Stroke prevention in sickle cell disease (STOP) study guidelines for transcranial Doppler testing

Area of Science:

• Neurology
• Pediatrics
• Hematology

Background:

• Sickle cell disease (SCD) is a genetic blood disorder associated with a high risk of stroke in children.
• Transcranial Doppler (TCD) ultrasound is a non-invasive tool used to assess cerebral blood flow velocities.
• The Stroke Prevention in Sickle Cell Disease (STOP) trial investigated the efficacy of TCD screening in preventing strokes.

Purpose of the Study:

• To detail the TCD scanning and reading protocols used in the STOP trial.
• To explain the measurement and significance of time-averaged mean of the maximum (TAMM) velocities.
• To provide guidance for appropriate application of STOP TCD data.

Main Methods:

• Children with SCD and no history of stroke underwent TCD screening.
• Children with elevated TAMM velocities in specific cerebral arteries were randomized to either transfusion or standard care.
• Follow-up data on stroke incidence were collected and analyzed.

Main Results:

• The standard care arm experienced 11 strokes over an average follow-up of 20 months.
• The transfusion arm experienced only 1 stroke during the same period.
• This highlights a significant reduction in stroke risk with transfusion therapy.

Conclusions:

• TCD screening is effective in identifying children with SCD at high risk for stroke.
• Prophylactic transfusions significantly decrease stroke incidence in these high-risk children.
• Understanding the STOP TCD methodology is crucial for accurate interpretation and application of screening results.