Category: Pediatrics
Keywords: Sickle Cell Trait, Sudden Death, Pediatrics, Military, Sports Medicine, Law Enforcement, Medical Legal (PubMed Search)
Posted: 9/18/2009 by Adam Friedlander, MD
(Updated: 11/22/2024)
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You've probably long been taught that Sickle Cell Trait is an irrelevant piece of the PMH, unless you are a genetic counselor. Well, thanks to Dr. Rolnick and a literature search, I (and now you) know that that is incorrect.
Though Sickle Cell Trait (SCT) does not cause exactly the same pathologies as Sickle Cell Disease (SCD), there are believed to be a variety of RBC abnormalities associated with HgbS (such as measurably lower RBC deformability, and low levels of sickling under extreme heat and exercise conditions) which contribute to increased exercise-related sudden death. In one NEJM study of all deaths among 2 million (MILLION) military recruits over a 4 year period, the relative risk of otherwise unexplained sudden death for black recruits with HgbAS vs. black recruits without HgbS was 27.6 (p<0.001), and 39.8 (p<0.001) for all recruits (HgbAS vs. no HgbS).
I must say that this topic is not controversy-free, however, I should also note that my search for "Sickle Cell Trait and Sudden Death" turned up quite a few articles directed at plaintiff's attorneys.
The take-home point is that SCT is likely not a benign condition, and you must be cautious in telling patients that it is. Again, this phenomenon is best described in patients undergoing extreme physical exertion, but hopefully this will change how you think about SCT.
