Google
Trusted Resources: Evidence & Education
Scientific literature and patient education texts
Central Nervous System Complications and Management in Sickle Cell Disease
source: Blood
year: 2016
authors: DeBaun MR, Kirkham FJ
summary/abstract:With advances in brain imaging and completion of randomized clinical trials (RCTs) for primary and secondary stroke prevention, the natural history of central nervous system (CNS) complications in sickle cell disease (SCD) is evolving. In order of current prevalence, the primary CNS complications include silent cerebral infarcts (39% by 18 years), headache (both acute and chronic: 36% in children with sickle cell anemia [SCA]), ischemic stroke (as low as 1% in children with SCA with effective screening and prophylaxis, but ∼11% in children with SCA without screening), and hemorrhagic stroke in children and adults with SCA (3% and 10%, respectively). In high-income countries, RCTs (Stroke Prevention in Sickle Cell Anemia [STOP], STOP II) have demonstrated that regular blood transfusion therapy (typically monthly) achieves primary stroke prevention in children with SCA and high transcranial Doppler (TCD) velocities; after at least a year, hydroxycarbamide may be substituted (TCD With Transfusions Changing to Hydroxyurea [TWiTCH]). Also in high-income countries, RCTs have demonstrated that regular blood transfusion is the optimal current therapy for secondary prevention of infarcts for children with SCA and strokes (Stroke With Transfusions Changing to Hydroxyurea [SWiTCH]) or silent cerebral infarcts (Silent Infarct Transfusion [SIT] Trial). For adults with SCD, CNS complications continue to be a major cause of morbidity and mortality, with no evidence-based strategy for prevention.
organization: Vanderbilt University School of Medicine, USA; UCL Institute of Child Health, UKDOI: 10.1182/blood-2015-09-618579
read more full text
+myBinderRelated Content
-
Social Determinants of Health and Emergency Department Use Among Children With Sickle Cell DiseaseSickle cell disease (SCD) is associated ...
-
The Regional Comprehensive Sickle Cell Center at Robert Wood Johnson Medical SchoolThe Division of Hematology provides spec...
-
Testing Gene Editing for Sickle Cell Diseasehttps://www.youtube.com/watch?v=RGetNC2B...
-
FDA Awards $2M to Phase 2 Trial of Vitamin D for Reducing Risk of SCD Respiratory ComplicationsThe U.S. Food and Drug Administration (F...
-
Optimizing Hydroxyurea Therapy for Sickle Cell AnemiaHydroxyurea has proven efficacy in numer...
-
Edith Peterson Mitchell, MDEdith Peterson Mitchell, MD, is board ce...
-
Developing Hydroxyurea, the First FDA-Approved Therapy for Sickle Cell Diseasehttps://www.youtube.com/watch?v=0kIz5ZKy...
To improve your experience on this site, we use cookies. This includes cookies essential for the basic functioning of our website, cookies for analytics purposes, and cookies enabling us to personalize site content. By clicking on 'Accept' or any content on this site, you agree that cookies can be placed. You may adjust your browser's cookie settings to suit your preferences. More Information
The cookie settings on this website are set to "allow cookies" to give you the best browsing experience possible. If you continue to use this website without changing your cookie settings or you click "Accept" below then you are consenting to this.
Powered by
Support for this site is provided by
This platform is made possible through a partnership with the Sickle Cell Disease Association of America, Inc. (SCDAA) and its member organizations. SCDAA's mission is to advocate for people affected by sickle cell conditions and empower community-based organizations to maximize quality of life and raise public consciousness while advancing the search for a universal cure.