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Perfusion
adequate arterial blood flow through the tissues and blood that is pumped by the heart to oxygenate body tissues.
hemoglobin A
Normal adult hemoglobin. The molecule has two alpha chains and two beta chains of amino acids.
hemoglobin S
sickle cell hemoglobin. At least 40% (and often more) of the total hemoglobin is composed of an abnormal beta chain.
hypoxia
deficiency in the amount of oxygen reaching the tissues.
hemolytic
destroying worn-out red blood cells and releasing their hemoglobin for reuse
crises
Periodic episodes of extensive cellular sickling in a patient with Sickle cell disease (SCD)
hypoxemia
deficient amount of oxygen in the blood
teratogenic
known to have potential to cause developmental defects in the embryo or fetus
microcytic
a small red blood cell. Chronic iron deficiency.
macrocytic anemia
A form of anemia characterized by large, immature red blood cells
pernicious anemia
lack of mature erythrocytes caused by inability to absorb vitamin B12 into the bloodstream
intrinsic factor
A substance produced by the mucosa of the stomach and intestines that is essential for the absorption of vitamin B12.
glossitis
inflammation of the tongue. Big beefy red tongue.
paresthesias
Abnormal sensations such as burning, numbness, or tingling
leukopenia
Abnormally low white blood cell count
Thrombocytopenia
low platelet count
pancytopenia
abnormal reduction of all blood cells
warm antibody anemia
autoimmune hemolytic anemia that occurs in warm body temps. May be triggered by drugs, chemicals, or other autoimmune problems.
cold antibody anemia
autoimmune hemolytic anemia that occurs at cold temps.
most common symptom of SCD crisis?
Pain
sickle cell disease
Genetic disorder in which red blood cells have abnormal hemoglobin molecules and take on an abnormal shape. Results in chronic anemia, pain, disability, organ damage, increased risk for infection, and early death as a result of poor perfusion.
conditions that cause sickling
hypoxia, dehydration, infection, venous stasis, pregnancy, alcohol consumption, high altitudes, low or high environmental or body temperatures, acidosis, strenuous exercise, emotional stress, and anesthesia.
repeated vaso-occlusive event (VOE) results in
long term damage to tissues and organs. Most often affected are the spleen, liver, heart, kidney, brain, joints, bones, and retina.
Sickle cell disease
genetic disorder with an autosomal recessive pattern of inheritance.
Occur in people of all races and ethnicities but is most common among African Americans in the U.S.
Sickle cell trait and Sickle cell disease
how to assess for skin changes in a patient with decreased perfusion and anemia.
examine the lips, tongue, nail beds, conjunctivae, palms, and soles of the feet every 8 hours for subtle color changes.
another skin sign of SCD is jaundice. How do you inspect for Jaundice in patients with darker skin?
inspect the roof of the mouth for a yellow appearance. Examine the sclera closest to the cornea to assess jaundice more accurately. Jaundice often causes intense itching.
The hematocrit of patients with SCD
Low between 20% and 30% because of RBC shortened life span and destruction.
WBC in a patient with SCD
Usually high
Imaging assessment in SCD
bone changes occur as a result of chronically stimulated marrow and low bone oxygen levels.
Other diagnostic assessment in patient with SCD
ECG changes document cardiac infarcts and tissue damage. Echocardiograms may show cardiomyopathy and decreased cardiac output (low ejection fraction)
result of poor perfusion and gas exchange from obstructed blood flow in a SCD patient
pain
Hydroxyurea (droxia)
Medication may reduce number of sickling and pain episodes by stimulating fetal hemoglobin production. Drug increases the risk for lukemia. It also suppresses bone marrow function including immunity and regular follow-up to monitor complete blood counts for drug toxicity.
the patient with SCD is at greater risk for
bacterial infection bc of decreased spleen function resulting from anoxic damage
major cause of death in adults with SCD
Acute chest syndrome, in which a vaso-occlusive event (VOE) causes infiltration and damage to the pulmonary system.
Interventions for SCD patient in crisis
assess for adequate perfusion
Hydration
Oxygen (possible ABG/CXR if o2 low)
Transfusion with RBCs
iron deficiency anemia
most common anemia worldwide. It can be a result from blood loss, poor GI absorption of iron, and inadequate diet.
Evaluation for any adult with iron deficiency anemia
abnormal bleeding especially from the GI tract.
symptoms of iron deficiency anemia
pallor, irritability, anorexia, and decrease in activity, fissures at the corner of the mouth; sometimes a slight heart murmur is heard
Vitamin B-12 deficiency anemia results in
failure to activate enzymes that move folic acid into precursor RBCs cells so cell division and growth into functional RBCs can occur.
Causes of vitamin B-12 deficiency
vegan diets or diets lacking dairy products, small bowel resection, chronic diarrhea, diverticula, tapeworm, or overgrowth of intestinal bacteria.
Indications of B-12 deficiency anemia are
pallor, jaundice, Glossitis, fatigue, and weight loss
Symptoms of folic acid deficiency
same as B12 deficiency
-no neurological alterations
common causes of folic acid deficiency
poor nutrition, malabsorption, and drugs.
Second most common cause of folic acid deficiency
Malabsorption syndromes such as Crohn's Disease
Management of Iron deficiency anemia
increase intake of iron from food sources. if iron losses are mild, oral iron supplements are started until the hemoglobin level returns to normal.
Management of B-12 deficiency anemia
teach patient to increase the intake of foods rich in B-12. Vitamin supplements may be prescribed when anemia is severe. Patients who have pernicious anemia are given B-12 injections.
Management of folic acid deficiency anemia
a diet rich in foods containing folic acid and vitamin B-12. May also be managed with folic acid replacement therapy.