Pain crises in children with sickle cell anemia

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Sickle cell anemia (SCA) illustrates some of the challenges of keeping current with the management of a chronic illness. As improvements in standard care are incorporated into practice, one needs to periodically reexamine the natural history of the condition, which may be modified by the new therapies. Thus, even in a disease studied as well as SCA, there is an ongoing need for continuing well-characterized multicenter studies.

In this issue of The Journal, Darbari et al use one such study to update us on a serious complication of SCA—the painful vasoocclusive crises. Although the perspective of the physician working in an emergency department might be that pain crises are common in SCA, one observation in the study is that frequent episodes (≥3 annually) only occur in about one-fifth of children. That being said, this is a worrisome subgroup because they have lower quality of life and higher mortality. There appears to be a number of factors in common with these adolescents: older age, alpha thalessemia trait, iron overload, high hemoglobin, and low LDH.

Physicians caring for children and adolescents with SCA now have an up-to-date data set that should help identify the children at risk for this complication and apply potential preventive interventions.

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