20 Yo African American Female With Sickle Cell Trait Complains Of Inability To Control Eyes - Page #4
 

Working Diagnosis:
Acute right thalamic ischemic stroke.

Treatment:
Patients symptoms rapidly improved over the course of an hour after Emergency Department arrival. Because of this, TPA was not offered.

Outcome:
Patient progressed to full recovery without any residual symptoms within several hours. An echocardiogram was performed which showed a PFO. Patient elected to have her PFO closed which was performed one week later. Lower extremity venous dopplers were performed which did not reveal any DVT.

Author's Comments:
-Sickle cell trait (SCT) was thought to be a benign carrier state with very little clinical sequelae
-We now know that SCT is associated with a number of complications in the athlete including exertional rhabdomyolysis and cardiac sudden death.
-The risk of VTE among those with SCT is twice that of individuals without.
-Our patient had that added risk of OCP use and we hypothesize her PFO was the conduit through which a venous thrombosis embolized to her brain.
-Small case-control study alluded to possible synergistic effect in incidence of VTE among SCT patients who use OCP. Not statistically significant.
-American Society of Hematology recently withdrew their recommendation to screen all athletes for SCT.
-We believe identifying athletes that are at risk for serious complications such as VTE and stroke is imperative and therefore promote routine hemoglobin solubility screening.

Editor's Comments:
This is an excellent case which highlights the complexity of sickle cell screening. In this case the question is "would screening have prevented this event?" This patient had the sickle cell trait, but was also complicted by oral contraceptives and a Patent Foramen Ovale (PFO). PFO's do not carry an increased risk of CVA, but in cases of cryptogenic stroke the incidence of PFO increases. Certianly having the knowledge of sickle cell trait would allow us to more acurately access risk for sudden cardiac death in our athletic population. This case emphasizes the need at this point in time to actively manage these athletes until we have more definitive data. The AMSSM and American Society of Hematolgy have both reccomended for universial preventitive precautions for these athletes. The NCAA and NATA both have released guidlines and concensous statements on those preventitive measures. It might also be important to consider cessation of OCP use in sickle cell trait athletes.

References:
-Hart RG, Kanter MC. Sroke. "Hematologic disorders in ischemic stroke. A selective review." 1990. 21:1111-1121.
-Austin H, Key NS, Benson JM, et al. Blood. "Sickle Cell Trait and the Risk of Venous Thromboembolism among Blacks." 2007.110;3,908-912.
-Austin H, Lally C, Benson JM, et al. American Journal of Obstetrics and Gynecology. "Hormonal Contraception, Sickle Cell Trait, and Risk of Venous Thromboembolism among African American Women." June 2009: 620;e1-3.
-Key N, Derebail VK. Hematology. "Sickle Cell Trait: Novel Clinical Significance." 2010, 418-422.
-Fisher DC, Fisher EA, Budd JH, et al. Chest. "The incidence of Patent Foramen Ovale in 1,000 consecutive patients." June 1995, 107;6, 1504-1509.

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