Sickle Cell Anemia and Stroke

What is a Stroke?

A stroke is a sudden and severe complication of sickle cell anemia. It affects between 6 to 8% of patients with sickle cell anemia, especially between two and ten years of age. A stroke may occur with a painful episode or an infection, but in most cases there are no related illnesses.

Although recovery from the stroke may be complete in some cases, frequently the stroke can cause brain damage, paralysis, convulsions, coma and even death.

A repeat stroke causes greater brain damage and increases the risk of death. Repeat strokes occur in at least 60% of the children who have already suffered one stroke unless treatment is given. Newer tests may help identify some children with sickle cell anemia who are at risk.

At this time, no test has been proven to predict those children with sickle cell anemia who are at risk for having strokes.

What Causes a Stroke?

The sickled cells in a child with sickle cell anemia have a hard time moving through the blood vessels in the brain. If some cells get "stuck" and can't move, other sickled cells pile up behind and cause a "log jam" that blocks the blood vessels. Oxygen can't get past the block to other parts of the brain, which causes the stroke.

Symptoms of a Stroke

• Jerking or twitching of the face, legs, arms
• Convulsions or seizures
• Strange, abnormal behavior
• Inability to move an arm and/or a leg
• Staggering or an unsteady walk when your children walked normally before
• Stuttered or slurred speech when your child had clear speech before
• Weakness in the hands, feet, or legs
• Changes in vision
• Severe headaches that won't go away with Tylenol
• Severe vomiting

Initial Treatment of a Stroke

Your child will be watched carefully in the hospital for any new or progressing symptoms. They may be put on medicine to control or prevent any convulsions caused by the stroke. Your child may need special x-ray tests (such as CT and MRI scans) and physical therapy. They will probably be transfused with blood that does not have any sickle cells in it.

Prevention of a Repeat Stroke

To prevent another stroke from happening, your child will probably need to receive blood transfusions every three to four weeks. Your child will be transfused with blood that does not have any sickled cells in it. This will fool their body into thinking it won't need to make any new blood with sickle cells. Although blood transfusions may help prevent any more strokes from happening, we do not know how many years your child must continue to be transfused.