Stroke in children with sickle cell disorder is preventable —Expert

Professor Ikeoluwa Lagunju is a Professor of Paediatrics at the College of Medicine, University of Ibadan and Consultant Paediatric Neurologist at the University College Hospital, Ibadan, Nigeria. She is an astute clinician and an accomplished researcher who pioneered the work on Transcranial Doppler (TCD) ultrasonography for primary stroke prevention in children with sickle cell disease (SCD) in Nigeria. She sheds light on the condition, how it affects children with SCD and how these children can be helped.

STROKE is a condition resulting from sudden loss of blood supply to a part of the brain, either as a result of an obstruction or sudden rupture of the blood vessel supplying that part of the brain. According to medical experts, it is a severe disease because it has the potential to cause death and major disabilities in affected individuals. There are two main types of stroke; the one that occurs as a result of blockage of the blood vessels is called ischemic stroke, while the other that results from rupture of the blood vessels is called hemorrhagic stroke.

Do Children have strokes?
Yes, children do have strokes. Many people are of the impression that stroke occurs only in adults but this is not true! There are some conditions that occur in children and make them vulnerable to developing a stroke. The most common of these predisposing conditions is Sickle Cell Disorder (SCD). Others include congenital heart disease and brain infections.

Why Do Children with SCD develop a stroke and how common is it?
Stroke occurs in about 10 per cent of children with SCD. It is currently estimated that 10 out of every 100 child with SCD would have suffered a stroke by their 20th birthday. Children with hemoglobin SS are more likely to have a stroke while those with other forms of SCD like hemoglobin SC and S Sickle Cell Disorder would have suffered a stroke by their 20th birthday. Children with hemoglobin SS are more likely to have a stroke while those with other forms of SCD like hemoglobin SC and S thalassaemia suffer stroke less frequently.
Ischemic stroke is more commonly seen than hemorrhagic stroke in children with SCD. The repeated sickling of the red blood cells results in damage of the linings of the blood vessels of the brain, particularly, the larger arteries that carry blood to the brain. After several years of repeated sickling, the vessel lumen gradually gets severely narrowed and with the formation of a blood clot, the blood vessel suddenly gets occluded. The occlusion of the blood vessel then results in failure of blood supply to the brain and death (also called infarction) of that part of the brain.
The risk of developing ischemic stroke in children with SCD is highest between the ages of three and nine years and once a child suffers a first stroke, he/she is likely to suffer recurrent episodes of this severe complication. Occurrence of stroke in children with SCD below the age of two years is extremely rare and hemorrhagic stroke is usually seen between the ages of 20 and 29 years.

What are the symptoms of stroke?
The symptoms of stroke in children could include the following:
•Sudden weakness (paralysis) of one arm and one leg on the same side of the body
•Associated weakness of one side of the face, which may make the mouth to appear twisted and fluids may drop out of the mouth while drinking
•Speech problems which can vary from a slurred to total speech loss
•Loss of consciousness
•Convulsion

What is the treatment for stroke in SCD?
The treatment of stroke in children with SCD is quite expensive and complex. It requires regular blood transfusion every month till the child is at least 18 years old. Another treatment option is the daily administration of a drug called hydroxyurea. The best treatment for stroke in children with SCD however, is prevention.

Is stroke in children with SCD preventable?
Stroke in SCD is preventable. It is now known that children with SCD who have an increased risk of developing a stroke can be identified by a test called Transcranial Doppler (TCD) Ultrasonography. TCD is a simple test, cheap, easy to perform and painless. The test helps to identify those children who are likely to develop a stroke so that appropriate measure can be taken to prevent this severe complication. The children so identified, are placed on monthly transfusions or daily oral hydroxyurea especially in situations where safe blood is not readily available as a means of stroke prevention. Facilities for TCD are available at the Sickle Cell Foundation Nigeria.

Why should we prevent stroke in SCD?
Stroke is a devastating and potentially fetal complication of SCD. It causes severe disability which may render the child immobile after repeated attacks. Other complications of stroke in SCD include learning disabilities, epilepsy and speech problems. Let us make primary stroke prevention in SCD our goal. We can, we should and we are doing it at the Sickle Cell Foundation, Nigeria.