Sickle Cell Anemia

1. acute anemia-aplastic crisis, splenic sequestrations crisis, hyperhemolytic crisis-

2. acute chest syndrome- fever, chest pain, hypoxemia wheezing, cough or respiratory distress of new pulmonary infiltrate

3. cycles of deoxygenation & oxygenation cause HbS molecule to polymerize damaging RBC structure leading to sickle shaped RBC's that die prematurely leading to hemolytic anemia

4. erythrocyte derangement, infection, chronic hemolysis (anemia), microvascular occlusions, tissue damage

5. compensatory increases in RBC production & adaptation to a lower hgb level are sufficient tp prevent major sx of anemia

5.1. chronic anemia-low serum erythropoietin concentration d/t renal disease, increased plasma viscosity &/or folate or iron deficiency

6. chronic anemia- CBC, peripheral blood smear typically show hgb -8-10, htct 20-30%, retic count 3-15%, sickled cells, mildly elevated WBC's

7. sickle cells block blood flow to organs depriving them of oxygen damaging nerves and organs

8. herediatry hemoglobinopathy -autosomal recessive pattern where each parent carries 1 copy of mutated gene

9. hemoglobin S- HbSS; sickle shaped hemoglobin within erythrococytes formed by genetic mutation in B globin

10. pain, sob, Spo2 decreases, infection, htn, tachycardia

11. organ systems effected by SCD; central nervous system-stroke, cognitive impairment; cardiopulmonary system- pulm htn, acute chest syndrome, restrictive lung disease; GU system- priapism, CKD, papillary necrosis of the kidney; hepatic system- hepatic sequestration, hepatic crisis & intrahepatic cholestasis; splenomegaly

11.1. complications in lungs= worsening cycle of hypoxemia, sickling & vaso-occlusion