Thanks to Dr. Bradby for today’s Morning Report!
Stroke in Sickle Cell Disease
Neurological and Hematological Emergency
- 24% of sickle cell disease patients have a stroke by the age of 45 years
- these people have an increased risk of secondary stroke 2-3 years after initial CVA (recurrence rate of 66%)
- Ischemic variant – 54% of all CVAs is highest during the first decade of life and after age 30
- In the 20s – hemorrhagic CVA is most predominant
- 10-30% of SCD patients have “silent strokes”
- Radiologic findings consistent with diffuse white matter disease
- Associated with cognitive deficiencies
Prevention:
- Hydroxyurea
- Chronic transfusion with goal of maintain HbS <30% (STOP 1, 2 trials – Optimizing Primary Stroke Prevention in Sickle Cell Anemia)
Management:
- Head CT, Stroke Labs, Consults
- Treatment: EXCHANGE Transfusion; no place for tPA in these patients
- Consults: Neurology, Hematology, MICU, Renal (for Shiley placement)
- Call blood back – need 8-10 units for exchange transfusion
- Call NY Blood Blank and ask to initiate exchange transfusion – this can take several hours
- Place Central Line – need a large bore femoral line for exchange (ex. Shiley)
- This is where your renal consult comes in if necessary
References:
- Luis A. Verduzco and David G Nathan. Sickle Cell Disease and Stroke. Blood. December 10, 2009. Vol. 114, no 25, p5117-5125.
- Switzer JA, Hess DC, Nichols FT, Adams RJ. Pathophysiology and treatment of stroke in sickle-cell disease: present and future. Lancet Neurol. 2006 Jun;5(6):501-12. Review. PubMed PMID: 16713922.
The views expressed on this blog are the author's own and do not reflect the views of their employer. Please read our full disclaimer here. Any references to clinical cases refer to patients treated at a virtual hospital, Janus General Hospital.
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Jay Khadpe MD
Editor in Chief of "The Original Kings of County"
Assistant Professor of Emergency Medicine
Assistant Residency Director
SUNY Downstate / Kings County Hospital
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