Final Blueprint Pt. 2 Flashcards
what is anemia and RBC disorders anemia?
Anemia = the # of RBCs or hemoglobin concentrations are below normal value.
RBC disorders anemia: decreased blood O2 carrying ability (cyanosis is not common in kids). Hemodilution – causes blood to return to the heart (heart murmur, increased cardiac workload (leads to cardiac failure). Slow growth, delayed sexual maturation, decreased RBC production, increased RBC loss, and increased RBC destruction.
what are the DX, TX, and care for anemia?
Diagnosis: physical exam (fatigue, decreased energy, pallor), CBC, and other DX labs and test.
Treatment: treat underlying cause, replace deficiency, RBC transfusion and fluids, O2 and bedrest.
Nursing considerations: detailed history, atraumatic care, education, and monitor for exertion and infection.
what is iron deficiency anemia and the @ risk population?
not enough iron or a loss of iron.
@ Risk population = preterm infants, toddlers (excessive cow’s milk intake and inadequate nutrition), adolescents (poor eating habits, heavy menses, and obesity)
what is the tx and nursing care for iron deficiency anemia?
Treatment: Ferrous iron oral supplements (3-6 mg/kg daily, >60 mg/kg severe toxicity deferoxamine, polyethylene glycol whole bowl irrigation, gastric lavage, crystalloid infusion, vit K, and FFP), take with vit C, and don’t take with milk products. Parenteral and intramuscular iron (expensive, risk of anaphylaxis, test dose given, common S/E = headache). Packed RBCs (2-3 mL/kg). O2 for severe tissue hypoxia.
Nursing considerations: education = meds (side effects, precautions), diet and nutrition (iron-fortified cereal and high iron foods)
what is beta thalassemia and its forms?
Microcytic anemia non-responsive to iron supplementation associated with people of Mediterranean origin.
Forms:
-Thalassemia minor: asymptomatic silent carrier
-Thalassemia trait: heterozygous and mild microcytic anemia
-Thalassemia intermedia: heterozygous or homozygous, moderate to severe anemia, and manifests as splenomegaly.
-Thalassemia major: AKA Cooley anemia, homozygous, and not compatible with life w/o transfusions.
what are the CM for beta thalassemia?
Anemia (pre-diagnosis): pallor, unexplained fever, poor feeding, splenomegaly or hepatomegaly.
Progressive anemia: signs of chronic hypoxia (headache, precordial and bone pain, decreased exercise tolerance, listlessness, and anorexia).
Other CM: small stature, delayed sexual maturation, bronzed, freckled complexion
Bone changes (older kids): enlarged head, prominent malar eminences, flat or depressed bridge of nose, enlarged maxilla, protrusion of the lip and upper central incisors and eventual malocclusion. Generalized osteoporosis.
what is the dx, tx, and nursing for beta thalassemia?
Diagnosis: HgB electrophoresis
Treatment: blood transfusions, chelation therapy (remove iron from tissues and blood, due to all blood transfusions), and splenectomy (when indicated).
Nursing considerations: promote transfusion and chelation therapy compliance, anxiety coping support for the kid b4 and during treatments and provide emotional support for the kid and family through their treatments.
what is aplastic anemia (AA) and how is it acquired?
rare and life-threatening bone marrow failure
Congenital (Fanconi syndrome (rare, hereditary disorder)
Acquired: most cases are idiopathic, parvovirus, hepatitis, infection, irradiation, immune disorders, meds, chemicals, and leukemia or lymphomas can lead to this.
what are the dx and S/S of AA?
Diagnosis: CBC w/ Diff (anemia, leukopenia, and decreased platelet count), blood smear, and bone marrow biopsy.
S/S: overwhelming infection, pallor and patchy brown or yellow skin, weak, fever, and dyspnea, and uncontrolled bleeding and ecchymosis.
what is the tx and nursing for AA?
Treatment: restore bone marrow function (immunosuppressive therapy (IST) antilymphocyte globulin (ALG) and antithymocyte globulin (ALG), bone marrow transplant (BMT), and HSCT.
Nursing considerations: education (disease, treatment, and central line access and care) and family and patient support.
group of bleeding disorders with a deficiency of one or more factors necessary for blood coagulation prolonged bleeding.
hemophilia
what are the types of hemophilia?
Factor VII Deficiency (hemophilia A)
-AKA classic hemophilia
-80-85% of all hemophiliacs
-Factor VII (AHF) is produced by the liver
-Longer periods of bleeding but not a faster rate
-Classified as severe, moderate, and mild
Factor IX Deficiency (hemophilia B)
-Produced by the liver
-AKA Christmas disease
Von Willebrand disease (vWD)
-Produced in endothelial cells
-vWD deficiency
what is the DX and CMs for hemophilia?
Dx: prolonged bleed, HX of bleed, know X-linked inheritance, and labs = PTT, factor assays, and genetic test
Most common bleeding episodes
Joints (knees, elbows, and ankles): stiff, tingling, decreased ROM, warm to touch, red, and swell
After procedure or trauma: circumcision, injections, and epistaxis
SubQ and intramuscular hemorrhages
Spontaneous hematuria
Hematoma
what is the TX and nursing for hemophilia?
Tx: factor VII concentrates, Desmopressin (DDAVP), corticosteroids, Amicar, PT, home infusion, and high dose of factor for active bleeds.
Nursing: education (treat bleeds early, RICE, safety, gentle oral hygiene, med alert ID, SubQ injections when possible – NO IM, NO ASA OR NSAIDS, maintain healthy wt, and epistaxis treatment) and monitor for bleeding (HA, slurred speech, and LOC).
what is kawasaki?
systemic vasculitis, ectasia (dilation of coronary artery leads to aneurysm (giant)), S/S = high fever, red eyes, ring around iris, strawberry tongue, rash (desquamates), and serious = MI, TX = high dose IVIG and salicylate therapy, and NC: grumpy kids, symptomatic, supportive.
what are the mixed defects?
Transpositional of the great vessels (TGV), total anomalous pulmonary venous return (TAPVR), Truncus arteriosus, and hypoplastic L heart syndrome (HLHS)
Survival depends on mixing of blood from the pulmonary and systemic circulation within the heart, variable S/S depending on defect, cyanosis (although not always visible), CHF, and may require multiple surgeries (many times in the 1st week of life)
what is TGV?
the Aorta exits off the R ventricle and the pulmonary artery off the L ventricle, no communication between the pulmonary and systemic circulations, incompatible with life unless another defect is present that allows the mixing of blood, rapid and sustained cyanosis, surgical repair (immediate), and IV prostaglandin E may be admin to maintain ductal patency until surgery (to force the PDA and ASD to stay open)
what is TAPVR?
R atrium receives all the blood that normally would flow into the L atrium, R side of the heart hypertrophies, is overworked, and may cause a backup of blood in the lungs, L side of the heart, especially the L atrium, may remain small, generally have other defects, such as ASD or PDA, that will help the child by allowing more blood to get from the R side of the heart to the L side and out to the body, and if no other defects, immediate and progressive cyanosis and immediate surgical repair.
what is truncus arteriosus?
during fetal development, failure of the pulmonary artery and aorta to divide, resulting in one single vessel that opens into both R and L ventricles. Resistance is less to pulmonary blood flow than to systemic blood flow, so more blood flow to the lungs. Moderate to severe CHF with variable cyanosis. Surgical repair in the 1st month of life, VSD is usually repaired 1st.
what is HLHS?
second most common CHD, severe underdevelopment of the L side of the heart, aortic valve, aorta, L ventricle, and mitral valve. Pulmonary congestion and edema. Child will be asymptomatic until ductus arteriosus closes, then poor perfusion with cyanosis, tachypnea, and dyspnea. IV prostaglandin E to keep ductus arteriosus open until taken to surgery.
