the etiology, pathophysiology, clinical manifestations, and diagnostic evaluation of hemophilia. Hemophilia Etiology Bleeding disorder d/t congenital def, dysfunction, or absence of certain protein -​ Clotting factor is deficient X-linked recessive d/o -​ ⅓ cases d/t gene mutation Patho Deficiency of factor 8 -​ Produced by liver and necessary for formation of thromboplastin in phase 1 of coagulation -​ Less F8 in blood = more severe dz C/M Prolonged bleeding -​ Hemorrhage from minor trauma -​ Hemarthrosis: bleeding into joint cavities -​ Most freq form of internal bleed -​ Early s/s: stiffness, tingling, ache in affected joint + decrease ROM of joint -​ Obvious s/s: warmth, red, swell, pain, loss of movement -​ Petechiae -​ Intracranial hemorrhage Diagnostic Platelet function test: normal in hemophilia -​ Clotting factor function abnormal -​ PTT Factor 8 & 9 assay Discuss the modes of transmission of hemophilia and the most prevalent types of this bleeding disorder. Transmission X-linked recessive d/o 60% of affected kids: positive family hx for dz ⅓ cases: gene mutation Prevalent types Factor 8 deficiency -​ Hemophilia A or classic -​ 80-85% of cases Factor 9 deficiency -​ Hemophilia B or Christmas dz Von Willebrand dz (VWD) -​ Heredity bleed d/o characterized by def, abnormality or absence of vWF protein Discuss the therapeutic and nursing care management of children with hemophilia Therapeutic M Primary therapy: replace missing clotting factor Avoid aspirin & NSAIDS Regular exercise & PT -​ Decrease # of spontaneous bleeding episodes -​ Passive ROM should NEVER be part of exercise regimen AFTER acute episode -​ Joint capsule can be easily stretched & bleed -​ Active ROM are best Education: perform venipuncture & how to administer F8 to kids over 2-3 y/o -​ Learn self-admin @ 8-12 y/op -​ Home tx: highly successful No cure but s/s can be controlled NC Earlier bleeding epi recognized = more effective tx High level of suspicion: h/a, slurred speech, LOC, black/tarry stool Prevent bleed -​ Decrease risk of injury -​ Appropriate exercises -​ Minimize accidental injuries Recognize & control bleed -​ RICE (rest, ice, compress, elevation) Prevent crippling effects of bleed -​ Prevention of bleed is ideal -​ Active ROM exercises -​ Joint elevated & immobilized ruing bleed