Recommendations for Routine Sickle Cell Trait Screening for NCAA Division I Athletes

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  • Point/Counterpoint

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    The primary objectives of the preparticipation physical evalua-tioenSecmataking

    ityseedecescaucarlinmasiocaranthedepreco

    Effective PPE screening must, to the best of our abilities and

    PM&R 2011 by the American Academy of Physical Medicine and Rehabilitation193 74, February 2011

    Prin j.pmrj.2010.12.017

    164-1482/11/$36.00 Vol. 3, 168-1

    ted in U.S.A. DOI: 10.1016/

    8n (PPE) are screening for conditions that may be life threat-ing or disabling, or may predispose to injury or illness [1].ondary objectives include identifying personal health infor-tion that initiates discussion with the athlete, for example,ingknowledge gained, educating the athlete basedon screen-results, and applying precautions as warranted.The PPE, historically, has had little impact on the mortal-of athletes in sports. In our college athlete screening, wek, preeminently, to protect life. Success in screening, then,pends on identifying those conditions, diseases, or pro-ses that cause death in college athletes. Four commonses of nontraumatic death in an athlete in action arediac, asthma, exertional heat stroke, and exertional sick-g [2]. These same 4 causes are echoed in the 16 nontrau-tic National Collegiate Athletic Association (NCAA) Divi-n I football deaths since 2000. Of the 16 deaths, 4 werediac, one was asthma, and one was exertional heat stroke,d the inconvenient truth was that exertional sickling wasleading cause of death and accounted for 10 of the 16

    aths. A complication of a condition, sickle cell trait (SCT),sent in 3%-4% of NCAA Division I football players, ac-unted for 63% of the deaths.

    resources, 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-binS and follow-up testing asnecessary to confirmSCT, excludeotherhemoglobin variants, andquantify hemoglobinS.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], andthepotential 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, inone case, the parents were unaware. Screening for SCT isRecommendations for Routine SicTrait Screening for NCAA Division

    CASE SCENARIO

    You are part of a panel determining recommendations foscreening for National Collegiate Athletic Association (NCAparticular, the panel would like your informed opinion whetshould be screened for sickle cell trait. The panel would likeanticipate screening will impact the morbidity and mortalityimpact on cost and time for appropriate administration ofmotion that NCAA Division I athletes should be screened fornderson, head athletic trainer for the University of Oklahoollege Athletic Trainers Society. Arguing against the motesting should not be part of the routine screening for Divisoperak, DO, fellowship director for the Primary Care Sporthe University of Pittsburgh, head team physician for Setoincent College, assistant team physician at Carnegie Mellonepresentative on the board of directors of the Big East Sport

    ott Anderson, ATC, RespondsCellhletes

    ine sickle cell traitvision I athletes. Inl Division I athletespinion on how youletes as well as theg. Arguing for thecell trait is Scott A.d president of the

    hat sickle cell traitAthletes, is Jeanneicine Fellowship atUniversity and St

    rsity, and physicianicine Society.

    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

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    169PM&R Vol. 3, Iss. 2, 2011ly established in the PPE monograph: Do you or some-e in your family have sickle cell trait or disease? Theestion within the question is should we have a laboratoryt to confirm that we know what we know, recognizingt accuracy of answers in PPE medical history forms ishly suspect [6]. The NCAAs screening requirement invision I institutions provides provision of documentedults of prior tests in lieu of PPE testing, but natal testults that do not track with the patient-athlete, along withinability to readily access past medical records renders, inst cases, a repeated test in the PPE as the sole source ofgnosis for the collegiate athlete.SCT status confirmation from the PPE leads to discussionhealth-related topics. Thus, targeted education, based on asitive SCT test, becomes the second pillar in an expandedrgin of safety for the collegiate athlete with SCT. Theletic trainer and team physician inform the athlete thatT is a condition, not a disease, and is consistent with armal, healthy life span. They discuss associated potentialmplications; genetic considerations with respect to familynning; any medical history relative to sickling events;ns, symptoms, and settings of exertional sickling; andcautions for the athlete with SCT. The college athlete whots positive is virtually assured of follow-up counseling,ereas some primary care physicians may feel ill preparedcounsel and/or reticent to refer for counseling [7].Tailored precautions for athletes with SCT offer the bestmise of preventing exertional sickling. The insult thatates the injury that is exertional sickling collapse is inten-y. Intensitymaymanifest in, inclusive of but not limited tobient heat stress, newness to altitude, acute illness, sus-ned intensity, heroic effort, or a relentless coach. Precau-n, among other points, entails modifying the work:restio in consideration of sports specificity to mitigate, if notolly offset, exertional sickling.Knowledge of SCT status facilitates a differential diagnosisthe field. Exertional sickling often presents with muscularmping and appearance of fatigue. In the absence of trait-tus awareness, exertional sickling that proved fatal hasen mistaken for exertional heat illness [8]. Symptoms ofkling in the athlete with SCT should always be assumedd managed as exertional sickling.We emphasize that SCT is no contraindication to partici-tion in sports [9,10]. A common criticism of screening ist athletes with SCT will be denied participation in sports.e risk of death for an athlete with SCT was established in74 when Polie Portier died of exertional sickling at theiversity of Colorado [11]. Despite 17 similar sicklingaths in college athletes since Polie Portier, no evidenceists that any athlete with SCT has been denied participationsports. Subsequent to Portiers death, the NCAA, in 1975,ommended screening for SCT, then rescinded the recom-

    ndation a few years later. Nonetheless, testing has oc-rred in NCAA institutions, at a variable rate, for 35 years.35 years of testing, there is no known case of any athleteing denied participation due to testing positive for SCT. In06, Clarke et al [12] published a survey of Division Ititutions from which one could estimate that 50% of thetitutions were testing for SCT in their PPE; yet, again, thereo evidence that any athlete has been denied an opportu-y to participate in sports. Undeniably, past discriminationsed on SCT status in societies other than sports is real, but,sports, it is a baseless fear.Another approach is universal precautions. Instead ofeening for SCT, apply precautions equally to all athletes.e U.S. Army proffered and popularized the concept thatertional heat illness is a trigger to sickling and that prevent-exertional heat illness will prevent SCT deaths. Despiteir universal precautions without screening, exertionalaths continue to occur in U.S. Army recruits with SCT, 6m 1992 to 2001, and at least one in 2008 and one in 2009.niversal precautions have not eliminated sickling deathsthe U.S. Army, nor will they in collegiate athletics.Death grabs the headlines, whereas, athletes who experi-ce nonfatal exertional sickling fly under the radar. Exer-nal sickling may cause severe ischemic rhabdomyolysis,ich results in compartment syndromes, myonecrosis, andte renal failure [13,14]. Splenic infarction occurs in ath-es with SCT newly exposed to elevation in altitude [15].ilure to consider sickling and its sequelae in a differentialgnosis and management has delayed proper care of SCTletes, and some have permanent consequences from whatould have been a reversible sickling complication.Given that athletes with SCT are participating in sports,orts medicine practitioners need to be armed to recognized manage exertional sickling as it presents. Knowledge,geted education, and tailored precaution become 3 pillarsan expanded margin of safety for the athlete with SCT inorts. Testing begets knowledge, knowledge begets targeteducation, education begets tailored precaution, and precau-n begets an expanded margin of safety in sports for thelete with SCT.

    FERENCES. Bernhardt DT, Roberts WO, eds. PPE Preparticipation Physical Evalu-ation. 4th ed. Elk Grove Village, IL: American Academy of Pediatrics;2010.

    . 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.

    . Day C. ONeal lawsuit settled for $2 million, athletic scholarshipplanned. Columbia Missourian March 12, 2009.

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

    . 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.

    . 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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    Although the death of any young person is tragic, I believeit ithird leading cause of death in athletes means and how thiscompares with other causes of mortality. The overall greatestrisk of death in athletes is cardiovascular conditions, whichareparecpecargreyea10vehres

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    plied to an athlete, especially one who has achieved great

    sport. The feared scenario is that an otherwise healthy youngman is passed over for a National Football League (NFL) orNational Basketball Association (NBA) draft pick because ateaSCrisThRylonteapla

    170 Anderson et al SICKLE CELL TRAIT SCREENING FOR DIVISION I ATHLETESthought to account for 0.46 deaths per 100,000 partici-nts [4]. Although there are no direct comparisons for SCT,ognize that, in the above cohort in which 7 athletesrished due to SCT, 100 were reported to have had fataldiac events [2]. Even more astonishing is that, by far, theatest risk of death in children from newborn through 19rs of age is unintentional events at a rate of 14.1 per0,000 [5], which includes occurrences such as motor

    icle and firearm accidents. We invest a large amount ofources and concern on the issues of screening when the

    chdem may not want to make any accommodations for hisT. There is evidence that high altitude and low oxygen arek factors for adverse events in an individual with SCT.ere have been professional athletes, such as Steelers safetyan Clark, who, after such an adverse event, opts to noger play in Denver. In opposition, Mr Clarks formermmate Santonio Holmes, who is also SCT positive, hasyed in Denver without any issues for years and at lasts important to gain some depth perception on what the success and may have the talent to make a career of his or her. Kemper AR, Uren RL, Moseley KL, Clark SJ. Primary care physiciansattitudes regarding follow-up care for children with positive newbornscreening results. Pediatrics 2006;118:1836-1841.

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

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

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

    . Ewing PC. Death of an athlete with sickle cell trait. Med World News1974;15:44.

    anne Doperak, DO, Responds

    rly in any medical career, every clinician is taught to askeral questions regarding any test or procedure being or-red. First, what is the purpose of this test? Second, what do Iect the results to show? Third, what is the risk to the patient?ally, fourth and probably most important, how will theults of this test influence how I will care for this patient?In the case of sickle cell trait (SCT) testing, the answer to thet question is simple. The blood test is ordered to identify amoglobin abnormality. This variant has been linked to splenicarction, renalmedullary carcinoma, and,most importantly inr debate, exertional rhabdomyolysis [1].The foundation for this debate is that, among 136 well-died, sudden nontraumatic deaths in high school andllege athletics over a decade, the third-most common causedeath was found to be sickling-associated rhabdomyolysis. Under rare and extreme circumstances, usually in a lowygen environment, the red blood cell will classically de-m and is believed to clog the vessels and lead to rapid andminant muscle breakdown and eventually to multiorganlure and death. However, it is important to note that incumented cases of death related to SCT, the relationship isrely coincidental and not causative, because there is anbility to discern histologically artifactual postmortemkling from antemorte...

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