Sickle Cell Anemia

Question 1

SCD - Pathophysiology

Answer 1

• Genetic disorder (autosomal recessive) of HgbS causing distortion of RBC shape (sickle) in response to dcr O2
• Anemia due to abnormal Hgb; cannot carry oxygen
• Vaso-occlusive events

Question 2

Sickle Cells

Answer 2

• Sticky/fragile; clump together; occlude blood vessels
• Leads to anoxia, ischemic infarctions, end-organ damage
  > can cause: PE, MI, stroke, kidney disease

Question 3

Vaso-Occlusive Event

Answer 3

• A blockage of blood flow by sickled RBCs, deprives tissues of oxygen (hands&feet)
  > bc they’re sticky, fragile, and clump together
  > can lead to anoxia, ischemic infarctions, end-organ damage
• Common presentation of sickle cell crisis
• Debilitating pain

Question 4

SCD - CMs

triggers

Answer 4

• Severity varies
• Periodic crises
  > pt usually recovers
• Precipitating Conditions (triggers):
  > hypoxia states
  > high altitudes
  > dehydration
  > venous stasis: blood not moving around
  > physical/emotional stress
  > anesthesia
  > infections
  > low or high envir’t or body temo

Question 5

SCD - Medical Surgical Management

Answer 5

• Pain management
  > opioids, watch for constipation
• Oxygen
• Hydration
• Body positioning
  > no bending
• Prevent infection
• Blood transfusion
  > esp w/ major symps or Hgb is <7.5
• Hydroxyurea
  > reduces sickling
  > antimetabolites: prevent sickling of cells
  > incrs risk of developing Cx w/in infants; prevent pregnancy

Question 6

SCD - Pt Education

Answer 6

• Teaching
  > to prevent crisis/triggers: stay hydrated, avoid lack of O2, treat infections quickly
  > manage drugs: women take birth control w/ Hydroxyurea
  > heredity aspects
• Emotional support & supportive care
  > disease not curable