UT Southwestern researchers investigate predictors for sickle-cell-anemia com...(o are at greater risk of having a stroke...),Health

Another study by Dr. Quinn and his colleagues appeared in the January issue of the journal Blood. That study examined how effectively a model developed by the Cooperative Study of Sickle Cell Disease (CSSCD) predicted severe disease in the newborn cohort.

Because sickle cell disease can affect children in many different ways, it is difficult to identify young children who are at high risk of adverse outcomes before irreversible organ damage occurs. Such outcomes include death, stroke, frequent pain or recurrent acute chest syndrome. The CSSCD criteria, which evaluates patients based on factors such as occurrences of dactylitis a type of painful swelling of the hands and feet in the first year of life, steady-state hemoglobin concentration in the second year of life, and steady-state leukocyte count in the second year of life, was created in hopes that a predictive model would allow early, tailored therapy to prevent adverse outcomes.

We found the CSSCD model was not better than random prediction when applied to the Dallas Newborn Cohort, said Dr. Quinn, the Blood studys lead author. Most subjects who experienced adverse events were predicted to be at low risk for adverse events, and no subject who was predicted to be at high risk actually experienced an adverse outcome. We concluded that the model was not clinically useful, at least not in the Dallas cohort.

Dr. Quinn said the findings suggest that the CSSCD model should not be used as the sole criterion to initiate early, high-risk intervention and that a robust early prediction model is still needed.

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