American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults

Central nervous system (CNS) complications are among the most common, devastating sequelae of sickle cell disease (SCD) occurring throughout the lifespan. These evidence-based guidelines of the American Society of Hematology are intended to support the SCD community in decisions about prevention, di...

Full description

Saved in:
Bibliographic Details
Published in:Blood Advances Vol. 4; no. 8; pp. 1554 - 1588
Main Authors: DeBaun, M.R., Jordan, L.C., King, A.A., Schatz, J., Vichinsky, E., Fox, C.K., McKinstry, R.C., Telfer, P., Kraut, M.A., Daraz, L., Kirkham, F.J., Murad, M.H.
Format: Journal Article
Language:English
Published: United States Elsevier Inc 28.04.2020
American Society of Hematology
Subjects:
Adult
Anemia
Anemia, Sickle Cell
Anemia, Sickle Cell - complications
Anemia, Sickle Cell - diagnosis
Anemia, Sickle Cell - drug therapy
Anemia-Clinical: Sickle cell anemia
Biomedical Imaging
Brain Disorders
Child
Clinical Guidelines
Clinical Research
Good Health and Well Being
Hematology
Hemoglobin
Hemoglobin, Sickle
Humans
Hydroxyurea
Hydroxyurea - therapeutic use
Neurological
Neurosciences
Pediatric
Prevention
Rare Diseases
RED CELLS
Sickle
Sickle Cell
Sickle Cell Disease
Stroke
United States
United States
Anemia-Clinical: Sickle cell anemia
RED CELLS
ISSN:2473-9529, 2473-9537, 2473-9537
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:Central nervous system (CNS) complications are among the most common, devastating sequelae of sickle cell disease (SCD) occurring throughout the lifespan. These evidence-based guidelines of the American Society of Hematology are intended to support the SCD community in decisions about prevention, diagnosis, and treatment of the most common neurological morbidities in SCD. The Mayo Evidence-Based Practice Research Program supported the guideline development process, including updating or performing systematic evidence reviews. The panel used the Grading of Recommendations Assessment, Development and Evaluation (GRADE) approach, including GRADE evidence-to-decision frameworks, to assess evidence and make recommendations. The panel placed a higher value on maintaining cognitive function than on being alive with significantly less than baseline cognitive function. The panel developed 19 recommendations with evidence-based strategies to prevent, diagnose, and treat CNS complications of SCD in low-middle– and high-income settings. Three of 19 recommendations immediately impact clinical care. These recommendations include: use of transcranial Doppler ultrasound screening and hydroxyurea for primary stroke prevention in children with hemoglobin SS (HbSS) and hemoglobin Sβ0 (HbSβ0) thalassemia living in low-middle–income settings; surveillance for developmental delay, cognitive impairments, and neurodevelopmental disorders in children; and use of magnetic resonance imaging of the brain without sedation to detect silent cerebral infarcts at least once in early-school-age children and once in adults with HbSS or HbSβ0 thalassemia. Individuals with SCD, their family members, and clinicians should become aware of and implement these recommendations to reduce the burden of CNS complications in children and adults with SCD.
Bibliography:ObjectType-Article-1
SourceType-Scholarly Journals-1
ObjectType-Feature-2
content type line 23
M.R.D. and L.C.J. are joint first authors.
ISSN:2473-9529
2473-9537
2473-9537
DOI:10.1182/bloodadvances.2019001142