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Care of the patient with hematologic problems

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Answer
diagnosis of SCD   based on the percentage of hemoglobin S (HbS) on electrophoresis.  
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Sickle cell trait (AS) HBS: AS rbc with permanent sickling:   HBS: 40% rbc with permanent sickling: 5-50%  
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Sickle cell disease HBS: SCD rbc with permanent sickling:   HBS: 80% to 100% rbc with permanent sickling: greater than 90%  
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Hematocrit of SCD   low bt 20 and 30% due to short life span of RBC  
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Bilurubin level in SCD patients   High  
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WBC levels in SCD patients   WBC count is elevated due to chronic inflammation cause by hypoxia and ischemia  
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Changes in skull under XRAY   a “crew cut” appearance X-rays of joints may show necrosis and destruction. Ultrasonography, computed tomography (CT), positron emission tomography (PET), and magnetic resonance imaging (MRI) may show soft-tissue and organ changes  
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Priority problems in patients with SCD:   1 Acute Pain related to poor tissue oxygenation 2 Chronic Pain related to joint destruction 3 Potential for sepsis 4 Potential for multiple organ dysfunction and death  
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Conditions that cause sickling   hypoxia, dehydration, infections, venous stasis, pregnancy, alcohol consumption, high altitudes, low or high environmental or body temperatures, acidosis, strenuous exercise, emotional stress, and anesthesia.  
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Cardiovascular changes in patients with SCD   risk for high output heart failure S3 heart sound may be present increased jugular-venous pulsation or distention Rapid heart rate  
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Priaprism   prolonged penile erection  
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Skin assessment   every 8 hour, assess for subtle color changes. in black people, look at the roof of the mouth for a yellow appearance yellow sclera may be misleading  
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Skin integrity   As many as 75% of adults with SCD have open sores or ulcers on the lower legs that are caused by poor tissue perfusion.  
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Liver and spleen in patients with SCD   LIVER AND SPLEEN MAY FEEL FIRM  
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CNS changes in patients with SCD   If the CNS has infarcts or repeated episodes of hypoxia, patients may have seizures or manifestations of a stroke, including pronator drift and a weakened hand grasp.  
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CARE OF THE PATIENT WITH SCD   DO NOT ELEVATE HEAD OF BED ABOVE 30 degrees. Do not raise the knee position of the bed. Avoid taking blood pressure with external cuff. Check circulation in extremities every hour Remove any constrictive clothing.  
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T or F use IM injections in patients with   False Avoid IM injections  
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Hydration in patients with SCD   hypotonic fluids at 250ml/hr for 4 hr later reduced to 125ml/hr if more is needed 200ml/hr minimus is needed durring crisis  
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Infection risk of patient with SCD   They are a greater risk because of reduced spleen fucntionality  
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Major cause of death in patients with SCD   Acute Chest Syndrome, in which a vaso-occlusive episode (VOE) causes infiltration and damage to the pulmonary system  
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Teachings of sickle cell patients   • Drink at least 3 to 4 liters of liquids every day. • Contact your health care provider at the first sign of illness or infection. • Avoid planes with unpressurized passenger cabins. • Avoid strenuous physical activities. Engage is low-impact exercis  
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Glucose-6-Phosphate Dehydrogenase Deficiency Anemia   hemolytic (blood cell–destroying) anemia inherited as an X-linked recessive disorder fully expressed in homo males partial expression (carrier state) is possible in hetero females  
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Patho of G6PD   break of RBC from exposure to some drugs (e.g., sulfonamides, aspirin, quinine derivatives, rasburicase, chloramphenicol, dapsone, high doses of vitamin C, and thiazide diuretics) and exposure to benzene and other toxins.  
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Warm antybody anemia/Cold antybody anemia   Warm = IgG (98.6F) COLD = IgM (86F) use of steroid to suppress immune function splennectomy indicated if steroids are not working, as well as chemotherapy  
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Serum feretin value in Iron deficiency anemIa   serum ferritin values are less than 10 ng/mL (normal range is 12 to 300 ng/mL).  
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Best time for taking iron supplement   Best if taken between meals to reduce GI distress  
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IM or IV iron administration   for IM, use the z-track method at dorsal gluteal site ferumoxytol (Feraheme): IV drugs associated with kidney disease  
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B12 anemia   megaloblastic anemia deficiency can be cause by less intrinsic factor or low consumption can cause glossitis (beefy red tongue), fatigue, weightloss, paresthesia  
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Shilling test   which measures the presence of vitamin B12 in the urine after the patient is given an oral dose of radioactive vitamin B12.  
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Aplastic anemia   a deficiency of circulating red blood cells (RBCs) because of failure of the bone marrow to produce these cells.  
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Pheresis   removal of  
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Polycythemia   too much RBCs associated with intense itching poor oxygenation of tissue  
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Myelodysplastic Syndromes   Myelodysplastic syndromes (MDSs) are a group of disorders caused by the formation of abnormal bone marrow cells. It results in anemia, neutropenia, thrombocytopenia  
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ecchymoses   bruises  
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treatment of thrombocytopenia begins at what platelet level:   50,000/mm3  
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Thrombotic Thrombocytopenic Purpura   Clot resulting from clumping of platelet patient at risk for bleeding  
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Hemophilia   X-linked recessive  
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Transfusion   double check another nurse administer within 4 hours use 20 gauge needle (larger = better) stay with patient for the 1st 15-20minutes  
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Transfusion in older adults   assess every 15 min for signs of fluid overload. and signs of Transfusion Reaction • Rapid thready pulse • Hypotension • Increased pallor, cyanosis 2 hours in bt transfusion administer blood slowly, taking 2-4hrs  
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Platelet infusion   done within 15 to 30 minutes use of smaller filter and shorter tubing  
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what to do if patient has history of reaction with platelet transfusion   A patient who has had a transfusion reaction in the past may be given diphenhydramine (Benadryl) and acetaminophen (Tylenol) before the transfusion to reduce the chances of a reaction.  
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false signs of transfusion reactions   being febrile and having rigors (severe chills)  
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Cryoprecipitate   clotting factor for people with clotting factor disorders administered IV push in 3 minutes  
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