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155 Cards in this Set
- Front
- Back
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a disorder in which the body launches an immunologic response against itself.
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autoimmune disorder
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a type of medication therapy in which there is a binding of a metallic ion with a structure so that the ion is inactivated (such as iron)
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chellation
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the production of erythrocytes (RBCs)
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erythropoiesis
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production of blood cells, normally occurs in the bone marrow but may occur in extramedullary sites
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hematopoiesis
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breakdown of RBC's
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hemolysis
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focal or general increase in tissue iron stores without associated tissue damage
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hemosiderosis
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process of vasoconstriction and coagulation to stop bleeding
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hemostasis
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reduction in all types of blood cells
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pancytopenia
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immature RBC
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reticulocyte
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In fetal life, various tissues produce RBC's but after birth, their production is controlled exclusively by by the _____ _____, particularly in the _____ bones.
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bone marrow
long |
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The function of RBC's is to transport oxygen to the tissues. Essential to this ability to carry oxygen is an appropriate amount of _______, whose production depends on sufficient amounts of circulating ____.
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hemoglobin
iron |
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Iron is absorbed from dietary intake by the intestines and is stored by the _____.
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liver
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What stimulates the production of RBCs?
What organ(s) is stimulated and what what hormone is then produced? |
a decrease in circulating oxygen
The kidneys are stimulated to produce erythropoietin. This hormone stimulates the production of RBC precursors and casuses them to mature rapidly. |
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Disorders of the ____ can affect an individual's ability to produce erythropoietin and thus can affect RBC production by the bone marrow.
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kidney
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The life span of erythrocytes in neonates is shorter/longer than in older infants and children because of increased/decreased destruction during rapid growth.
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shorter
increased |
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Where are some places hematopoises takes place?
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Hematopoises primarily in the marrow of the ribs, sternum, vertebrae, pelvis, skull, clavicles, and scapulas.
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___________ can occur as a result of hypoxia, such as that experienced at high altitudes or when oxygen is not sufficiently directed to the tissues, as in cyanotic heart disease. This is a ________ mechanism,
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polycythemia
compensatory |
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____ or leukocytes are formed in the bone marrow and in _______ tissue. They assist in the body's ability to distinguish "self" from "nonself."
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WBC's
lymphatic |
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______ are the cells that promote hemostasis- the prevention of blood loss. They are formed in bone marrow from megakaryocytes.
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platelets
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Platelets can circulate in the blood for about ___ days before they die; however, disease, fever, and infection can shorten a platelet's lifetime. Platelet disorders occur when the bone marrow cannot meet the production demands of the body.
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10
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______ are made in the thymus and are responsible for cell mediated immunity.
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T- Cells
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______ are responsible for humoral immunity and produce antibodies.
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B Cells
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____ _____ _____ are lymphocyte-like cells that can kill certain types of tumor cells and viruses directly.
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natural killer cells
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_____ end in phil, while ______ don't!
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Granulocytes , agranulocytes
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________ fight bacteria.
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neutrophils
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_______ fight parasites and respond to allergens.
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eosinophils
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_____ activate the inflammatory process and contain histamine.
They also produce heparin and speed fat removal. |
basophils
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________ have a U- or kidney shaped nucleus and phagocytize large cells, including necrotic tissue. They have an important role in chronic infection.
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monocytes
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________ are found in the bone marrow, spleen, thymus, lymph glands, tissues, and circulation. There are B and T types.
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lymphocytes
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What is the most common cause of anemia during infancy, childhood, and adolescence?
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iron-deficiency anemia
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Several factors can contribute to iron-deficiency anemia. What are the main 3?
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decreased iron intake
increased iron or blood loss periods of increased growth |
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True or False: iron-deficiency anemia occurs most often in children 9 to 24 months old as maternal iron stores are depleted.
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true
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True or False: premature infants are less likely to have iron-deficiency anemia than term infants.
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false, preterm infants are more likely
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Early transition from breast milk or infant formula to ___ ____ can precipitate and chronic diarrhea with occult intestinal bleeding in children younger than 2 years old due to the exposure to a protein found in this substance/food.
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cow's milk
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________ are also at risk for iron-deficiency anemia because they go through an increased growth stage and often have poor dietary habits. _______ further complicates blood loss for young women.
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adolescents
menstruation |
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Clinical manifestations of iron-deficiency anemia vary with the degree of anemia but may include which symptoms?
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pallor
pale mucous membranes pale conjunctiva tachycardia tachypnea lethargy irritablity |
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Before age 20, blood cells are produced mainly in what 3 bones?
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ribs
sternum vertebrae |
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Basophils increase when?
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in the healing phase of inflammatory response
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______ suppress the immune response.
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lymphocytes (T & B)
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____ or ________ are the cell fragments responsible for clotting and promote _______.
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platelets (thrombocytes)
hemeostasis |
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Platelets have a short or long life span?
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short
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If a child is younger than ___ iron-fortified formula or breast milk with iron-fortified supplements is recommended.
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12 months
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How often is iron given? What are some implications?
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TID
Give with Vit. C rich fluids empty stomach (between meals) avoid administration with milk or formula wipe off teeth/ use straw |
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What do stools like when iron supplements an taken?
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black, tarry
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In iron deficiency anemia, what might you hear if it is severe when you listen to the heart?
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murmor
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If the child is unresponsive, to oral iron supplements, what can be given and how?
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iron dextran (Imferon)
Given deep IM using Z track |
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True or False: After beginning iron supplement therapy, the reticulocyte count should peak in 4 to 30 days and the HGB levels should increase in 5 to 7 days. This is a good way to measure compliance with therapy.
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False. The reticulocyte count should peak in 5 to 7 days and the HGB levels should increase in4 to 30 days.
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If the child is older than 12 months, restrict milk intake to _____ oz per day or less.
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24
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Name some examples of iron rich foods. (9)
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egg yolks
liver meat prunes/ dried fruit dark, leafy green vegetables apricots beans cream of wheat iron fortified cereals |
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Name some sources of Vitamin C
(3) |
strawberries
orange juice tomatoes |
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True or False: iron deficiency anemia is most common between 4-6 months.
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False, infants still have maternal iron stores at this age
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True or False: blood transfusions are common therapies in iron deficiency anemia
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false. Response to iron therapy can often be positively predicted and blood transfusions are rarely indicated.
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What are some diagnostic evaluations for iron deficiency anemia
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nutritional intake
CBC serum ferritin level serum iron levels iron binding capacity |
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If serum iron levels are _____ iron binding capacity will be ______.
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decreased
increased |
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3 types of sickle cell disease
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sickle cell anemia
sickle beta-thalassemia sickle C disease |
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In Sickle Cell Disease, a group of hemoglobinopathies, _____ hemoglobin is partially or totally replaced with ____ hemoglobin.
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normal
abnormal (HbS) |
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IN Sickle cell disease, RBCs assume a sickle shape, which prevents them from flowing easily through the smallest/largest blood vessels.
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smallest
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Describe sickled RBCs
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stiff (stiffen into a rod)
rigid nonpliable |
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Sickled cells clump together, causing _______ in the small vessels. With reoxygenation, most of the sickled RBCs resume their normal shape. However, with repeated sickling and unsickling, the cells become irreversibly ____ and their life span is reduced from 120 days to ___ days.
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occlusions
12 |
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Sickled cells cause microvascular occlusion, leading to tissue ______, i_____, and organ _____.
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ischemia, infarct, organ damage
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What are some causes of cell sickling?
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acidosis
decreased oxygenation dehydration |
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What 3 organs are the most seriously affected by the complications of sickle cell disease?
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lungs, spleen, brain
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What is the spleen's normal function? The spleen of a child with sickle cell disease does not function much after what age?
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to filter bacteria in the blood
5 |
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What is the most frequent complication and reason for hospitalization in sickle cell disease?
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vaso-occlusive crisis
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How does someone get sickle cell disease? What ethnicities most commonly present this disease?
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inherited autosomal recessive
African-American Mediterranean Indian Middle Eastern |
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All of the clinical manifestations of Sickle Cell Disease are a result of the obstructions caused by the sickled RBCs and the increased destruction of sickled and normal RBCs caught in microcirculation obstructions. What are some of the clinical manifestations of sickle cell disease? (9)
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chronic hemolytic anemia
pallor jaundice fatigue renal dysfunction retinopathy delayed growth & puberty cholelithiasis (gall stones) Avascular necrosis of hips & shoulders |
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Symptoms of sickle cell disease are not usually present until what age? (hint: when infant begins to manufacture hemoglobin)
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4 to 6 months
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an acute episodic exacerbation of sickle cell disease is known as a what?
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sickle cell crisis
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What are some precipitating factors to a sickle cell crisis?
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infection
dehydration hypoxia trauma stress D- ****! |
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With sickle cell disease, are reticulocyes high or low?
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high because of the chronic loss and destruction as a result of the shorted life span of the RBCs
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In sickle cell disease, the _____ does not function properly or has been surgically removed because of complications. Surgical removal of this organ places the patient at high risk of what?
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spleen
infection because they are in an immunocompromised state |
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Because people with sickle cell disease have an improperly functioning spleen (if they have one) they are at greater risk of Strep. pneumoniae and H. influenzae. These are usually destroyed by the spleen. Because of this, these people should get what?
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routine vaccines against H. influenzae, pneumococcal polyvalent vaccine, as well as hepatitis B.
Also penicillin V therapy is recommended for all children with suspected or actual diagnosis to prevent pneumonia. |
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What are the 3 types of sickle cell crises?
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vaso-occlusive crisis
acute- sequestration crisis aplastic crisis |
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describe a vaso-occlusive crisis
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pain in joints & limbs
chest syndrome dactylitis cerebrovascular infarction (CVA caused by vaso-occlusion of vessels in brain) |
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what is dactylitis?
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swelling of hands or feet, pain, warmth in affected area
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What is chest syndrome?
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chest pain, fever, cough, abdominal pain
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Describe a acute sequestration crisis
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pooling of blood in spleen causing sleep enlargement
hypovolemic shock (can be life threatening) acutely- ill looking child pallor irritability |
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Describe an aplastic crisis
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decreased RBC production causes profound anemia
can be caused by exposure to toxic chemicals, ionizing radiation, some antibiotics, viral infections symptoms include pallor, lethargy, headache, fainting |
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_______ can be the first sign of bactermia.
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fever
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A child with SCD and a temperature of ____ or higher should receive prompt medical attention because of the risk for infectious complications.
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38.5 C
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Diagnosis of SCD is made at newborn screening in the first __ days of life. Tests can include a ___ and a hemoglobin _______.
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1-7
CBC electrophoresis |
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Therapeutic management for SCD includes what?
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prophylactic penicillin (antibiotic therapy)
hydration- oral & IV analgesics- NSAIDs, IV opioids RBC transfusion Rest oxygen (keep above 95%) PHARRO |
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Nursing considerations for a patient with SCD include strict ____, pain management, hydration, and monitor the child for signs of _____ and monitor the child's condition for complications. Should warmth or cold be used to soothe pain?
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I & O
infection warmth (not cold) |
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Teach patient & families with SCD about the need for follow up appts. and the prevention of precipitating causes such as (3)
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avoid low oxygen environment
avoid infection, get immunized prevent tissue deoxygenation |
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What is the opioid of choice for treating pain in a sickle cell crisis?
What should be avoided? |
morphine
(opiods and NSAIDS are recommended) Tell patients to avoid aspirin and don't take antipyretics for fever until directed by a doctor. |
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Beta-Thalassemia is an _____ caused by diminished _______ of beta ________ _____ of ________.
How does one get this disorder? |
an autosomal recessive disorder
an anemia caused by a diminished synthesis of beta polypeptide chains of HGB |
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Beta-Thalassemia is also known as ...
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thalassemia major
cooley's anemia |
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What ethnicities is Beta-Thalassemia found in?
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primarily Mediterranean
also has been reported in Asian and African populations |
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A buildup of iron in organs such as the spleen, liver, pancreas, skin and heart is known as _________
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hemosiderosis
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Symptoms of Beta-Thalassemia include...
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bronze skin tone
growth and maturation retardation characteristic facial features hepatosplenomegaly anemia (severe) pallor BiG CHAP |
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Explain the pathophysiology of Beta-Thalassemia
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a defect in beta chain of HGB synthesis which leads to the RBCs being hemolyzed as they are produced which frees large amounts of iron into the bloodstream. This causes hemosiderosis. The excessive erythropoises leads to thin, fragile bones. Hepatosplenomegaly occurs due to hemosiderosis.
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What are some characteristic facial features of Beta-Thalassemia
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enlarged head
protruding forehead prominent maxillary (upper lip) wide set eyes flattened nose green/yellow skin tone (bronze?) |
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The major complication of long term transfusion therapy for the tx of Beta-Thalassemia is ________. To prevent organ damage from excessive iron overload, ____ therapy is initiated. What medications are we talking about?
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hemosiderosis
chelation deferoxamine (Desferal) IV or SQ desferasirox (Exjade) PO children over 2 |
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The diagnostics for Beta-Thalassemia include exam of RBCs show they are m_____ and h______, reticulocyte count to see how fast ___ is being made, serum iron levels, total ___ binding capacity, and ________ electrophoresis.
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microlytic, hypochromic
RBC hemoglobin |
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true or false: hemoglobin electrophoresis determines the type of hemoglobin that is deformed.
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true
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management of Beta-Thalassemia includes
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erythrocyte transfusion
chellation therapy splenectomy |
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In erythrocyte transfusions for Beta-Thalassemia, _____ provides a RBC a longer life.
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neocytes
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If there is a splenectomy, what are the 3 recommendations?
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-immunizations
-prophylactic penicillin -aggressive antibiotic therapy for febrile illnesses |
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What is the only known cure for Beta-Thalassemia?
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bone marrow transplant
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Hemophilia is a lifelong blood disorder with no cure. How does one get this disorder?
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It is hereditary
x-linked autosomal recessive |
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Hemophilia involves a deficiency in what?
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the factor necessary to coagulate blood
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Name the 2 types of hemophilia and their deficiency.
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classic hemophilia (hemophilia a)
deficiency in factor VIII (75%) Christmas disease (hemophilia b) deficiency in facor IX (15%) |
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What are the s/s of hemophilia?
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bruise easily
epistaxis (bloody nose) hematuria (maybe) prolonged bleeding (with pokes/cuts/tooth loss etc) Recurrent bleeding (hemarthrosis) in same joint Swelling, pain, bleeding stiffness in joints/ muscles |
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Diagnosis for hemophilia includes a history of ______; prolonged ___, ____, and _____ time, _____ level, ____ count, and factor ___ & ___ assays.
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bleeding
prothrombin time (PT) partial thromboplastin time (PTT) bleeding fibrinogen platelet XIII & IV |
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What is the therapeutic management for a patient with hemophilia?
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factor replacement (recombinant antihemolytic factor)
DDAVP - for milld A (synthetic ADH) Avoid activities that induce bleeding, avoid aspirin |
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How do we treat a bleeding part?
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RICE
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For a patient with hemophilia, apply gentle pressure for 10 to 15 minutes to superficial wounds and assess area for bleeding. Pressure facilitates what? Patients with hemophilia should be caught up on their what?
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clot formation
immunizations |
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Severe side effects of chelation therapy that should be reported to the primary HCP can include...
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hearing loss, ringing in the ears, fever, diarrhea, visual disturbances, allergic reactions, respiratory compromis
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True or False: Von Willebrand's Disease (VWD) is the most commonly inherited bleeding disorder and includes at least 20 subtypes.
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true
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True or False: Von Willebrand's Disease is an x- linked autosomal recessive disorder and only affects males.
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False.
It is an autosomal dominant disorder and affects both males and females |
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Children with Von Willebrand's Disease have either __________ or _______ of von Willebrand's ______
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underproduction or dysfunction
protein |
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VWD protein occurs together with factor ____ in circulation, making it a carrier for this factor.
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VIII
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One of Vonn Willebrand's protein's important functions is to bind and attract ____ to the endothelial tissue injury, thus facilitating the formation of a ____. Deficiency of Von Willebrand's protein may result in a corresponding ______ of factor VIII.
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platelets
clot deficiency |
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Clinical manifestations and the need for treatment for Von Willebrand's Disease vary depending on the severity of the disorder. What are some clinical manifestations?
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-epistaxis
-bleeding from gums -prolonged bleeding from cuts -excessive bleeding after surgery or trauma -menorrhagia in females |
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In diagnosing Von Willebrand's Disease, a thorough history will ascertain whether or not episodes of bruising are proportional to thedegree of trauma. A family history of _____ disorders is relevant. Lab tests may include a bleeding time and PTT, which will likely be _____. What is the most clinically significant lab test for diagnosing the disorder? What will it show?
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bleeding
normal quantitative immunoelectrophoretic assay, which will reveal a discrepancy in the quantity and function of Von Willebrand's factor in the plasma. |
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The therapeutic management for Von Willebrand's Disease is aimed at controlling bleeding episodes and replacing the missing or dysfunctional factor in the blood. What is the treatment? What are 2 nursing implications for this disease?
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DDAVP- IV or intranasally
(vasoconstricts) -wear a medic alert bracelet Avoid NSAIDS and ASA (thins blood) |
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With all blood disorders, the patient should wear a ____ _____ ______ and should avoid ____ and _____ (medications).
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medic alert bracelet
aspirin, NSAIDS |
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If DDAVP is administered during a bleeding episode for Von Willebrand's Disease, the parent should monitor the child's ____ intake for 24 h following administration to prevent ______, a complication seen in young children.
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fluid
hyponatremia |
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Thrombocytopenia is when platelet count falls below _____ per mm3
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150,000
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Immune thrombocytopenic purpura (ITP) is an _____ hemorrhagic disorder characterized by ______, a _____ rash, ______ bone marrow, and the absence of signs of other identifiable causes of _________.
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acquired
thrombocytopenia purpura normal thrombocytopenia |
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Immune thrombocytopenic purpura is classified as either acute, or chronic. How is each defined?
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acute- less than 6 months
chronic- greater than 6 months |
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What season(s) is Immune thrombocytopenic purpura most prevalent?
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late winter and spring
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While acute Immune thrombocytopenic purpura affects males and females equally, chronic Immune thrombocytopenic purpura affects _____ more often.
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females
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The etiology of Immune thrombocytopenic purpura is unknown, but in the majority of children, it follows a _____ illness and is considered to be an _______ process.
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viral
autoimmune |
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What are some s/s of Immune thrombocytopenic purpura ? 3
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-sudden onset of bruising & petechiae
-bleeding of mucous membranes -bleeding of gums (otherwise in good health) |
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How is Immune thrombocytopenic purpura diagnosed?
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low platelet count, < 50,000 /mm3
normal physical exam may be hx of recent live virus vaccination, illness, fever, bruising, petechiae, purpura, |
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Immune thrombocytopenic purpura results in destruction of circulating ____ and decreased ____ _____ production of new platelets.
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platelets
bone marrow |
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The greatest risk of a low platelet count is _______ ________, so a _______ assessment is important. This is especially important if platelets fall below _____.
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intracranial hemorrhage
neurologic 20,000 |
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The therapeutic management for Immune thrombocytopenic purpura includes monitoring the ______ count, and medications such as _____ and _____ . If the ITP is still not controlled, a _____ may be indicated.
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platelet
IVIG steroids (methylprednisone, prednisone) splenectomy Steroids block the autoimmune destruction of platelets. |
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True or False: Immune thrombocytopenic purpura is usually self limiting
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true
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If a splenectomy is indicated, avoid until after age ____ and there needs to be adequate measures taken to prevent infection.
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5
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What should be avoided if receiving IG (immunoglobulin)?
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live viruses because the IG can suppress the normal immune response
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In treating Immune thrombocytopenic purpura, oral steroids are taken at what dose?
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1-2 mg/kg/day
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True or False: steroids need to be weened off gradually.
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true
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_____is an acquired hemorrhagic syndrome that involves overstimulation of clotting and anticlotting process in response to a disease or injury (caused by something else). There is excessive bleeding and excessive clotting at the same time.
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disseminated intravascular coagulation (DIC)
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What is the leading cause of DIC?
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sepsis
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What are some other causes of DIC?
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disease
injury shock trauma hypoxia burns poisonous snake bite necrotizing entercolitis viral or bacterial infection pregnancy induced |
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Explain the pathophysiology of DIC
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A clotting mechanism is triggered
--> increase thrombin, and rapid fibrinogen converts to fibrin --> destruction of platelets, then obstruction and necrosis of vessel -->fibrinolytic mechanism activated with less clotting factors available -->leads to hemolysis of RBC's basically a hyperstimulation of clotting mechanism and anti-clotting mechanism where clots are destroyed faster than they can be produced. Eventually run out of clotting factors |
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What are some initial clinical manifestations of DIC?
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Excessive bruising
Petechiae Purpura Mild GI bleeding Bleeding/oozing from mild tissue trauma such as IM injections, NG insertion site, catheter site |
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What are some signs of worsening DIC?
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worsening of bleeding
progressive organ failure intracranial hemorrhage hemoptysis hypoxia (all leads to organ failure) |
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what is hemoptysis?
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the expectoration of blood or blood streaked sputum
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What are some confirmatory diagnostic lab findings for DIC?
platelet count? RBC count? fibrinogen level? PT time? level of fibrin degradation products? You will see what on the smear? |
decreased RBC count
decreased platelet count decreased fibrinogen level Prolonged PT Elevated levels of fibrin degradation products. RBC fragments on smear |
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What is an example of a fibrin degradation product?
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d dimer
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_______ are the substances left behind whet clots dissolve in blood (relating to DIC)
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fibrin degradation products
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Therapeutic management for DIC includes treating the underlying cause. Treatment and nursing considerations? (hint: mnemonic is 5 letters)
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HAVAS
Heparin (sometimes, it’s controversial) Administer blood- platelets, plasma, RBC (rapid mass transfusion) Vitamin K Avoid tissue trauma, monitor labs, support family Strict I & O |
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True or False: patients with DIC are usually in an outpatient setting and the nurse must monitor vital signs, look for signs of bleeding, and administer medications as ordered.
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False. These patients are in the ICU. Everything else is true.
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What is the opioid of choice for treating acute pain in DIC?
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morphine
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What is aplastic anemia?
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a condition in which the bone marrow stops producing the cells it normally makes, resulting in pancytopenia
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How is aplastic anemia contracted?
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congenital or acquired
congenital- Fanconi syndome acquired- drugs, chemicals, radiation exposure, viruses idiopathic- no known cause in 70% cases |
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What is pancytopenia?
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reduction of all blood cell types
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What are the clinical manifestations of aplastic anemia?
(7) |
A PEEP FIT
anorexia petechiae (low platelet) ecchymosis (low platelet) epistaxis (low platelet pallor (anemia) fatigue(low O2) infection (low WBC) tachycardia (compensating) |
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Explain what is required for diagnosis of aplastic anemia
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1. granulocytes <500
2.platelets < 20,000 3.reticulocytes <1% 4. bone marrow sample: <25% of normal cellularity two of these must be present for diagnosis, one must be #4 |
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____ ____ biopsy for aplastic anemia should reveal the presence or absence of precursors of the mature cells found in a peripheral blood sample. In aplastic anemia, these precursors are notably absent from the sample. This type us described as hypocellular and often contains a predominance of lymphocytes and yellowish fatty tissues.
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bone marrow
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Therapeutic management for aplastic anemia is based on the severity of the symptoms. If there is exposure to a known causative agent, the exposure is discontinued immediately. Describe some treatment options.
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Platelet & RBC transfusion
Bone Marrow transplant Stem Cell transplant (if matches found) If matches are not found, immunosuppresive therapy cyclosporine, antithymocyte globulin effectively treat the condition |
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Name some Nursing considerations for aplastic anemia..
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infection & bleeding precautions...
no rectal temps, meds, or exams no injections extra soft toothbrush avoid contact sports infection precautions- private room, screen visitors for illness, visitors/hcp may need to gown up, |
