Bloodless Management of Significantly Elevated Transcranial Doppler Velocity Value in a Jehovah’s Witness Child with Sickle Cell Disease: A Tertiary Centre Experience

Abstract

Objective: To show the possibility of the management of SCD patients with significantly elevated transcranial Doppler (TCD) velocity values suggestive of a high risk of stroke. Case Presentation: A 3-year old Jehovah’s Witness male with SCD who presented with a 9-month history of elevated TCD velocities ranging between 193 and 203cm/s despite the daily intake of hydroxyurea, iron-free blood tonic and proguanil. Multiple investigations revealed neutrophil counts of approximately 3000 cells/mm3, and rising Haemoglobin F values. Exchange blood transfusion was not provided owing to the caregivers’ religious convictions against blood transfusion. Following a rise in TCD value to 227cm/s, hydroxyurea dosage was increased to 30mg/kg and omega 3 supplements initiated at 1000iu twice daily. A month later, an alarming TCD value of 242cm/s was detected with elevated neutrophil count of 8670 cells/mm3. The urgent need for exchange blood transfusion was again rejected by the caregivers. On the suspicion of omega-3 induced neutrophilia and Vitamin A overdose which can lead to raised intracranial pressures, omega-3 was withdrawn and the tonic substituted with folic acid and multivitamin tablets. Hydroxyurea was further increased to 35mg/kg. Within two weeks, a repeat TCD ultrasound showed a remarkable drop in velocity to 188cm/s. The neutrophil counts also significantly declined to 3630 cells/mm3. The child is currently stable and no longer at high risk of stroke. Conclusion: Hydroxyurea continues to play a key role in the management of stroke in SCD patients. The impact of omega 3 and blood tonics in the routine management of SCD in children require further evaluation.