The 10 questions included by the Cerebrovascular Disease Panel based on importance in preventing or decreasing neurological morbidity in children and adults with SCD
| . | PICO questions on preventing or decreasing neurological morbidity in SCD . |
|---|---|
| 1 | Should transfusion (vs no transfusion or hydroxyurea therapy) be used for children aged 2-16 y with HbSS or HbSβ0 thalassemia and abnormal nonimaging TCD measurements? |
| 2 | Between 2 and 16 y of age, should children with HbS/Lepore disease, HbSE disease, HbS/O Arab disease, or HbS/D disease phenotypes or other compound heterozygous SCD phenotypes other than HbSC have TCD screening at the same frequency and interval as children with HbSS or HbSβ0 thalassemia? |
| 3 | Should annual screening with TCD be used for children between 2 and 16 y of age with HbSS or HbSβ0 thalassemia phenotypes from low-middle–income settings? |
| 4 | Should simple blood transfusion vs exchange transfusion be used for children and adults with SCD and suspected acute symptomatic stroke, including TIA? |
| 5 | Should RBC transfusion targeted to keep HbS levels below 30% (vs no treatment), RBC transfusion targeted to keep HbS levels above 30% and Hb above 9 g/dL, or hydroxyurea at MTD therapy be used for children with SCD with a history of stroke? |
| 6 | Should cerebral revascularization surgery (including EDAS, EDAMS, pial synangiosis, or direct anastomosis) plus transfusion therapy vs regular blood transfusion therapy alone be used for patients with SCD and moyamoya syndrome? |
| 7 | Should IV thrombolysis with tPA vs no treatment with tPA be used for adults with SCD presenting with acute ischemic stroke and no hemorrhage on CT scan within 4.5 h of onset of symptoms? |
| 8 | Should clinicians perform or refer for screening for developmental delay and cognitive impairment vs no screening in children and adults with SCD? |
| 9 | Should cognitive rehabilitation therapy vs no rehabilitation be used for children and adults with SCD and cognitive impairment? |
| 10 | Should screening with MRI for SCIs vs no screening be used for children and adults with HbSS or HbSβ0 thalassemia? |
| . | PICO questions on preventing or decreasing neurological morbidity in SCD . |
|---|---|
| 1 | Should transfusion (vs no transfusion or hydroxyurea therapy) be used for children aged 2-16 y with HbSS or HbSβ0 thalassemia and abnormal nonimaging TCD measurements? |
| 2 | Between 2 and 16 y of age, should children with HbS/Lepore disease, HbSE disease, HbS/O Arab disease, or HbS/D disease phenotypes or other compound heterozygous SCD phenotypes other than HbSC have TCD screening at the same frequency and interval as children with HbSS or HbSβ0 thalassemia? |
| 3 | Should annual screening with TCD be used for children between 2 and 16 y of age with HbSS or HbSβ0 thalassemia phenotypes from low-middle–income settings? |
| 4 | Should simple blood transfusion vs exchange transfusion be used for children and adults with SCD and suspected acute symptomatic stroke, including TIA? |
| 5 | Should RBC transfusion targeted to keep HbS levels below 30% (vs no treatment), RBC transfusion targeted to keep HbS levels above 30% and Hb above 9 g/dL, or hydroxyurea at MTD therapy be used for children with SCD with a history of stroke? |
| 6 | Should cerebral revascularization surgery (including EDAS, EDAMS, pial synangiosis, or direct anastomosis) plus transfusion therapy vs regular blood transfusion therapy alone be used for patients with SCD and moyamoya syndrome? |
| 7 | Should IV thrombolysis with tPA vs no treatment with tPA be used for adults with SCD presenting with acute ischemic stroke and no hemorrhage on CT scan within 4.5 h of onset of symptoms? |
| 8 | Should clinicians perform or refer for screening for developmental delay and cognitive impairment vs no screening in children and adults with SCD? |
| 9 | Should cognitive rehabilitation therapy vs no rehabilitation be used for children and adults with SCD and cognitive impairment? |
| 10 | Should screening with MRI for SCIs vs no screening be used for children and adults with HbSS or HbSβ0 thalassemia? |
EDAMS, encephalo-duro-arterio-myo-synangiosis; EDAS, encephalo-duro-arterio-synangiosis; Hb, hemoglobin; MTD, maximum tolerated dose; PICO, population, intervention, comparison, and outcomes; RBC, red blood cell; SCI, silent cerebral infarct; TIA, transient ischemic attack.