Heidi James, MD, and Vanessa Cardy, MD
Screening for Stroke in Pediatric Sickle Cell Disease
Vanessa and Heidi kick off the show by discussing a case of stroke in a pediatric patient with sickle cell disease and reviewing current stroke prevention screening for this population.
- Overview of sickle cell disease (SCD):
- SCD is an autosomal recessive disorder.
- Red blood cells (RBCs) sickle in shape and have a shorter lifespan.
- SCD is predominantly seen in people of African descent but also in increasing numbers of Hispanic patients.
- The viscosity and ischemia that occurs in SCD is multifactorial, involving physical, inflammatory, and cellular factors.
- Vaso-occlusive crises with intense pain
- Severe and acute anemia
- Acute chest syndrome
- Guidance on stroke prevention:
- Annual transcranial Doppler looking for elevated cerebral vascular flow is recommended.
- High cerebral flow rates = higher risk of strokes.
- Offer periodic packed RBC (PRBC) transfusions.
- Screening and transfusion results in 90% decline in strokes.
- Many patients and families are not aware of this screening modality.
- Why? Many with SCD are from underserved groups and communities.
- American Society of Hematology recommendations:
- Annual transcranial Dopplers of children >2 y and adults
- Identification of silent strokes to increase resource access for patients with deficiencies
- Monthly transfusions for patients aged 2-16 y with abnormal scan
- If transfusion is NOT an option, start hydroxyurea.
- Adults and children <2 y:
- Consider transfusion of RBCs without waiting for sub-specialty consults or computed tomography scan confirmation of stroke.
PEARL: Annual transcranial Doppler looking for elevated cerebral vascular flow in pediatric patients with SCD and treatment with monthly PRBC transfusions can prevent strokes in this population.
See EM:RAP Special Report June 2021 - Sickle Cell Disease: Personal Insights and Screening
Clinical Alert: Periodic Transfusions Lower Stroke Risk in Children with Sickle Cell Anemia
National Heart, Lung, and Blood Institute (NHLBI), September 18, 1997
American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults
DeBaun MR, Jordan LC, King AA, et al. Blood Adv. 2020;4(8):1554-1588. doi:10.1182/bloodadvances.2019001142