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Sickle Cell and Strokes

Posted Sep 22 2010 2:25am
Written by Sickle Cell Warrior on 22 September 2010

One of the most severe complications with sickle cell is strokes.  So many of the warriors I know have had a stroke, or potential to develop strokes at one time or another. The main causative factor here are those pesky sickle cells that love to clump together wrecking havoc all over the body. If sickle cells clump up in a particular blood vessel leading or delivering blood to the brain; it is possible to develop a stroke.

What is a Stroke? Stroke is an interruption of blood supply to the brain due to vessel blockage by a blood clot (i.e., ischemic stroke) or rupture of a vessel causing blood to leak into the brain (i.e., hemorrhagic stroke). Patients with SC are at increased risk for both types of strokes.

Risk Factors: There are many factors that put people with sickle cell at risk for strokes.

  • Age: Children ages 2-9 are at the highest risk for strokes and ‘, which occurs when there are no physical signs of a stroke, but there is cognitive or developmental delays. In fact, children with sickle cell have a much greater chance of having strokes than non-sickle cell having children. This is why it’s important to schedule routine tests for your kids to monitor their brain tissue.
  • Low hemoglobin levels and severe anemia
  • Previous transient ischemic attack (TIA)s,
  • Elevated blood pressure
  • Previously having acute chest syndrome,
  • Crises
  • Previous  infarctions (tissue death)
  • High leukocyte count.

Signs and Symptoms:

Although clinical signs and symptoms depend on the location of the stroke, the most common sign in patients with sickle cell is sudden weakness on one side of the body. You may feel numb, be completely paralyzed or feel tingly in both the arm and leg of the affected side. Other symptoms may include some loss of senses (sight, taste, smell etc), difficulty speaking, uneven gait, or inability to use both arms the same.

Prevention:

To prevent a stroke; many physicians encourage routine blood transfusions. Getting regular monthly (or bi-monthly) blood transfusions has been shown to reduce the risk of a first stroke significantly. This is because it curtails more than half of the risk factors (anemia, low hemoglobin, acute chest, frequent crises, infarctions etc.) However, you have to keep in mind the risk of iron overload and other transfusion related disorders if you go with this option.

Alternatively, you could go the natural remedy route and try using supplements like L-Arginine to help promote circulatory and cardiovascular health. L-Arginine  makes the blood vessels expand, thereby preventing the ‘narrowing’ effect seen in many strokes. There is no empirical evidence that supports this treatment alternative, but many warriors have stated that it is effective, especially in the 20s and 30s.

Diagnostics:

Besides the symptoms listed above, the doctor can order a

  • Cranial MRI or CT scan to show abnormalities that indicate stroke
  • Transcranial Doppler ultrasound (TCD) to show elevated blood velocity, suggesting vessel hardening, damage and increased risk of stroke
Since children with sickle cell have a higher chance of developing stroke, most pediatricians order regular TCDs about every 4-6 months to monitor for silent infarcts. Please be sure to take your child to these appointments.

Treatment:
The good news is that with proper management, majority of [SC+ stroke] patients recover about 90% back to baseline. Prompt treatment ensures that there are no physical delays but cognitive delays may still exist.

Treatment of stroke in patients with SCA depends on the stroke type. Initial management includes diagnostic tests to determine the type, location, and size of the stroke. Stabilizing blood pressure, temperature, fluid balance, and other body functions is crucial. Stroke patients with SCA often require transfusions to lower levels of hemoglobin S (HbS; i.e., the sickle-shaped RBCs) to 30% or less.

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