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Sickle Cell Trait in Athletes Jason Blackham MD CAQSM Clinical Assistant Professor Internal Medicine And UI Sports Medicine Center.

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Presentation on theme: "Sickle Cell Trait in Athletes Jason Blackham MD CAQSM Clinical Assistant Professor Internal Medicine And UI Sports Medicine Center."— Presentation transcript:

1 Sickle Cell Trait in Athletes Jason Blackham MD CAQSM Clinical Assistant Professor Internal Medicine And UI Sports Medicine Center

2 Introduction ] Cases ] History ] Pathophysiology ] Complications ] Screening ] Symptoms ] Cautions ] What to do about it?

3 Case #1 ] 19 yo healthy African American Div I freshman DE during preseason conditioning ] Atraumatic, painless tea to bright red urine ] Cramping in paraspinal muscles ] No recent heat illness, dysuria, polyuria, fever, myalgias, sore throat, rashes, trauma, or previous episodes. ] Used whey protein shake daily. ] MEDs: Occasional ibuprofen, none in past 7 days ] FHx: Sister with sickle cell disease

4 Case #1 Exam ] VS: 97.7°F, 18, 72, 130/75, 242 lbs ] GEN: Healthy, NAD ] ABD: BS normoactive, soft, NT, ND, no HSM, no CVAT ] GU: Male genitalia normal without lesions, discharge, or testicular mass ] SKIN: No rashes, petechiae, skin lesions ] EXT: No edema

5 Case #1 LABS ] UA - SG 1.020, 1+ prot, 4+ heme, LE + ] Micro - RBC TNTC with dysmorphia ] Urine Culture - Negative ] Normal CBC, CMP, CK, PT, PTT, INR

6 Case #1 LABS ] No exercise or lifting for one week ] UA - SG 1.015, 1+ prot, 4+ heme, LE + ] Micro - RBC TNTC, no dysmorphia ] Heme Electrophoresis - Sickle cell trait ] Normal abdomen/pelvis CT ] Referred to urology ] Cystoscopy - bleeding from L kidney

7 Case #1 Treatment ] Epsilon aminocaproic acid and Na bicarb for 2 weeks ] UA - SG 1.010, no prot, heme, LE, RBC ] Morning urine - SG 1.020 ] Gradually transitioned back to conditioning without recurrence

8 Case #2 ] 16 yo African American high school football player in North Carolina ] Summer football practice without pads ] 1 hr ] Severe Cramps ] ? of mental status changes ] Fell in exhaustion. ] EMS took to ER.

9 Case #2 LABS ] Rectal temp 36.3 Pulse 109 ] PE unremarkable ] WBC 15.4 ] Cr 1.3 0.7 ] AST 1320, ALT 465 ] CK 138,120 8,936 ] UA- trace protein, 3+ blood, no RBC. ] Sickle cell trait ] 5L NS IV in ED. ] Admitted to ICU for ] Heat exhaustion ] Rhabdomyolysis

10 Case #2 ] Next year ] After returning from knee arthroscopy ] Conditioning at football practice ] Dizziness, weakness, mental status changes ] Cramping, ? of syncope ] Cr 1.4, CK 489, UA SG 1.010, trace Prot. ] EMS took to ER ] Felt better after 1L NS.

11 Case #2 Treatment ] Calculated sweat loss to recommend appropriate fluid intake ] Recommended guidelines for exercise limitations ] Returned to play gradually and finished season his senior year

12 Sickle Cell Trait ] History ] Pathophysiology ] Complications ] Screening ] Symptoms ] Cautions

13 Sickle Cell Trait History ] 1970 Four Deaths in Military Recruits ] 4 more with exertional rhabdomyolsis ] 1974 Colorado football player died ] 1970-1985 Several collapses and deaths in military. ] Air Force temporarily banned SCT applicants

14 Deaths - SCT ] Sudden death in athletes ] 1- Cardiovascular ] 2- Heat illness ] 3- Rhabdomylosis with SCT ] 4- Asthma Med Sci Sports Exer 1995;27(5):641-647 Arch Intern Med 1996 156(20):2297-2302

15 Deaths – Military data ] 1987 ] RR 28 compared with black recruits CI 11-84 ] RR 40 compared with all recruits ] Rate 1/3200 per training cycle ] 1994 ] RR 21 compared with black recruits CI 10-43 ] Rate 1/5,500 per training cycle NEJM 1987;317(13):781-7 Semin Hematol 1994;31(3):181-225

16 College Football Deaths with SCT Eichner GSSI #103, 2006;19(4)

17 College Football Deaths ] 2006-2007 ] Rice, after running ] University of Southern Florida ] Deaths from exertional rhabdomyolysis or cardiac death from arrhythmia

18 Pathophysiology ] Point mutation on Beta- chain of hemoglobin ] Homozygous ] Sickle cell disease ] Conformational change + sickling ] Heterozygous ] Sickle cell trait ] Normally benign

19 Pathophysiology ] In the kidney medulla ] Hyperosmolar ] Hypoxic – anaerobic ] Acidotic ] Sickling in vasa recta leading to obstruction ] Microscopic infarction of medulla ] Papillary necrosis ] Rupture of arterioles NEJM 1985;312(25):1623-31 J Am Soc Nephrol 1997;8:1034-40 Am J Hematol 2000;63:205-11

20 But with exercise ] Lactic acidosis especially muscle capillaries ] Elevated body temperature ] Hyperosmolar drives fluid out of RBC ] Increases concentration of hemoglobin S ] Hypoxia in muscle ] Leads to sickling, necrosis, rhabdomyolysis Phys Sportsmed 1990;18(11):53-63 Phys Sportsmed 1993;21(7):51-64

21 Risk factors for sickling ] Altitude ] Heat stress ] Rapid conditioning ] Sustained maximal exertion Phys Sportsmed 1993;21(7):51-64

22 Complications ] Hematuria ] Inability to concentrate urine ] Glaucoma- bleeding in anterior chamber ] Splenic infarction ] Cramps ] Exertional rhabdomyolysis ] Increased risk of heat illness ] Sudden collapse Phys Sportsmed 1993;21(7):51-64 Sem Hematology 1994;31(3):181-225

23 Renal ] Hematuria ] 2.5% of hospitalized Vets, RR 1.98 ] Expert opinion, 3-4% ] 80% from LEFT kidney ] Epidemiology in athletes and effect of exercise is not known ] Papillary necrosis ] Infarctions in medulla ] Inability to concentrate urine ] Disrupted countercurrent exchange in medulla ] Progresses with age and may lead to dehydration NEJM 1979;300(18):1001-5 NEJM 1985;312(25):1623-31 J Am Soc Nephrol 1997;8:1034-40

24 Spleen ] RBCs sickle in hypoxic environment ] Removed in spleen ] Plug up vessels in spleen ] Thrombosis leads to splenic infarction ] Most cases are at altitude >7000 ft Semin Hematol 1994;31(3):181-225

25 Spleen ] LUQ severe pain ] n/v ] Splinting, left pleural effusion and atelectasis ] Palpable spleen ] Fever ] Elevated WBC ] LDH elevated higher than CK, AST, ALT ] Usually self limited not requiring surgery

26 Muscle ] Rhabdomyolysis ] Necrosis

27 Screening ] Recommendations to screen for SCT ] 6-14%, average 8% of African Americans ] Is it preventable?

28 Presentation ] Ischemic pain in low back, buttock and leg muscles with weakness ] Cramps ] Sudden without warning ] Muscles give out and look normal ] Occurs early in season and training sessions ] Normal body temperature ] With oxygen, fluids, cold tub ] Feel fine in 10-15 minutes ] Can talk when collapse

29 Precautions for SCT athletes ] Acclimatize gradually ] Monitor hydration ] Avoid diuretics ] Consider testing urine concentrating ability in first AM void ] Modify workouts, condition gradually ] Avoid sprints or repeats over 500m, and timed runs over ½ mile Semin Hematol 1994;31(3):181-225 Phys Sportsmed 1993;21(7):51-64 NCAA Sports Medicine Handbook 2006-7, pg 74-5 GSSI #103, 2006;19(4)

30 Precautions for SCT athletes ] No participation during illness ] Avoid or acclimatize to altitude ] If cramping, heat illness or unusual symptoms ] IV fluids, supplemental O 2, cooling ] If doesnt improve, transport to ED Semin Hematol 1994;31(3):181-225 Phys Sportsmed 1993;21(7):51-64 NCAA Sports Medicine Handbook 2006-7, pg 74-5 GSSI #103, 2006;19(4)

31 Precautions and Screening ] Does it help? ] No prospective data in sports ] After military implemented protocols, number of cases reduced ] 1982-1986 compared with 1977-1981 ] RR dropped to 11 ] Rate dropped from 32 to 14 per 100,000 Semin Hematol 1994;31(3):181-225

32 Key Points ] 3 rd cause of death in athletes ] Distinguish from heat cramps ] Complications ] Hematuria, splenic infarction, rhabdomyolysis ] May be preventable

33 References ] Eichner. Phys Sportsmed 1990;18(11):53-63 ] Jones et al. Clin J Sport Med 1997;7(2)119-25 ] Heller, et al. NEJM 1979;300(18):1001-5 ] Scully, et al. NEJM 1985;312(25):1623-31 ] Diggs. Aviat Space Environ Med 1984;55(5):358-64 ] Zadeii, et al. J Am Soc Nephrol 1997;8:1034-40 ] Kark et al. Semin Hematol 1994;31(3):181-225 ] Kark et al. NEJM 1987;317(13):781-7 ] Ataga et al. Am J Hematol 2000;63:205-11 ] Warren et al. Pediatrics 1999;103(2):22-4 ] Eichner. Phys Sportsmed 1993;21(7):51-64 ] Eichner Gatorade Sports Science Institute, Sports Science Exchange 103, 2006;19(4):1-6 ] NCAA Sports Medicine Handbook 2006-7, pg 74-5 ] Van Camp et al. Med Sci Sports Exer 1995;27(5):641-647 ] Thompson et al. Arch Intern Med 1996; 156(20):2297-2302


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