By Scott Anderson and Eugene Egdorf, of the Lanier Law Firm
Perspective is warranted in response to the issue of sickle cell trait (SCT) screening of athletes. A prominent purveyor of obfuscation is the American Society of Hematology (ASH).
Christian and Green characterize requisite “extreme conditions accompanied by intense physical activity, severe dehydration, and high temperatures” as leading to death. Sustained intense physical activity is the insult that leads to exertional sickling in a working athlete with SCT, but conditions for our NCAA DI football dead were far from extreme. ASH echoes this misinterpretation of conditions of risk in their “preventive interventions”, i.e., 1) monitoring heat acclimatization and work-rest cycles based on the training environment, and, 2) implementing guidelines for hydration.
ASH’s heat and hydration philosophy has basis in an unpublished U.S. Army study (1982-1991) led by John Kark, M.D. The ASH meeting notebook for its 2011 Workshop to Address Screening for Sickle Cell Trait and Athletic Participation includes a Kark, at al, abstract claiming NCAA football SCT-related deaths “…were actually associated with multiple risk factors for exertional heat illness.”[12] ASH attestations are manifest in Kark’s five-fold factors of 1) competitive activity with incomplete acclimation to heat, 2) sustained hot weather, 3) high body mass index, 4) poor fitness, 5) and/or heat-retaining clothing. Only one of the ten NCAA football SCT-related dead meet the incomplete acclimation criteria. Sustained hot weather suffers validity in that only one of the ten exerted in a temperature greater than 90-degrees. Our population of NCAA football dead is described as “well-built…almost no fat at all”, “well nourished…in excellent physical condition”, “well developed”. The majority were smaller skill players inconsistent with high body mass index. Fitness is difficult to quantify but most of the ten were weeks or months into training, debunking poor fitness as a factor. Heat retaining clothing doesn’t hold up to analysis as nine of the ten dead NCAA football players were working out in shorts and t-shirt; the tenth is indeterminate. The heat/hydration hypothesis is serially proven wrong when tested in the cases of SCT-related deaths.
ASH trumpets ‘universal precaution’, their management plan borrowed from the Army, averring that regardless of SCT status, the Army’s ‘‘universal strategy for prevention of exertion-related heat illnesses [has] almost eliminated deaths in all trainees’’. Tracking the tragic trail of contemporary SCT-related deaths in the military reveals they are occurring at a rate Dr. Kark predicted from data in his previously referenced unpublished study. Similar to athlete deaths, few or none of the military SCT-related deaths meet exertional heat illness criteria. Declarations of ‘heat’ create a dubious denial of the syndrome of exertional sickling and constitute a dangerous dismissal of the risk these athletes face. SCT-related complication in the working athlete is not a heat-related illness and ‘universal precaution’ absent screening is a failed military protocol that should not be foisted on athletics.
Rarity is relative, as regards prevalence. There is a grave risk to the working athlete with SCT. Recent research reveals an NCAA DI football player with SCT has a 37-fold higher risk of exercise-related death versus those without SCT.[13] In that decade of study 1:827 NCAA DI football players with SCT died in a conditioning workout. Next is NCAA DI basketball players’ sudden cardiac death risk of 1:3000.[14]
Christian and Green illustrate Devard Darling as an example of discrimination toward an athlete with SCT, that he was “treated differently” once status was known. Devard’s status was ‘known’ in his pre-participation physical examination, the same time as his twin, Devaughn. Devard was not “treated differently” until Devaughn died and a cardiac cause was wrongly surmised with a pseudo-fear that Devard carried the same ‘cardiac’ anomaly. Devard was ‘discriminated’ against and “treated differently” due to his ‘cardiac condition’. Washington State University athletic medical professionals absolved the errant cardiac assumption, acknowledged his SCT, and granted full participation in their tackle football program. Screening for SCT in NCAA institutions can be tracked to 1975, with no evidence that any athlete has been denied opportunity to participate in sport.
Beyond the oddity that is ASH on the subject of athletes with SCT are the more numerous National Athletic Trainers Association, College of American Pathologists, American Medical Society for Sports Medicine, the American College of Sports Medicine, the American Academy of Pediatrics, the American College of Emergency Physicians, and the National Academy of Sports Medicine that support SCT screening for athletes “as an important component of a three-pronged approach to preventing SCT-related deaths 1) knowledge, 2) education, 3) precautions.” There is no known case of an athlete with SCT dying an exertion-related death when status knowledge was followed with education and tailored workout precautions were heeded.
The February 2014, death of a DI NCAA football player has a reported, but an as yet unconfirmed, SCT tie. If proven, the case will merit investigation, particularly regarding the third prong, precautions practiced. ASH opposition to identifying athlete SCT status is grounded in the lack of peer-reviewed scientific evidence that SCT screening will actually save lives. ASH’s stance is secure as the ‘evidence’ will likely never exist. However, the August 2010, index event of NCAA Bylaw requiring SCT status identification in the DI PPE is telling. In the decade, prior to August 2010, on average one DI football player died an exertional sickling death each year. From a pattern of predictable SCT-related death in DI football…no death…none in 2011…not in 2012…zero in 2013. February 2014, marked four years since Bennie Abram of Ole Miss died an ECAST death. I count that as three lives saved.
Lastly, an op-ed from an esteemed institution of academic medicine in a recent issue of JAMA reflects where the culture has moved in care for the athlete with SCT.[15] The op-ed, in its content, supports NCAA DI Bylaw 17.1.5.1 calling for SCT status knowledge in the PPE and is a ringing endorsement of NCAA Sports Medicine Handbook Guideline 2R: The Student-Athlete With Sickle Cell Trait.[16],[17]
[12] Kark JA et al. Prevention of exercise-related death unexplained by preexisting disease (EDU) associated with sickle cell trait (SCT) without hemoglobin (Hb) screening or Hb specific management. ASH Workshop to Address Screening for Sickle Cell Trait and Athletic Participation. Friday, June 3, 2011. Meeting notebook pp 58-59.
[13] Harmon, K.G., Drezner, J.A., Klossner, D., Asif, I.M. (2012). Sickle cell trait associated with a RR of death of 37 times in NCAA football athletes: a database with 2 million athlete-years as the denominator. British Journal of Sports Medicine, 46, 325-330.
[14] Harmon KG, Asif IM, Klossner D, Drezner JA. Incidence of Sudden cardiac death in national collegiate athletic association athletes. Circulation 2011;123(15):1594-600
[15] Taylor C, Kavanagh P, Zuckerman B. Sickle cell trait—neglected opportunities in the era of genomic medicine. JAMA 2014;311(15):1495-96
[16] National Collegiate Athletic Association. (2013). 2013-14 NCAA Division I manual. Indianapolis, IN: NCAA.
[17] National Collegiate Athletic Association. (2013). 2013-14 NCAA Sports Medicine Handbook. Indianapolis, IN: NCAA.
