STROKE IN SICKLE CELL DISORDER

Stroke does occur in children! Stroke is one of the causes of severe disability and mortality among children with sickle cell and cause of sorrow in their families.

Childhood stroke has long been known to be a devastating complication of sickle cell disease. About 10% of children with sickle cell between the ages 2 to 16 years are at risk of stroke, the incidence being highest during the first 10 years of life – mostly between ages 2 and 5 – and lowest before the age of 2.

TCD: There is a procedure called TransCranial Doppler (TCD) ultrasonography, which is used for early identification of children at risk of developing stroke, so that timely, life-saving prevention interventions can be instituted; such as blood transfusion and some special medications. It was mindful of this capacity to lay the ground for stroke prevention and equally help prevent the agony of the consequences of stroke on the patients and their families that the Sickle Cell Foundation Nigeria commenced TCD services at the National Sickle Cell Centre, Idi Araba, Lagos in 2011.

A study carried out at the end of September 2013 at the National Sickle Cell Centre, Idi Araba. Lagos, showed that between March 2011 and September 2013 (31 months), a total of 2,369 children with sickle cell disorder (SS and SC) were scanned with TCD. Of these, 98.4% (2,331) were Hb SS, while the remaining 1.6% were Hb SC patients (38). This supports the evidence that the complication of STROKE in Sickle Cell Disorder is more prevalent among those with Hb SS than in those with Hb SC.

Prophylactic/Preventative Treatment of Stroke in Sickle Cell Disorder
The stroke preventive measures we have at present for preventing those children at risk of developing stroke from the devastating condition are as follows:
1. Repeated Blood Transfusions: The child at risk receives blood transfusion with Haemoglobin AA blood (every 3 – 4 weeks) – the aim being to reduce the Haemoglobin S (HbSS) cells in the child’s blood to less than 30%.

2. Exchange Blood Transfusion: This is even more effective than regular blood transfusion. As the name implies, the aim is to exchange the child’s blood with donated Hb AA blood. It is the standard treatment.

3. Hydroxyurea therapy: This medication is taken by mouth daily for stroke prevention in the children found to be at risk of stroke. It modifies the disease by reducing the circulating HbSS and increasing the HbF – which has a high affinity for oxygen and helps to reduce the incidence of a stroke.