5. 2. Assessment:
      1. Increased susceptibility to infection (cause: unknown; most common cause of death in children under 5).
      2. Inherited as autosomal recessive disorder.
      3. Precipitated by conditions of low oxygen tension, dehydration, vascular obstruction, increased blood viscosity.
      4. Signs of anemia:
         1. Pallor (in dark - skinned children, do not rely on pallor alone - check hemoglobin and hematocrit).
         2. Jaundice, due to excessive hemolysis.
         3. Irritability, lethargy, anorexia, malaise.
      5. Vaso-occlusive crisis: severe pain (variable sites, e.g., chest, back, abdomen), fever, swelling of hands and feet, joint pain and swelling, all related to hypoxia, ischemia, and necrosis at the cellular level. Most common; usually non - life threatening.
      6. Splenic sequestration crisis: blood is sequestered (pooled) in spleen; precipitous drop in hemoglobin levels and blood pressure, increased pulse rate, shock, and ultimately death from profound anemia and cardiovascular collapse.
   3. Analysis/nursing diagnosis:
      1. Altered tissue perfusion related to anemia and occlusion of vessels.
      2. Pain related to vaso-occlusion.
      3. Impaired physical mobility related to pain, immobility.
      4. Knowledge deficit related to disease process and treatment (e.g., prevention of sickling or infection; genetic counseling).
   4. Nursing care plan/implementation:
      1. Goal: prevent sickling.
         1. Avoid conditions of low oxygen tension (hypoxia), which causes RBCs to assume a sickled shape.
         2. Provide continuous extra fluids to prevent dehydration, which causes sluggish circulation. Avoid temperature extremes.
         3. Avoid activities that may result in overheating, to prevent dehydration; suggest appropriate clothes; limit time in sun.
         4. If dehydrated due to acute illness, supplement with IV fluids and additional oral fluids to reestablish fluid balance.