VII. TEACHING POINTS

1. 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 SCD.
2. If a child with SCD needs a transfusion, the physician should communicate with the local blood bank. Children with SCD are at higher risk for developing allo-antibodies to C, E, and Kell. Extended typing of blood can usually be completed.
3. Over 20% of children with sickle cell anemia (SS) will have a silent cerebral infarct by the age of eighteen. Reports have associated infarcts with severe acute chest syndrome. While children with SCD 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.
4. 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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