Exam 2 Blueprint Hematology Flashcards
what is sickle cell anemia?
Hereditary disorder where the RBCs are sickle or crescent-shaped due to the presence of hemoglobin S. (normal blood has HGB A, SCA = HGB S replaced HGB A.
Mostly African American descent (1 in 375 births) and Hispanics (1 in 1,200 births)
what are the CM of SCD?
Vaso-occlusive crises (VOC): severe pain (might start out as soft pain), priapism, acute chest syndrome (ACS), and stroke (CVA)
Sequestration crises: splenic and hepatic, may cause hypovolemic shock.
Aplastic crises - causes extreme anemia
Hyper-hemolytic crises (anemia, jaundice, etc.)
what is the diagnosis and treatment of SCD?
Diagnosis: newborn screen, sickledex heel/ finger stick @ birth, and hemoglobin electrophoresis if + on their sickledex.
Treatment: hydroxyurea (once a day, small dose), penicillin (BID), O2 (ONLY when hypoxic), rest, pain meds, hydration, electrolytes, blood transfusion (can exchange sickle cells with new cells), ABX, and splenectomy.
what are the nursing considerations of SCD?
Pain Control. Education: hydration, meds, vaccinations, follow-up visits, associated risks, and fever monitoring. Psychosocial support: child life specialists, psychiatrists/psychologist, social workers, and school counseling.
what is anemia?
the # of RBCs or hemoglobin concentrations are below normal value.
what is 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 is the diagnosis, treatment, and nursing considerations for RBC disorder 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?
not enough iron or a loss of iron.
what is the @ risk population for iron deficiency anemia?
preterm infants, toddlers (excessive cow’s milk intake and inadequate nutrition), adolescents (poor eating habits, heavy menses, and obesity)
what is the treatment for iron deficiency anemia?
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.
what are the nursing considerations for iron deficiency anemia?
education = meds (side effects, precautions), diet and nutrition (iron-fortified cereal and high iron foods)
what is beta thalassemia?
Microcytic anemia non-responsive to iron supplementation associated with people of Mediterranean origin.
what are the forms of beta thalassemia?
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 diagnosis and TX 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).
