Point/Counterpoint

Recommendations for Routine Sickle CellTrait Screening for NCAA Division I Athletes

CASE SCENARIO

You are part of a panel determining recommendations for routine sickle cell traitscreening for National Collegiate Athletic Association (NCAA) Division I athletes. Inparticular, the panel would like your informed opinion whether all Division I athletesshould be screened for sickle cell trait. The panel would like your opinion on how youanticipate screening will impact the morbidity and mortality of athletes as well as theimpact on cost and time for appropriate administration of testing. Arguing for themotion that NCAA Division I athletes should be screened for sickle cell trait is Scott A.Anderson, head athletic trainer for the University of Oklahoma and president of theCollege Athletic Trainers Society. Arguing against the motion, that sickle cell traittesting should not be part of the routine screening for Division I Athletes, is JeanneDoperak, DO, fellowship director for the Primary Care Sports Medicine Fellowship atthe University of Pittsburgh, head team physician for Seton Hill University and StVincent College, assistant team physician at Carnegie Mellon University, and physicianrepresentative on the board of directors of the Big East Sports Medicine Society.

Guest Discussants:

Scott A. Anderson, ATCHead Athletic Trainer, University ofOklahoma, Norman, OK; Co-Chair, Inter-Association Task Force on Sickle Cell Traitand the Athlete; President, College AthleticTrainers SocietyDisclosure: nothing to disclose

Jeanne Doperak, DOUniversity of Pittsburgh Physicians;Department of Orthopaedic Surgery,University of Pittsburgh Medical CenterSports Medicine, Pittsburgh, PADisclosure: nothing to disclose

Feature Editor:

Gary P. Chimes, MD, PhDUniversity of Pittsburgh Medical Center,Pittsburgh, PA.Address correspondence toG.C.; e-mail: garypchimes@gmail.comDisclosure: nothing to discloseDisclosure Key can be found on the Table ofContents and at www.pmrjournal.org

Scott Anderson, ATC, Responds

The primary objectives of the preparticipation physical evalua-tion (PPE) are screening for conditions that may be life threat-ening or disabling, or may predispose to injury or illness [1].Secondary objectives include identifying personal health infor-mation that initiates discussion with the athlete, for example,taking knowledge gained, educating the athlete based on screen-ing results, and applying precautions as warranted.

The PPE, historically, has had little impact on the mortal-ity of athletes in sports. In our college athlete screening, weseek, preeminently, to protect life. Success in screening, then,depends on identifying those conditions, diseases, or pro-cesses that cause death in college athletes. Four commoncauses of nontraumatic death in an athlete in action arecardiac, asthma, exertional heat stroke, and exertional sick-ling [2]. These same 4 causes are echoed in the 16 nontrau-matic National Collegiate Athletic Association (NCAA) Divi-sion I football deaths since 2000. Of the 16 deaths, 4 werecardiac, one was asthma, and one was exertional heat stroke,and the inconvenient truth was that exertional sickling wasthe leading cause of death and accounted for 10 of the 16deaths. A complication of a condition, sickle cell trait (SCT),present in 3%-4% of NCAA Division I football players, ac-

counted for 63% of the deaths.

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Effective PPE screening must, to the best of our abilities andresources, identify those athletes with a condition, disease, orpredisposition to the 4 aforementioned causes of death. Sensi-tive and affordable testing for SCT in the PPE is available,beginning with a hemoglobin solubility test to detect hemoglo-bin S and follow-up testing as necessary to confirm SCT, excludeother hemoglobin variants, and quantify hemoglobin S. The costof not testing must also be considered: athletes with SCT whocarry a risk of serious or fatal sickling crisis go undetected, theincalculable cost of a human life lost to exertional sickling, thefinancial fallout in gaining closure in death of an athlete [3], andthe potential impact upon careers [4]. A futuristic ideal is to havetrait status identified at birth, which will be charted, communi-cated, and follow the patient and/or athlete and offset futurerepetitive testing and expense.

SCT status as a priority point of personal health informa-tion is evidenced by required natal hemoglobinopathy testingin all 50 states and the District of Columbia. However, resultsof a recent survey found that only 37% of families were toldof a positive newborn test for SCT [5]. In testing for SCT inour PPE at the University of Oklahoma, we identified 20football players with SCT. Only 3 knew their status, and, in

one case, the parents were unaware. “Screening” for SCT is

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DOI: 10.1016/j.pmrj.2010.12.017

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firmly established in the PPE monograph: “Do you or some-one in your family have sickle cell trait or disease?” Thequestion within the question is should we have a laboratorytest to confirm that we “know” what we “know,” recognizingthat accuracy of answers in PPE medical history forms ishighly suspect [6]. The NCAA’s screening requirement inDivision I institutions provides provision of documentedresults of prior tests in lieu of PPE testing, but natal testresults that do not track with the patient-athlete, along withan inability to readily access past medical records renders, inmost cases, a repeated test in the PPE as the sole source ofdiagnosis for the collegiate athlete.

SCT status confirmation from the PPE leads to discussionof health-related topics. Thus, targeted education, based on apositive SCT test, becomes the second pillar in an expandedmargin of safety for the collegiate athlete with SCT. Theathletic trainer and team physician inform the athlete thatSCT is a condition, not a disease, and is consistent with anormal, healthy life span. They discuss associated potentialcomplications; genetic considerations with respect to familyplanning; any medical history relative to sickling events;signs, symptoms, and settings of exertional sickling; andprecautions for the athlete with SCT. The college athlete whotests positive is virtually assured of follow-up counseling,whereas some primary care physicians may feel ill preparedto counsel and/or reticent to refer for counseling [7].

Tailored precautions for athletes with SCT offer the bestromise of preventing exertional sickling. The insult thatreates the injury that is exertional sickling collapse is “inten-ity.” Intensity may manifest in, inclusive of but not limited tombient heat stress, newness to altitude, acute illness, sus-ained intensity, heroic effort, or a relentless coach. Precau-ion, among other points, entails modifying the work:restatio in consideration of sports specificity to mitigate, if notholly offset, exertional sickling.Knowledge of SCT status facilitates a differential diagnosis

n the field. Exertional sickling often presents with muscularramping and appearance of fatigue. In the absence of trait-tatus awareness, exertional sickling that proved fatal haseen mistaken for exertional heat illness [8]. Symptoms ofickling in the athlete with SCT should always be assumednd managed as exertional sickling.

We emphasize that SCT is no contraindication to partici-ation in sports [9,10]. A common criticism of screening ishat athletes with SCT will be denied participation in sports.he risk of death for an athlete with SCT was established in974 when Polie Portier died of exertional sickling at theniversity of Colorado [11]. Despite 17 similar sicklingeaths in college athletes since Polie Portier, no evidencexists that any athlete with SCT has been denied participationn sports. Subsequent to Portier’s death, the NCAA, in 1975,ecommended screening for SCT, then rescinded the recom-endation a few years later. Nonetheless, testing has oc-

urred in NCAA institutions, at a variable rate, for 35 years.

n 35 years of testing, there is no known case of any athleteeing denied participation due to testing positive for SCT. In006, Clarke et al [12] published a survey of Division I

nstitutions from which one could estimate that 50% of thenstitutions were testing for SCT in their PPE; yet, again, theres no evidence that any athlete has been denied an opportu-ity to participate in sports. Undeniably, past discriminationased on SCT status in societies other than sports is real, but,

n sports, it is a baseless fear.Another approach is “universal precautions.” Instead of

creening for SCT, apply precautions equally to all athletes.he U.S. Army proffered and popularized the concept thatxertional heat illness is a trigger to sickling and that prevent-ng exertional heat illness will prevent SCT deaths. Despiteheir “universal precautions without screening,” exertionaleaths continue to occur in U.S. Army recruits with SCT, 6rom 1992 to 2001, and at least one in 2008 and one in 2009.Universal precautions” have not eliminated sickling deathsn the U.S. Army, nor will they in collegiate athletics.

Death grabs the headlines, whereas, athletes who experi-nce nonfatal exertional sickling fly under the radar. Exer-ional sickling may cause severe ischemic rhabdomyolysis,hich results in compartment syndromes, myonecrosis, and

cute renal failure [13,14]. Splenic infarction occurs in ath-letes with SCT newly exposed to elevation in altitude [15].Failure to consider sickling and its sequelae in a differentialdiagnosis and management has delayed proper care of SCTathletes, and some have permanent consequences from whatshould have been a reversible sickling complication.

Given that athletes with SCT are participating in sports,sports medicine practitioners need to be armed to recognizeand manage exertional sickling as it presents. Knowledge,targeted education, and tailored precaution become 3 pillarsin an expanded margin of safety for the athlete with SCT insports. Testing begets knowledge, knowledge begets targetededucation, education begets tailored precaution, and precau-tion begets an expanded margin of safety in sports for theathlete with SCT.

REFERENCES1. Bernhardt DT, Roberts WO, eds. PPE Preparticipation Physical Evalu-

ation. 4th ed. Elk Grove Village, IL: American Academy of Pediatrics;2010.

2. Van Camp SP, Bloor CM, Mueller FO, Cantu RC, Olson HG. Nontrau-matic sports death in high school and college athletes. Med Sci SportsExerc 1995;27:641-647.

3. Day C. O’Neal lawsuit settled for $2 million, athletic scholarshipplanned. Columbia Missourian March 12, 2009.

4. Witt G. Wheeler Brown dismissed as A&T athletics director. News &Record October 15, 2010.

5. Kavanaugh PL, Wang CJ, Therrell BL, Sprinz PG. Communication ofpositive newborn screening results for sickle cell disease and sickle celltrait: variations across states. AJM Genet C Semin Med Genet 2008;148C:15-22.

6. Carek PF, Futrell M, Hueston WJ. The preparticipation physical exam-ination history: who has the correct answers? Clin J Sport Med 1999;

9:124-128.

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7. Kemper AR, Uren RL, Moseley KL, Clark SJ. Primary care physicians’attitudes regarding follow-up care for children with positive newbornscreening results. Pediatrics 2006;118:1836-1841.

8. Gardner JW, Kark JA. Fatal rhabdomyolysis presenting as mild heatillness in military training. Mil Med 1994;159:160-163.

9. Diggs LW, Flowers E. High school athletes with the sickle cell trait (HbA/S). J Natl Med Assoc 1976;68:492-493.

0. NATA Consensus Statement: sickle cell trait and the athlete, June 2007.Available at http://www.nata.org/NR062107.

11. Ewing PC. Death of an athlete with sickle cell trait. Med World News

1974;15:44.

esources and concern on the issues of screening when the

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12. Clarke CE, Paul S, Stilson M, Senf J. Sickle cell trait preparticipationscreening practices of collegiate physicians. Clin J Sport Med 2006;16:440.

13. Hale MH, Clugston JR, Prine BR, Pass AN, Gupta A. Severe low back painin a football player. Poster presentation for American Medical Society forSports Medicine 17th Annual Meeting. Las Vegas, NV. March 2008.

14. Schnebel B, Eichner ER, Anderson S, Watson C. Sickle cell trait andlumbar myonecrosis as a cause of low back pain in athletes [abstract].Med Sci Sports Exerc 2008;40(Suppl):537.

15. Ortega JO. Nausea, vomiting, and abdominal pain in a collegiate

basketball player. Clin J Sport Med 2006;16:443-444.

Jeanne Doperak, DO, Responds

Early in any medical career, every clinician is taught to askseveral questions regarding any test or procedure being or-dered. First, what is the purpose of this test? Second, what do Iexpect the results to show? Third, what is the risk to the patient?Finally, fourth and probably most important, how will theresults of this test influence how I will care for this patient?

In the case of sickle cell trait (SCT) testing, the answer to thefirst question is simple. The blood test is ordered to identify ahemoglobin abnormality. This variant has been linked to splenicinfarction, renal medullary carcinoma, and, most importantly inour debate, exertional rhabdomyolysis [1].

The foundation for this debate is that, among 136 well-studied, sudden nontraumatic deaths in high school andcollege athletics over a decade, the third-most common causeof death was found to be sickling-associated rhabdomyolysis[2]. Under rare and extreme circumstances, usually in a lowoxygen environment, the red blood cell will classically de-form and is believed to clog the vessels and lead to rapid andfulminant muscle breakdown and eventually to multiorganfailure and death. However, it is important to note that indocumented cases of death related to SCT, the relationship ispurely coincidental and not causative, because there is aninability to discern histologically artifactual postmortemsickling from antemortem sickling, and there is no directevidence that links the pathogenesis of these deaths to micro-vascular obstruction by rigid erythrocytes [3].

Although the death of any young person is tragic, I believet is important to gain some “depth perception” on what thehird leading cause of death in athletes means and how thisompares with other causes of mortality. The overall greatestisk of death in athletes is cardiovascular conditions, whichre thought to account for 0.46 deaths per 100,000 partici-ants [4]. Although there are no direct comparisons for SCT,ecognize that, in the above cohort in which 7 athleteserished due to SCT, 100 were reported to have had fatalardiac events [2]. Even more astonishing is that, by far, thereatest risk of death in children from newborn through 19ears of age is unintentional events at a rate of 14.1 per00,000 [5], which includes occurrences such as motorehicle and firearm accidents. We invest a large amount of

umbers show that focusing our attention on seat belts andrearm safety would save dozens more lives.

Moving on to the second question in our algorithm, whichoses the question, what do we expect the results to show?his is a simple data-driven answer that the majority of those

ested will be negative. The SCT genetic fingerprint evolvedn Africa. This heritable variation is protective against ma-aria. Darwinism in practice now shows that the generalopulation carries the SCT, with an expected black predom-

nance. In fact, 8% of African Americans, 0.5% of Hispanics,nd 0.2% of whites make up the approximately 2 millionarriers that are affected in the United States [6]. This wouldean that a Division 1 screening program would most likely

dentify 400-500 carriers each year, the majority of whomill be African American. Consistent with this expectation,

ll 7 of the deaths reported in the reference study above werethletes of African American descent [2].

This quickly takes us to our third question, which in-olves looking at the risk of the test to the patient. The answero this question is probably much greater than the averageeader would anticipate. The tangible risk is low, very low. Aimple venipuncture will sometimes cause bleeding, bruising,ain at the site, and, rarely, infection. The intangible risk is muchigher. What does this label mean to a young athlete? How doe protect the patient’s privacy? Who pays for the test?Discrimination becomes a concern once any label is ap-

lied to an athlete, especially one who has achieved greatuccess and may have the talent to make a career of his or herport. The feared scenario is that an otherwise healthy youngan is passed over for a National Football League (NFL) orational Basketball Association (NBA) draft pick because a

eam may not want to make any accommodations for hisCT. There is evidence that high altitude and low oxygen areisk factors for adverse events in an individual with SCT.here have been professional athletes, such as Steelers safetyyan Clark, who, after such an adverse event, opts to no

onger play in Denver. In opposition, Mr Clark’s formereammate Santonio Holmes, who is also SCT positive, haslayed in Denver without any issues for years and at lastheck will continue to do so as long as the league allows. This

ebate may be one that a professional organization will just

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quite frankly not want to undertake; so, an equally talentedplayer will be signed who is not SCT positive. For those whothink this would not or could not occur, we have seen asimilar bias in the past. In 1970, the U.S. Air Force Academybarred individuals with SCT from enlisting, and some airlinesrefused to then hire them as pilots and flight attendants.Could our testing inadvertently cheat a young African Amer-ican man out of his dream of being a professional athlete?

Opponents to the above theory would say that HIPAA(Health Insurance Portability and Accountability Act) and con-fidentiality laws should protect the athlete from this scenario.However, that leads to our second point in the risk debate,which is, how do we protect the test results? As you will readlater in this article, one of the only interventions for SCT ismodification of the athlete’s workouts. Given this, the entirecoaching staff, including the strength and conditioning coaches,will need to be aware of the player’s status. As if this were notenough, should an adverse event occur, prompt managementand knowledge of the carrier state, in theory, should help withthe athlete’s care. Therefore, the entire medical staff that cares forthe athlete, from the physician to the athletic trainers, and, evenin some cases the athletic training students, will need to haveknowledge of an athlete’s status. The U.S. Navy goes so far toidentify each recruit with a neck tag and red belt for trainingexercises. I have no knowledge of a Division I program withvisual identification on the field, but, with wide dissemination ofinformation, the concept of confidentiality becomes difficult, ifnot impossible.

Is cost a risk? Yes, when it may exclude athletes fromparticipating if they cannot afford the testing. The averagecost of a basic sickle cell test is variable across the country.Our institution in Pittsburgh is charged $12.50 per test. MostDivision I schools will have approximately 150 incomingfreshman each year, with a total cost of approximately $1875.Although all programs are on economic contracture thesedays, I believe that the majority of Division I schools wouldbe able to find this amount in their budgets. Where it be-comes more burdensome is down the road. Typical policystarts at the professional and/or Division 1 level and tricklesdown through the collegiate pyramid to high schools. I canimagine that one, not so far off, day many of my less-fortunatehigh school athletes may not have the extra money to coverthe blood test and as a result may be unable to participate.

This brings us to the final and, in my opinion, the mostimportant question: how will the results affect treatment?The answer to this question I believe is the strongest reasonwhy we should not be testing all Division I athletes at thecurrent time. As mentioned previously, the risk of a cata-strophic event from SCT rises in extreme environments thatcauses low oxygen and undue stress on the body. Specifi-cally, these include the following: dehydration, illness,poorly controlled asthma, extreme fatigue, and altitude.These factors simply can be addressed by universal precau-

tions. We should be protecting all of our athletes as if they

had SCT by keeping them well hydrated and by followingNCAA guidelines for acclimatization. Every player’s asthmashould be well controlled, and anyone who is febrile shouldbe held from play. Most schools that are currently testinghave variable provisions, and many do not make any partic-ular accommodations for athletes who are SCT positive.Although there is a policy for testing, there is no set guidelinewith what to do with the positive results. This leaves manywith the impression the test is done for medical-legal impli-cations rather than the well being of the athlete.

The other point that remains unclear is if the knowledge ofa positive test prevents deaths. The NCAA has collected dataon 8 university deaths from SCT over the past decade. Ofthese 8 athletes, 4 were known to be SCT positive beforesuccumbing [7]. This statement alone raises doubt that thesystem we have currently in place is a best-practice model.

In athletics, we often refer to military data, because the 2groups tend to be made up of young, healthy individuals whoroutinely engage in rigorous physical activity in environmen-tal extremes. Each branch of the military has developed itsown policy regarding sickle cell testing. The U.S. Navy, asmentioned above, screens and labels individuals with a redbelt during training. The U.S. Air Force screens and offers theoption of discharge to those individuals who test positive.The U.S. Marines screen but do not alter the routine of thosewho are positive. Finally, the U.S. Army, in 1996, ceasedscreening and just use across-the-board precautions [3].

Perhaps our best resource for planning the future is byooking at the past. In 1970, under the leadership of Presidentixon, the country implemented a nationwide sickle cell

creening program. The policy was not well planned out, andany of the same issues that have been raised above led to an

utcry of discrimination and lack of provisions for positive testesults. Since that time, these programs have either been discon-inued or heavily modified [8]. Although a screening program inome form, not just including Division I athletics, but for theverall increasingly active population at large, may be beneficial,t needs to be more carefully constructed. The goal should be torovide the maximum benefit targeted to the highest risk pop-lation. There must not only be policies in place for screeningut also clear establishment of how to address the positiveesults while assuring patient privacy. In addition, there needs toe a greater commitment of research time and money to thisisease process to make more evidence-based practices. In 1973,ew England Journal of Medicine ran an editorial [9] on sickle cell

esting that stated the policy was “introduced before evidence that aompulsory program is needed, desired or in the best interest of theffected community.” In 2010, I could not have said it any better.

REFERENCES1. Eichner ER. Sickle cell trait. J Sport Rehab 2007;16:197-203.2. Van Camp SP, Bloor CM, Mueller FO, Cantu RC, Olson HG. Nontrau-

matic sports death in high school and college athletes. Med Sci Sports

Exerc 1995;27:641-647.

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3. Mitchell BL. Sickle cell trait and sudden death—bringing it home. J NatMed Assoc 2007;99:300-305.

4. Maron BJ, Gohman TE, Aeppli D. Prevalence of sudden cardiac deathduring competitive sports activities in Minnesota high school athletes.J Am Coll Cardiol 1998;32:1881-1884.

5. National MCH Center For Child Death Review. Available at: http://www.childdeathreview.org/nationalchildmortalitydata.htm. AccessedNovember 2010.

. Bonham VL, Dover GJ, Drody LC. Screening student athletes for sickle

cardiovascular, which echoes Maron. Maron et al [5] and Van

cell trait—a social and clinical experiment. N Engl J Med 2010;363:997-999.

7. Thomas K, Zarda B. In NCAA, question of bias over a test for a genetictrait. New York Times. April 11, 2010.

. Rutkow IM, Lipton JM. Some negative aspects of state health depart-ments’ policies related to screening for sickle cell anemia. Am J PublicHealth 1974;64:217-221.

. Whitten CF. Sickle cell programming - an imperiled promise. N Engl

J Med 1973;286:1129-1132.

Scott Anderson, ATC, Rebuts

Dr Doperak and Mitchell [1] are inarguably accurate withrespect to “no direct evidence links the pathogenesis of these[exertional sickling] deaths to microvascular obstruction byrigid erythrocytes.” Reality recognizes little likelihood thatthere will ever be “direct evidence,” given that no institutionalreview board will grant permission for a study to establishdirect evidence due to the plausibility of a causal relationshipof morbidity and mortality in exertion with SCT.

A causal relationship exists in antemortem sickling atautopsy as evidenced by extensive sickling despite transfu-sion of normal red blood cells; splenomegaly due to conges-tion of sickled red cells occurs over time versus postmortem[2]; extensive presence of sickled red blood cells in the brainand cardiac conduction system [3]; and, major “congestion”identified “behind” the sickling, a strong suggestion that thestill-beating heart was trying to pump blood through organschoked with sickling.

Skeptics give grudging ground to “rare and extreme cir-cumstances” of sickling-associated rhabdomyolysis that leadsto death, but, with a curious exclusion of athletes. Theircaveats are that events occur in the untrained, the settings arelimited to extreme environmental heat, altitude, and/or de-hydration. Sickling-associated rhabdomyolysis collapse anddeath in working athletes has occurred weeks into a trainingregimen, at altitude and at sea level, in even moderate ambi-ent conditions, and virtually year round.

Discrimination discussion deserves reprise due to thescare in the scenario of a denied professional career. Inci-dence of SCT in NFL players denies discrimination as itsimultaneously shows no contraindication to participation insports at the highest levels for the athlete with SCT [4]. Boththe NFL and the NBA test for SCT. Ryan Clark’s case does notmake him a “poster child” for discrimination. Rather, Clark’scase evidences the outcome ignorance breeds as SCTstatus is unknown and unknowing caregivers languishwhile the athlete-patient suffers, undiagnosed and un-treated, through what should be a readily recognized com-plication of SCT: splenic infarct with elevation in altitude.Return to play for Clark, and others, becomes an electiveaction based on knowledge.

Dr Doperak cites the greatest risk of death in athletes is

Camp et al [6] read the same data and drew different conclu-sions on SCT deaths: Maron’s one death to Van Camp’s 7deaths. Maron [7] subsequently revealed that his study de-sign did not include a systematic analysis for SCT. A reason-able question is raised regarding hypertrophic cardiomyop-athy (HCM) as being an over-called cause of death in blackmale athletes versus exertional sickling as one acknowledges:the incidence of HCM at birth is the same for black males andwhite males, yet HCM is a called cause of death at twice therate in black athletes versus white athletes, plus morpholog-ical attributes of athletes’ heart and an NFL study showingblack male athletes with larger hearts and thicker walls [8], allin combination with exclusion of SCT as a potential cause ofdeath [9].

Confidentiality concerns are another pseudoscare. Colle-giate athletic medicine holds an obligation to HIPAA and theFamily Educational Rights and Privacy Act. The Family Ed-ucational Rights and Privacy Act mandates stricter guidelinesfor release of information than does HIPAA. Athletic medi-cine specialists are professionals who practice in safeguardingthe athlete per federal law.

In 1992, the NCAA rescinded a recommendation for SCTtesting, not because of discrimination or “program’” failurebut for sociopolitical reasons, which triggered a descent intoignorance regarding SCT and the athlete. In 2010, in the bestinterests of the athlete, NCAA Division I joined all 50 states,the District of Columbia, the U.S. Marines, the U.S. Air Force,the U.S. Navy, the NFL, and the NBA in testing for SCT . . .but only if SCT status is absent from their personal healthrecord.

REFERENCES1. Mitchell BL. Sickle cell trait and sudden death—bringing it home. J Nat

Med Assoc 2007;99:300-305.2. Anzalone ML, Green VS, Buja M, et al. Sickle cell trait and fatal rhabdo-

myolysis in football training: a case study. Med Sci Sports Exerc 2010;42:3-7.

3. Wirthwein DP, Spotswood SD, Barnard JJ, Prahlow JA. Death due tomicrovascular occlusion in sickle-cell trait following physical exertion. JForensic Sci 2001;46:399-401.

4. Murphy JR. Sickle cell hemoglobin (Hb AS) in black football players.JAMA 1973:225:981-982.

5. MaronBJ,Shirani J,PoliacLC,et al. Suddendeath inyoungcompetitiveathletes:

clinical, demographic, and pathologic profiles. JAMA 1996;276:199-204.

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6. Van Camp SP, Bloor CM, Mueller FO, Cantu RC, Olson HG. Nontrau-matic sports death in high school and college athletes. Med Sci SportsExerc 1995;27:641-647.

7. Maron BJ. Sickle cell trait and sudden death in athletes [letter]. JAMA

1996;276:1472.

hese protective measures only work if they are used

8. Magalski A, Maron BJ, Main ML, et al. Relation of race to electrocardio-graphic patterns in elite American football players. J Am Coll Cardiol2008;51:2250-2255.

9. Eichner ER. Sickle cell trait in sports. Curr Sports Med Rep 2010;9:347-

351.

Dr Doperak Rebuts

I applaud my colleague for his compelling argument andagree that athlete’s health and safety is our primary concern.However, Mr Anderson’s 2 key points, namely the number ofdeaths since 2000 attributed to SCT and that universal pre-cautions have not been successful in the military population,are lacking only one very important thing: references!

The number of nontraumatic deaths in NCAA DivisionI football has been reported frequently in popular mediasources such as CBS Sports and USA Today. Many of thesenewspaper and magazine articles cite similar but not exactnumbers to those reported in our debate. CBS Sportsreports 10 Division I football deaths since 2000, 5 ofwhich were from SCT [1]. USA Today reports a total of 21

eaths 9 of which are linked to SST [2]; noting, that Mrnderson reports 16 deaths, of which 10 were from SCTithout an identifiable source. The NCAA football injury

urveillance document published in 2007, which includesata though the 2004 season and discusses concussionnd heat illness, does not even mention SCT in the 12-age record [3]. Several literature searches for these dataroduce only an editorial from Current Sports Medicineeports [4]. This article lists the same numbers as Mrnderson but cites the sole source as a January 2010resentation given by . . . you guessed it . . . Mr Anderson!ased on these discrepancies, using this misleading data toake a sweeping policy change before NCAA confirma-

ion and publication is premature and impulsive. In addi-ion, the numbers are only for Division I football, and thegures are not broken down by gender or race. Because theata collection was limited to football, one would safelyssume that the deaths were all men, and, based onnformation obtained from another one of Mr Anderson’srevious presentations, most if not all, were African Amer-

can. These specifics, if true, do make a good case foresting all African American NCAA Division I footballlayers for SCT. However, the data provided by my col-

eague does not provide insight into the original questionf why we should test all Division I athletes.

Mr Anderson’s second key point, that “universal pre-autions have not eliminated sickling deaths in the Army”s also without any scientific data to support this broadtatement. It is true that there have been nontraumaticeaths in the military linked to SCT over the past 2ecades; however, there is no proof that universal precau-ions were being followed by those individuals affected.

appropriately. One could compare a similar scenario ofuniversal precautions for bloodborne pathogens in health-care. Despite strict Occupational Safety and Health Ad-ministration standards to protect both the provider andthe patient against diseases such as human immunodefi-ciency virus and hepatitis, there are still a number ofreported cases secondary to exposure each year. If thoseindividuals infected did not wear gloves or use the appro-priate universal precautions when the exposure occurred,you cannot fault the system but rather the individual.Similarly, if a team or military unit is not following guide-lines for hydration and a death occurs, then this is not afailure of the structure but a human error in choosing todisregard the system. Even the American Society of Hema-tology in a September 2010 meeting reports “the militaryexperience suggests that prevention of complications incollege athletes should be possible by applying universalprecautions without the need to single out affected indi-viduals for specific treatment [5].”

The question of discrimination, although addressed byMr Anderson, is still more speculation than fact. Althoughhe does site references that state “no discrimination hasever occurred,” I would challenge, how do they reallyknow? These scenarios are often played out in a way thateven the athlete is not aware of being the victim. In anycase, I introduced the issue as a possibility and one that Ibelieve in reviewing the data unarguably and unfortu-nately still exists.

In conclusion, I do believe that there is a place for SCTtesting in athletics. However, those creating policy need moreresearch and information to answer the question, what is themost effective application of these resources? Referring backto the American Society of Hematology, which concluded itsworkshop by stating, “Given that SCT affects approximately3 million individuals in the United States, this may be anissue of genuine public health concern. It is very clear,however, that better data are needed before proceeding tomass screening, with all of its potential for undesirable stig-matization [5].” I, too, oppose mass screenings and advocatemore research and targeted testing.

REFERENCES1. CBS Sports. Non-traumatic deaths in college football since 2000. Avail-

able at: http://www.cbssports.com/collegefootball/story/11739638. Ac-cessed November 2010.

2. USA Today. Lawsuit prompts NCAA to screen athletes for sickle cell.

3

4

5

174 Anderson et al SICKLE CELL TRAIT SCREENING FOR DIVISION I ATHLETES

Available at: http://www.usatoday.com/cleanprint/?1290390980336.Accessed November 2010.

. Randall D, Ferrara M, Agel J, et al. Descriptive epidemiology of collegiatemen’s football injuries: National Collegiate Athletic Association injurysurveillance system, 1988-1989 through 2003-2004. J Athl Train 2007;

42:221-233.

. McGrew CA. NCAA football and conditioning drills. Curr Sports MedRep 2010;9:185-186.

. Framing the research agenda for sickle cell trait: NHLBI workshop focuses oncurrent scientific understanding of the health implications for individuals withSCT. Available at: http://www.hematology.org/Publications/Hematologist/

2010/5633.aspx. Accessed November 2010.

Gary Chimes, MD, PhD Responds

Testing for SCT is a contentious issue among sports medicineclinicians, and I thank Scott Anderson and Jeanne Doperakfor appropriately establishing the parameters for the discus-sion. Both of them raise important issues that are relevant forboth sports medicine clinicians and for clinicians at large;namely, what is the appropriate threshold for monitoring fora condition with potential morbidity?

I believe we would all be in agreement that there arecertain conditions that have such a high degree of morbiditythat they necessitate appropriate screening for the safety ofthe athlete or patient. For example, it obviously would not besafe for a football player to play with an acutely torn anteriorcruciate ligament, so all reasonable injury monitoring pro-grams will monitor for anterior cruciate ligament tears. Onthe other side of the injury spectrum, although glaucoma is acondition that would present an athlete with an increasedrate of morbidity, it is uncommon enough in young athletesso that glaucoma is not a condition that is regularly checkedin injury monitoring programs. The key question for thisPoint/Counterpoint, then, is whether SCT is more similar toACL injuries or to glaucoma.

One of the challenges in answering this question is inknowing the actual numbers of athletes affected. Injury mon-itoring for NCAA Division I football players is quite good, atleast compared with other sports, but, even so, the crux of thedisagreement between our 2 discussants was largely based on

consideration is the total cost of monitoring, not just for SCT,but for all conditions. For the sake of argument, let us assumethat we agree that SCT is something that should be moni-tored. However, there may be other conditions that havesimilar rates of morbidity but that have not yet been broughtto the public attention, because there have not been high-profile deaths. For example, it is likely that a bleeding disor-der such as von Willebrand disease has associated morbidityand even deaths in athletes, yet is not routinely tested inathletes. Similarly, obstructive sleep apnea may also be anissue. So may some of the more mild forms of osteogenesisimperfecta. Even if all of these conditions are reasonable tocheck in isolation, the aggregate cost of testing for all of theseconditions may not be, either because of cost or because ofadministrative burden.

On some level, testing athletes is a zero sum game, be-cause we do not have infinite resources for testing all condi-tions, so I would argue that there are 2 thresholds that need tobe crossed before we justify testing. First, is there a prevent-able morbidity that could be reduced with testing? Second, isthis the particular issue that we should be addressing or arethere more pertinent issues that have a higher morbidity?

Does SCT testing meet either of these thresholds? ScottAnderson makes a compelling case that it does, and JeanneDoperak presents a compelling argument that it does not. In

a dispute on the actual injury rates. Another issue that needs this case it is left for you, the reader, to decide.