Sickle Cell Disease: Prevention, Diagnosis, and Treatment of Cerebrovascular Disease in Children and Adults

Patient Guideline Summary

Publication Date: April 1, 2020

Last Updated: November 21, 2023

Objective

This patient summary means to discuss key recommendations from the American Society of Hematology for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults.

Overview

Sickle cell disease is an inherited genetic defect that causes RBCs to deform under certain conditions. Those deformed cells plug small blood vessels, causing tissue damage.
- Other genetic defects in RBC formation may combine with sickle cell trait to produce similar consequences.

We will use the abbreviation SCD throughout this summary to refer to sickle cell disease, RBC to refer to red blood cells and ASH for the American Society of Hematology.

Plugging blood vessels to the brain causes strokes.

This patient summary focuses on prevention, diagnosis, and treatment of strokes due to sickle cell disease.

Screening

Children with sickle cell disease should be screened at regular intervals using transcranial Doppler ultrasound (TCD) imaging to detect silent damage to blood vessels in the brain.
- Abnormal results may suggest further imaging studies by magnetic resonance (MRI).
At least one MRI screening should be done in all early-school-age children and in all adults with SCD to detect silent blood vessel damage.
Doctors should evaluate adults and follow children with SCD for development in the areas of learning and behavior. Referral to specialists is recommended if any irregularities are discovered.

Treatment

Standard preventive treatment is a drug called hydroxyurea and/or repeated RBC transfusions.

These treatments do not cure the disease. They only reduce symptoms and the frequency of attacks.

For sudden strokes, immediate transfusion or apheresis (a modified transfusion that replaces sickle RBCs with normal, transfused RBCs) is recommended.
A second-choice treatment for adults with an acute stroke within 4.5 hours of its onset is thrombolysis (dissolving the blood clot with intravenous tissue plasminogen activator [tPA]).

Thrombolysis is not recommended for children.

Surgery to repair or bypass occluded blood vessels is an option for older, permanent strokes.
In all cases, the choice of and benefits from treatment must be carefully and thoroughly evaluated to assure that they exceed the risks.

Abbreviations

ASH: American Society Of Hematology
MRI: Magnetic Resonance Imaging
RBC: Red Blood Cell
SCD: Sickle Cell Disease
TCD: Transcranial Doppler
tPA: Tissue Plasminogen Activator

Source Citation

DeBaun MR, Jordan LC, King AA, Schatz J, Vichinsky E, Fox CK, McKinstry RC, Telfer P, Kraut MA, Daraz L, Kirkham FJ, Murad MH. American Society of Hematology 2020 guidelines for sickle cell disease: prevention, diagnosis, and treatment of cerebrovascular disease in children and adults. Blood Adv. 2020 Apr 28;4(8):1554-1588. doi: 10.1182/bloodadvances.2019001142. PMID: 32298430; PMCID: PMC7189278.

Disclaimer

The information in this patient summary should not be used as a substitute for professional medical care or advice. Contact a health care provider if you have questions about your health.