Transcript Sickle Cell Trait in Athletes

Sickle Cell Trait in Athletes
Jason Blackham MD CAQSM
Clinical Assistant Professor Internal Medicine
And
UI Sports Medicine Center
Introduction
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Cases
History
Pathophysiology
Complications
Screening
Symptoms
Cautions
What to do about it?
Case #1
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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
Case #1 Exam
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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
Case #1 LABS
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UA - SG 1.020, 1+
prot, 4+ heme, LE +
Micro - RBC TNTC
with dysmorphia
Urine Culture Negative
Normal CBC, CMP,
CK, PT, PTT, INR
Case #1 LABS
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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
Case #1 Treatment
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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
Case #2
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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.
Case #2 LABS
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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
Case #2
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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.
Case #2 Treatment
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Calculated sweat loss to recommend
appropriate fluid intake
Recommended guidelines for exercise
limitations
Returned to play gradually and finished
season his senior year
Sickle Cell Trait
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History
Pathophysiology
Complications
Screening
Symptoms
Cautions
Sickle Cell Trait History
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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
Deaths - SCT
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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
Deaths – Military data
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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
College Football Deaths with SCT
Eichner GSSI #103, 2006;19(4)
College Football Deaths
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2006-2007
 Rice, after
running
 University of
Southern Florida
Deaths from
exertional
rhabdomyolysis or
cardiac death from
arrhythmia
Pathophysiology
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Point mutation on Betachain of hemoglobin
Homozygous
 Sickle cell disease
 Conformational
change + sickling
Heterozygous
 Sickle cell trait
 Normally benign
Pathophysiology
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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
But with exercise
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Lactic acidosis especially muscle capillaries
Elevated body temperature
Hyperosmolar drives fluid out of RBC
 Increases concentration of hemoglobin S
Hypoxia in muscle
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Leads to sickling, necrosis, rhabdomyolysis
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Phys Sportsmed 1990;18(11):53-63
Phys Sportsmed 1993;21(7):51-64
Risk factors for sickling
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Altitude
Heat stress
Rapid
conditioning
Sustained
maximal exertion
Phys Sportsmed 1993;21(7):51-64
Complications
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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
Renal
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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
Spleen
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RBC’s 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
Spleen
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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
Muscle
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Rhabdomyolysis
Necrosis
Screening
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Recommendations
to screen for SCT
6-14%, average 8%
of African Americans
Is it preventable?
Presentation
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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
Precautions for SCT athletes
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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)
Precautions for SCT athletes
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No participation during illness
Avoid or acclimatize to altitude
If cramping, heat illness or unusual
symptoms
IV fluids, supplemental O2, cooling
 If doesn’t improve, transport to ED
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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)
Precautions and Screening
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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
Key Points
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3rd cause of death in athletes
Distinguish from heat cramps
Complications
 Hematuria, splenic infarction,
rhabdomyolysis
May be preventable
References
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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