1. Sickle Trait in Athletes Andy Peterson MD MSPH

4. Sickle Cell Disease/Trait Substitution of a valine for glutamic acid as the sixth amino acid of the beta globin chain. Poorly soluble (alpha2-betaS2) hemoglobin Sickles and sticks together when deoxygenated

11. Sickle Cell Disease SS disease - rare in athletes Vaso-occlusive crises –“A vicious viscous cycle” Frequent ischemic injuries –Dactylitis –Lung = acute chest –Stroke –Splenic Sequestration –Kidney Infarct (papillary necrosis) –Rhabdomyolysis –Bone Marrow Failure –Priapism –Soft tissue/ulcers/painful crisis Infections (pneumococcus, salmonella) Significant Disability Early Death –Median life expectancy 45 years 1 1. Platt, OS, Brambilla, DJ, Rosse, WF, et al. Mortality in sickle cell disease: Life expectancy and risk factors for early death. N Engl J Med 1994; 330:1639.

12. Sickle Trait Hgb AS No hematologic findings 25 million Americans 8.5% of African Americans Inconsequential in most people

13. Challenges Exercise Altitude Heat

16. Rare complications Splenic infarct at altitude Papillary necrosis Rhabdomyolysis

17. Splenic Infarct Ryan Clark at Denver 2005 – “Splenic contusion” 2007 – splenectomy 2009 – held out

18. Splenic Infarct Acclimatize Can occur as low as 5500 feet Presents with –LUQ pain –Nausea –Vomiting Commonly misdiagnosed

19. Splenic Infarct Descend Rest Hydration Oxygen Rarely splenectomy

20. Hematuria Papillary necrosis Sickling in medula Hyposthenuria

21. Hematuria/Papillary necrosis Hydrate Avoid 2 nd kidney injury

23. Sudden Death With Sickle Trait Sudden death among 2 million military recruits 1977-1981: –32.2/100,000 black recruits with AS –1.2/100,000 black recruits without hgb S –0.7 in nonblack recruits without hgb S 3 Army recruits 2008-2010 –All trying to make 2 mile time standard Kark, JA, Posey, DM, Schumacher, HR, et al. Sickle cell trait as a risk factor for sudden death in physical training. N Engl J Med 1987; 317:781

24. Sudden Death With Sickle Trait Most deaths occurred during basic training 1 Most occurred during 1-3 mile runs 2 Sudden collapse was rare - most deaths 2 hours to 2 days later (rhabomyolitis) 2 Risk of fulminant exertional rhabdomyolitis 200-fold higher in AS 2 1. Kark and Ward. Exercise and hemoglobin S. Semin In Hematol. 31:181-225. 2. Gardner and Kark. Fatal rhabdomolitis presenting as mild heat ilness in military training. Milit Med. 159:160-163.

25. Sudden Death With Sickle Trait Martin et al. Am J Med. 1989. –Exercised 15 military recruits with AS –Maximal exercise at 1270m and Sim4000m –1270m = 2.3% sickled cells –Sim4000m = 8.5% sickled cells –One recruit had 25% sickled cells and vSpO2 28%

26. NCAA Football 2000-2010 No deaths on the field of play 16 conditioning deaths 1 weight lifting 15 running or agility 4 Cardiac 1 Asthma 1 Exertional Heat Stroke 10 SCT

27. Math 10 SCT deaths 5 non-SCT deaths 3-4% prevalence of SCT = RR 16-21!

28. The other math 1/25,000 die of SCT per year 0.5/25,000 die of non-SCT per year = 0.00004 absolute risk increase/year (4/1,000 th of a percent attributable risk) If limit to AA’s, 0.00008 ARI

29. 10 most recent football SCT deaths #5 serial sprints for 5 to 25 minutes #4 fast-tempo, multi-station drills with short rest #1 gassers for 30 minutes

30. –1974ColoradoRan 700m –1985 ArkansasRan 3/4 mile –1986 MississippiRan 1 mile –1987IndianaRan 1200m –1989 UtahRan 3/4 mile –1990New MexicoRan 800m –1992GeorgiaRan 1000m –1995ArizonaRan 900m –2000TennesseeRan 800m –2001FloridaIntense drills 1h –2004 OhioRan for 10 minutes –2005MissouriField Drill 1h –2006TexasRan 1600m –2008 FloridaDrills –2008 North CarolinaRan hill 15 times –2009North CarolinaRan 700 yards –2010 MississippiStation Drills

31. Common AS sickling situations –Running for time –Suicides/gassers –Hard efforts 800-1200m –High heat –Dehydration –Altitude –Early in workouts

32. Multiple other studies support –Weisman et al. Clin Res. 1988 –Weisman et al. Am Rev Resp Dis. 1988 –Gozal et al. Med Sci Sports Exerc. 1992 –Freund et al. Int J Sports Med. 1995 –Bile et al. Med Sci Sports Exerc. 1996 –Sara et al. Clin J Sport Med. 2003 –Bergeron et al. Clin J Sport Med. 2004 –Marlin et al. Int J Sports Med. 2005 –Connes et al. Eur J Appl Physiol. 2006 –Monchanin et al. Med Sci Sports Exerc. 2006

33. Heat Cramps –Early warning signs –More painful/locking –Pain stops exercise –“Hobble to a halt” –Rock hard muscles –Slow response to Tx Sickling –Abrupt onset - no warn –Less painful (claudication) –Weakness stops exercise –“Slump to a stop” –Soft muscles –Rapid response to Tx

34. Management/Prevention Acclimation Progressive adaptation Increased time between hard efforts Pre-hydrate Respond quickly –Cool –Hydrate –Oxygen –Have AED ready if Sx

35. SPORT SPECIFFIC CONDITIONING!

36. –1974ColoradoRan 700m –1985 ArkansasRan 3/4 mile –1986 MississippiRan 1 mile –1987IndianaRan 1200m –1989 UtahRan 3/4 mile –1990New MexicoRan 800m –1992GeorgiaRan 1000m –1995ArizonaRan 900m –2000TennesseeRan 800m –2001FloridaIntense drills 1h –2004 OhioRan for 10 minutes –2005MissouriField Drill 1h –2006TexasRan 1600m –2008 FloridaDrills –2008 North CarolinaRan hill 15 times –2009North CarolinaRan 700 yards –2010 MississippiStation Drills

37. Dale Lloyd II Rice 2006 Family sued NCAA Required screening

39. NCAA emphasis Year round conditioning Longer rest Symptom recognition Modification at altitude Hydration Set own pace Build slowly Adequate rest Sport specific conditioning Avoid exercise with illness Supplemental O2 at altitude Climate of acceptance

40. Questions