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Continuous Manual Exchange Transfusion for Patients with Sickle Cell Disease: An Efficient Method to Avoid Iron Overload
Published on: March 14, 2017
Transcranial Doppler ultrasonography and prophylactic transfusion program is effective in preventing overt stroke in
Henrietta Enninful-Eghan1, Reneé H Moore, Rebecca Ichord
1Division of Hematology, Children's Hospital of Philadelphia, Department of Pediatrics, University of Pennsylvania, Philadelphia, PA 19104, USA.
Insights
Implementing transcranial Doppler (TCD) ultrasonography significantly reduced stroke incidence in children with sickle cell disease. While effective, the program led to increased transfusion rates for stroke prevention, suggesting a need for further screening and treatment research.
Area of Science:
- Neurology
- Pediatrics
- Hematology
Background:
- Sickle cell disease (SCD) poses a high risk for cerebrovascular complications, including stroke.
- Transcranial Doppler (TCD) ultrasonography is a key screening tool for identifying children with SCD at elevated stroke risk.
Purpose of the Study:
- To evaluate the effectiveness of a TCD screening program in reducing the incidence of first stroke in children with SCD.
- To assess changes in transfusion therapy rates for stroke prevention following the implementation of TCD screening.
Main Methods:
- A retrospective study compared stroke incidence and transfusion rates in 475 SCD patients before and 530 patients after initiating a TCD screening program.
- Data were analyzed over two 8-year periods to determine the program's impact.
Main Results:
- The incidence of overt stroke decreased significantly from 0.67 to 0.06 per 100 patient-years post-TCD implementation (P<.0001).
- The rate of transfusion therapy for stroke prevention increased from 0.67 to 1.12 per 100 patient-years (P=.008).
- Two strokes occurred post-TCD, one in a patient too young for screening and another with isolated anterior cerebral artery abnormalities.
Conclusions:
- The TCD screening program successfully reduced the rate of first overt stroke in children with SCD.
- Increased transfusion use accompanied the stroke reduction, highlighting the need for ongoing evaluation and potential refinement of screening protocols and alternative treatments.
Objective:
To assess the impact of our transcranial Doppler ultrasonography (TCD) program on the incidence of first stroke and the rate of transfusion for stroke prevention in children with sickle cell disease.
Study Design:
In this single-institution, retrospective study, we compared the incidence of stroke and of transfusion for stroke prevention in 475 patients observed in the 8-year period before instituting TCD screening with the rate in 530 children in the 8-year period after.
Results:
The incidence of overt stroke in the pre-TCD period was 0.67 per 100 patient-years, compared with 0.06 per 100 patient-years in the post-TCD period (P<.0001). Of the 2 strokes in the post-TCD period, 1 occurred in a child too young for the screening protocol, and 1 occurred in a child with high velocities solely in the anterior cerebral arteries. The rate of transfusion therapy for stroke prevention increased from 0.67 per 100 patient-years to 1.12 per 100 patient-years since instituting our program (P=.008).
Conclusions:
Our program has been successful in reducing the rate of first overt stroke, but with increased use of transfusion. Additional modifications to screening might further reduce the risk of first stroke, and studies of alternative treatments may be beneficial.
