Blood smear findings: Sickle cells; also helmet, burr, schisto-cytes, acanthocytes

Complications of sickling:

  1. Hand-Foot syndrome
  2. Splenic effects: Sequestration, progressive infarctions; lose ability to phagocytose encapsulated bacteria
  3. Renal effects: Lose concentrating ability
  4. "Painful crises"
  5. "Acute chest syndrome"
  6. Cerebrovascular Disease-About 10% of pts with SCA have a CVA by age 20
  7. Gallstones and Cholecystitis
  8. Stroke
    1. Highest incidence is 2-5yo
    2. Evidence of infarction seen on 20-35% of children with sickle cell on MRI
    3. Transcranial Doppler studies can identify patients at highest risk

Sickle trait has been associated with splenic infarction, usually during strenuous physical exercise after a recent arrival at a high altitude. May also be linked to exercise-related rhabdomyolysis and exercise-associated sudden death.

Treatment (note--this list is by NO means complete--ER)