NUR 2310: STUDY GUIDE FOR EXAM 2 Cardiovascular Disorders: o Transposition of the Great Vessels (TGV) Pulmonary artery receives blood from the left ventricle, and the aorta received blood from the right ventricle. S/S Tachycardia, tachypnea, difficulty feeding, poor weight gain, pallor, cool and moist. Newborns w minimal communication defects are cyanotic at birth Genetic Considerations Gestational diabetes, genetics, boys more than girls Diagnostic Tests CBC and ABG CXR ECG, MRI, EKG Pharmacology/Non-Pharmacologic as indicated Nursing Process/Management Medication- prostaglandin E may be given to maintain patent ductus arteriosus Mechanical vent Inverventional cardiac catheter procedure Balloon atrial septostomy Surgery: Arterial Switch Open surgery done within 1-2 weeks of birth Mustang or Senning Procedure- directs the blood with oxygen to the pumping chamber that goes to the lungs Nursing Process/Management Catheterization Preop -NPO, baseline o2, assess and mark pulses, explain procedure to child, prepare child and family Catheterization postop -check pulse distal to sites, monitor temp and extremity color, vital signs q15min, monitor bp, monitor dressing for bleeding or hematoma, monitor intake and output, monitor blood glucose levels o Congestive Heart Failure (CHF) Decrease in cardiac output necessary to meet the metabolic needs of the body S/S Pulmonary venous congestion: tachypnea, labored breathing during feeding, cough, crackles, grunting, cyanosis,diaphoresis during feeding Systemic venous congestion: peripheral edema, fluid retention,, ascites, enlarged liver, jvd Impaired cardiac output: tachycardia, pallor, tiring w play, cool extremities, weak pules, hypotension. Oliguria, irritability, delayed cap refill Increased Metabolic demand: Diaphoresis, slow weight gain, , failure to thrive, weight loss Clinical Manifestations Depends on affected side Right sided hf moves unoxygenated blood to the pulmonary circulation Failure results in the backup of blood in systemic venous systems Left side of heart moves oxygenated blood from pulmonary circulation to the systemic circulation Failure causes backup into the lungs When body compensates- peripheral vasoconstriction=cold/blue hands and feet and tachycardia and tachypnea Infants Early: easily tired, weight gain, diaphoresis, frequent respiratory infection Late: Tachypnea, tachycardia, parlor or cyanosis, nasal flaring, grunting, or retractions, cough or crackles, s3 gallop Older children Early: Exercise intolderance, dyspnea, addominal pain or distension, peripheral edema, changes in skin color Late: Anorexia, wheezing, crackles, fluid, volume excess, jugular vein distension Complications Laboratory Diagnostic Tests Decreased cardiac output, increased fluid volume, risk for impaired skin integrity, imbalanced nutrition, compromised family copinh Pharmacology/Non-Pharmacologic as indicated Nursing Process/Management Loop diuretics are potassium wasting, you need to use potassium sparing like spironolactone and aldactone Nursing Process/Management Assessment of child and family: provide of emotional support, discharge planning and teaching Promote ocygenation: improve respiration: give oxygen to reduce dyspnea Cardiovascular function: reduce the work of the heart Meds: Digoxin (Lanoxin): Solws hr, increases cardiac filling time, decreases cardiac output, used w increased pulmonary blood flowAssess apical pulse for 1 minute if brady (lower than 90), monitor for digoxin toxicity- blurred vision, bradycardia Furosemide (Lasix): rapid diuresis not potassium sparing, monitor for hypokalemia below 3.9 for infant 3.9 newborn Teach parents to avoid competitive sports, nutritional guidance to prevent anemia, no vacations to high altitudes Give pt family a scale, first indication is sudden weight gain o Rheumatic heart disease (RF) Autoimmune disease caused by beta-hemolytic streptococcus infection that causes pharyngitis Genetic Considerations Family History of RF Clinical Manifestations Symptoms usually develop within a few weeks after strep throat: shortness of breath, irregular heart beat , chest pain, fever, rash, tiredness, loss of appetite, irritability, poor concentration, and behavioral problems Polyartritis, nontender, subcutaneous nodules over bony prominence , chorea (sudden movememts) Damage to heart valves: carditis, mitral/aortic stenosis, mitral regurgitation, chf, afib Diagnostic Test Elevated c reactive proteins (CRP titer) Throat culture: GABHS serum antistreptolysin-O (ASO titer) - most definitive Radiology, EKG, ECG Therapeutic Management Antibiotics: Penicillin G, V, Sulfidiazine, or erythromycin if allergic to others Antipyretics/analgesic Antiinflammatory joint disease- Aspirin Steroids-Prednisone- for severe cardiac symptoms Anticonvulsant:severe symptoms of chorea Treatment of recurrent RF Prophylaxtic antibiotivs Salicyliates control imfalmmatory Nursing Process/Management Reassure joint damage Prevent GABhs, PROPER TREATMENT OF STREP o Kawasaki Disease (KD) Mucotaneous lymph node syndrome leading cause of acquired heart disease in children, weakens the walls of the vesssels, often results in aneurysms Categories and stages Acute: sudden high fever unresponsive to antipyretics and antibiotics Subacute: Lasts from the end of fever through clinical signs Convalescent: clinical signs have resolved but labs are not normal, end when labs are normal at 6-8 weeks S/S Acute: Irritability, Hyperemic conjunctivae, fever over 102.2 that lasts for 5 days, inflamed mouth, red toungue, red hands and feer, nvd, pain in abdomen Subacute: lasting 2 or more weeks No fever, cracked lips and fissures, swollen and peelings hands and feet, swollen painful joints, cardiac disease, thrombocytosis Convalescent:6-8 weeks after onset Symptoms slowly decrease, lingering signs of inflammation, lines on nails Diagnostic Tests Leukocytosis shift to left ESR C-reactive protein EKG ECG XRAY Therapeutic Management No live vaccines w blood products High dose IV immunoglobin G 7-10 days Salicyliate: 6 weeks or longer Anticoagulant therapy: warfarin Coronary aneurysms Nursing Process/Management Avoid MMR and Varicella 80-100 mg/kg for antiinflammatory o Tetralogy of Fallot (ToF) 4 defects Pulmonary stenosis Hypertrophy of the right ventricle Overriding aorta Ventral Septal Defect Squatting, or knee to chest for infant Boot shaped heart, surgery if not squatting S/S Genetic Considerations Pathophysiological Changes Clinical Manifestations Complications Laboratory Diagnostic Tests Therapeutic Management Pharmacology/Non-Pharmacologic as indicated Nursing Process/Management Nursing Process/Management o Atrial Septal Defect (ASD) Categories and stages S/S Genetic Considerations Pathophysiological Changes Clinical Manifestations Complications Laboratory Diagnostic Tests Therapeutic Management Pharmacology/Non-Pharmacologic as indicated Nursing Process/Management Nursing Process/Management o Ventricular Septal Defect (VSD) Categories and stages S/S Genetic Considerations Pathophysiological Changes Clinical Manifestations Complications Laboratory Diagnostic Tests Therapeutic Management Pharmacology/Non-Pharmacologic as indicated Nursing Process/Management Nursing Process/Management o Patent Ductus Arteriosus (PDA) Categories and stages S/S Genetic Considerations Pathophysiological Changes Clinical Manifestations Complications Laboratory Diagnostic Tests Therapeutic Management Pharmacology/Non-Pharmacologic as indicated Nursing Process/Management Nursing Process/Management o Coarctation of the Aorta (CoA) Categories and stages S/S Genetic Considerations Pathophysiological Changes Clinical Manifestations Complications Laboratory Diagnostic Tests Therapeutic Management Pharmacology/Non-Pharmacologic as indicated Nursing Process/Management Nursing Process/Management o Hypoxemia Categories and stages S/S Genetic Considerations Pathophysiological Changes Clinical Manifestations Complications Laboratory Diagnostic Tests Therapeutic Management Pharmacology/Non-Pharmacologic as indicated Nursing Process/Management Nursing Process/Management Musculoskeletal Disorders o Scoliosis o Hip Dysplasia o Club Foot Hematological Disorders o Leukemia (ALL) Malignant diseases of the bone marrow and lymphatic system MOST COMMON FORM OF CHILDHOOD CANCER LEADING CAUSE OF DEATH FROM DISEASE IN CHILDREN PAST INFANCY Categories and stages 2 forms (ALL & AML) 4 phases of therapy Intro therapy- 4-6 week CNS prophylactic therapy: intrathecal chemo Intensification therapy Eradicate residual leukemic cells, prevent resistant leukemic clones Maintenance therapy, preserve remission S/S Assess for hypoxia, fatigue, anemia, pain, cachexia (wasting syndrome, muscle loss), Testicular exam Lethargy= decrease Hgb Decrease amount of oxygen to the brain Pallor-anemia Petechia and brusing- thrombocytopenia, lymphadenopathy, hepatosplenectomy Changes on mental status (CNS)-papilledema, nuchal rigidity (Stiff neck), cranial nerve palsy Bone Pain-arthritis Testicular check- unilateral painless testicle enlargement Infection- fever (low or high); neutropenia, panocytopenia (all cells are reduced) Palpable mass Genetic Considerations Trisomy 1- down syndrome= BIG RISK 20 times greater Children w 50 chromosomes- best prognosis (No environmental factors) Complications Laboratory CBC, Peripheral blood smear, serum chemistry- potassium, phosphorus, calcium, uric level acid, LDH, Coagulation studied- pt,aPTT, fibrinogen, d-dimer Diagnostic Tests Bone marrow aspiration=best dx Lumbar Puncture Pathological Evaluation Immunophenotyping Cytogenic studies Molecular studied (FISH, RT-PCR, Southern blot) Therapeutic Management Collaborative approach, decision w fam and child Surgery, Chemo, Radiation, Biotherapy, HSCT, Complimentary Therapies, End of Life Pharmacology/Non-Pharmacologic as indicated Nursing Process/Management Antineoplastics- vincristine, MTX, imatinib Corticosteroids- prednisone and dexamethasone Antimicrobials- TMX/SMP and pentamidine Antifungals- fluconazole Non pharm IV fluids Cranial irradiation: prevents overt CNS relapse Allogeneic HSCT Surgical options Placement of central venous catheter Chemo, blood products, antibiotics, blood sample Nursing Process/Management Assess for bleeding and infection q8h Monitor for toxic side effects of chemo or tumor cell lysis Check renal function for children on cyclophosphamide Educate fam- handwashing, preventing infection, oral care For bone marrow suppression- isolation and transmission precautions o Hemophilia Bleeding disorder as a result of a defiencies of coagulation factors Categories and stages Hemophilia A (Factor XIII deficient) – MOST COMMON= X LINKED RECESSIVE Von Willebrand disease- bleeding disorder caused by low levels of clotting protein (fibrinogen) Hemophilia B (Christmas disease, factor IX deficiency-hereditary) Hemophilia C (factor XI deficiency) Genetic Considerations X link inheritance. Mom is carrier, son gets it Risk: family history, males Pathophysiological Changes Clinical Manifestations Prolonged bleeding from minor trauma Hematoarthritis: bleeding into joints that will need to be drained if severe Spontaneous or traumatic bleeding HEMATOMA- HEAD INJURY SUPER BAD Petechiae isn’t common bc platelette count is normal 150k-450k Diagnostic Tests Bleeding episodes, ptt bleeding time, identification of defencient factor (factor assay) Therapeutic Management IV administration of the deficient clotting factor- monitor for allergic or anaphylaxis Prophylactically or on demand, dose varies by injury Synthetic vasopressin Pharmacology/Non-Pharmacologic Desmopressin- reduce intercranial pressure, mild cases ONLY, IV or intranasal Aminoproic Acid and tranexamic acid- prevent recurrent bleeds NOT ongoing bleedings Nursing Process/Management NO NSAID, good dental hygene Monitor clothing times and coagulation studies, factor replacement RICE, monitor for internal bleeding, esp: intercranial o Idiopathic Thrombocytopenia Purpura (ITP) Most frequent hemmoragic in children, more common in girls 3:1, autoimmune disorder, after viral infection Categories and stages S/S Genetic Considerations Pathophysiological Changes Clinical Manifestations Complications Laboratory Diagnostic Tests Therapeutic Management Pharmacology/Non-Pharmacologic as indicated Nursing Process/Management Nursing Process/Management o Sickle Cell Anemia (SCA) Categories and stages S/S Genetic Considerations Pathophysiological Changes Clinical Manifestations Complications Laboratory Diagnostic Tests Therapeutic Management Pharmacology/Non-Pharmacologic as indicated Nursing Process/Management Nursing Process/Management o Iron Deficiency Anemia (IDA) Most common hematologic disorder, decreased RBC and/or hemoglobin, loss or destruction of existing RBC S/S Pallor, glossitis (soft tongue), cheilitis (mouth cracking at corners), decreased peripheral resistance, hemodilution, may have murmur, fatigue, PICA, growth retardation Diagnostic Tests CBC-Decreased RBC, decreased hemoglobin and hematocrit, hemoglobin is usually less than 10-11 CBC w differential Energy level and activity Therapeutic Management 1. Nutrition, 2, Supplement, 3. Transfusion Treat underlying cause=transfuse hemmorhage, nutrition if diet Supportive care-IV, oxygen, bed rest, diet, family education Supplemental iron: NEEDED FOR 6 MONTHS, supplemental folic acid-ferroud sulfate prophylaxis- 1-2mg/kg, mild-moderate- 3 mg/kg, severe 4-6mg/kg