Term
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Definition
| Sickle cell anemia is a genetic disease that affects hemoglobin. Under low oxygen conditions the hemoglobin sticks together causing long fibers that make the red blood cells change to a sickle shape. This causes them to get stuck in blood vessels causing parts of the body to not receive the oxygen they need. |
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Term
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Definition
| The red blood cells change shape causing the bone marrow to try to make more cells to make up for the loss, but it cannot keep up which causes anemia. The shape causes the cells to clump and cut off block flow in the vessels. As a result children’s growth is delayed, they are fatigued, and they have extreme pain. |
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Term
| What causes pain in patients with SCA? |
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Definition
| Because of the blockage the tissues are not getting enough oxygen. They must switch to anaerobic respiration. The fatigue is because anaerobic respiration does not produce as much energy as the cells normally would. The pain is because of the lactic acid that is a byproduct of anaerobic respiration. This pain is made worse because the reduced circulation means that the lactic acid remains in the tissues. |
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Term
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Definition
| Characterized by ischemia that causes mild to severe pain that can last from minutes to days |
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Term
| SCA: Sequestration crisis |
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Definition
| The pooling of a large amount of blood usually in the spleen and infrequently in the liver that causes a decreased blood volume and ultimately shock. |
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Term
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Definition
| Diminished RBC production usually caused by viral infection that may result in profound anemia |
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Term
| SCA: Hyperhemolytic crisis |
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Definition
| An accelerated rate of RBC destruction characterized by anemia, jaundic, and reticulocytosis |
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Term
| SCA: Acute chest syndrome |
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Definition
| Clinically similar to pneumonia. It is presence of new pulmonary infiltrate and may be associated with chest pain, fever, cough, tachycardia, wheezing, and hypoxia |
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Term
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Definition
| Common genetic disorder where there is a deficiency in the rate of production of specific globin chains in hemoglobin. When it disintegrates it damages RBCs causing severe anemia. |
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Term
| B-Thalassemia: Goals of treatment and nursing care |
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Definition
Goals: Provide therapy to maintina Hgb high enough to have enough RBCs to support normal normal growth and development. Do this by giving frequent blood transfusions.
Nursing care: Promote compliance with transfusion treatment, assist the child with their anxiety, help the parents adjust, and monitor for transfusion reactions. |
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Term
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Definition
| Type of bone marrow failure where the formation of formed elements of the blood (leukocytes, thrombocytes, and Hgb) are all depressed. It can be primary (congenital, autosomal recessive) or secondary (acquired). Goal of therapy is to restore function of the bone marrow. |
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Term
| Goal of therapeutic treatment of hemophilia |
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Definition
| Replace missing clot factor. |
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Term
| 2 most common misconceptions about pathophysiology of leukemia |
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Definition
-In the acute form of leukemia, the leukocyte count is low, even though leukemia is an overproduction of WBCs
-There is a misconception that the immature cells deliberately attack and destroy the normal blood cells or vascular tissue, but in fact cellular destruction happens by infiltration and subsequent competition for metabolic elements |
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Term
| Nursing care of child with leukemia |
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Definition
| Therapeutic regime itself causes a lot of unpleasant and dangerous symptoms for the child. These symptoms must be managed to make the child more comfortable and as safe as possible. |
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Term
| Absolute Neutrophil Count |
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Definition
-The number of neutrophil granulocytes present in the blood.
-To calculate the total percent of neutrophils you multiply the WBC count by the percent of neutrophils. |
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Term
| What are the current statistics of pediatric cancers? |
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Definition
-11,630 children under the age of 15 will be diagnosed with cancer in 2013
-1310 children are expected to die from cancer in 2013
-Cancer is the leading cause of death by disease in children 1-14 years of age
-There is an 80% 5 year survival rate for cancer |
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Term
| Most common pediatric cancer |
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Definition
| Leukemia (about 1/3 of cases of cancer in children) |
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Term
| Which cancer has the best and worst prognosis? |
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Definition
-AML has the worst prognosis
-Hodgkin Lymphoma has the best prognosis |
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Term
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Definition
-Inadequate supply of dietary iron
-Children 12-36 months at greatest risk |
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Term
| Sickle Cell Anemia Etiology |
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Definition
-Inherit the HgbS gene from each parent
-If only one gene for Hgbs then have sickle cell trait
-Normal adult hemoglobin partially or completely replaced by hemoglobin S |
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Term
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Definition
| Hereditary, chronic, hemolytic disorder. It predominately affects African-Americans but also people of Mediteranean decent. Under certain conditions, RBCs will assume a crescent or sickle shape |
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Term
| Signs and symptoms of a patient with sickle cell anemia (not in crisis) |
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Definition
-Growth retardation
-Chronic anemia
-Delayed sexual maturation
-Marked susceptability to infection and sepsis |
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Term
| Signs and symptoms of sickle cell anemia (in crisis) |
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Definition
-Severe pain
-Muscle spasms
-Hot, swollen joints, fever
-Hematuria
-Paralysis, seizure, coma
-Severe abdominal pain and distension
-Later jaundice d/u hemolysis
-Crisis may be fatal r/t cerebral infracting & cardiac complications |
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Term
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Definition
Enmeshing of sickling cells block microcirculation intermittenly
-Hypoxia results then tissue edema then ischemia and possible tissue death
--Extremities-swollen
--Cerebrum stroke |
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Term
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Definition
-High mortality rate
-Cause usually result of lung tissue damage from vasoocclusion
-Infiltrates develop appears as Respiratory Distress
-Poor oxygenation to tissues-cell death results |
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Term
| Diagnosing and treatment of sickle cell disease |
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Definition
-Most states mandatory newborn screening
-Sickledex-screening test
-Electrophoresis
Treatment:
-Aimed at preventing sickling
-Short term O2 therapy
-Analgesics
-Bedrest
-Bone marrow transplant
-Hydration |
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Term
| Nursing care for sickle cell |
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Definition
Educate parents about disease
-Don't fly in under-pressurized planes
-Don't exercise in high altitudes
-May use PCN G prophylactically |
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Term
| Thalassemia and treatment |
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Definition
Problem with production of Hemoglobin in RBC. Do not have enough hemoglobin or RBCs to transport O2 throughout the body.
-Frequent blood transfusions
-Hemosiderosis can result: defective RBCs stored in various organs |
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Term
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Definition
| Form of iron overload disorder. It is an accumulation of iron in the liver and/or heart but also endocrine organs, in patients who receive frequent blood transfusions (such as those with Thalassemia, Sickle cell disease, anemia, or myedysplastic syndrome) |
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Term
| ITP (Idiopathic thrombocytopenic purpura) |
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Definition
| -Idiopathic: cause is unknown; Thrombocytopenic: blood does not have enough platelets; Purpura: excessive bleeding/bruising |
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Term
| Relationship between ITP and immune system |
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Definition
-Antibodies destroy platelets
-Antibodies see platelets as bacteria and work to eliminate them
-ITP is preceded by a viral illness (URI, varicella, smallpox/measles vaccine, mononucleosis, flu) |
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Term
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Definition
Hemophilia A: classic
Hemophilia B: Christmas disease
Von Willabrands Disease |
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Term
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Definition
-Spontaneous bleeding
-Bleeding into joints and associated pain adn swelling
-Gastrointestinal tract and urinary tract hemorrhage
-Blood in the urine or stool
-Prolonged bleeding from cuts, tooth extraction and surgery |
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Term
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Definition
-Spontaneous bleeding
-Bleeding into joints and associated pain adn swelling
-Gastrointestinal tract and urinary tract hemorrhage
-Blood in the urine or stool
-Prolonged bleeding from cuts, tooth extraction and surgery |
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Term
| Interventions for Hemophilia |
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Definition
-Replace the factor as ordered by physician
-Manage pain utilizing analgesics as ordered: no salicylate products.
-Maintaining joint intergrity during acute phase: immobilization, elevation, ice.
-Physical therapy to prevent flexion contraction and to strengthen muscles and joints
-Provides opportunities for normal growth and development |
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