Exam 2 Flashcards

Question

Management of Hydrocephalus: Shunt Complications (4) Nursing Care (6)

Answer 1

Complications: infection, malfunction, subdural hematoma, peritonitis Nursing care - keep head flat to prevent rapid drainage (flex head prior to shunt placement; place on unoperated side - shunt is for life and revised when growth - do not pump shunt - educate family on name and model of shunt - no scalp IVs - test any drainage for glucose (CSF leak)

Answer 2

Ventriculoatrial (VA) - drains fluid from ventricles into atria of the heart usually done when hx of abdominal surgery - contraindicated with CHD or elevated CSF protein Ventriculoperitoneal shunt (VP Shunt) - drains fluid from ventricles into peritoneum - preferred method b-c longer and allows growth - observe for abdominal distention (s/s of peritonitis) - contact sports prohibited

Answer 3

Infections: Septicemia, Bacterial endocarditis, Wound infection, Meningitis Treatment - massive-dose antibiotics - shunt externalization (at level of auditory meatus, clamp during ambulation) - shunt removal

Answer 4

Acute inflammation of the meninges and CSF which spreads via vascular dissemination (due to Hib, GBS, Meningococcal) Seasonal variation: late winter and early spring Prevention: Hib vaccine; prophylaxis in pregnancy Diagnosis: Lumbar puncture unless increased ICP then CT scan Severe complications: seizures, subdural effusions, deafness (from CN VIII damage), sepsis, obstructive hydrocephalus, hemiparesis (r/t thrombi in veins)

Answer 5

* Fever, chills * Nuchal rigidity * Photophobia * + Brudzinski’s sign (neck stiffness causes hip and knees to flex when neck flexed) * + Kernig's sign (unable to straighten leg >135 degrees w/o pain) * Joint problems * s/s of increased ICP (poor feeding, vomiting, irritability, seizures, high pitched cry)

Answer 6

CSF color: cloudy, turbulent, purulent ICP Pressure: elevated Culture: positive Presence of following in CSF: WBCs: elevated esp. polymorphonuclear leukocytes Protein: elevated Glucose: Decreased

Answer 7

CSF color: clear or slightly cloudy ICP Pressure: normal or elevated Culture: negative Presence of following in CSF: WBCs: elevated Protein: normal or slight elevation Glucose: normal

Answer 8

- Droplet isolation due to nasopharyngeal secretions - 10 day Antibiotic therapy (IV) - not for viral (GIVEN ASAP TO IMPROVE PROGNOSIS) - Control fever (hydration, antipyretics) - Pain management- meds - positioning (sidelying) - Reduce ICP (elevate HOB and decreased stimulation)

Answer 9

Causative organism: virus from vector (mosquitoes and ticks) Patho: inflammation of CNS by virus Key S/s: stiff neck, headache, Ataxia, speech difficulties (also fever, malaise, NV)

Answer 10

Epilepsy: Two or more unprovoked seizures Seizure: Caused by excessive and disorderly neuronal discharges in the brain r/t an underlying disease process (such as trauma, meningitis, encephalitis, epilepsy, stress, illness)-- Most common neurologic dysfunction in children

Answer 11

- Local onset and involves a relatively small location of the brain - Simple Partial (w/o impaired awareness; may have aura) - Complex Partial (w/ impaired awareness and automatisms)

Answer 12

- Involves both hemispheres without local onset - loss of consciousness and motor impairment at onset - no aura

Answer 13

Tonic-Clonic seizures (grand mal and most dramatic) - Tonic (stiffening, eyes roll up) - Clonic (intense jerking w/ rhythmic contraction and relaxation longer than tonic phase Absence (petitt mal): Sudden onset of brief loss of consciousness (5-10 sec), blank stare and automatisms; does not fall Atonic (drop): Sudden, momentary loss of muscle tone Myoclonic: Sudden brief, shocklike muscle movements

Answer 14

- transient disorder of childhood (rare after 5 yrs of age) - fever > 38C (100.4 C) - treat w/ antipyretics and PRN benzos at home (alongside seizure precautions) - call 911 if > 5 min

Answer 15

- clusters of Jerking of tummy, raising arms, blinks, occur every 3- 5 seconds lasting up to 30 minute - abnormal EEG w/ hyperarrhythmia - treat w/ ACTH (monitor for immunosuppression, glucose, hypertension) OR prednisolone - treat w/ ketogenic diet (monitor ketones and glucose)

Answer 16

Drug therapy (ideally monotherapy for full control; slow discontinuation) Ketogenic diet (high fat, to preserve glucose) Vagus nerve stimulation (palliative, generator in chest sends electrical impulse to Vagus nerve (CN X) at onset of seizure after activation w/ magnet) Corpus Callosotomy (ligation of area via surgery)

Answer 17

- Time and record behavior surrounding Seizure - Keep patient safe – ABCs - Oxygen - Seizure precautions (no restrain, nothing in mouth during, suction equipment at bedside, ease to floor, ) - aspiration precautions (post seizure NPO and suction; place on side)

Answer 18

- Apnea - Cyanosis - Pupils dilated and non-reactive - Incontinence

Answer 19

Diazepam or Lorazepam (Ativan) -oral, buccal, rectal, IV Fosphenytoin- (form of phenytoin) phenytoin (DILANTIN) - Do not take w/ milk - can cause gum hyperplasia Levetiracetam (Keppra) - rash and mood swings risk Carbamazepine (Tegretol) - SJS risk Phenobarbital - for status epilepticus - take adequate vitamin D and folic acid Valproic Acid - liver toxicity

Answer 20

- bones join together too early and surgery separates them (posterior fontanel < 8 wks; anterior fontanel < 12-18 months) Treatment: Surgery Complications: Edema, Bleeding (black eyes), Infection

Answer 21

Cause: giving aspirin or Pepto bismol to children with a viral infection S/s: cerebral edema, liver involvement Diagnostics: Liver biopsy (fatty liver) Complications: Coma, Brain herniation, Death

Answer 22

- other anomalies (trisomies 21, 13, 18, turner, DiGeorge) - Maternal Chronic illness (diabetes, PKU) - Maternal Alcohol consumption, Substance abuse - Exposure to environmental toxins - In utero Infections (CMV, toxoplasmosis, Rubella, HIV) - Medications (phenytoin, valproic acid, amphetamine, OCs) - Family history of heart defects - IUGR or HBW

Answer 23

- Tachypnea/tachycardia - Activity/Exercise intolerance (poor feeding, fatigue during eating) - Developmental delay, FTT - Frequent respiratory tract infections - cyanosis (unresponsive to O2) - Unusual pulsations – JVD, diminised peripheral pulses, pallor - clubbing of fingers - heart sounds (murmurs, distinct, muffled; irregular; thrills) - Enlarged organs (cardiomegaly, hepatomegaly, splenomegaly)

Answer 24

Primary parenchymal lung disease ( Pneumonia, meconium aspiration syndrome) -Problem is with O2 diffusion - Responds to increased FiO2 Primary cardiac disease - combo of decreased pulmonary flow and intracardiac mixing of “blue” and “pink” blood - Doesn’t respond much to increased FiO2

Answer 25

ECG - electrical activity of heart (assess child, not monitor; takes 15 min) ECHO -ultrasound of structure, size, blood flow through heart (takes an hr and child be still) Exercise/Stress test - monitor during exercise Syncope/ Tilt Table Test- determine cause of fainting (normal: BP stable, HR increases w/ horizontal to vertical position; positive: Pt passes out, hypotension, bradycardia/tachycardia w/ horizontal to vertical position due to heart or BV problem Cardiac MRI- still and moving pics of heart and major BVs to analyze structure and function (sedation for child < 7; contraindicated w/ metal implants (pacemakers, cochlear)

Answer 26

Diagnostic- prior to surgical repair – Right and/or Left cath. Right through vein, left through artery to Helps plan for procedures - Angiogram - visualize the arteries surrounding the heart w/ contrast dye and X-ray images Interventional – repair or other intervention Electrophysiology studies – evaluate and destroy (ablate) accessory pathways that cause some tachydysrhythmias

Answer 27

- Assess dye allergy (iodine) - Age-appropriate teaching (informed consent from parent) - NPO at least 4-6 hours (IV fluids for infants) - Assessment: VS baseline cardiopulmonary, perfusion, assess and MARK all pulses, height & weight - Sedation- oral or IV - Assess for infection may be postponed if severe diaper rash

Answer 28

- Monitor blood glucose (hypoglycemia possible), VS, dressing, I & O, continuous cardiac monitoring - Bedrest for 4-6 hr, Strict. - Keep affected extremity straight for 2hr, then limited ambulation for 3 days - avoid baths and activity for 48-72 hrs - HOB flat x 2 hrs, elevate 30˚ 2 hrs after completion of procedure. - Check cath site and distal extremity pulses, color and perfusion, q15 min x 4 occurrences, then q 30 min x 4 occurrences , then q1h x 3 h, - Pulse - normal to be weaker in affected extremity for a few hours - remove dressing in 24 hrs

Answer 29

- If bleeding, apply pressure at cardiac catheter entry site (2.5 cm above entry site) - swelling - hematoma - arrhythmia (possible and reason for monitoring) - fever

Answer 30

↑ Pulmonary blood flow - Atrial Septal Defect (ASD)* - Ventricular Septal Defect (VSD)* - Patent Ductal Arteriosus (PDA)* Obstruction of blood flow (out of the heart) - Coarctation of aorta* - Pulmonic Stenosis * - Aortic Stenosis ↓ Pulmonary blood flow - Tetralogy of Fallot (TOF)* Mixed blood flow - Transposition of Great Arteries/Vessels (TGA)* - Hypoplastic Left Heart Syndrome (HLHS)

Answer 31

- Abnormal connection between 2 sides of heart increased blood volume on R side - Left to Right shunt - Decreased systemic blood flow due to increased pulmonary blood flow

Answer 32

- If small, may be asymptomatic and close spontaneously - Fatigue (poor feeding, activity intolerance) - Heart Murmur - Risk for Endocarditis - Risk for pulmonary vascular obstructive disease - risk for CHF - Growth delay (FTT, poor weight gain)

Answer 33

Abnormal opening b/ atria so L-R shunt Key S/s - usually asymptomatic - murmur (systolic w/ fixed split-second sound) - atrial dysrhythmias (r/t RA and RV enlargement) - hypertrophy of right side Complications: HF by 30-40 yrs if untreated; embolism

Answer 34

Surgical- Pericardial/Dacron patch w/ cardiac bypass Non-surgical- Amplatzer Septal Occluder placed by cardiac cath (need Low dose aspirin x 6 months)

Answer 35

Abnormal opening b/w heart ventricles (most common defect) causing higher systemic resistance and ventricle pressure Key S/s - Murmur (loud, harsh)-- best heard at left sternal border - right side hypertrophy Complications: HF

Answer 36

Palliative- PA banding to decrease pulmonary blood flow Surgical- Patch (large defect) or suture w/ cardiac bypass- preferred Non-surgical- Device closure with catheterization; more risk than ASD for complete AV block

Answer 37

failure of fetal ductus arteriosus to close at birth causing shunt from aorta to pulmonary artery S/s: Murmur (machinery like), widened pulse pressure, pulmonary arterial HTN Complications: right sided hypertrophy

Answer 38

Medical - prostaglandins to keep PDA if other defects present (side effect = apnea) - prostaglandin inhibitors (Indomethacin (Indocin) or ibuprofen) in newborns closes PDA Nonsurgical - Coiling to occlude in cath lab for > 1 yr if small Surgery (if large) - Ligation of PDA with surgery (thoracotomy) - Thoracoscopic placement of clip

Answer 39

What keeps it open: - Low pO2/ hypoxia - Prostaglandins - Nitric oxide What constricts it? - O2 - Norepinephrine, acetylcholine, bradykinin - Indomethacin, ASA

Answer 40

- Localized narrowing near the insertion of the DA - Increased pressure proximal to defect (upper extremities); left ventricle and left atrium - Decreased pressure distal to the defect (lower extremities)

Answer 41

- bounding pulses in UE’s - Weak or absent LE pulses, cool LE’s - Heart failure (acidosis and hypotension) - HTN in UE shows as dizziness, fainting, headaches, epistaxis in older children - Risk for stroke - Risk for aortic aneurysm

Answer 42

Medical - Initially, may need Prostaglandin E to maintain PDA (palliative) Nonsurgical - Balloon angioplasty - stent placement for patency of aorta Surgical - end to end anastomosis with prosthetic graft or portion of L subclavian artery - Cardiac Bypass not required, thoracotomy incision used - give mechanical ventilation and inotropic support (Digoxin) pre-op - manage HTN post-op with meds (Nitroprusside or ACE inhibitors)

Answer 43

- VSD (pressure equal in right and left ventricles) - Overriding aorta (determines blood distribution) - Pulmonic stenosis (decreases pulmonary blood flow) - Right ventricular hypertrophy

Answer 44

- Mild to severe cyanosis at birth - Murmur - Clubbing - Polycythemia/clot formation (emboli) - Failure to thrive - Tet spells (anoxia with crying, feeding -- risk for seizures, loss of consciousness, emboli, death)

Answer 45

Palliative - during Tet spell, put in knee-to-chest OR squat position - BT shunt w/ subclavian artery Complete repair - Close VSD - Resect pulmonary stenosis and patch pulmonary valve stenosis to enlarge right ventricle outflow via bypass and median sternotomy

Answer 46

- PA leaves the LV - Aorta leaves the RV - Must have other defect to allow for mixing (patent foramen ovale, PDA, or VSD)

Answer 47

- Severe cyanosis at birth with minimum communication of saturated and desaturated blood unless large VSD or PDA - Symptoms of heart failure (pulmonary congestion) - Murmur (+/-) - Cardiomegaly within a few weeks

Answer 48

Medical - Prostaglandin E to maintain PDA and O2 sat ≥75% (side effect – apnea (likely need intubation/ventilator)) - IMMEDIATELY Nonsurgical - Balloon Atrial Septostomy (BAS; Rashkind) - cath lab Surgery - arterial switch in first few weeks of life (transection of great arteries and anastomosing pulmonary artery to aorta to establish normal circulation)

Answer 49

- Air filters on IV lines to prevent R→L shunt of air bubbles - Monitoring (Central/Arterial lines, Cardiac monitor and pulse oximeter, Chest tube (strip only if cardiac), Pacemaker on abdomen)) - Ventilator usually via nose - If murmur expected, you should hear it - Strict I and O (UOP > 1 mL/kg/hr; NPO if intubated) - Monitoring Labs (K+ (never bolus potassium!), Ca+) - do not lift by arms until 6 weeks post-op (scoop instead) - maintain thermoregulation (hypothermia possible post- op so use radiant heat warmer for infants

Answer 50

inability of the heart to pump an adequate amount of blood into the systemic circulation to meet metabolic demands Causes - structural abnormalities r/t blood volume and pressure in heart (heart defects) - Myocardial failure (cardiomyopathy, dysrhythmias, electrolyte imbalance (severe)) - excessive demands on heart muscle (Sepsis, severe Anemia)

Answer 51

- restlessness - anorexia, FTT, fatigue, weakness - Tachycardia, gallop - diaphoresis - pallor or mottling (purply) - poor perfusion (cool extremities, weak pulses, delayed cap refill, decreased urine output) - cardiomegaly

Answer 52

- Tachypnea - respiratory distress (Crackles, cough, retractions, flaring, grunting, wheezing) - cyanosis - orthopnea - activity intolerance (dyspnea)

Answer 53

Hepatomegaly weight gain edema (peripheral edema, periorbital edema) ascites neck vein distention

Answer 54

- Prevent crying and keep them calm - thermoregulation (radiant warmer; shivering increases demand) - cluster care to prevent sleep interruption - semi-fowler positioning

Answer 55

- Monitor apical pulse - Hold if HR under 70 bpm in older child or 90-100 in young child/infant OR sig lower than previous reading - Do not repeat dose if vomiting, notify health care team if misses more than 2 doses - Give water after dosing to prevent tooth decay

Answer 56

Therapeutic Effect - Increases force of contraction/contractility (+ inotrope)- prolonged PR - Decreases HR (- chronotropic) - reduced ventricular rate - Increases renal perfusion Toxicity - bradycardia - NV - visual changes (see halos) - poor feeding, anorexia - dysrhythmias

Answer 57

Therapeutic Effect - Vasodilation decreases PVR and SVR (preload) - Decreased BP and afterload - Reduces aldosterone which prevents fluid retention Side effects: angioedema, Cough, hyperkalemia, hypotension, renal dysfunction Care: Check BP before and after administration

Answer 58

- Monitor potassium (s/s hypokalemia = muscle weakness, irritability, drowsiness, tachy/bradycardia) - Potassium supplements - K+ rich foods (bran cereals, potatoes, tomatoes, bananas, oranges, leafy veggies) - Daily weights, I & O - give early in day

Answer 59

- feed when well rested at first sign of hunger b-c fatigue can decrease feeding - increased caloric needs w/ Polycose, corn oil - Preemie nipple or enlarge opening - Allow at least 30 minutes for feedings (then gavage remaining feeding) - stroke jaw to encourage sucking

Answer 60

- give oxygen w/ caution b-c can cause vasoconstriction but decreases PVR - raise HOB 45 degrees

Answer 61

Inflammatory condition impacting joints, heart, brain and skin after untreated Strep infection Incidence: 2-6 weeks after a group A streptococcal infection Complications: Rheumatic Heart Disease (mitral valve damage) Prevention of recurrence: Penicillin or erythromycin BID; or IM penicillin q28 days for 10 yrs if carditis (5 yrs w/o carditis)

Answer 62

- Carditis (New murmur or valve regurgitation; Cardiomegaly; Pericardial friction rub) - Polyarthritis (Swollen, hot, red, very painful joint pain); arthralgias - Chorea (Sudden aimless, irregular movements of extremities, exacerbated by stress) - Nontender SubQ nodules - Erythema marginatum (Pink nonpruritic rash) - Fever (> 38.5 C) - ESR > 60 mm or SRP > 3 mg/dL - Prolonged PR

Answer 63

- Antibiotics (Penicillin) - ASA for 2 weeks (aspirin to prevent clots, control inflammation, fever, discomfort) - prednisone if no response to aspirin - Bedrest initially followed by quiet activities - heart valve repair or replacement for RHD

Answer 64

infection of valves and inner lining of heart which can damage or destroy valves, deposit fibrin and platelet thrombi Transmission: Agents enter blood from any localized infection, brushing teeth, flossing, chewing, invasive procedures (GI, GU, dental, cardiac) Risk factors: CHD Complications: heart failure, dysrhythmias

Answer 65

- Heart (HF, dysrhythmias, new/changed murmur) - arthralgias - headache - weight loss - Vegetations on ECHO - inflammation signs (fever, malaise, +/- blood cultures, increased ESR, leukocytosis) - Splinter hemorrhages (thin black lines under the nails) - Osler nodes (red, painful intradermal notes on pads of phalanges) - Janeway lesions (painless hemorrhagic areas on palms and soles) - Petechiae on oral mucous membranes

Answer 66

- Prosthetic heart valve repair - History of endocarditis - Heart transplant with history of abnormal valve function - Cyanotic CHD not fully repaired (includes shunts or conduits) or for first 6 months after repair - Repaired congenital heart disease with residual defects (i.e. persisting leaks or abnormal flow near prosthetic patch or prosthetic device)

Answer 67

- Acute stage (inflammation of capillaries, arterioles, venules and pancarditis) esp coronary arteries - Progression to muscular arteries (may lead to coronary artery aneurysms or MI) - Vessels reach max enlargement in 4-6 weeks from onset of fever - disease resolves in 6-8 weeks

Answer 68

- Fever (>102.2) for 5 days - bilateral nonexudative conjunctivitis - cervical lymphadenopathy (unilateral) - rash esp trunk and perineal - mucositis (strawberry tongue, red lips) - Swelling or erythema of palms and soles (peripheral edema) - desquamation of skin (in 2-3 weeks; painless) - IRRITABILITY (last up to 2 months) - temporary arthritis, lethargy - Inflammation of myocardium (myocarditis, valvulitis, arrhythmias)

Answer 69

Elevated ESR, CRP Anemia Leukocytosis Do Baseline Echo Platelet rises in 2nd week

Answer 70

Purpose: high dose to reduce risk of coronary artery abnormalities) Nursing Care/Pt education - usually 1 infusion - same care as blood products (2 nurse check, frequent VS) - monitor cardiac status and anaphylaxis - avoid MMRV for 11 months after admin

Answer 71

Aspirin (anti inflammatory and reduce clot formation) - given 6-8 wks; indefinitely if cardiac abnormalities - avoid chickenpox and flu to avoid Reye's - avoid contact sports b-c low platelets w/ ASA - Warfarin, Plavix (clopidogrel), lovenox (enoxaparin) if aspirin inadequate)

Answer 72

- skin (cool compress, lotion, soft cloths) - oral care (lip lubrication, soft foods, clear liquids) - Monitor VS, I & O, weight, ECG - Passive ROM to increase flexibility - environment (quiet, respite for parents)

Answer 73

Inadequate tissue perfusion to meet the metabolic needs results in cellular dysfunction and organ failure Consequences: hypotension, tissue hypoxia, metabolic acidosis

Answer 74

Hypovolemic (blood loss (Trauma, IC bleed, GI bleed), plasma loss (sepsis, acidosis), ECF loss (NVD)) Distributive (Anaphylaxis, sepsis) Cardiogenic (post op CHD, pump failure (myocarditis, trauma, CHF), Dysthymias (SVT, AV block))

Answer 75

Degree of tachycardia- present Perfusion to extremities- pallor, diminished UO, LOC- irritable, thirsty BP- normal, narrow pulse pressure

Answer 76

Perfusion to extremities - cool and pale, decreased skin turgor, poor cap refill LOC - confusion and somnolence BP - low BP Other- metabolic acidosis, tachypnea

Answer 77

Perfusion to extremities - Anuria; thready, weak pulse; periodic breathing or apnea LOC- stupor or coma BP- hypotension

Answer 78

- VS q15 minutes; output hourly - Ventilatory support- Early endotracheal intubation - IV NS or LRs ( 20 ml/kg push) - Albumin (b-c remain in system longer than NS) - Catecholamines to improve heart pumping (Dopamine and Epinephrine) - legs flat and above heart

Answer 79

Patho: increased RBC destruction b-c sickled hgb unable to obtain oxygen causing obstruction, inflammation (local hypoxia), adhesion Types: homozygous HgbSS, heterozygous HgbSC, Beta thalassemia (seen in mediterranean) Incidence: autosomal recessive seen in AA; manifests after 4-6 months when fetal Hgb disappears

Answer 80

- Mandatory screening of all newborns - Sickledex- detects HgbS but cannot differentiate trait from disease; heel stick - Hgb electrophoresis (definitive diagnosis)- separates various forms of Hgb via fingerprint of protein - CBC- anemia

Answer 81

Vaso-occlusive crisis (ischemia and pain) – main - Painful joints and hands/feet (dactylitis) - Abdominal pain - retinal (detachment, blindness or visual changes, icteric sclera) - renal (Hematuria, dark urine, enuresis) - Priapism - AVN (avascular necrosis) of hip or shoulder - lordosis or kyphosis - leg ulcers

Answer 82

Sequestration crisis- RBC breaking down quickly * Hepatomegaly (jaundice) * Splenomegaly (fibrous or rupture; functional asplenia)– may need spleen removed * Circulatory collapse/shock (cardiomegaly, systolic murmur, tachycardia, Fatigue)

Answer 83

- high reticulocyte count (reticulocytosis) - reduced RBC production

Answer 84

- Chronic Anemia (hgb 6-9 g/dl) - Susceptibility to sepsis (r/t functional asplenia) - Growth retardation - Delayed sexual maturation

Answer 85

- Anything that increases body’s need for oxygen or alters transport of oxygen - Hypoxia - Fever (notify provider if > 38.5) - Infection, trauma - dehydration (b-c increases blood viscosity) - Physical and emotional stress

Answer 86

Acute Chest Syndrome (ACS) - r/t pulmonary infiltrates - Report chest pain, fever of 38.5 C or higher, congested cough, tachycardia, dyspnea, retractions, decreased O2 sat - Call provider immediately Cerebral Vascular Accident (CVA) - r/t HgbSS blocking BVs in brain - Report seizures, abnormal behavior, weakness or inability to move an extremity, slurred speech, visual changes, vomiting, or severe headache - If hx of CVA or beta thalassemia, child may get monthly chronic transfusions for prevention

Answer 87

- Hydration - Hydroxyurea- ↑ Hgb F (fetal hemoglobin), lower leukocyte and reticulocyte levels - Oxybryta (decreases # of crises in 4yrs +)

Answer 88

- Penicillin prophylaxis – 2m to 5yrs (prevents pneumococcal sepsis) - Vaccines (Hib, pneumococcal, meningococcal, influenza) - Transcranial Doppler annually for children 2-16 years- Identify blood clots early via measuring intracranial vascular flow

Answer 89

- Rest - Hydration -oral or IV therapy (tell parents specific amount) - Electrolyte replacement - Blood replacement (phoresis of blood or whole-blood exchange) - Antibiotics for infection - Monitoring of reticulocyte (baby RBCs) count regularly to evaluate bone marrow function - HSCT is only potential for cure (stem cell transplant) -- complications (graft rejection, immunosuppression, graft vs host disease, relapse)

Answer 90

- PCA preferred - Mild: acetaminophen, Ibuprofen, codeine, toradol - Severe: opioids, ketorolac - avoid meperidine (Demerol) b-c risk for seizures and product breakdown - monitor for GI bleeds from NSAIDS - Apply heat or massage affected area - Avoid cold compresses (we want blood flow to area b-c pain from tissue anoxia)

Answer 91

- Wear medical ID band - Frequent rest with physical activity - Avoid contact sports with splenomegaly - avoid oxygen admin b-c can depress RBC production and worsen anemia - refer to crisis as pain episode

Answer 92

Indication: during crisis or monthly if CVA risk or Beta thalassemia Risk: infection, hyperviscosity, alloimmunization, Hemosiderosis and hemochromatosis (iron deposits with/without tissue damage) Nursing Care - Oral chelation agents (deferasirox, deforxamine) to remove excess iron - oral chelation given IV or subQ over 8-10 hours (during sleep)

Answer 93

Bleeding disorder r/t congenital deficiency of specific coagulation proteins (Factor VIII or Factor IX) Incidence: x-linked recessive so usually boys with carrier mother Diagnosis: Factor VIII and Factor IX assays; PTT, DNA testing

Answer 94

Severe (Factor VIII activity <1%)- spontaneous bleeding without trauma Moderate (Factor VIII activity 1-5%)- bleeding with trauma Mild (Factor VIII activity 5-40%)- bleeding with severe trauma or surgery

Answer 95

- excessive Bruising - prolonged bleeding (epistaxis) - Hemarthrosis (bleeding in joint -> bony changes and deformities) - signs: stiff, tingling, ache, inflammation signs - hematuria - hemorrhages (brain = fatal, GI = anemia) - subQ and IM hemorrhages - Hematoma (pain, swell, limited motion)

Answer 96

- Factor VIII Infusions -replace missing clotting factors; every other day or 3x/week for severe - Desmopressin (DDAVP-synthetic vasopressor) (IV or nasal spray)- increases factor VIII activity for mild - Aminocaproic acid- prevents clot destruction; give Factor infusion first - Corticosteroids- for hematuria, acute hemarthrosis, chronic synovitis - Cox-2 inhibitors- for hemophilic arthropathy

Answer 97

- Regular exercise and PT may reduce bleeding episodes - Noncontact Sports- golf, swimming, jogging, fishing, bowling - Oral (Soft bristled toothbrush w/ warm water) - Electric shavers vs razors - SubQ instead of IM injections - Avoid aspirin or ibuprofen (NSAIDs)

Answer 98

- factor replacement - RICE- rest, ice, compression, elevation - Keep cold packs ready - Only active ROM (NO PASSIVE) for hemarthrosis

Answer 99

- Medical ID bracelet - teach child 8-12 yrs to give injections

Answer 100

destruction of pancreatic beta cells causing absolute insulin deficiency

Answer 101

- Polydipsia - Polyuria (enuresis) - Polyphagia - Weight loss - Infections (Vaginitis (candida) or diaper rash) - Fruity odor to breath - Delayed wound healing - Blurred vision - Changes in LOC (very hyper, irritable, Fatigue/lethargy/ drowsy) - Rapid respirations - Abdominal pain - Hot dry skin

Answer 102

- Fasting BG of > 126 mg/dL - random BG of > 200 with classic signs of diabetes - oral glucose tolerance test of > 200 mg/dL in the 2 hour sample (Balanced diet 3 days prior to test; NPO for 8 hours prior; BG levels drawn q 30 minutes x 2 hours) - HbA1C > 6.5% ,<8 %- determines long term control (120 days) for children 6-12 yrs

Answer 103

- Weigh/measure food for 3 months w/ progression to estimation of portion sizes - 3 well balanced meals at regular intervals, afternoon, and night-time snack - Allow child to participate in food choices (balanced colors) - Extra food needs to be consumed for increased activity (10-15 g of carbohydrate for 30-45 minutes of activity) - Avoid concentrated sweets - Use Sugar substitutes in moderation b-c sorbitol metabolizes to fructose then glucose and can lead to osmotic diarrhea

Answer 104

- done ac and hs at minimum - Keep records of results with food intake and any other events such as increased activity or illness that may alter BG - Check strips for expiration and no needle sharing - If using pump, have backup in case it malfunctions AND change needle q48-72hrs - teach school age to give their insulin w. pinch technique and rotate sites

Answer 105

- Do not hide needles from the child (be direct, honest, show them) - Check BG before exercise. May need decreased dose of insulin when exercising or a snack. - wear Medic-Alert bracelet - may need increased insulin during illness or stress - check glucose and urine for ketones q3h when sick

Answer 106

- Sweating/chills - Trembling/shakiness/anxiety/nervousness - Tiredness/ paleness/ palpitations - Blurry vision - Mood Changes (tearful or euphoria - headaches/ confusion/dizziness - Hunger

Answer 107

If conscious - simple glucose (½ cup orange juice or soda; 8 oz milk, Small box raisins, 3-4 hard candies or Life Savers, 1 tsp of sugar or honey, 1 candy bar) - give complex carb and protein afterward If unconscious - cake frosting or glucose paste on gums - glucagon

Answer 108

- BG >240 - Fever higher than 38.9, fever doesn’t respond to acetaminophen, or lasts over 12 hours - + urine ketones - Disoriented or confused - Rapid breathing - Vomiting more than once - Diarrhea over 5 times or more than 24 hours - Unable to tolerate liquids - Illness > 2 days

Answer 109

- Short stature (proportional height and weight) - Delayed epiphyseal closure and bone age - Increased insulin sensitivity - Delayed dentition (crowded or mispositioned) and Underdeveloped jaw - Delayed sexual development - premature aging

Answer 110

Growth curve and familial height pattern- Assess height and weight velocity on growth charts MRI and x-ray of left hand/wrist to predict remaining growth potential CT and skull x-rays -determine presence of tumor or other structural defects

Answer 111

- draw baseline IGF-1 and IGFBP-3 (Insulin-like Growth factor) levels - give GH-releasing hormone (levodopa, clonidine, propranolol, glucagon, insulin) or arginine - obtain blood samples q30 x 3 hr - NPO and limit activity 10-12 hours prior to test

Answer 112

- Child should wear identification bracelets - Do note infantilize child - Emphasis abilities and strengths vs physical size (helps develop positive self-image - final height usually less than normal

Answer 113

Route: subQ 6-7 days/week at bedtime for GH releases 45-90 mins after sleep Nursing Care - Give until bone age > 14 in girls and > 16 in boys or when growth is under 1 inch/year - Height is usually attained slower than peers

Answer 114

Function: Thyroid hormone regulates BMR and controls growth and tissue differentiation Synthesis impacted by dietary iodine and tyrosine Consequences of Delayed treatment: poor intellectual development and growth ( 0-3: IQ 89; 3-6 m: IQ 71; After 6 months: IQ 54)

Answer 115

- mandatory screening of all newborns - blood studies if positive screen - Labs (low TH, high TSH)

Answer 116

Poor feeding Lethargy, somnolence, sleepy Constipation Bradycardia Respiratory difficulty (dyspnea on exertion) Large Fontanels

Answer 117

Short forehead and depressed nasal bridge Puffy eyes Large Tongue (macroglossia) Thick dry skin w/ yellow discoloration Dry Coarse but sparse Hair Abdominal Distention Hypotension

Answer 118

Short w/ infantile proportions Obese Abnormal Reflexes Awkward Mobility (growth cessation) Intellectual Disability Thyromegaly

Answer 119

S/S of overdose/toxicity - Tachycardia - dyspnea - irritability - insomnia - fever/sweating - weight loss Nursing Care - Keep TSH levels between 0.5-2 mU/L - given for life so compliance key

Answer 120

- lessens life expectancy (by 5-20 yrs) - Psychosocial consequences (low self-esteem, depression, social isolation, anxiety) - comorbidities (DM2, sleep apnea, HTN, dyslipidemia, asthma, gallbladder disease, metabolic syndrome, fatty liver, PCOS) - stress incontinence (r/t bladder pressure) - Orthopedic (knee or hip pain indicate SCFE and leg bowing i.e. Blount’s disease) - early puberty

Answer 121

- Eat only at the table without other distractions like TV or reading a book. - Use fruits for desserts. - Use smaller plates, plastic utensils - Eat slowly and put fork down between bites - Limit “sit time” at computers, TV, video games to < 2 hrs/day - physical activity 60 min/day - choose low fat, low sugar, low sodium, high fiber foods

Answer 122

- Behavior modification (Appetite regulation via positive reinforcement and peer groups) - Pharmacological agents (ex. Orlistat) - Surgical/ bariatric—not recommended due to many metabolic complications

Answer 123

Positioning - turn q2h - skin care regularly - maintain proper alignment - use active and passive ROM Nutrition - protein - bone healing (calcium and vitamin D) - adequate calories

Answer 124

- Assessment (pain, ROM, tenderness, erythema etc.; key for diagnosis) - X-rays (Difficult to use for diagnosis because of localized swelling but accesses skeletal trauma - CT Scans and MRI (find injuries, areas of infections, and cancer) - Ca 2+ (varies w/ bone breakdown from immobility making hydration crucial) - CBC, WBC and blood cultures - ESR elevation - Alkaline phosphatase - increases with growth

Answer 125

Contusions: damage to soft tissue, subcutaneous tissue, and muscle causes escape of blood into tissues leading to ecchymosis (black-and-blue discoloration) (s/s. Swelling, pain, disability, crush injuries) Dislocation - Displacement of normal position of opposing bone ends or of bone ends to socket Strain - microscopic tear in the muscles and tendons Sprain - A ligament is torn or partially torn.

Answer 126

RICE (rest, ice, compression, elevation) ICES (Ice, compression, elevation, support

Answer 127

* Plastic deformation: bone is bent, not broken * Buckle: appears raised or bulging due to compression of porous bone * Simple or closed: does not produce a break in the skin * Greenstick: bone is angulated beyond limits of bending; compressed side bends and tension side fails leading to incomplete fracture * Open or compound: fractured bone protrudes through the skin * Complicated: bone fragments have damaged other organs or tissues * Comminuted: small fragments of bone are broken from fractured shaft and lie in surrounding tissue

Answer 128

- injury at epiphyseal plate (cartilage growth plate which is weakest point of bone) Complication: longitudinal or angular growth deformities Management - frequent surveillance if type 1 (separation of growth plate) - Surgery (open reduction and internal fixation) if type 3 (half of bone under plate broken)

Answer 129

- RICE (no heat) - splint (immobilize)- soft splint (pillow or folded towel) or rigid splint (rolled newspaper or magazine) - cover wounds w/ sterile or clean dressing - surgery (external fixation (rings to bone w/ wires), internal fixation, distraction (separate bones to encourage growth of new bones)) - calm and reassure family

Answer 130

* Osteoblasts stimulated by break in bone and form bulging growth of new bone tissue * Callus forms (deposition of calcium salts) * Remodeling (smooths out irregularities produced by osteoblasts and callus)

Answer 131

* regain alignment and length of bony fragments (reduction) * Retain alignment (immobilization) - until callus forms * Restore function - ROM and weight bearing after site stable * Prevent deformity or injury

Answer 132

Traction—forward force produced by attaching weight to distal bone fragment; HCP Adjust by adding or subtracting weights Countertraction—backward force provided by body weight; Increased by elevating foot of bed Frictional (patient’s contact w/ bed)

Answer 133

- Relieves fatigue in involved muscles - Fatigues muscles to reduce muscle spasms (rare) - Position distal and proximal bone ends (Realignment) to promote healing - Prevents deformity and contractures - Immobilize healing bone and prevent further injury

Answer 134

Manual- applied by hand placed distal to the fracture site during application of a cast or for closed reduction Skin- Buck’s (leg extended) OR Bryant (legs up 90 degree for DDH) Skeletal - pins, wire, tongs (ex. 90-90 degree (common at hip and knee) or Halo vest (cervical)

Answer 135

- Diazepam (muscle relaxant for spasms) - Provide Skin care (q2h turns, braden scale, hygiene, pressure alt mattress) - Pin site Care daily or weekly (cover w/ rubber or padding; antiseptic (chlorhexidine) or topical antibiotic) - NURSES DO NOT LIFT OR DISCONTINUE WEIGHTS!!!! LET THEM HANG FREELY - prevent constipation (fluids, fiber, stool softener) - promote circulation (ROM, bandages not restrictive)

Answer 136

Neonatal period: 2-3 weeks due to thickened periosteum and more blood supply Early childhood: 4 weeks Later childhood: 6-8 weeks Adolescence: 8-12 weeks

Answer 137

* Pain – unrelieved by pain medication 1 hr after admin, especially w/ passive ROM * Pulselessness - no distal pulse palpable * Pallor - discoloration OR cyanosis; cool temp; cap refill > 3 sec * Paresthesia - tingling or burning * Paralysis – inability to move affected part; * Pressure - swelling, tenseness or warmth of affected limbs and extremities Pallor, paralysis, and pulselessness are late

Answer 138

- fasciotomy (cut skin and reduce pressure) - do not elevate - administer fluids if needed - bivalve cast if excessive edema (i.e., cut calf in half a hold w/ elastic bandage) - CALL HCP

Answer 139

- infection (hot spots on dried cast or foul smell) - Loose cast (no longer useful and can lead to skin irritation or pressure sores) - Compartment syndrome – medical emergency

Answer 140

- Plater of Paris dries in 2 to 48 hours - Fiberglass dries in a couple of hours - handle w/ palms until dry - let child know some warmth may be felt but no burning

Answer 141

- Elevate initially w/ pillow (do not let stay dependent for long) - Encourage use of unaffected muscles - No coat hangers or straws in cast - Use fan or cool air to circulate air if high humidity (no heated air) - for spica, use cast petaling (tape edges w/ waterproof tape around perineal) - if open cast, cut out window to view wound AND outline any blood - Crutches should fit properly w/ soft rubber tip to prevent slipping and well padded (support on hand grips NOT axilla)

Answer 142

- reassure muscle atrophy will go away as strength regained - avoid cold temps after removal - use sunscreen after removal - apply mineral oil or lotion to remove - Soak in bathtub - Do not pull or forcibly remove w/ vigorous scrubbing (may cause excoriation and bleeding)

Answer 143

- Barlow (thigh adducted, and light pressure applied to femoral head to see if slips out of acetabulum then slips back when pressure is released) - unstable hip - Ortolani sign (abduct knees and put forward pressure behind trochanter than backward pressure; audible clunk/thunk if femoral slips forward into acetabulum meaning it is dislocated) - disappears when adduction contractures develop at about 6-10 weeks - Galeazzi sign: Limb shortening on affected side - Trendelenburg sign: When child stands on one foot and bears weight on affected hip, pelvis tilts downward on normal side instead of upward, as it would with normal stability - Asymmetric thigh and gluteal folds seen when prone - lordosis - waddling gait (bilateral hip dislocation

Answer 144

1. Acetabular dysplasia ( mildest form of DDH) - neither subluxation nor dislocation - delay in acetabular development (osseous hypoplasia of the acetabular roof that is oblique and shallow - cartilaginous roof is intact)

Answer 145

2. Subluxation ( largest percentage of DDH) - subluxation ( incomplete dislocation of the hip) - femoral head remains in contact with the acetabulum - stretched capsule and ligamentum teres cause the head of the femur to be partially displaced - Pressure on the cartilaginous roof inhibits ossification and produces a flattening of the socket.

Answer 146

3. Dislocation - femoral head loses contact with the acetabulum and is displaced posteriorly and superiorly over the fibrocartilaginous rim - ligamentum teres is elongated and taut.

Answer 147

- Avoid lotions and powders because they can cake and irritate the skin. - Always place the diaper under the straps - Gently massage healthy skin under the straps once a day to stimulate circulation - Do not adjust harness

Answer 148

Birth to age 6 months - Pavlik harness for abduction of hip for 6-12 week continuously Ages 6-24 months - recognized when child begins to stand and walk - need closed reduction and spica cast - may use traction for full abduction of hip Older child (4yrs +): - need open reduction w/ preop traction - tenotomy (lengthen contracted muscles) - osteotomy ( construct acetabular roof) - correction and healing is very difficult

Answer 149

- Affected foot or feet smaller - shorter, empty heel bed - midfoot medial creases - If unilateral, affected limb shorter and calf atrophy present - Foot pointed down (plantar flexed) and inward

Answer 150

- Positional: primarily from IUGR; no bony abnormality; responds to simple passive exercise and casting - Congenital (idiopathic): in normal child w/ wide range of rigidity & prognosis; Requires casting w/ surgical intervention (heel-cord tenotomy) - Syndromic (teratologic): seen w/ anomalies (such as myelomeningocele or arthrogryposis); requires surgical correction and resistant to treatment

Answer 151

* Correction of deformity * maintain correction until muscle balance is regained * frequent follow-up care to monitor for recurrence of deformity w/ x-rays

Answer 152

- Weekly gentle manipulation and stretching of medial side of foot w/ serial long leg casts ideal shortly after birth - Casting (6-10 weeks) followed by percutaneous heel-cord tenotomy (corrects equinus deformity) - After tenotomy, long leg cast for 3 weeks - After casting, Ponseti sandals w/ bar set in abduction to maintain correction and prevent recurrent - surgery at 6m-1 yr if casting fails

Answer 153

- Spontaneous movement of femoral neck from femoral head in acetabulum due to increased weight Incidence: boys before or during rapid growth Diagnosis: physical exam, hx, Anteroposterior and frog-leg hip x-ray Complications: avascular necrosis, further slippage, deformity

Answer 154

- Obese - limp on affected side w/ altered gait - internal rotation and loss of abduction of affected leg - affected limb shortens - groin, hip, and knee pain - inability to bear weight

Answer 155

- strict bedrest and Buck’s traction immediately - surgery within 24 hrs w/ pin placement OR surgical hip dislocation - post-op no weight bearing to gradual weight- bearing - Restriction from certain sports until fusion or closure of proximal femoral physis to prevent further slippage

Answer 156

* Neurologic injury or spinal cord injury * hypotension from acute blood loss * SIADH * Superior mesenteric artery syndrome( due to duodenal compression by aorta and superior mesenteric artery leading to obstruction; causes epigastric pain, copious NV, and eructation(belch); alleviated w/ left lateral or prone position (worsened by supine)

Answer 157

- spinal deformity involving lateral curve, spinal rotation, thoracic hypokyphosis Diagnosis: x-ray to determine skeletal maturity Levels - <10 degrees = postural variation - 20 or less = may not require intervention; can do exercises or braces - Greater than 40 = requires surgery

Answer 158

- started at School Age (Screening at 10 AND 12 yrs. for girls or at 13 OR 14 yrs. for boys) - child in only underpants - Child bends at waist, trunk parallel to floor, arms hanging free - observe the back for asymmetry of hips, scapula, shoulder height, pelvic obliquity; widened angle between arm and body - Hold hips stable and have child turn left / right to assess flexibility of curve

Answer 159

Types: Boston, TLSO, Milwaukee (usually for kyphosis and has neck ring) Notes - Braces are worn for 23 hours per day - Not curative - slows or stops the progression of the curvature until child reaches skeletal maturity - discontinued once growth completed

Answer 160

- depends on degree of curvature (>45-50 degrees), pain, OR if exercise & brace fails Types - Spinal fusion is more common and less invasive than rod insertion (via posterior or anterior approach) - VEPTR (done for younger children with severe scoliosis via lengthening the ribs by using a prosthetic rib to enhance the breathing effort of children with scoliosis and straighten spine) - instrumentation

Answer 161

- Bed rest - logrolling - PCA / pain control b-c very painful surgery - Teds / SCD’s - frequent assessment (check for sensation and ileus (stool softeners), retention (foley)) - early mobility (usually by day 2; PT on 1st day) - Brace may or may not be used.

Answer 162

- inflammation in joints for > 6 weeks causing pain, swelling, and scar tissue (limited ROM) Incidence: autoimmune condition in girls < 16; total remission in most by adulthood Classification: dependent on joints affected, systemic symptoms, and presence of absence of rheumatoid factors

Answer 163

- 10% have positive rheumatoid factor - ESR/CRP elevated - Leukocytosis w/ exacerbations of systemic JIA - Antinuclear antibodies common but not specific to JIA (can identify those at risk for uveitis) - Radiographs – best to show soft-tissue swelling and joint space widening from increased synovial fluid

Answer 164

Joint signs - limp - favoring an extremity - inflammation (pain, swelling, edema, redness) Systemic signs - Rash - fever - lymphadenopathy - splenomegaly and hepatomegaly - uveitis (inflammation in anterior chamber of eye; diagnosed by slit lamp eye exam)

Answer 165

- Ibuprofen (NSAIDs)- GI bleeds - Aspirin (NSAIDs)- be wary of viral illness b-c reyes syndrome - Methotrexate or Sulfasalazine (DMARD) - teratogenic, hepatotoxic, Bone marrow suppression - Corticosteroids (given intra-articular) - risk for infection, adrenal insufficiency, avascular necrosis, cushingoid, osteoporosis, HTN, diabetes - Etanercept or Adlimulmab (Biologic DMARDs) - infection risk

Answer 166

Goal of therapy - NO CURE - preserve joint function - limit abnormalities and deformity - promote normal growth and development - decrease the discomfort (relieve symptoms)

Answer 167

- Physical therapy (strengthen joints and prevent deformities) - pain relief (usually avoid opioids unless severe) - moist heat ( hot pack, warm bath) - exercise - promote general health and nutrition (match caloric intake to energy needs) - allow child to rest as needed - promote independence ( parent only help when necessary)

Answer 168

- DNA analysis (genetic detection of absence of dystrophin (muscle protein product due to muscle fiber degeneration)) - possible at 12 weeks gestation - Serum CK (elevated in first 2 yrs of life) - EMG (decreased amplitude and duration of motor unit potentials) - Muscle Biopsy

Answer 169

Muscle atrophy and fatty infiltrates on extremities leads to progressive degeneration of muscle fibers Incidence: x-linked; often wheelchair bound by 12 yrs

Answer 170

- osteoporosis and fractures - contractures - scoliosis - obesity - respiratory infection r/t reduced pulmonary vital capacity from weak respiratory muscles; also difficult clearing secretions via cough b-c weak muscles - cardiopulmonary issues due to respiratory, oropharyngeal and facial muscle involvement (heart failure and sleep apnea) - disuse atrophy

Answer 171

- waddles (due to weak butt muscles) causes frequent falls - lordosis (sway back, awkwardly holds shoulders back; due to weak pelvis - Gower’s sign (walking hands up knees and legs to stand up) - Mild to moderate mental impairment (Decrease in verbal skills) - Higher emotional disturbance - fatty muscle hypertrophy (thigh, calf, upper arm)

Answer 172

- encourage normalcy - okay to grieve since chronic fatal disease - Guilt from passing this to son lies on woman - Genetic counseling for female relatives, siblings - provide for rest periods ( child can self-regulate activity tolerance) - prevent infections ( avoid crowds; get pneumococcal and influenza vacs)

Answer 173

- Prevent contractures (passive ROM, soft casts, surgery, braces) - Promote pulmonary function ( breathing exercises, incentive spirometer q4h; mechanically assisted coughing) - Position changes when in wheelchair (allow for time to lie flat) - Keep active as long as possible (don't wheelchair confine early)

Answer 174

Corticosteroids (prednisone, deflazacort) - Increased muscle strength, bulk, power and decreased progression of weakness (Prolonged ambulation) - decreased incidence of scoliosis and cardiomyopathy) - Increased pulmonary function Oral albuterol - increase lean body mass and decrease fat mass - no change in strength