Sickle cell disease is a condition that affects the red blood cells. It can lead to other complications, such as stroke.

Sickle cell disease (SCD) is a condition where a person’s red blood cells are misshapen or “sickle-shaped.” According to the American Stroke Association (ASA), SCD affects 1 in 2,400 children and 1 in 400 Black children.

A stroke occurs when there is a blockage to the brain’s blood supply, such as blood clotting in the blood vessels or bleeding around the brain. Both situations are more likely to happen to a person with SCD.

This article looks at the cause of stroke in SCD, how doctors diagnose and treat it, and how to reduce the risk.

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For a person with SCD, their misshapen red blood cells can cause a blockage within the blood vessels. If this blockage stops blood from getting to the brain, it can cause a stroke.

Bleeding is also a complication of SCD. According to a 2020 study, bleeding in patients with SCD includes neurological, renal, and ocular bleeding complications. Neurological bleeding on or around the brain could lead to a hemorrhagic stroke.

Learn more about SCD here.

There are two main types of sickle cell stroke:

Ischemic stroke: This type of stroke happens when misshapen red blood cells block a person’s blood vessels. According to a 2021 study, ischemic stroke is common in children aged 2–10 with SCD. The same study says incidents tend to lower between the ages of 20–29 but peak again at 35.

Intracranial hemorrhage: This type of stroke is caused by bleeding in or around a person’s brain. Adults aged 20–30 are the most likely to experience a hemorrhagic stroke, but it is not uncommon in children. The rupture of an aneurysm commonly causes cerebral hemorrhage in people with SCD.

Learn more about ischemic stroke here.

According to the ASA, another common type of stroke seen in children with SCD is the “silent stroke.” It can occur in up to 39% of children before the age of 18. Doctors can detect signs of a silent stroke on an MRI scan of the brain, but there are no other outward signs. Silent strokes can cause problems with:

  • thinking
  • learning
  • problem-solving

They are also a risk factor for future strokes.

Ischemic and hemorrhagic stroke share similar symptoms, but the time of onset sometimes differs.

Symptoms of ischemic stroke

The symptoms of ischemic stroke tend to appear suddenly. They include:

  • numbness or weakness in the face, arm, or leg, particularly on one side of the body
  • difficulty speaking
  • confusion
  • severe headaches with no known cause
  • dizziness, and loss of balance or coordination
  • trouble seeing out of one or both eyes
  • difficulty walking

There may be additional symptoms in women, including:

  • pain in the legs or arms
  • nausea
  • heart palpitations
  • chest pain
  • shortness of breath

Symptoms of intracranial hemorrhage

The symptoms of intracranial hemorrhage can appear suddenly or take time to appear over a few days or weeks as pressure on the brain increases. They include:

  • increasing headache
  • vomiting
  • drowsiness and progressive loss of consciousness
  • confusion
  • dizziness
  • unequal pupil size
  • slurred speech
  • loss of movement or paralysis on one side of the body
  • seizures
  • unconsciousness

Learn what to do if someone is having a stroke here.

If doctors suspect a stroke, they will do an initial assessment to look for typical symptoms. This can include:

  • testing a person’s movement, sensation, speech, vision, and balance
  • taking a person’s pulse to check for an irregular heartbeat
  • assessing a person’s blood pressure

A person with a suspected stroke should have a brain scan within an hour of arriving at the hospital. Doctors may use the following methods to determine whether a person is having an ischemic or hemorrhagic stroke: a computerized tomography (CT) scan or a magnetic resonance imaging (MRI) scan.

The following techniques may help determine the location, type, and cause of stroke and to rule out other conditions:

If doctors diagnose stroke in a person with SCD, they will recommend an urgent blood transfusion.

If the stroke is ischemic, a person may also receive thrombolytic medication. Doctors use this medication to break up blood clots and restore blood flow to the brain.

A person experiencing an ischemic stroke may also receive blood-thinning medication, such as aspirin or warfarin.

To treat hemorrhagic stroke resulting from a ruptured aneurysm, a person may have an open craniotomy. In this procedure, a surgeon opens a portion of the skull and uses a metal clip to stop blood loss from the aneurysm.

A person may receive additional medication to help with conditions that can lead to a stroke, such as high blood pressure or diabetes.

According to a 2015 study, routine transcranial Doppler screenings along with regular blood transfusion therapy can decrease the prevalence of overt stroke in children from 11% to 1%.

A 2019 study found that hydroxyurea, a medication taken orally, is a safe and cost-effective option for reducing the risk of silent stroke in children. However, further research is needed to confirm its effectiveness.

Although women with SCD can have a healthy pregnancy, they will need to speak with a doctor as some sickle cell disease medications may harm the baby.

General advice about reducing stroke risk includes:

According to the Centers for Disease Control and Prevention (CDC), a person with SCD must go to an emergency room if they experience any of the following:

  • fever above 101°F
  • breathing difficulties
  • chest pain
  • sudden swelling in the belly area
  • sudden weakness or loss of movement or sensation
  • seizure
  • a painful erection of the penis lasting more than 4 hours

A person should contact a doctor if they experience pain anywhere in the body that will not go away with treatment or sudden vision problems.

A person with SCD has an increased risk of stroke. Stroke can happen when sickle-shaped red blood cells clog an artery or when there is bleeding in or around the brain.

Doctors can identify a stroke using an MRI or CT scan. They may use surgical procedures or medication to treat the stroke.

A person with SCD can reduce their risk of stroke by having regular blood transfusions and transcranial Doppler screenings. Taking prescribed medications and adopting healthy lifestyle habits may also help prevent stroke.