Sickle Cell Anemia

• Sickle Cell Anemia


  A hemoglobinopathy in which normal adult hemoglobin (HgbA) is replaced by abnormal sickle hemoglobin (HgbS)
• Sicking of cells causes clumping and obstruction of blood flow.
• Normal shape of RBC: Biconcave disk

  RBC shape in SCA: Crescent-shaped
• Hemophilia

  Etiology: Autosomal recessive gene

  • both parents are carrier
  • 1 out of 4 children = 25 %
• The most acute symptoms occur during periods of exacerbation called "crisis":

  • Vaso-occlusive crisis - "painful episodes" due to distal ischemia and pain in areas of involvement
  • Sequestration crisis - pooling of blood in the liver and spleen with decreased blood volume and shock
  • Aplastic crisis - diminished RBC production resulting in profound anemia
  • Hyperhemolytic crisis - an accelerated rate of RBC destruction characterized by anemia, jaundice, and reticulocytosis
• Sickle Cell Crisis

  Vaso-Occlusive Crisis

  • Manifestations: Fever; painful swelling of hands, feet, joints, or affected area; and abdominal pain
• Sickle Cell Crisis

  Vaso-Occlusive Crisis

  • Caused by stasis of blood with clumping of cells in the microcirculation, ischemia, and infarction
• Sickle Cell Crisis

  Sequestration Crisis

  • Caused by pooling and clumping of blood in the spleen (hypersplenism)
• Sickle Cell Crisis

  Aplastic Crisis

  • Manifestations: Profound anemia and pallor
• Sickle Cell Crisis

  Hyperhemolytic Crisis

  • Manifestations: Anemia, jaundice, and reticulocytosis
• Sickle Cell Crisis

  Hyperhemolytic Crisis

  • Caused by an accelerated rate of red blood cell destruction over a short time
• Triggering Factors of Sickle Cell Anemia:

  • Cold temperature - vasoconstriction decreasing perfusion
  • Dehydration - due to poor circulation, main
  • Stress -  SNS causing vasoconstriction
  • High altitudes - decreased atmospheric O2
  • Infection –  human parvovirus (GIT & Respi)
• SCA: Management

  1. Hydration - to prevent viscosity of blood
  2. Oxygenation - to prevent sickling
  3. Pain reliever - Morphine (check RR first; antidote - narcan)
  4. BT - PRBC (4 hours)
• Hydroxyurea - antineoplastic drug given to SCA but give with caution; given only to patients with 6 episodes per year or sickle cell crisis