Sickle cell disease is an inherited disease of red blood cells. It is characterized by pain episodes, anemia (shortage of red blood cells), serious infections and damage to vital organs.

  1. Symptoms of sickle cell disease vary and range from mild to severe, and symptoms may be less severe or different in children who have inherited a sickle cell gene from one parent and a different abnormal hemoglobin gene from the other.
  2. Acute chest syndrome: Infection or trapped red blood cells in the lungs cause this syndrome. Signs include chest pain, coughing, difficulty breathing, and fever.
  3. Aplastic crisis: This is when the bone marrow temporarily slows its production of red blood cells due to infection or another cause. Signs include paleness and fatigue.
  4. Hand-foot syndrome (also called dactylitis): This may be the first sign of sickle cell anemia in some infants. Signs include painful swelling of the hands and feet and fever.
  5. Infection: Children with sickle cell disease are at increased risk for certain bacterial infections.
  6. Painful crises: These may occur in any part of a child's body. They may be brought on by cold or dehydration.
  7. Splenic sequestration crises: The spleen becomes enlarged by trapping (or sequestering) the abnormal red blood cells. This leads to fewer cells in the general circulation. Early signs include paleness, weakness, an enlarged spleen, and pain in the abdomen.
  8. Stroke: Poor blood flow in the brain can occur from the sickle-shaped cells blocking small blood vessels. This may lead to a stroke.