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93 Cards in this Set
- Front
- Back
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What are complications of sickle cell anemia?
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-Strokes
-Swelling of hands and feet -Spleen problems (splenomegaly) -Infections -Infarctions -Crises (painful sequestration/segregation, aplastic- unable to regenerate RBC tissue) -Kidney disease -Liver disease -Lung problems -Erection/priapism -Eye problems |
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What can the accumulation of sickle cells do?
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Obstruct capillary blood flow
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What type of hemoglobin molecules do sickle cell anemia people form?
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Hemoglobin (Hgb) S
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When does Hgb S tend to polymerize (come out) in a person with sickle cell anemia?
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-Low PaO2 (partial pressure of oxygen in the blood, <35 mmHg)
-Low pH (acidosis) -Low temperature -High osmolality (high chemical makeup in the blood) |
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How can the sickling of cells be reversed?
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-Reoxygenation
-Hydration -Hypotonic solution (due to pt.'s high serum osmolarity; hydrates the body's cells) -Warmth |
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What types of complications result from a "crises" episode in sickle cell disease?
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-Vaso-occlusion
-Pain -Aplastic anemia -Hyperhemolysis (rapid destruction of RBCs can lead to shock) -Stroke caused by obstruction (manifested by seizures) -Chest pain (dyspnea, pulmonary hypertension) -Infection due to loss of spleen protection (may lead to death) |
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Why does sickle cell disease cause anemia?
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Sickled RBCs are destroyed quickly, causing anemia. Sickled RBCs lose normal functioning.
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What is a splenic sequestration crises?
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Splenic sequestration crises are acute, painful enlargements of the spleen, caused by intrasplenic trapping of red cells, resulting in a precipitous fall in hemoglobin levels with the potential for hypovolemic shock
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What does aplastic anemia cause?
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Aplastic anemia is a disease in which the bone marrow, and the blood stem cells that reside there, are damaged. This causes a deficiency of all three blood cell types (pancytopenia): red blood cells (anemia), white blood cells (leukopenia), and platelets (thrombocytopenia).
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What is the main risk of aplastic anemia?
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Infection
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Where do sickle cell anemia patients present pain during a crisis?
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-Abdomen
-Back -Chest -Arms -Legs |
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A nurse is treating a patient with sickle cell disease crises. Which hypotonic solutions (with osmolality of 275 mOsm/kg or less) can the nurse use as a treatment? Select all that apply.
A. Half-normal saline solution B. Dextrose 5% in lactated Ringer's solution C. 0.33% sodium chloride solution D. Dextrose 2.5% in water |
A. Half-normal saline solution, C. 0.33% sodium chloride solution, D. Dextrose 2.5% in water:
Dextrose 5% in LR solution is a hypertonic solution. |
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What causes sickling?
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-Fever
-Emotional stress -Increased blood viscosity -Low blood oxygen -Infection |
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How long is the life span of an RBC that has been sickled?
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10-20 days
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What is the normal RBC life span?
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120 days
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What happens to the bone marrow space in reaction to shortened RBC life span?
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Bone marrow space enlarges to produce more RBCs
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At what age does a child with SCD lose their spleen?
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About age 5
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Without the spleen, the body is prone to bacterial infections. Which infection do SCD people usually adhere?
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Streptococcus pneumoniae
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What organ is the first to be affected from SCD?
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The spleen
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What two life-threatening risks can splenic dysfunction cause?
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-Sepsis
-Sudden death |
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Define dactylitis
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Dactylitis is a painful inflammation of the fingers or toes, usually associated with sickle cell anemia. Results in symptoms of pain and swelling of soft tissue.
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Most common symptom of SCD in children is...
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Dactylitis
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What complication can dactylitis cause?
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Infarction of short tubular bones by sickled cells (short stature in people with SCD)
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Define infarction
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Infarction= death of tissue due to inadequate blood supply
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What is the main prevention of strokes in people with SCD?
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Blood transfusions
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What are the 3 main problems in sickle cell crises?
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-Hypoxia
-Pain -Ischemia |
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How is sickle cell disease transmitted?
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Sickle cell disease is a deformity of chromosome 11. When both parents carry a sickle cell trait, they can produce a child with SCD (25% chance).
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The treatment for sickle cell crisis is...
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-Oxygen
-Hydration -Morphine |
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The primary risk of repeated transfusions is...
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Risk of iron overload
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Nurse is providing teaching for newly diagnosed 4 month old with sickle cell disease. Which teaching is not appropriate?
A. Give daily antibiotics to 4 month old with SCD B. Provide infant with hydration C. Avoid immunizations D. Get immediate medical attention if infant presents fever |
C. Avoid immunizations:
Patient with SCD is at risk for infection. Immunizations are vital. |
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Each unit (100 mL) of platelets raise the platelet count by how much?
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Each unit of platelets raise the platelet count by 5,000 after 1 hour
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One unit of packed red blood cells (PRBCs) increase the hemoglobin by...
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1 g/dL
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What complications and features might an adult with sickle cell disease (who did not have SCD treatment as a child) have?
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-Short
-Skin ulcers -Bone disease -Poor tissue perfusion -History of multiple myocardial infarctions -Cognitive impairment -Blindness -On dialysis -Most likely in poverty |
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Define ischemia
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Ischemia is an inadequate blood supply to an organ or part of the body.
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How long does a vaso-occlusive crises from SCD last?
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About 4-6 days
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How does SCD affect the musculoskeletal system?
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SCD causes severe pain in bones and joints
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Define hematuria
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Hematuria is blood in the urine.
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What complications can a vaso-occlusive crises cause?
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-Blockage of blood vessel
-If blockage abdominal, abdominal problems (anorexia, vomiting) -Pain in bones and joints -Hematuria -Obstructive jaundice -Visual disturbances |
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SCD can cause acute chest syndrome. What is acute chest syndrome? What causes acute chest syndrome?
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Acute chest syndrome in SCA is defined by pulmonary infiltrates seen on a chest radiograph; associated with one or more new symptoms: fever, tachycardia, pleuritic chest pain, leukocytosis (increase in WBCs), cough, sputum production, dyspnea, or hypoxia.
It is due to vascular occlusion and/or infection; in children, ACS is often due to bacterial pneumonia, especially S pneumoniae–preventable by pneumococcal vaccine. |
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To lower the risk of neurological complications in SCD, how often should a patient schedule chronic transfusion therapy?
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Every 3-4 weeks to keep Hgb S levels <30% and Hgb 6-10 g/dL
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What does Sickledex screen for?
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Sickledex will detect Hgb S, but will not differentiate sickle cell trait from the disease
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When do symptoms of SCD emerge in a person born with SCD?
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6 months when Hgb F declines
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What percentage of RBCs contain hemoglobin S in carriers of SCD?
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30-40% of RBCs have hemoglobin S, the remainder of RBCs contain Hgb A
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Severe sickle cell syndrome is distinguished by a hemoglobin count of...?
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6-10 g/dL Hgb
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How does hydroxyurea function as a treatment for SCD?
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Produces more Hgb F over Hgb S
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What are the side effects of hydroxyurea?
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-Increases risk of leukemia
-Risk of stroke -Carcinogenic -Teratogenic (causes birth defects) |
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What test differentiates sickle cell trait from sickle cell disease?
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Hemoglobin electrophoresis
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What is the typical hematocrit range for those with SCD?
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20-29% hematocrit
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What electrophoresis result indicates normal hemoglobin?
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"FA"= normal results
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What electrophoresis result indicates sickle cell trait?
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"FAS"= sickle cell trait
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What two tests should a positive SCD child receive? How often should they have these tests?
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CBC and retic count every 3 months
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Which three vaccines should be given to a child with SCD?
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-Pneumococcal vaccines (PCV)
-Meningococcal vaccine (MCV4) -Yearly influenza vaccine |
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How much penicillin prophylaxis is given to 2-month-old child with SCD? At what age does a child with SCD need to increase their penicillin prophylaxis dosage?
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125 mg orally, twice a day; By 3 years, penicillin dose increases until age 5
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What is the purpose of an annual transcranial doppler screen?
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Screens major intracranial vessels for strokes (for ages 2-16 years)
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What supplementation do children with SCD need?
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Folic acid supplementation
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What SCD problems require hospitalization?
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-Fever greater than 38.3 C (101 F)
-Pneumonia, chest pain, or other pulmonary symptoms -Sequestration crisis (splenomegaly with decreased Hgb or HCT) -Aplastic crisis (decreased HCT and reticulocyte count) -Severe painful crisis -Unusual headache, visual distrubances (stoke signs) -Priapism |
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What does the SCD treatment drug Deferoxamine do?
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Deferoxamine treats iron overload by binding to free iron and eliminating it in the urine.
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How often is the pneumococcal vaccine given to children with SCD?
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At 2 and 5 years, then every 5 years thereafter
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When can SCD be diagnosed prenatally by DNA analysis of cells by chorionic villus sampling?
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9 weeks
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Define hemosiderosis
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Hemosiderosis is an overload of iron in the body resulting from repeated blood transfusions.
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What drug choices are used to treat mild-moderate SCD pain?
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Acetominophen (tylenol), Ibuprofen, or Codeine
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What class of drugs are given for moderate-severe pain from SCD?
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Opioids
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What opioid drug is extremely potent with a high risk of apnea and diaphragm rigidity when given too fast IV?
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Fentanyl
(also causes increased nausea and vomiting in kids when given IV) |
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How much Fentanyl IV should be given to relieve SCD pain?
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0.001-0.005 mg/kg IM or IV of Fentanyl
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What are three opioids that can be given to treat SCD pain?
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-Codeine
-Morphine sulfate -Oxycodone |
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How much Codeine should be given to treat SCD?
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1-2 mg/kg every 4-6 hours
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How does Ketorolac tromethamine work?
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Ketorolac decreases the production of prostaglandin precursor
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How much Ketorolac is given for SCD pain relief?
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0.5 mg/kg every 4-6 hours
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How much morphine sulfate is given to reduce SCD pain?
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0.1 mg/kg every 3-4 hours
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What is the risk of giving morphine sulfate to kids?
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Morphine sulfate may precipitate bronchospasm, urticaria and pruritis, and vasodilation (especially if kids are asthmatic)
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What are symptoms of obstructed vessels from clumped sickle cells?
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-Hypoxia
-Acute chest pain -Fever -Cough -Increased respiratory rate -RBC destruction |
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What are symptoms of a vaso-occlusive crisis?
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-Pain (acute abdominal, hand/foot syndrome)
-Splenomegaly congested with blood infarction -Hemolytic crisis (anemia, jaundice) -Stroke (cerebral infarction) -Kidney ischemia |
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Pain medications for SCD should be given in which route?
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IV route
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What is the most common blood type?
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O positive
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How many days can a unit of PRBC last in storage?
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45 days
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How long can a unit of platelets last in storage?
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5 days
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Define apheresis
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Apheresis is a procedure in which blood is drawn from a donor and separated into its components, some of which are retained, such as plasma or platelets, and the remainder returned by transfusion to the donor.
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How much hematocrit will 1 unit of packed red blood cells (PRBC) raise?
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One PRBC unit will raise the heamtocrit of a standard adult patient by 3%
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Why are packed red blood cells given?
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PRBCs are given to replace red cell mass when tissue oxygenation is impaired by acute or chronic anemia. Often given to chemotherapy patients.
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What is albumin used for?
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Albumin is used as a plasma expander
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What is the benefit of using albumin?
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Albumin does not have the risk of viral infection
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Define thrombocytopenia
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Thrombocytopenia= Platelet count less than 20,000 mm3
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What is the life span of platelets?
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Platelets survive 7-10 days
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Why would platelets be transfused?
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Platelets are used to control and prevent bleeding associated with thrombocytopenia or platelet dysfunction
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What are the adverse reactions from a platelet transfusion?
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-Febrile with rise in temp (over 38 C or over 1 degree above pre-transfusion temp)
-Septic reaction (high temperature and shock) -Post-transfusion purpura -Transfusion associated acute lung injury |
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Define purpura
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Purpura are small hemorrhages in the skin, in mucous membranes, or serosal surfaces. They result from leakage of blood out of a vessel and under the skin.
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Is frozen fresh plasma used as a plasma expander?
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No, FFP is never used as a plasma expander
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Why would frozen fresh plasma be infused?
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FFP is indicated when a patient has multiple coagulation factor deficiencies and is bleeding.
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When is cryoprecipitate used?
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Cryoprecipitate is used for:
-Hypofibrinogenemia -Von Willebrand's disease -Situations calling for a "fibrin glue" |
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What are 3 deficiencies that Leukemia causes?
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-Anemia
-Neutropenia (low level of neutrophils in the blood) -Thrombocytopenia (low level of platelets in the blood) |
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Why are irradiated RBCs used?
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Irradiated RBCs are used to prevent graft-verus-host reactions in immunosuppressed patients.
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Why are washed RBCs used?
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Washed RBCs are used in patients with deficiency in immunoglobulin A or who have severe allergic type reactions with RBC trasfusions
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What are washed RBCs?
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RBCs that have been washed in sterile saline to remove WBCs, lytic mediators, non-self antigens
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