Children with sickle cell disease more prone to stroke —Expert

A paediatric radiologist, Dr Dele Kammen, has said that children with sickle cell disease stand a higher risk of developing stroke and hip bone fracture, urging that parents ensure their care is prioritised right from childhood.

Dr Kammen, a paediatric radiology at the University of San Francisco, stated this in a lecture entitled “Sickle Cell Disease Imaging and Current Concepts: Imaging Manifestations and Treatment” at the University College Hospital, Ibadan.

The expert stated that the burden of stroke, a leading cause of death in children with sickle cell disease could be reduced through chronic blood transfusion.

She declared that the incidence of stroke in sickle cell disease is 15 per cent and that this increases between ages six and nine, adding that stroke accounts for 12 per cent of death before age of 20.

The expert, adding that children could also suffer from what is called a silent stroke, said that children with sickle cell disease can be effectively screened using the transcranial doppler to determine those that may be at risk of stroke.

According to her, “In the United States, 20 per cent of the children with sickle cell disease is on chronic blood transfusion. And through chronic blood transfusion, their risk of developing a stroke is reduced from 11 per cent to one per cent.

She explained that chronic blood transfusion helps to reduce the risk of stroke by improving blood oxygenation and key to treating chronic and acute complications of sickle cell disease.

However, she highlighted challenges of chronic blood transfusion to include high cost, unavailability of blood, transfusion reactions and iron overload.

The expert, however, stated that hydroxyl urea is also an approved drug that sickle cell patients can use to improve their well being and decrease complications of sickle cell disease, including sickle cell crisis.

Dr Kammen added that studies from Ibadan had shown that hydroxyl urea significantly reduces transcranial Doppler levels in children, and as such also a potential treatment for prevention of complications of sickle cell disease like stroke.

The expert, however, warned that use of hydroxyl urea need to be monitored effectively given that it can lead to suppression of the bone marrow and abnormal liver function test.

On hip bone collapse, she stated that persons with sickle cell need vigorous physical therapy to help to delay hip bone collapse, which can only be repaired by hip bone replacement.

Professor Ikeoluwa Lagunju, head, Department of Paediatrics, University College Hospital, said UCH has been offering trans cranial Doppler tests in the last eight years, adding that “in a cohort of patients been studied, and using hydroxyl urea,  we have only had one case of stroke.”