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123 Cards in this Set
- Front
- Back
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What is erythropoiesis
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production of rbc's
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Explain the events leading up to RBC production
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decreased levels of O2 stimulate the kidneys to release ESF for RBC production
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What triggers the release of ESF
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decreased levels of O2
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What is the normal life span of a properly functioning RBC
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120 days
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What is the primary function of RBCs
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transporting O2
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How much stored iron is a healthy neonate born with
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4-6mos worth
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Iron is necessary for what r/t RBC
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HgG bound to RBC
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What is HgbF
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neonatal hemoglobin
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What kind of hemoglobin is neonatal hgb replaced be?
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HgbA
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HgbF has a higher/lower affinity for O2
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higher
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At what age is HgbF replaced by HgbA
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6-12mos
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In sickle cell disease, HbF is replaced by...
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HbS
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Define anemia
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RBC and/or Hb concentration(s) below normal, resulting in decreased O2 carrying capacity in blood
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Is anemia a disease
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no; it indicates that something is going on
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What causes anemia
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loss/destruction of existing RBCs or impaired/decreased production of RBC
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What is the most common hematologic disease in infancy
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anemia
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What is the significance of measuring reticulocytes r/t anemia
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to determine the cause; low count = impaired production; high count = RBC destruction
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What is a reticulocyte
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immature RBCs
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T/F - anemic pt's have low blood density
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T
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How is anemia typically discovered in pt's
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CBC
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What is the significance of the recommendation that children <2yrs should not drink >1L milk
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interferes with iron absorption
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CBC of an anemic pt r/t - Hct, RBC, and Hb
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all decreased
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Some common foods high in iron
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nuts, fish, beans, some cereals
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One of the first signs associated with anemia (excluding CBC for diagnosis)
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tachycardia; decreased Hb = decreased O2 = tachycardia to pump out more blood and more O2!!!
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Signs of anemia...
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cardiac - tachycardia, slow cap refill, pallor, murmur, palpitations, dizzy; respiratory - SOB; other - fatigue, decreased apetite, cool/moist, frequent infection
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Classifications of anemia (2)
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1-RBC depletion
2-RBC morphology |
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anemia - examples of excessive blood loss r/t RBC depletion
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hemorrhage
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anemia - examples of RBC destruction r/t RBC depletion
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chemotherapy
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anemia - examples of impaired RBC production r/t RBC depletion
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kidney failure, impaired BM production
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Examples of morphological changes r/t anemia
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shape, size, color
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define microcytic & its common causes r/t anemia
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small RBC; iron deficiency; lead poisoning
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define macrocytic nd its common cause r/t anemia
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large RBC; folic acid/ B12 deficiency
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An example of a change in RBC shape
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sickle cell
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Decreased O2 carrying capacity of the blood leads to...
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decreased amount of O2 delivered to cells
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Why would we see delayed growth r/t anemia
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cells aren't receiving enough O2 to stimulate growth
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Describe the viscosity of the blood r/t anemia
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less viscous, decreased density of RBC's, tachycardic in order to put out more RBC's
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name some clinical manifestations seen in pt's with anemia
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muscle weakness, fatigue, irritability, dizziness, headache, decreased attention span, shortness of breath, pale, signs of shock, pica, depression
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why might we see signs of shock in pt's with anemia
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decreased viscosity, low BP, tachycardia
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define pica, give examples
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eating non-edible substances r/t iron deficiency; ie/ lead paint, dirt
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What is most important regarding management of anemia
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treat the underlying cause
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Why might it be important to check an anemic pt's stool?
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checking for blood in case cause of blood loss is internal bleeding
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What is the most common form of anemia during infancy and childhood
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iron deficiency anemia
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What two age groups are most commonly subjected to anemia
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premi's; adolescence during puberty
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Causes of iron deficiency anemia
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1-decreased iron supply
2-decreased iron absorption 3-increase in iron demand 4-abnormal Hb |
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Two disorders resulting in decreased iron absorption
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Crohn's, CF
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What can affect Hb synthesis
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folic acid and B12 deficiency
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CBC findings r/t iron deficient anemia
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1-decreased RBC
2-decreased Hb 3-decreased MCHC 4-decreased MCV 5-increased iron binding capacity 6-increased reticulocyte count 7-microcytic 8-hypchromic |
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What is MCV
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mean cell volume; the measure of the average RBC size; determines microcytic vs macrocytic
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What is MCHC
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mean corpuscular hb concentration; measure of hemoglobin concentration in a given volume of packed RBCs
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Describe microcytic and hypochromic
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small and pale
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What are options for iron supplements
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1-dietary
2-vitamin C 3-ferrous sulfate |
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If dietary sources cannot adequately supply iron stores what are useful supplements
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ferrous sulfate & vitamin C
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How does vitamin C work to increase iron stores
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increases iron absorption
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How do we want to administer vitamin C and how? (if we are using a syringe)
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back of the mouth to avoid staining the teeth
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What color are stools r/t iron deficiency
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tarry green
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Why is it a good idea to administer iron supplements with meals
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can cause abdominal pain
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When using iron supplements, iron levels should increase by ____ /week
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0.7-1.0g/dl
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After starting iron supplement therapy, it usually takes ______ to see an increase in iron levels
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2-3 weeks; re-check counts after a month
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What is an autosomal recessive gene; give an example of a disorder
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parent doesn't have the disease but both parents have to carry the trait; sickle cell anemia
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Define hemoglobinopathy
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when normal adult HgA is partially or completely replaced by HgS
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HgS is representative of...
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sickle cell disease
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When using iron supplements, iron levels should increase by ____ /week
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0.7-1.0g/dl
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After starting iron supplement therapy, it usually takes ______ to see an increase in iron levels
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2-3 weeks; re-check counts after a month
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What is an autosomal recessive gene; give an example of a disorder
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parent doesn't have the disease but both parents have to carry the trait; sickle cell anemia
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Define hemoglobinopathy
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when normal adult HgA is partially or completely replaced by HgS
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HgS is representative of...
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sickle cell disease
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What is the most common genetic disease in the US
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sickle cell anemia
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Are symptoms of sickle cell anemia common during infancy? Explain...
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no; presence of HgF
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T/F sickle cell disease is part of newborn screening
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T
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Pathophysiology of sickle cell
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1-increased RBC destruction 2-shortened life span
3-obstruction 4-entanglement and meshing of RBC |
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Explain some complications of the rigid shape of sickled cells
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abrasion and scarring of vessel walls affecting their ability to constrict and dilate; painful episodes from obstruction and meshing
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What are the two biggest triggers of sickle cell crisis
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poor hydration and low O2
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Triggers of sickle cell crisis
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1-altered O2, hypoxia
2-increased O2 demand 3-fever 4-emotional or physical stress 5-increased blood viscosity (low blood volume) |
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What organs are affected by sickle cell disease
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ALL OF THEM!!!
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Why might you find an enlarged spleen in pt's with sickle cell anemia
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rigid shape of sickled cells cause obstruction in spleen
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What is a complication of sickle cell obstruction in the spleen?
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hypovolemic shock; decreased blood volume
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What is dactylitis? It's significance?
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pain and swelling in hands and feet; often first sign of sickle cell anemia
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Example of a nursing priority r/t sickle cell crisis
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pain, pain and more pain; pain assessment and intervention!
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Name the different sickle cell anemia crises
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1-vaso-occlusive
2-sequestration 3-aplastic 4-hyperhemolytic 5-megaloblastic anemia 6-chest syndrome 7-CVA |
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T/F you can an anemic crisis alone or in combination
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T
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Vaso-occulsive crisis
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r/t ischemia; can be anywhere
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Sequetration Crisis
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pooling in spleen or liver; hypovolemic shock is main concern
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What is main concern of sequestration crisis?
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hypovolemic shock
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Aplastic anemia
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decreased RBC production in BM
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Chest syndrome
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vaso-occlusive crisis of pulmonary vasculature; mimics pneumonia
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Hyperhemolytic crisis
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increased destruction of RBCs; jaundice is symptom
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Jaundice is a sign of which anemic crisis
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hyperhemolytic
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Megaloblastic crisis
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over-sized RBCs; inadequate folate, B12
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Cause of megaloblastic anemia
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inadequate folate, B12
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What is the significance of fifth disease r/t sickle cell anemia
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exacerbates symptoms of anemia; can lead to aplastic anemia where pt is unable to produce RBCs
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What is cholestasis r/t sickle cell anemia
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pooling of bile r/t obstruction
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Why might we see avascular necrosis with sickle cell anemia
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inadequate blood/O2 supply
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what sites do we commonly see avascular necrosis r/t sickle cell?
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hips and shoulder jt
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Prevention for sickle cell anemia
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hemodilution!
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Treatment during crisis
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bed rest
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Order of pain meds prescribed
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nsaids - oxycodone - IV opiods - methadone
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At what age is it considered appropriate to start PCA treatment
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~6yrs
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Examples of pain intervention
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morphine, dilaudid, PCA pump, phentinol patch
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Complications r/t pain meds
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constipation, nausea, decreased urine output, puritis
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How is hydration an effective form of managing sickle cell anemia
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increases blood volume, decreases viscosity, helps with vaso-occulsion
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How is bed rest an effective form of managing sickle cell anemia
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decreases O2 demand
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What is the concer when using O2 therapy as form of management r/t sickle cell anemia
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decreased O2 is stimulation for kidneys to release ESF to increase RBC production; if O2 levels remain high then RBC production doesn't get stimulated
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What pt's with sickle cell anemia should receive blood transfusions
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Hb < 6
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What Hb concentration is considered too high for pt's with sickle cell anemia? Explain.
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Hb > 10; don't want blood too viscous
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Complications of blood transfusions
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introduction of antibodies; iron deposits
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What is despirol
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chelating agent; binds to iron for excretion
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What is asplenia?
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spleen usually atrophies r/t obstruction
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What is te #1 cause of death in pt's <5yrs r/t sickle cell anemia
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PCP r/t decreased functioning of the spleen
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What medications are pt's with sickle cell anemia usually prescribed
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1-folic acid
2-antibiotics 3-pain meds 4-hydroxurea |
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Explain the difference in vaccine regimen for pt's with sickle cell anemia
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additional vaccine for PCP at 2yrs; also menomune vaccine
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What is the normal regimen for PCP vaccine
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2, 4, 6 months - 13 different strains
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What is Prevnar?
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additional vaccine against PCP that pt's with sickle cell disease receive at 2yrs; polysaccharide vaccine; protects against an additional 23 strains
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What is Menomune?
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polysaccharide meningitis vaccine; pt's with sickle cell receive this vaccination at 2yrs which is earlier on then seen with healthy children
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What is Toradol and explain the caution with using it
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strong NSAID, can only be on it for 5 days because of it's effect on the kidneys
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If a pt is on Toradol what assessment is important that nurses monitor
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kidney function; creatinine levels
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Aside from working as a pain med itself, what additional benefit does Toradol have?
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decreases opioid dose
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Ice or heat for vaso-occlusive crisis
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heat
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what is guided imagery
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used as alternative treatment for pain associated with sickle cell anemia; child's "favorite place"
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How does hydroxyurea work? Why do we need to use caution with it?
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increases HbF - neonatal hemoglobin; can depress BM
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Are pt's with sickle cell anemia candidates for BMT?
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yes
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Why are pt's with sickle cell anemia placed on prophylaxis PCN
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greater risk of PCP
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AT what point are pt's with sickle cell anemia usually taken off prophylaxis PCN?
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after immunizations are complete
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What is the importance of folic acid r/t sickle cell anemia?
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RBC production
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