VII. TEACHING POINTS

1. Sickle cell disease is an autosomal recessive disorder due to the inheritance of an abnormal hgb that under low oxygen conditions will form polymers and alter the red cell membrane. It is associated with chronic hemolytic anemia and recurrent vaso-occlusive crises. These crises can be associated with tissue infarct in the bones, lungs, liver, central nervous system, pancreas, and kidneys.
   
   
2. Patients with sickle cell disease may have a variable clinical course due in part to the amount of hgb S in their red cells. However, recently two types of clinical profiles have been defined by their LDH levels. Patients with high LDH levels may have marked anemia, pulmonary hypertension, leg ulcers, and episodes of priapism. Patients with low LDH levels may have chronic pain episodes.
   
   
3. Initial management of acute chest syndrome requires recognition of the clinical syndrome: chest pain, desaturation, fever, and infiltrate on CXR. Hematologists should also consider asthma as a contributing factor to acute chest syndrome. Asthma is a common chronic disease in African American children, and asthma has been associated with an increased rate of pain as well as an increased incidence of acute chest syndrome in children with sickle cell disease.
   
   
4. If a child with sickle cell disease needs a transfusion, the physician should communicate with the local blood bank. Children with sickle cell disease are at higher risk for developing allo-antibodies to C, E, and Kell. Extended typing of blood can usually be completed.
   
   
5. Over 20% of children with sickle cell anemia will have a silent cerebral infarct by the age of eighteen. Reports have associated infarcts with severe acute chest syndrome. While children with sickle cell disease and acute chest syndrome are in the ICU setting, both the hematologists and intensivists should monitor the child’s fluid status and neurological condition. Fluid overload may lead to hypertension and then reversible posterior leukoencephalopathy or infarct.
   
   
6. Even though infarcts without a focal correlating neurological finding are deemed “silent,” affected children still may have long-term sequelae. Almost 75% of children with silent infarcts may have cognitive deficits or receive special education services in school. Families, medical teams, and educators will need to communicate with each other to address these children’s needs.
   
   

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