Stroke and Sickle Cell Disease

What is a stroke?

A stroke is caused by damage to brain tissue that happens when the blood flow to the brain is slowed or stopped. This results in not enough oxygen getting to the brain and causes part of the brain to die and not work as well.

If your child has sickle cell disease, they are more likely to have a stroke than someone who does not have sickle cell disease.

Symptoms of stroke

Signs and symptoms of stroke in children include: 

• Weakness or numbness, usually on one side of the body  
• Problems walking 
• Slurred speech or trouble speaking
• Confusion or difficulty understanding   
• Severe headache
• Vision changes
• Dizziness 
• Droopy face or mouth, not able to smile normally
• More sleepy than normal or hard to wake up
• Changes in behavior
• Problems with thinking, learning, or memory
• Seizures

Risk factors for stroke in sickle cell disease

In people with sickle cell disease, red blood cells become hard, sticky, and shaped like a banana (sickle-shaped). Over time, sickle cell disease can damage blood vessels. Sickle cells can clump together and block blood vessels. This can affect blood supply to the brain and cause a stroke.

Up to 1 in 10 children with certain types of sickle cell disease will have an increased risk of stroke. These types include hemoglobin SS disease (also called sickle cell anemia) and sickle beta zero thalassemia.

Screening for stroke in sickle cell disease

Transcranial Doppler (TCD) ultrasound is a painless test to measure blood velocity in your child’s brain. This exam can see if your child is at high risk for having a stroke. If the test shows your child is at high risk, treatment can be given to help prevent a stroke. Your care team may do this test yearly to monitor for risk of stroke.

Learn more about TCD and sickle cell disease.

Diagnosis of stroke

Imaging tests that take pictures of the brain are used to diagnose stroke. These include MRI or a CT scan (also called a CAT scan). The pictures will show any brain damage. This will let the doctor know if your child has had a stroke.

Treatment of stroke in sickle cell disease

Blood transfusions

A blood transfusion is a procedure to give your child blood from a healthy donor that does not have sickle cells. This helps dilute the sickle cells so that blood can flow better and lowers the chance of having a stroke. Sometimes, the sickled red blood cells can be replaced with normal red blood cells, and this is called an exchange transfusion.

If your child has a stroke, there may be as high as a 70% chance of having another stroke unless your child gets treatment. A blood transfusion will be needed every month to help prevent future strokes.

A bone marrow transplant is a treatment that can potentially cure sickle cell disease in children who have had a stroke. This is where the bone marrow cells that are responsible for making sickled red blood cells are replaced with bone marrow from a healthy donor (sometimes a relative).

Rehabilitation therapy

After a stroke, damage to parts of the brain can cause different problems. Your child might have trouble walking, talking, balancing, or learning. One side of the body might be weak. With therapy, symptoms often improve. 

Types of rehabilitation after stroke may include:

• Physical therapy
• Occupational therapy
• Speech-language therapy

A stroke can be life threatening. Be sure to have all screening and monitoring recommended by your care team. Seek medical help right away if your child shows any signs or symptoms of a stroke.

Questions to ask your care team

• How does sickle cell disease increase the risk of stroke?
• What are signs and symptoms of stroke in children?
• What is my child's risk of stroke?
• What screening tests and monitoring does my child need?
• Will my child need treatments to help prevent stroke?
• How do blood transfusions help prevent stroke?
• Are there any other options other than blood transfusions?
• What emergency warning signs of stroke should I watch for?

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