The "pain rate" (episodes per year) is a measure of clinical severity and correlates with early death in patients with sickle cell anemia over the age of 20. Even when the fetal hemoglobin level is low, one can predict that small increments in the level may have an ameliorating effect on the pain rate and may ultimately improve survival. This outcome is particularly encouraging to investigators studying hydroxyurea and other treatments designed to increase the fetal hemoglobin level.

Three easily identifiable manifestations of sickle cell disease that may appear in the first two years of life (dactylitis, severe anemia, and leukocytosis) can help to predict the possibility of severe sickle cell disease later in life.

Patients with risk factors for silent infarcts should be evaluated for cerebrovascular disease. If evidence of infarction is found, consideration must be given to therapeutic intervention. At present, the appropriate treatment has not been determined.

Examined pain coping strategies in 72 children and adolescents with sickle cell disease and their parents using the Coping Strategies Questionnaire, and found that pain coping strategies could be reliably assessed. Children high on Negative Thinking and Passive Adherence were less active, required more health care services, and were more psychologically distressed during painful episodes. Children high on Coping Attempts were more active and required less frequent health care services. Parents high on Coping Attempts and low on Passive Adherence had children who were more active.